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Fludarabine combinations are very affective in follicular lymphoma (FL) with high rates of complete response and prolonged survival. However, late toxicities could be a concern. The aim of the present study was to analyze the long-term impact on survival, relapse and late toxicities of a trial of treatment with fludarabine, mitoxantrone and cyclophosphamide (FCM regimen) for untreated patients with advanced stage FL. One hundred and twenty patients enrolled in a phase 2 trial of treatment with FCM regimen between 2000 and 2003 were evaluated. After a median follow-up of 12 years, 52 patients eventually relapsed/progressed with 10 year progression-free survival (PFS) of 46 %. Ten patients showed histological transformation to aggressive lymphoma with a risk of transformation of 2 and 9 % at 5 and 10 years, respectively. Three patients developed therapy-related myelodysplastic syndrome/acute myeloid leukaemia (MDS/AML) and seven solid neoplasms with an overall risk of 3 and 8 % at 5 and 10 years, respectively. Twenty-six patients eventually died during the follow-up. Overall survival at 10 years was 83 %. In conclusion, FCM regimen allows excellent long-lasting response in previously untreated patients with FL. The incidence of late events including histological transformation and secondary neoplasia is low but not negligible. [ABSTRACT FROM AUTHOR]

The introduction of rituximab and, more recently, new targeted drugs and its subsequent impact on the outcome of patients with lymphoma has called into question the role of high-dose therapy with autologous stem cell rescue (HDT-ASCR) in the therapeutic algorithm of these diseases. However, this strategy remains essential for the management of some types of lymphoma, both Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL) with some well-established indications. Thus, the role of HDT-ASCR to consolidate first-line therapy in young and fit patients with mantle cell lymphoma is well accepted, as it is indisputable the importance of this strategy to provide the best chance of cure in patients with relapsed HL or diffuse-large B-cell lymphoma (DLBCL). In contrast, the role of HDT-ASCR in the management of patients with follicular lymphoma (FL) or T-NHL is much more controversial. In what there is general agreement is in that the preferred source of stem cells is peripheral blood stem cells, with exceptional occasions in which the difficulties of obtaining peripheral blood progenitors prevents this strategy. [ABSTRACT FROM AUTHOR]

The article presents a discussion about salvage chemotherapy for aggressive B cell lymphoma. Burkitt lymphoma (BL) and diffuse large B cell lymphoma (DLBCL) are the common kinds of aggressive B cell lymphoma. It is suggested that salvage chemotherapy should be followed by high-dose therapy (HDT) and an autologous stem cell transplant (ASCT).

Matched unrelated donor stem cell transplantation (MUD-SCT) provides the only curative option for patients with follicular lymphoma (FL) who fail conventional therapies and do not have a sibling donor. The purpose of this study was to analyse the outcome of patients with FL treated with MUD-SCT included in the European Group for Blood and Marrow Transplantation registry. 131 patients treated with reduced-intensity conditioning (RIC, n = 87) or conventional myeloablative (CONV, n = 44) MUD-SCT between 2000 and 2005 were included. Median time from diagnosis to MUD-SCT was 47 months and the median number of previous therapeutic regimens was 4 (previous autograft: 47%). RIC recipients were significantly older, with a longer interval from diagnosis to MUD-SCT and had failed a previous autograft more frequently than CONV recipients. Non-relapse mortality (NRM) was 24% and 30% at 100-d and 1-year, respectively. After a median follow-up of 36 months, 17% of the patients developed disease progression, the 3-year progression-free survival (PFS) being 47%. Three-year overall survival (OS) for the whole series was 51%. On multivariate analysis, RIC regimens were associated with at lower NRM and a significantly longer PFS and OS. This retrospective study demonstrated that MUD-SCT results, even in heavily pre-treated populations, in a meaningful PFS and OS. [ABSTRACT FROM AUTHOR]

Bcl-2/IgH rearrangement is the molecular hallmark of follicular lymphoma (FL) which is present in 70-90% of the cases at diagnosis. The clinical significance of this feature is controversial. The aim of this study was to analyze the bcl-2/IgH rearrangement by means of a PCR technique, and to correlate molecular findings with clinical characteristics and outcome. Sixty-nine patients (median age, 53 years; male/female ratio: 35/34) diagnosed with FL in a single institution were included in the study. A total of 77 DNA samples were analyzed. 54 were obtained from lymph node biopsy and 23 from peripheral blood or bone marrow. Bcl-2/IgH rearrangement was assessed for both the major breakpoint region (MBR) and the minor cluster region (mcr) breakpoints by a PCR technique. Thirty-nine out of sixty patients (65%) with assessable samples were found to have a bcl-2/IgH rearrangement in the MBR breakpoint, whereas bcl-2/IgH rearrangement in mcr was observed in one patient (2%) and no rearrangement at MBR or mcr in the remaining 20 patients (33%). Regarding the initial characteristics, patients with bcl-2/IgH rearrangements at MBR or mcr were younger (<65 years) than those with no rearrangement at these sites (p = 0.0001). No differences were found according to bcl-2/IgH rearrangement in terms of complete response rate, time to treatment failure and overall survival. In our series bcl-2/IgH rearrangement at MBR or mcr, which was found in 67% of the patients, was not correlated with response to treatment, survival nor time-to-treatment-failure. [ABSTRACT FROM AUTHOR]

In this report we analyse the presenting features of a series of patients diagnosed with Waldenström macroglobulinaemia (WM) in Spain over the last 10 years. Criteria for diagnosis required a serum monoclonal IgM protein >= 30 g/l and > 20% bone marrow lymphocytes. Two hundred and seventeen patients were included in the study, with a median age of 69 years and male/female ratio of 2:1. The most common symptoms at diagnosis were anaemia (38%), hyperviscosity (31%), B symptoms (23%), bleeding (23%) and neurological symptoms (22%). Sixty-one patients (27%) were asymptomatic at diagnosis and, to date, 32 of them have not received chemotherapy. Variables predicting a shorter survival free of therapy were haemoglobin < 12·5 g/dl and high β2microglobulin (β2M). The 83% of patients who did receive treatment were distributed as follows: chlorambucil/prednisone (43%), intermittent chlorambucil (11%), continuous chlorambucil (26%), cyclophosphamide/vincristine/prednisone (COP, 13·5%) and other (6·5%). Response to therapy was complete in 2%, partial in 48% and minor in 10%. Finally, 28% and 13% of patients presented stable and progressive disease, respectively, which was more common among patients treated with COP. Progression-free survival was 43% at 5 years, with three independent predictors for shorter progression-free survival (PFS): COP treatment, age > 65 and B symptoms at diagnosis. The 10-year projected overall survival (OS) was 55%. The two most frequent causes of death were development of second malignancies (31%), or infections (19%). The two main variables predicting a poor OS were hyperviscosity and high β2M. In summary, this study favours the use of chlorambucil-based therapy as the standard treatment for WM, and describes a subset of patients who should be considered as suffering a smouldering form and who therefore do not require treatment for a long period of time. [ABSTRACT FROM AUTHOR]

Examines the association of pyoderma gangrenosum skin disease with the administration of alpha 2b-Interferon in a chronic granulocytic leukemia patient. Pathogenesis of the disease in myeloproliferative disorders; Effectiveness of cyclosporin A and prednisone in treating the dermatoses.

In this issue of I Leukemia and Lymphoma i , Candelaria et al. [[1]] report the results of a double-blind randomized multicenter international study comparing rituximab in combination with cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) with the biosimilar RTXM83 in combination with CHOP chemotherapy in newly diagnosed patients with diffuse large B-cell lymphoma (DLBCL). Efficacy, pharmacokinetics, and safety of the biosimilar CT-P10 in comparison with rituximab in patients with previously untreated low-tumour-burden follicular lymphoma: a randomised, double-blind, parallel-group, phase 3 trial. Comparative assessment of pharmacokinetics, and pharmacodynamics between RTXM83™, a rituximab biosimilar, and rituximab in diffuse large B-cell lymphoma patients: a population PK model approach. [Extracted from the article]

Summary: Peripheral T‐cell lymphomas (PTCLs) are a heterogeneous group of haematological cancers with generally poor clinical outcomes. However, a subset of patients experience durable disease control, and little is known regarding long‐term outcomes. The International T‐cell Lymphoma Project (ITCLP) is the largest prospectively collected cohort of patients with PTCLs, providing insight into clinical outcomes at academic medical centres globally. We performed a long‐term outcome analysis on patients from the ITCLP with available 10‐year follow‐up data (n = 735). The overall response rate to first‐line therapy was 68%, while 5‐ and 10‐year overall survival estimates were 49% and 40% respectively. Most deaths occurred prior to 5 years, and for patients alive at 5 years, the chance of surviving to 10 years was 84%. However, lymphoma remained the leading cause of death in the 5‐ to 10‐year period (67%). Low‐risk International Prognostic Index and Prognostic Index for T‐cell lymphoma scores both identified patients with improved survival, while in multivariate analysis, age >60 years and Eastern Cooperative Oncology Group performance status 2–4 were associated with inferior outcomes. The favourable survival seen in patients achieving durable initial disease control emphasizes the unmet need for optimal front‐line therapeutic approaches in PTCLs. [ABSTRACT FROM AUTHOR]

Primary refractory (PREF) follicular lymphoma (FL) has a completely different clinical course from that of FL that responds to front-line treatments. In addition to having poor responses to salvage therapies, it seems that patients with PREF are at increased risk of histological transformation (HT). The Aristotle consortium presented the opportunity of investigating the risk of HT in a very large series of cases. Thus, we investigated the risk of HT in patients with PREF FL compared with that of responding patients or in stable disease and ultimately their outcome. Six thousand three hundred thirty-nine patients from the Aristotle database were included in the analysis. These patients had a histologically confirmed grade 1, 2 or 3a FL diagnosed between 1997 and 2013. The primary end-points were the cumulative incidence (CI) of HT at the first progression or relapse and the survival after transformation. The 5-year CI of HT among patients with PREF was 34% (95% confidence interval (CI): 27–43), whilst it was 7.1% (95% CI: 6.0–8.5) in the group of patients with partial response (PR) or stable disease (SD) (PR + SD) and 3.5% (95% CI: 3.0–4.2) in the group of patients achieving complete response (CR). The 5-year survival after relapse (SAR) was 33% (95% CI: 28–39) for the PREF group, 57% (95% CI 54–61) in patients with PR, 51% (95% CI 43–58) in the SD group after first-line therapy and 63% (95% CI: 66–72) in patients with CR after initial treatment (p -value <0.001). The 5-year SAR for those patients with PREF who developed HT was 21% (95% CI: 12–31), clearly diminished when compared with those patients with PREF who did not experience HT (38% [95% CI: 31–44]) (p -value = 0.001). Patients with PREF FL have a dismal outcome and an associated very high rate of HT that further worsens their poor prognosis. • Five per cent of patients with follicular lymphoma (FL) were primary refractory to initial treatment. • These patients have increased risk of developing histological transformation (HT). • HT in this group of patients worsens their already poor survival. • Hidden transformation at diagnosis should be investigated in this group of patients. [ABSTRACT FROM AUTHOR]

• This article addresses the possible role of allogeneic hematopoietic cell transplantation (allo-HCT) for relapsed and refractory diffuse large B cell lymphoma in the chimeric antigen receptor-engineered T (CART) cell era. • Allo-HCT should be explored for patients failing CART therapy. • Allo-HCT should be considered when CART therapy is not feasible or available. • Allo-HCT and CART outcome data should be collected and analyzed in registries. Chimeric antigen receptor-engineered T (CART) cells are a promising new treatment option for patients with multiply relapsed and refractory (R/R) diffuse large B cell lymphoma (DLBCL). Because of the favorable outcome data reported for CART cells, uncertainty is emerging if there is still a role for allogeneic hematopoietic cell transplantation (allo-HCT) in the treatment of R/R DLBCL. This article provides an overview of available evidence and theoretical considerations to put these 2 types of cellular immunotherapy (CI) into perspective. Altogether, current data suggest that CART cells are preferred now over transplantation as first-choice CI in many clinical situations. However, the majority of patients will fail CART therapy, resulting in an unmet medical need where allo-HCT could be beneficial. In contrast, employing allo-HCT instead of CART cells as first CI should be presently restricted to situations where CART cell therapy is deemed not feasible or useful, such as patients with refractory cytopenia or incipient myelodysplastic syndrome. However, allo-HCT remains a standard treatment option as first CI for patients with chemosensitive R/R DLBCL when CARTs are not available or transplantation is preferred by the patient. Continuous collection and analysis of CI outcome data by professional registries appear to be of key importance for developing rational strategies of CI allocation and sequencing. [ABSTRACT FROM AUTHOR]

Bortezomib is a novel proteasome inhibitor with significant antimyeloma activity. Its toxicity is manageable, and the most frequent adverse effects mainly consist of gastrointestinal symptoms, peripheral neuropathy, neuropatic pain, and thrombocytopenia. Severe liver toxicity has not been previously recognized. A patient with relapsed multiple myeloma who developed bortezomib-induced severe recurrent hepatitis is described. The importance of recognizing this rare potential toxicity is highlighted in order to discontinue this agent if liver adverse reaction is suspected. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented in response to an article on monoclonal gammopathy, published in a previous issue.

The frequency of aggressive subtypes of B‐cell non‐Hodgkin lymphoma (B‐NHL), such as high‐grade B‐cell lymphomas (HGBL) with MYC and BCL2 and/or BCL6 rearrangement (HGBL‐DH/TH) or Burkitt‐like lymphoma (BL) with 11q aberration, is not well known in the HIV setting. We aimed to characterise HIV‐associated aggressive B‐NHL according to the 2017 WHO criteria, and to identify genotypic and phenotypic features with prognostic impact. Seventy‐five HIV‐associated aggressive B‐NHL were studied by immunohistochemistry (CD10, BCL2, BCL6, MUM1, MYC, and CD30), EBV‐encoded RNAs (EBERs), and fluorescence in situ hybridisation (FISH) to evaluate the status of the MYC, BCL2, and BCL6 genes and chromosome 11q. The 2017 WHO classification criteria and the Hans algorithm, for the cell‐of‐origin classification of diffuse large B‐cell lymphomas (DLBCL), were applied. In DLBCL cases, the frequencies of MYC and BCL6 rearrangements (14.9 and 27.7%, respectively) were similar to those described in HIV‐negative patients, but BCL2 rearrangements were infrequent (4.3%). MYC expression was identified in 23.4% of DLBCL cases, and coexpression of MYC and BCL2 in 13.0%, which was associated with a worse prognosis. As for BL cases, the expression of MUM1 (30.4%) conferred a worse prognosis. Finally, the prevalence of HGBL‐DH/TH and BL‐like with 11q aberration are reported in the HIV setting. The phenotypic and genotypic characteristics of HIV‐associated aggressive B‐NHL are similar to those of the general population, except for the low frequency of BCL2 rearrangements in DLBCL. MYC and BCL2 coexpression in DLBCL, and MUM‐1 expression in BL, have a negative prognostic impact on HIV‐infected individuals. [ABSTRACT FROM AUTHOR]

Keywords: combination antiretroviral therapy; HIV-BL; lymphoma EN combination antiretroviral therapy HIV-BL lymphoma 13 14 2 12/21/20 20210101 NES 210101 Over the last two decades, there have been significant improvements in the prognosis of patients living with human immunodeficiency virus (HIV) (PLWH) with increasing use of combination anti-retroviral therapy (ART). A higher incidence of Burkitt lymphoma (BL) remains despite improvement in ART, and typically occurs in those with CD4 counts of >200 × 10 SP 9 sp /l2 and constitutes ~20% of HIV-associated lymphomas. A larger Phase II trial with detailed short- and long-term toxicity, as well as cost analysis, including both HIV-positive and -negative patients may hold promise for a new standard therapy in patients with BL. [Extracted from the article]

Abstract: The presence of IgM paraproteinemia in low-grade lymphomas is usually considered a clinical syndrome known as Waldenstrom''s macroglobulinemia (WM). In the WHO classification, WM is associated to lymphoplasmacytic lymphoma (LPL); it is a clinicopathologic entity characterized by a monoclonal expansion of predominantly small B-lymphocytes with variable plasmacytoid differentiation. LPL constitutes less than 5% of all NHL and it is associated with hepatitis C virus infection in 26% of cases. Cells of LPL/WM are B cells positive for monocytic Ig light chains, IgM, pan-B-cell markers, and negative for CD3 and CD103. The t(9;14)(p13;q32) is present in 50% of LPL, and determines PAX-5 over-expression. 6q21 deletion is observed in 42% of cases. LPL occurs in older adults. Clinical presentation usually consists of disseminated disease, but extranodal involvement and leukemic phase are rare. Most WM patients have symptoms attributable to tumour infiltration and/or monoclonal protein. In fact, a monoclonal serum paraprotein of IgM type and hyperviscosity symptoms may occur in more than 20% of cases (WM). Hyperviscosity syndrome is usually manifested by bleeding, blurring or loss of vision, dizziness, headache, and neurologic symptoms. Malignant infiltration of the CNS (Bing–Neel syndrome) is uncommon. LPL/WM is an indolent malignancy that is not usually curable with conventional treatments. The median survival of patients with LPL or WM is 50–60 months, transformation to large cell lymphoma may occur. Stage definition is irrelevant in WM considering that initiation of therapy is decided on the bases of prognostic factors and the development of disease-related symptoms and signs. The main adverse prognostic factors are older age, B symptoms, anemia, low albumin serum levels, raised SGOT, and high beta 2-microglobulin values. Several therapeutic alternatives for newly diagnosed or relapsed LPL/WM are available; however, the best location for every strategy is a matter of investigation. Several new drugs are being assessed in prospective trials. As a significant progress in this field, response criteria and therapeutic recommendations were updated during the Third International Workshop on WM (7–10 October 2004, Paris, France). [Copyright &y& Elsevier]

Abstract: Follicular lymphomas constitute ∼30% of all non-Hodgkin lymphomas. These lymphomas are characterized by at least partially follicular growth pattern, but diffuse areas may be present. The proportions of follicular or diffuse areas vary also from case to case, which seems to be associated with prognosis. Follicular lymphomas should not be divided into distinct subtypes, but rather shows a continuous gradation in the number of large cells. On the bases of this grading, three groups have been defined: grades 1–3. There is a consensus that grade 3 follicular lymphomas, namely grade 3b, should be discriminated from lower-grade cases. The cells of follicular lymphomas express surface immunoglobulin, more frequently IgM+/−IgD>IgG>IgA, B-cell-associated antigens, CD10+/−; they are CD5−, CD23−/+, CD43−, and CD11c−. Follicular lymphomas express bcl-2 proteins, which is useful in distinguishing reactive from neoplastic follicles. t(14;18) is present in 70–95% of follicular lymphomas, involving rearrangement of bcl-2 gene. Clinical behavior of follicular lymphomas is heterogeneous and differs according to the histologic grade and extension of disease. Moreover, the evaluation of these malignancies is conditioned by therapeutic decision, which is also determined by main prognostic factors. The International Prognostic Index for aggressive lymphomas is not optimal for follicular lymphomas. Conversely, the Italian Lymphoma Intergroup Index and, more recently, the Follicular Lymphoma International Prognostic Index (FLIPI), designed in pre-rituximab era, seem to correlate well with outcome. Several active therapeutic approaches from the “wait and watch” strategy to the allogeneic transplantation are available for management of patients with follicular lymphoma. Therapeutic decision is mostly conditioned by patient''s characteristics, stage, histologic grade, tumor burden, and risk-predicting factors. [Copyright &y& Elsevier]

OBJECTIVE: To investigate the predictors of monoclonal gammopathy of undetermined significance (MGUS) by considering not only the initial features but also the pattern of evolution of the M protein during the first years after diagnosis. PATIENTS AND METHODS: This study consisted of 359 patients diagnosed as having MGUS at a single institution. Patients who showed a definite and progressive increase in their M protein size according to serum electrophoresis during the first 3 years of follow-up were considered to have evolving MGUS, whereas all others were considered to have nonevolving MGUS. RESULTS: Of the 359 patients, 330 had nonevolving MGUS, whereas 29 fulfilled the criteria for evolving MGUS. Overall, 32 patients developed malignant transformation. The progression rates at 10 and 20 years of follow-up for the evolving and the nonevolving types were 55% vs 10% and 80% vs 13%, respectively. Multivariate analysis revealed that the features significantly associated with a higher risk of progression were evolving MGUS (relative risk [RR], 12.14; P<.001), IgA MGUS (RR, 2.93; P=-.O06), and M protein concentration (RR, 2.18; P=-.04). CONCLUSION: The evolutionary pattern of serum M protein (progressive increasing vs stable) during the first years of follow-up is the most important risk factor for disease progression in patients with MGUS. [ABSTRACT FROM AUTHOR]

The purpose of this study was to analyze the results of second autologous hematopoietic stem cell transplantation (ASCT2) for patients with relapsed/refractory Hodgkin lymphoma (HL) after a first transplantation (ASCT1). Outcomes for 56 patients receiving an ASCT2 registered in the EBMT database were analyzed. The 4-year cumulative incidences of non-relapse mortality and disease relapse/progression were 5% and 67%, respectively. The 4-year overall survival (OS) and progression-free survival (PFS) were 62% and 28%. In univariate analysis, relapse of HL within 12 months of ASCT1 was associated with a worse OS (35% versus 76%, p = 0.01) and PFS (19% versus 29%, p = 0.059). Chemosensitivity at ASCT2 predicted better outcomes (4-year OS 72% versus 29%, p = 0.002; PFS 31% versus 12%, p = 0.015). This series shows that ASCT2 is a safe procedure and a relatively effective option for patients with late relapses after ASCT1 and with chemosensitive disease who are not eligible for an allogeneic transplant. [ABSTRACT FROM AUTHOR]

Summary. The objectives of the present study were to compare the interleukin 6 (IL-6) and tumour necrosis factor-alpha (TNF-alpha) serum levels of individuals with monoclonal gammopathy of undetermined significance (MGUS) with those of healthy controls, and to ascertain the predictor value of these cytokines in the evolution from MGUS to multiple myeloma. After a median follow-up of 7 years from the initial cytokine measurements, nine patients with MGUS have evolved to a malignant condition. The actuarial probability of malignant transformation in patients with increased IL-6 and TNF-alpha was not significantly higher than in those with normal values. [ABSTRACT FROM AUTHOR]

Keywords: coronavirus; lymphomas; leukaemia; myeloma; virology EN coronavirus lymphomas leukaemia myeloma virology e64 e67 4 07/20/20 20200715 NES 200715 Since being identified in China in December 2019, coronavirus disease 2019 (COVID-19) has rapidly evolved into a global pandemic with over 4 million cases and more than 270 000 deaths.1 Following the first reported cases in the United Kingdom (UK) in late January 2020, numbers have continued to rise, with 223 060 cases and 32 065 deaths reported as of May 11, 2020.2 Initial reports from China have indicated that COVID-19 has an overall mortality rate of 1-4%. Of 12 patients who had multiple myeloma, five patients had chronic lymphocytic leukaemia, four patients had each of diffuse large B cell lymphoma and acute lymphoblastic leukaemia, three patients had follicular lymphoma, two patients had acute myeloid leukaemia, along with one patient with each of aplastic leukaemia, myelofibrosis, monoclonal gammopathy of undetermined significance, mantle cell lymphoma and myelodysplastic syndrome. [Extracted from the article]

The article presents a stud that determines the effectiveness of lymph2Cx assay in assessing cell-of-origin (COO) subtypes in HIV-related diffuse large B-cell lymphoma (DBCL). It offers details of the method of the study which observes 47 patients who were previously diagnosed with HIV-related DBCL. It outlines the result which indicates that Lymph2CX is useful in determining COO of HIV-related DBCL.

Abstract More than 60% of patients with non-Hodgkin lymphoma (NHL) are age >60 years at presentation. The purpose of this study was to compare the potential risks and benefits of allogeneic hematopoietic cell transplantation (alloHCT) in elderly patients with NHL with younger patients in a large sample, also taking into account comorbidity information. All patients age ≥18 years who had undergone alloHCT from a matched sibling or unrelated donor for NHL between 2003 and 2013 and were registered with the European Society for Blood and Marrow Transplantation were eligible for the study. The primary study endpoint was 1-year nonrelapse mortality (NRM). A total of 3919 patients were eligible and were categorized by age: young (Y), 18 to 50 y (n = 1772); middle age (MA), 51 to 65 y (n = 1967); or old (O), 66 to 77 y (n = 180). Follicular lymphoma was present in 37% of the patients; diffuse large B cell lymphoma, in 30%; mantle cell lymphoma, in 21%, and peripheral T cell lymphoma, in 11%. At the time of alloHCT, 85% of the patients were chemosensitive and 15% were chemorefractory. With a median follow-up of 4.5 years in survivors, NRM at 1 year was 13% for the Y group. 20% for the MA group, and 33% for the O group (P <.001), whereas relapse incidence and overall survival (OS) at 3 years in the 3 groups were 30%, 31%, and 28% (P =.355) and 60%, 54%, and 38% (P <.001), respectively. Multivariable adjustment for confounders, including sex, NHL subset, time from diagnosis, chemosensitivity, donor, and conditioning, confirmed older age as a significant predictor for NRM and OS, but not for relapse risk. Although comorbidity was a significant predictor of NRM in a subset analysis restricted to the 979 patients with comorbidity information available, age retained its significant impact on NRM. In conclusion, our data show that alloHCT in patients age >65 y provides similar NHL control as seen in younger patients but is associated with a higher NRM that is not fully explained by comorbidity. Thus, although alloHCT is feasible and effective in very old patients, the increased NRM risk must be taken into account when assessing the indication for alloHCT for NHL in this age group. [ABSTRACT FROM AUTHOR]

Summary: Different models to investigate the prognosis of peripheral T cell lymphoma not otherwise specified (PTCL‐NOS) have been developed by means of retrospective analyses. Here we report on a new model designed on data from the prospective T Cell Project. Twelve covariates collected by the T Cell Project were analysed and a new model (T cell score), based on four covariates (serum albumin, performance status, stage and absolute neutrophil count) that maintained their prognostic value in multiple Cox proportional hazards regression analysis was proposed. Among patients registered in the T Cell Project, 311 PTCL‐NOS were retained for study. At a median follow‐up of 46 months, the median overall survival (OS) and progression‐free survival (PFS) was 20 and 10 months, respectively. Three groups were identified at low risk (LR, 48 patients, 15%, score 0), intermediate risk (IR, 189 patients, 61%, score 1–2), and high risk (HiR, 74 patients, 24%, score 3–4), having a 3‐year OS of 76% [95% confidence interval 61–88], 43% [35–51], and 11% [4–21], respectively (P < 0·001). Comparing the performance of the T cell score on OS to that of each of the previously developed models, it emerged that the new score had the best discriminant power. The new T cell score, based on clinical variables, identifies a group with very unfavourable outcomes. [ABSTRACT FROM AUTHOR]

Background: Allogeneic hematopoietic stem cell transplantation (allo-HCT) remains the only potentially curative treatment option for relapsed follicular lymphoma (FL), yet questions remain about the optimal timing. This study analyzed long-term outcomes and associated factors among recipients of allo-HCT with FL.Methods: Patients with relapsed FL who underwent allo-HCT from 2001 to 2011 with a human leukocyte antigen (HLA)-matched donor were included. Outcome analyses for overall survival (OS), progression-free survival (PFS), transplant-related mortality (TRM), and disease relapse/progression were calculated. A multivariate analysis was performed to determine factors associated with outcomes, and a prognostic score for treatment failure was developed in a subset analysis of patients.Results: In all, 1567 patients with relapsed FL were included; the median follow-up was 55 months. The 5-year probabilities of OS and PFS were 61% and 52%, respectively. The 5-year cumulative incidences of disease progression/relapse and TRM were 29% and 19%, respectively. Chemoresistant disease, older age, heavy pretreatment, poor performance status (PS), and myeloablative protocols were predictors for worse survival. The prognostic score, using age, lines of prior therapy, disease status, and PS, stratified patients into 3 groups-low, intermediate, and high risk-with 5-year PFS rates of 68%, 53%, and 46%, respectively, and 5-year OS rates of 80%, 62%, and 50%, respectively.Conclusions: Allo-HCT should be considered for patients with relapsed FL and available HLA-matched donors. Outcomes are better in earlier phases of the disease, and reduced-intensity conditioning should be preferred. The prognostic score presented here can assist in counseling patients and determining the time to proceed to transplantation. Cancer 2018;124:1733-42. © 2018 American Cancer Society. [ABSTRACT FROM AUTHOR]

Summary: Brentuximab vedotin (BV) is an anti‐CD30 antibody‐drug conjugate. Preliminary data suggest that BV might improve outcomes after allogeneic stem cell transplantation (SCT) for Hodgkin lymphoma (HL) when used as pre‐transplant salvage therapy. Between 2010 and 2014, 428 adult patients underwent an allogeneic SCT for classical HL at participating centres of the European Society for Blood and Marrow Transplantation. We compared the outcomes of 210 patients who received BV prior to allogeneic SCT with that of 218 patients who did not receive BV. The median follow‐up for survivors was 41 months. Patients in the BV group were more heavily pre‐treated (median pre‐allograft treatment lines: 4 vs. 3). The two groups were comparable in terms of disease status, performance status, comorbidities, prior autologous SCT, type of donor, conditioning and in vivo T cell depletion. In multivariate analysis, pre‐allograft BV had no impact on acute graft‐versus‐host disease (GVHD), non‐relapse mortality, cumulative incidence of relapse, progression‐free survival or overall survival (OS), but significantly reduced the risk of chronic GVHD (hazard ratio = 0·64; 95% confidence interval = 0·45–0·92; P < 0·02). Older age, poor performance status, use of pre‐transplant radiotherapy and active disease at SCT adversely affected OS. Patients allografted for HL after prior exposure to BV do not have a superior outcome after allogeneic SCT except for a lower risk of chronic GVHD. However, BV may improve the outlook of allogeneic SCT by helping otherwise refractory patients to achieve a more favourable disease status, facilitating allotransplant success. [ABSTRACT FROM AUTHOR]

The purpose of this study was to analyze the impact of graft-versus-host disease (GVHD) on the relapse rate of different lymphoma subtypes after allogeneic hematopoietic cell transplantation (allo-HCT). Adult patients with a diagnosis of Hodgkin lymphoma, diffuse large B cell lymphoma, follicular lymphoma (FL), peripheral T cell lymphoma, or mantle cell lymphoma (MCL) undergoing HLA-identical sibling or unrelated donor hematopoietic cell transplantation between 1997 and 2009 were included. Two thousand six hundred eleven cases were included. A reduced-intensity conditioning (RIC) regimen was used in 62.8% of the transplantations. In a multivariate analysis of myeloablative cases (n = 970), neither acute (aGVHD) nor chronic GVHD (cGVHD) were significantly associated with a lower incidence of relapse/progression in any lymphoma subtype. In contrast, the analysis of RIC cases (n = 1641) showed that cGVHD was associated with a lower incidence of relapse/progression in FL (risk ratio [RR], .51; P = .049) and in MCL (RR, .41; P = .019). Patients with FL or MCL developing both aGVHD and cGVHD had the lowest risk of relapse (RR, .14; P = .007; and RR, .15; P = .0019, respectively). Of interest, the effect of GVHD on decreasing relapse was similar in patients with sensitive disease and chemoresistant disease. Unfortunately, both aGVHD and cGVHD had a deleterious effect on treatment-related mortality and overall survival (OS) in FL cases but did not affect treatment-related mortality, OS or PFS in MCL. This study reinforces the use of RIC allo-HCT as a platform for immunotherapy in FL and MCL patients. [ABSTRACT FROM AUTHOR]

BACKGROUND The treatment and outcomes of patients with human immunodeficiency virus (HIV)-associated Hodgkin lymphoma (HL) continue to evolve. The International Prognostic Score (IPS) is used to predict the survival of patients with advanced-stage HL, but it has not been validated in patients with HIV infection. METHODS This was a multi-institutional, retrospective study of 229 patients with HIV-associated, advanced-stage, classical HL who received doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD) plus combination antiretroviral therapy. Their clinical characteristics were presented descriptively, and multivariate analyses were performed to identify the factors that were predictive of response and prognostic of progression-free survival (PFS) and overall survival (OS). RESULTS The overall and complete response rates to ABVD in patients with HIV-associated HL were 91% and 83%, respectively. After a median follow-up of 5 years, the 5-year PFS and OS rates were 69% and 78%, respectively. In multivariate analyses, there was a trend toward an IPS score >3 as an adverse factor for PFS (hazard ratio [HR], 1.49; P=.15) and OS (HR, 1.84; P=.06). A cluster of differentiation 4 (CD4)-positive (T-helper) cell count <200 cells/μL was associated independently with both PFS (HR, 2.60; P=.002) and OS (HR, 2.04; P=.04). The CD4-positive cell count was associated with an increased incidence of death from other causes (HR, 2.64; P=.04) but not with death from HL-related causes (HR, 1.55; P=.32). CONCLUSIONS The current results indicate excellent response and survival rates in patients with HIV-associated, advanced-stage, classical HL who receive ABVD and combination antiretroviral therapy as well as the prognostic value of the CD4-positive cell count at the time of lymphoma diagnosis for PFS and OS. Cancer 2015;121:423-431. © 2014 American Cancer Society. [ABSTRACT FROM AUTHOR]

Abstract: Hodgkin lymphoma (HL) prognostic models based on factors measured at time of autologous hematopoietic cell transplantation (AHCT) are limited by small sample sizes. Models based on information at diagnosis are often not uniformly collected or available at transplantation. We propose an easily implementable prognostic model for progression-free survival (PFS) post-AHCT based on factors available at transplantation in a large international cohort of HL patients. The outcomes of 728 AHCT recipients for relapsed/refractory HL were studied. Patients were randomly selected for model development (n = 337) and validation (n = 391). The multivariate model identified 4 major adverse risk factors at the time of AHCT with the following relative weights: Karnofsky performance score <90 and chemotherapy resistance at AHCT were each assigned 1 point, whereas at least 3 chemotherapy regimens pre-AHCT and extranodal disease at AHCT were each assigned 2 points. Based on the total score summed for the 4 adverse risk factors, 3 risk groups were identified: low (score = 0), intermediate (score = 1 to 3), or high (score = 4 to 6). The 4-year PFS for the low- (n = 176), intermediate- (n = 261), and high- (n = 283) risk groups were 71% (95% confidence interval [CI], 63% to 78%), 60% (95% CI, 53% to 66%), and 42% (95% CI, 36% to 49%), respectively. The prognostic model was validated in an independent cohort. The Center for International Blood and Marrow Transplant Research model is based on factors easily available at the time of AHCT and discriminates patients with favorable post-AHCT outcomes as well as an intermediate-risk group. This model should assist in the prospective evaluation of alternative treatment strategies. [Copyright &y& Elsevier]

Gain of function mutations in the H3K27 methyltransferase EZH2 represent a promising therapeutic target in germinal center lymphomas. In this study, we assessed the frequency and distribution of EZH2 mutations in a large cohort of patients with follicular lymphoma (FL) (n = 366) and performed a longitudinal analysis of mutation during the disease progression from FL to transformed FL (tFL) (n = 33). Mutations were detected at 3 recurrent mutation hot spots (Y646, A682, and A692) in 27% of FL cases with variant allele frequencies (VAF) ranging from 2% to 61%. By comparing VAF of EZH2with other mutation targets (CREBBP, MLL2, TNFRSF14, and MEF2B), we were able to distinguish patients harboring clonal EZH2 mutation from rarer cases with subclonal mutations. Overall, the high incidence of EZH2 mutations in FL and their stability during disease progression makes FL an appropriate disease to evaluate EZH2 targeted therapy. [ABSTRACT FROM AUTHOR]

The guideline group was selected to be representative of UK-based medical experts. Ovid MEDLINE, EMBASE and NCBI Pubmed were searched systematically for publications in English from 1980 to 2012 using the Me SH subheading 'lymphoma, CNS', 'lymphoma, central nervous system', 'lymphoma, high grade', 'lymphoma, Burkitt's', 'lymphoma, lymphoblastic' and 'lymphoma, diffuse large B cell' as keywords, as well as all subheadings. The writing group produced the draft guideline, which was subsequently revised by consensus by members of the Haemato-oncology Task Force of the British Committee for Standards in Haematology ( BCSH). The guideline was then reviewed by a sounding board of ~50 UK haematologists, the BCSH and the British Society for Haematology ( BSH) Committee and comments incorporated where appropriate. The ' GRADE' system was used to quote levels and grades of evidence, details of which can be found in Appendix I. The objective of this guideline is to provide healthcare professionals with clear guidance on the optimal prevention of secondary central nervous system ( CNS) lymphoma. The guidance may not be appropriate to patients of all lymphoma sub-types and in all cases individual patient circumstances may dictate an alternative approach. Acronyms are defined at time of first use. [ABSTRACT FROM AUTHOR]

Abstract: Lymphomatoid granulomatosis (LG) is a very rare, Epstein-Barr virus–associated lymphoproliferative disorder of B cells. Prognosis is poor, particularly after relapse and no curative treatment exists. We report the results of high-dose therapy and autologous stem cell transplantation (ASCT) or reduced-intensity conditioning and allogeneic stem cell transplantation (alloSCT) in patients with multiply relapsed LG. A European Group for Blood and Marrow Transplantation survey identified 10 patients who had received 9 ASCT and 4 alloSCT. All patients had active disease at the time of transplantation. With a median follow-up of 5.1 (range, 1.4 to 6.3) years, 6 patients are alive and disease-free. Two ASCT patients died of septicemia early after transplantation, and 1 committed suicide after being in continuous complete remission 19 months after ASCT. Another patient allografted 4 years after ASCT remained disease-free but died of severe graft-versus-host disease 3 months after alloSCT. High-dose therapy followed by ASCT and alloSCT are effective therapeutic options and should be considered in all patients with refractory and multiply relapsed LG. [Copyright &y& Elsevier]

This analysis reviews the response rate (RR), treatment toxicity and overall survival (OS) for human immunodeficiency virus (HIV)-positive patients with high-risk diffuse large B-cell lymphoma (DLBCL) and the impact of treatment intensification. Fifty patients, treated with either rituximab, cyclophosphamide, doxorubicin, vincristine and prednisolone (R-CHOP) ( n = 35) or cyclophosphamide, vincristine, doxorubicin, methotrexate/etoposide, ifosfamide, cytarabine (CODOX-M/IVAC) ± R ( n = 15) chemotherapy, were included. Baseline characteristics did not differ between the two treatment groups. Forty-seven patients (94%) received rituximab and 48 (96%) received combination anti-retroviral therapy, with chemotherapy. The RR and treatment-related mortality were not significantly different between the two groups. Overall, 68% achieved complete remission. There were significantly more infections and non-hematological toxicities in the CODOX-M/IVAC ± R group. With a median follow-up of 28 months, 2-year progression-free survival (PFS) and OS are 68% and 70%, respectively, with no significant differences in remission duration, PFS or OS between the groups. In our cohort, the outcome for HIV-positive patients with high-risk DLBCL is favorable. Treatment intensification is feasible, but demonstrated no advantage over R-CHOP. [ABSTRACT FROM AUTHOR]

Despite the use of highly active antiretroviral therapy (HAART), AIDS-related lymphoma remains common. We investigated the tumor, microenvironment, and viral components in 41 AIDS-related diffuse large B-cell lymphomas (AR-DLBCLs) in the pre- and post-HAART era. The outcome has improved and the frequency of the prognostically unfavorable immunoblastic histology has decreased after HAART. Compared with sporadic cases, AR-DLBCL demonstrated increased hyperproliferation (P< .001) and c-Myc rearrangements, reduced CD4+ (P< .001) and FOXP3+ T cells (P< .001), increased activated cytotoxic cells (P< .001), but no difference in tumor-associated macrophages. Our analysis showed that AR-DLBCL is highly angiogenic with higher blood-vessel density than sporadic cases (P< .001) and highlighted the role of Epstein-Barr virus in angiogenesis. We recognized viral profiles and as a second step examined the reactive cytotoxic cell infiltrates. Our observation of markedly higher numbers of cytotoxic cells in AR-DLBCL with LMP1 and/or p24 compared with cases lacking viral antigens (P< .001) has important clinical implications, implicitly linked to the immunosurveillance theory. Whereas early initiation of HAART should improve immunosurveillance and reduce the incidence of LMP1-positive AR-DLBCL, cases without viral antigens appear able to avoid immunologic reaction and likely require additional strategies to improve surveillance. [ABSTRACT FROM AUTHOR]

BACKGROUND: Plasmablastic lymphoma (PBL) is a rare and aggressive B-cell lymphoma strongly associated with human immunodeficiency virus (HIV) infection. The authors conducted a multi-institutional, retrospective study to describe characteristics and determine prognostic factors in HIV-associated PBL. METHODS: For this study, the investigators included consecutive, HIV-positive patients diagnosed between the years 2000 and 2010 whose tumors had a plasmablastic morphology, were cluster of differentiation 20 (CD20)-negative, and expressed markers of plasmacytic differentiation. RESULTS: Fifty patients from 13 institutions were evaluated. The median age was 43 years, and there was a male predominance. The median count of cells that were positive for CD4 (a glycoprotein expressed on the surface of T-helper cells, monocytes, macrophages, and dendritic cells) was 206 cells/mm3. At presentation, 90% of patients had extranodal involvement, 69% presented with advanced stage disease, and 27% had oral involvement. Rearrangements of v-myc myelocytomatosis viral oncogene homolog ( MYC) were detected in 41% of the tested patients. Eighty-five percent of patients received chemotherapy, with 63% receiving cyclophosphamide, doxorubicin, vincristine, and prednisone and 37% receiving more intensive regimens. The complete response (CR) rate was 66%. The median overall survival (OS) was 11 months regardless of the intensity of chemotherapy. In the survival analysis, an Eastern Cooperative Oncology Group performance status ≥2, advanced stage, and MYC rearrangements were associated significantly with a worse outcome, whereas attaining a CR with chemotherapy was associated with a better outcome. CONCLUSIONS: The prognosis of PBL in HIV-infected individuals remains poor in the highly active antiretroviral therapy era. Intensive chemotherapy regimens do not seem to increase survival in patients with HIV-associated PBL. Cancer 2012. © 2012 American Cancer Society. [ABSTRACT FROM AUTHOR]

Summary An intention-to-treat (ITT) analysis was performed in 103 unselected patients with relapsed/refractory classical Hodgkin lymphoma (CHL) comparing early relapse (<12 months) or failure of first-line therapy (ER/FTF) with late relapses (LR). Seventy one percentage proceeded to high-dose therapy/autologous stem cell rescue (HDT/ASCR) following salvage treatment. By ITT, 5-year overall survival (OS) was 50% for ER/FTF compared to 73% for LR patients ( P = 0·012). However OS was equivalent for both groups if salvage treatment response was adequate to proceed to HDT/ASCR. ER/FTF patients remain a high-risk group largely due to a failure of salvage therapy: a point at which novel interventions could impact survival. [ABSTRACT FROM AUTHOR]

The article discusses guidelines on the management and investigation of follicular lymphoma (FL) in Great Britain. It says that the objective of the guidelines is to provide healthcare professionals with guidance on FL management in patients. It mentions that the guidelines have used various nomenclature such as Development, Grading of Recommendations Assessment, and Evaluation in patients with FL to evaluate levels of evidence and to supply recommendations.

Summary We have previously reported presentation serum selenium level to be predictive of outcome in diffuse large B-cell lymphoma. This has now been studied in a further 430 patients, 163 with acute myeloid leukaemia (AML), 156 with Hodgkin Lymphoma (HL), and 111 with Follicular Lymphoma (FL). Serum selenium was below the UK normal reference range in 45% of patients, and correlated with serum albumin ( r = 0·24-0·46, P < 0·001-0·003) in all tumour types. Independent predictors of presentation selenium were; French-American-British subtype and albumin ( P < 0·001 for both) in AML, haemoglobin ( P = 0·002) and B-symptoms ( P = 0·01) in HL, and albumin ( P < 0·001) in FL. In AML and HL, response to first line therapy was lower in patients with low serum selenium, but selenium was no longer predictive of response when other variables were entered into a multivariate model. Low selenium was also associated with a worse overall survival in FL [Hazard Ratio (HR) 2·3, 95% confidence interval (CI) 1·4, 4·0] and a trend to a worse overall survival in AML (HR 1·43, 95% CI 0·96, 2·13) by univariate Cox regression analysis, but not by multivariate analysis. In conclusion, low serum selenium is associated with a worse outcome in patients with haematological malignancies, but is not independently predictive, suggesting that it reflects other factors. [ABSTRACT FROM AUTHOR]

The combination of bortezomib and rituximab was evaluated in patients with mantle cell lymphoma (MCL), follicular lymphoma (FL) and Waldenström macroglobulinaemia (WM), in a Phase I and later, a randomized Phase II study. In the randomized study, 42 patients with recurrent/refractory disease received either: bortezomib 1·3 mg/m on days 1, 4, 8 and 11 of a 3-week cycle with rituximab 375 mg/m on day 1 (21 patients) or: bortezomib 1·6 mg/m and rituximab on days 1, 8, 15 and 22 of a 5-week cycle (with rituximab being given only in cycles 1 and 4).Twenty-eight patients were withdrawn (toxicity 16, progression 7, and 'patient choice' 5). The main toxicities were neurological, gastro-intestinal and haematological. The overall response rate was 28/42(67%) and by histology: MCL 11/19, FL 8/15, and WM 9/10. Ten of 28 responding patients remained progression-free at 1-3·5 years. Toxicity and efficacy were equivalent between the two groups. The combination has significant toxicity but is effective, particularly in patients with WM. [ABSTRACT FROM AUTHOR]

Background: HIV-associated multicentric Castleman disease is a rare lymphoproliferative disorder with marked systemic symptoms attributed to cytokine disarray. Many therapeutic approaches in small series of patients have proved largely unsuccessful to date. Objective: To investigate the efficacy and clinicopathologic variables associated with first-line treatment for HIV-associated multicentric Castleman disease with the anti-CD20 monoclonal antibody rituximab. Design: Single-group, open-label, phase II trial. Setting: 3 teaching hospitals in England. Patients: Previously untreated patients with histologically proven HIV-associated multicentric Castleman disease. Intervention: 4 infusions of rituximab, 375 mg per m2 of body surface area, at weekly intervals. Measurements: Response was evaluated clinically and radiologically and by measuring plasma Kaposi sarcoma-associated herpesvirus viral load. Results: 21 consecutive patients (18 men) with plasmablastic multicentric Castleman disease were recruited. The median follow-up was 12 months (range, 1 to 49 months). One patient died before completing therapy, 20 achieved remission of symptoms, and 14 (67%) achieved a radiologic response. The overall and disease-free survival rates at 2 years were 95% (95% Cl, 86% to 100%) and 79% (Cl, 49% to 100%), respectively. Plasma acute-phase proteins, immunoglobulins, and Kaposi sarcoma-associated herpesvirus viral load decreased after rituximab therapy. The main adverse effect was reactivation of Kaposi sarcoma. Limitation: The study had no comparison group. Conclusion: Rituximab may be clinically valuable as initial therapy for HIV-associated multicentric Castleman disease. [ABSTRACT FROM AUTHOR]

Background and objectives: Patients with follicular lymphoma (FL) in advanced stages are currently deemed incurable with standard treatments. However, FL is considered to be eradicable in the small group of patients presenting with localized disease. The objective of this study was to analyze the clinical features and the outcome of a series of patients with FL in early stages with a long follow-up. Patients and methods: A total of 48 patients (25m/23f; median age: 50 yr) diagnosed consecutively with FL in Ann Arbor stage I (25 cases) or II (23) at a single institution with a median follow-up of 9.5 yr were included in the study. Main biological and clinical characteristics at diagnosis, including Follicular Lymphoma International Prognostic Index (FLIPI) were analyzed; treatment and response were assessed and analyzed for prognosis. Results: The histologic subtypes were: FL type I, 20 cases (42%); type II, 24 (50%); type III, three (6%); and unclassifiable, one (2%). Distribution according to FLIPI was: low risk (36 cases) and intermediate risk (five cases). Treatment mainly consisted of combination chemotherapy (CHOP in 34 cases) plus involved-field radiotherapy in 26 cases. Forty patients (89%) achieved a complete response (CR), three (7%) a partial response, and two (4%) were non-responders; the remaining three patients did not receive therapy. No initial variable predicted CR achievement. About 57% of the patients in CR eventually relapsed with a relapse risk of 46% at 10 yr. Intermediate-risk FLIPI predicted failure-free survival. Histologic transformation was observed in six patients with a 10-yr risk of transformation of 13%. Twelve patients died during follow-up, in two cases as a result of unrelated causes. Overall survival (OS) at 10 yr was 79%. The FLIPI was the sole variable predicting OS. Conclusions: Although the majority of patients with localized FL achieve CR, the risk of relapse is high. The FLIPI is of prognostic value in these patients. [ABSTRACT FROM AUTHOR]

It has been suggested that in blast crisis (BC) of chronic myeloid leukaemia (CML) the clinical and laboratory features of patients with ‘lymphoid’ phenotype differ from those of patients with non-lymphoid BC. In order to assess any differences, 97 patients consecutively diagnosed with BC that followed a known chronic phase of CML were analysed. 19 patients had ‘lymphoid’ BC: in 17 the blasts expressed a B-lineage phenotype; in the remaining two they corresponded to T lymphoblasts. Four cases of B-lineage phenotype BC were considered as biphenotypic, due to the co-expression of myeloperoxidase and one or two other myeloid markers (CD33, CD13 and CD68) on the blast cells; in the other six cases of B-lineage BC the blasts expressed one or both of the myeloid markers CD33 (n = 4) and CD13 (n = 3). Patients with ‘lymphoid’ BC seldom had an acceler-ated phase prior to BC (1/19 v 36/78 with non-lymphoid BC, P = 0.002), had less frequent splenomegaly (9/19 v 59/78, P = 0.03) and hepatomegaly (5/19 v 45/78, P = 0.02) and showed a higher degree of marrow blast infiltration (mean value 74 ± 24% v 38 ± 23%, P < 0.0001), lesser blood basophilia (2.2 ± 2.5% v 8.2 ± 7.8%, P < 0.0001), and higher serum albumin levels (P = 0.001) than those with non-lymphoid BC. 13 patients with ‘lymphoid’ BC (68.4%) showed a favourable response to chemotherapy regimens including vincristine and prednisone and, overall, ‘lymphoid’ BC patients survived significantly longer than the remainder (median survival 12 months v 4.7 months, P = 0.006). These results indicate that ‘lymphoid’ BC of CML has a distinct clinicohaematological profile and confirm the better prognosis of such patients. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented discussing use of low dose continuous chemotherapy (LD56) for treatment of patients with recurrent/refractory lymphoma.

Thalidomide is active in patients with refractory myeloma. Seventeen patients (nine men/eight women, median age 73 years) with multiple myeloma (MM) were treated with thalidomide. Fifteen patients had refractory disease and two untested relapse. The median dose of thalidomide was 500 mg (range, 200–800 mg). Nine of the 17 patients (53%) responded. The response rate was significantly higher in patients with no extramedullary disease than in those with soft tissue masses (75% CI: 43–95% versus 0%; P = 0·01)). Of note, no decrease in the size of soft tissue plasmacytomas was observed in all the five patients who had extramedullary involvement. This data suggests that the mechanism of action and effectiveness of thalidomide might depend on the site of the tumour cells. [ABSTRACT FROM AUTHOR]

A response by the authors of an article about a guideline on central nervous system prophylaxis in diffuse large B cell lymphoma patients in a 2013 issue is presented.