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Nodal marginal zone lymphoma (NMZL) is one of the three well-recognized entities within the broad category of marginal zone lymphoma, representing approximately 10% of the cases in this group. Patients typically present with nodal disease, usually at advanced stages, and a thorough work-up and staging are necessary in order to exclude occult extranodal involvement. NMZL shares many similarities with splenic marginal zone (SMZL) and mucosa-associated lymphoid tissue (MALT) lymphomas, such as cytology, immunophenotype, genetic abnormalities and maybe even a common cell of origin: a post-germinal memory B-cell. NMZL is characterized by an indolent course, but its prognosis is generally considered less favorable than that of SMZL and MALT lymphoma, with reported 5-year overall survival rates ranging between 55% and 89%. Therapeutic recommendations for NMZL are derived from small retrospective series or studies on larger cohorts of indolent lymphomas, which include a limited number of patients with NMZL. For localized disease, radiotherapy appears to be the treatment of choice, while rituximab-containing therapy is recommended for advanced stage disease. Due to the rarity of NMZL it is very difficult to perform prospective trials, and an international collaborative effort is necessary in order to better understand the biological features and provide evidence-based treatment recommendations. [ABSTRACT FROM AUTHOR]

Autoimmune hemolytic anemia and thrombocytopenia (AIHA/AITP) frequently complicate the course of non-Hodgkin lymphomas, especially low-grade, but they are very rarely observed in Hodgkin lymphoma (HL). Consequently the frequency and the profile of patients with HL-associated AIHA/AITP have not been well defined. Among 1029 patients with HL diagnosed between 1990 and 2010, two cases of AIHA (0.19%) and three of AITP (0.29%) were identified at the presentation of disease. These patients were significantly older, and more frequently had features of advanced disease and non-nodular sclerosing histology, compared to the majority of patients, who did not have autoimmune cytopenias at diagnosis. ABVD combination chemotherapy (doxorubicin, bleomycin, vinblastine, dacarbazine) provided effective control of HL and the autoimmune condition as well. During approximately 6600 person-years of follow-up for the remaining 1024 patients, seven (0.7%) patients developed autoimmune cytopenias (three AITP, three AIHA, one autoimmune pancytopenia) for a 10- and 15-year actuarial incidence of 0.95% and 1.40%, respectively. Their features did not differ compared to the general population of adult HL. In this large series of consecutive, unselected patients, those who presented with autoimmune cytopenias had a particular demographic and disease-related profile. In contrast, patients developing autoimmune cytopenias during follow-up did not appear to differ significantly from those who did not. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented in response to the article regarding the toxicity in patients with relapsed hairy cell leukemia who are treated with rituximab.

Evaluates the histochemical expression of retinoblastoma gene product (pRb) and WAF1/Cip1 in patients with non-Hodgkin's lymphomas (NHL). Patient characteristics; Detection of pRb in all cases of NHL; Percent of cases with p21 protein detection; Absence of significant prognostic value of pRb and p21 expression.

Determines the efficiency of recombinant human erythropoietin in the restoration of disease-related anemia in B-chronic lymphoproliferative disorders. Demonstration of whether the correction is related to the serum erythropoietin levels before treatment; Comparison of the degree of response with patients' clinical status.

Reports on a patient with primary non-Hodgkin's lymphoma of the gallbladder. Diagnosis of the patient as a T-cell lymphoblastic lymphoma; Inclusion of surgery and postoperative chemotherapy as part of treatment modalities; General prognosis for the disease; Achievement of complete remission.

Evaluates the efficacy of Epirubicin, Bleomycin, Vinblastine and Decarbazine (EBVD) combination chemotherapy followed by low dose involved field radiation therapy in patients with clinical stage I-IIA Hodgkin's disease. Dose administration; Survival rate of patients; Percentage of reduction of tumor load; Response rate.

PET/CT was not performed prior to surgery but the localized nature of the lesion was confirmed by the negative post-surgery PET/CT after the complete resection of the lesion, which had been transformed to anaplastic large cell lymphoma (ALK-negative). It is important to note that there were no other patients within the two cohorts of pediatric and adult cHL patients with 9 and 410 relapsed/refractory cases respectively, in whom a surgery-only approach was applied and failed. At least 70-80% of the patients with Hodgkin lymphoma (HL) can be cured with first-line therapy with the improvement of staging methods and the introduction of increasingly effective chemotherapy regimens during the last 50 years [[1]]. [Extracted from the article]

Yet, the description of a polymorphic cell infiltration in LN biopsy, the absence of clinical lymphadenopathy at presentation, the history of psoriatic arthritis as well as the preceded treatment with anti-tumor necrosis factor (TNF)- and cyclosporine raised diagnostic concerns. Although the patient could not finally avoid R-CHOP immunochemotherapy, this case highlights that a trial of NBA withdrawal under very close monitoring could be a rational therapeutic choice even in patients with aggressive ID-LPDs. Keywords: diffuse large B-cell lymphoma; psoriasis; infliximab; apremilast; novel biologic agent; anti-TNF EN diffuse large B-cell lymphoma psoriasis infliximab apremilast novel biologic agent anti-TNF 1695 1700 6 10/12/23 20231001 NES 231001 The association of psoriasis with cancer has been a matter of debate, especially in the era of the novel biologic agents (NBA). Lastly, taking into consideration our case with the re-appearance of the lymphoma despite the change of NBA as well as all the other cases described in Table 1, the appearance of LPDs in psoriatic patients is a more complex process influenced by clinical disease duration and extension, the time length of immunosuppression and the nature of the various treatments administered. [Extracted from the article]

The article features commentaries on the paper presented by Van Grotel and others on the treatment of pediatric nodular lymphocyte predominant Hodgkin's lymphoma (NLPHL), with only chemotherapy without alkylating agents. While positively commenting on the work, further investigation of the results with the addition of rituximab to the standard anthracycline-based chemotherapy is recommended. NLPHL is considered an indolent disease, and less toxic treatment even in cases of multiple relapses.

We have analyzed the immunohistochemical expression of a wide range of molecules along with the proliferation rate separately in the proliferation centers (PCs) and in the rest of the tumor area, in lymph node or spleen sections of patients with chronic lymphocytic leukemia (CLL). Fas, FasL and c-FLIP were observed both within and outside the PCs in all cases. However, only the difference in FasL expression between the PCs and the non-PC areas attained statistical significance. Median survivin expression in the PCs was higher compared to the non-PC areas. Cleaved caspase 3 was expressed at very low levels both within and outside PCs, while BCL-2 protein was expressed at high levels in all cases in both tumor compartments. Multivariate analysis demonstrated that concurrent overexpression of Fas/FasL/c-FLIP in the PCs was correlated with worse outcome for progression-free survival as well as for overall survival. [ABSTRACT FROM AUTHOR]

The article discusses research which was conducted to determine the validity of the simplified risk stratification/ prognostic score of the Splenic Marginal Zone Lymphoma Working Group in patients with splenic marginal zone lymphoma. Researchers evaluated 177 patients with a median age of 65. They found that the working group's prognositic system was valid.

The article presents a case of a 17-year-old white male who developed transient acute myopericarditis without pericardial effusion (PcE) during all-transretinoic acid (ATRA)-based induction. Topics discussed include abnormally granulated promyelocytes having the unique ability to undergo differentiation into mature granulocytes upon exposure to ATRA, increasing incident of coagulopathy-induced hemorrhage, and myocardial enzymes and serum creatinine levels found in limits.

The article presents two case studies related to no evidence of splenic disease in patients with splenic marginal zone lymphoma (SMZL) undergoing splenectomy for autoimmune hemolytic anemia after monotherapy with rituximab. Topics include the first case was a 56 year-old male diagnosed with SMZL in September 2012, and the second case of a 60-year old woman first diagnosed with SMZL in May 2010 after she presented with abdominal pain and early satiety.

Splenic marginal zone lymphoma (SMZL) is a rare chronic B-cell lymphoproliferative disorder recognized as a distinct entity in the World Health Organization (WHO) classification. SMZL usually runs an indolent clinical course with a median survival of more than 10 years. However, in a proportion of patients (10-20%) SMZL behaves more aggressively, with a median survival of less than 4 years. Many efforts are ongoing to establish commonly accepted prognostic factors as a guide to therapy for this disorder. Data on the treatment of SMZL come from reported retrospective series including relatively limited numbers of patients. Despite these limitations, much progress has recently been made in the management of patients with SMZL. The oldest and most commonly used first-line therapeutic modality is splenectomy, which offers rapid alleviation of splenomegaly-related symptoms along with an improvement of cytopenias in the majority of patients, with a median PFS of 5 years. However, SMZL is a systemic disease, and splenectomy is not carried out with eradicative intent. Furthermore, splenectomy is a major surgical procedure with significant morbidity or even mortality, especially in older patients. Chemotherapy has only moderate activity in this form of MZL. Recent data suggest that rituximab is a very effective therapy with minimal toxicity and could replace splenectomy as first-line treatment. The overall response rate is > 90%, with almost half of responses being complete, while the 5-year progression-free survival is approximately 70%. The combination of rituximab with chemotherapy requires further evaluation. Based on the current data, splenectomy could be abandoned as first-line treatment for patients with SMZL. [ABSTRACT FROM AUTHOR]

The optimal treatment approach for patients with mantle cell lymphoma (MCL) is not well defined. Intensive therapeutic regimens result in high response rates and prolonged progression-free survival but at the expense of significant toxicity. We report here our results of the administration of rituximab plus chlorambucil (R-Chl) as first line treatment in patients with MCL. Twenty consecutively diagnosed patients were treated with this combination in which an induction and a maintenance arm were included. During induction, rituximab was administered at a dose of 375 mg/m2 on day 1, while chlorambucil was given afterward at a dose of 10 mg/day for 10 consecutive days for eight cycles and then as a single agent for an additional four cycles. Maintenance consisted of rituximab administration every 2 months for 1 year. Most patients had indolent disease features such as a low mantle-cell international prognostic index (MIPI) score. The overall response rate was 95% (90% CR, 5% PR). Among patients in CR, 78% presented a molecular remission. The 3-year progression-free survival was 89%. There were no serious side effects. These results show that the R-Chl combination could be an effective therapeutic option as first line treatment in MCL, especially for patients with indolent disease characteristics. [ABSTRACT FROM AUTHOR]

In the present study, we assessed the clinical and pathological data of 76 patients with the diagnosis of non-gastric extranodal marginal zone B-cell lymphoma. The most commonly affected sites were salivary glands, skin, ocular adnexa, lung, intestine and Waldeyer's ring. Ann Arbor stage I disease was present in 39 patients (51%), stage II in 10 (13%) and stage IV in 27 (36%). In 17 cases (21%), the lymphoma presented at multiple mucosal sites. Lymph node and bone marrow involvement were present in 21% and 16%, respectively. Most cases were in the low or low-intermediate risk group. Treatment was heterogeneous and included chlorambucil in 59% either alone or in combination with other agents. Complete and partial remission was achieved in 79% and 7%, respectively, with an overall response rate of 86%. The 5- and 10-year overall survival and cause-specific survival rates were 94%, 82% and 95%, 91%, respectively. The 5- and 10-year progression free survival was 56% and 41%, respectively. The only feature associated with inferior outcome was disease localisation to the lung. [ABSTRACT FROM AUTHOR]

Assesses different treatment strategies for indolent lymphomas. Use of chemotherapy based treatment when the patient develops constitutional symptoms or symptoms related to tumor burden; Treatment of elderly patients with oral alkylating agents; Production of superior progression free survival rates by adding rituximab to conventional chemotherapy.

The standard CHOP regimen may cure 30-40% of patients with advanced aggressive non-Hodgkin's lymphoma (ANHL). Mitoxantrone is an anthracenedione, which is active in NHL and its toxicity profile may be more favorable than doxorubicin with respect to alopecia, mucositis and cardiotoxicity. This study was designed to compare the effectiveness of an escalated dose of mitoxantrone with that of standard doxorubicin, used in the CHOP regimen in patients with ANHL. One hundred and forty three eligible patients with ANHL were randomized to receive 6 cycles of either CHOP (n = 71) or intensified CNOP (iCNOP) (n = 72), with mitoxantrone 20 mg/m², i.v., d.1 instead of doxorubicin. Complete responders (CR) were again randomized either to receive interferon-α (IFN-α) maintenance (3 MU t.i.w., s.c.) or not. The CR rate was 70 vs. 76% for iCNOP and CHOP (p = 0.45), and the overall response rate was 81 vs. 83%, respectively (p = 0.71). The 5-year failure free survival (FFS) was 48 and 50% in the iCNOP and CHOP arm, respectively (p = 0.45), and the 5-year overall survival (OS) was 61 vs. 64% (p = 0.56). IFN-α did not prolong relapse free survival (p = 0.91). iCNOP produced less alopecia (p = 0.001) but more febrile episodes (p = 0.04) than CHOP, while requiring more frequent G-CSF support (p = 0.01). Two cases of acute myelogenous leukemia (AML) were recorded, both in the iCNOP arm (p = 0.14). In conclusion, iCNOP was equally effective to CHOP in patients with ANHL, producing more leukopenia and febrile episodes, but less alopecia. The development of two cases of secondary AML in the iCNOP arm is of concern. [ABSTRACT FROM AUTHOR]