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The article discusses the treatment of splenic marginal zone lymphoma (SMZL) and the lack of randomized trials, highlighting the use of rituximab infusions for the treatment. It examines the bone marrow for complete response (CR), the impact of rituximab on the disease progression, and the overall survival (OS) rate.

Sickle cell disease (SCD) is one of the most frequent and severe monogenic disorders, affecting millions of individuals worldwide. SCD represents a fatal hematological illness, characterized by veno-occlusive events and hemolytic anemia. Hemolytic anemia is caused by abnormal sickle-shaped erythrocytes, which induce parenchymal destruction and persistent organ damage, resulting in considerable morbidity and mortality. During the coronavirus disease 2019 (COVID-19) pandemic, patients with SCD were characterized as a 'high-risk' group due to their compromised immune system, caused by functional hyposplenism, as well as systemic vasculopathy. COVID-19 is characterized by endothelial damage and a procoagulant condition. The present study describes the clinical features, management and outcomes of 3 patients with SCD who were hospitalized due to COVID-19, who all had favorable outcomes despite the complications. [ABSTRACT FROM AUTHOR]

The aim of the present study was to identify factors associated with the risk of development of gastrointestinal acute graft-versus-host disease (GI-aGVHD), as well as to evaluate the impact of various baseline parameters on response to treatment, nonrelapse mortality (NRM), and overall survival (OS) in pediatric patients with GI-aGVHD after allogeneic hematopoietic stem cell transplantation (allo-SCT). We retrospectively analyzed 300 pediatric patients who underwent allo-SCT from HLA-matched related or volunteer unrelated donors in our institution. GI tract involvement was observed in 46 out of 133 patients with aGVHD grade II-IV. Severe aGVHD (grade III-IV) was more frequently observed among patients with GI-aGVHD in comparison with patients without GI involvement (P < .001). Treatment with steroids resulted in durable responses in 22/46 patients; 14 additional patients responded to salvage therapy, whereas 10 were refractory to all treatments administered. Using Cox regression analysis, we observed that serum albumin level ≥3 mg/dL on day 5 after the initiation of therapy with steroids was statistically significantly associated with decreased hazard of NRM and improved OS (P = .021 and P = .026, respectively). In our study, serum albumin level, early (+ day 5) after the onset of steroids in patients with GI-aGVHD, was a predictor of treatment outcome. Prospective randomized trials need to be performed to verify the predictive significance of serum albumin and the need for early intensification of immunosuppressive treatment. [Copyright &y& Elsevier]

Abstract: Several cord blood banks store cord blood units from healthy siblings of patients, who are candidates for stem cell transplantation. We analyzed the quality characteristics of 50 cord blood units collected from families with β-thalassemia major and the outcome of subsequent stem cell transplantations during a 15-year period. All cord blood units were found suitable for banking based on a minimum net volume of 40 ml. The mean volume of the units was 98.9 ml; the mean total nucleated cell count (NC) was 7.8×108 and the mean CD34+ cell count was 2.8×106. Eight out of twelve HLA matched collections were released for transplantation. All but one recipient belonged to Pesaro II-III risk classes. Three patients received a cord blood graft with >5×107 NC/kg . One of them with Pesaro class I disease engrafted, whereas the other two who failed to engraft, were re-transplanted with bone marrow from the same donor later. Cord blood grafts containing NCs <4×107/kg combined with reduced volume bone marrow from the same donor were used in all 5 remaining cases and stable engraftment was achieved. All patients survived, 7/8 thalassemia-free. Cord blood banking from healthy siblings of children with β-thalassemia major can result in a successful transplantation in cases in which there is HLA compatibility. However, in high-risk patients, the use of combined cord blood and bone marrow grafts seems necessary in order to ensure stable engraftment, especially when cord blood unit cell counts are low. [Copyright &y& Elsevier]

The prognostic value of baseline serum free light chain ratio (sFLCR) was investigated in 94 multiple myeloma (MM) patients. sFLCR was calculated as κ/ λ or λ/ κ, depending on the patients’ dominating monoclonal light chain. Median baseline sFLCR was 3·57 in κ-MM patients, 45·09 in λ-MM. ‘High’ sFLCR (≥ the observed median value for κ- and λ-MM respectively) correlated with elevated serum creatinine and lactate dehydrogenase, extensive marrow infiltration and light chain type MM. The 5-year disease-specific survival was 82% and 30% in patients with sFLCR lower than and equal or greater than the median, respectively ( P = 0·0001). sFLCR was an independent prognostic factor. [ABSTRACT FROM AUTHOR]

Primary cutaneous marginal zone lymphoma (PCMZL) is an indolent disease. Treatment options include excision, local irradiation, interferon- α or chemotherapy. We present two patients with PCMZL and multiple skin lesions successfully treated with intralesional administration of the anti-CD20 monoclonal antibody rituximab. The first presented with four red skin lesions and the second with two. Biopsy of the largest lesion revealed marginal zone B-cell lymphoma in both patients. There was no evidence of systemic involvement in either patient. Both patients were treated with intralesional rituximab for 18 consecutive weeks. Skin lesions gradually regressed. Apart from mild local pain during the injection, no other adverse effects were observed. In conclusion, rituximab can be safely administered intralesionally in patients with PCMZL and can produce disease remission. [ABSTRACT FROM AUTHOR]

Purpose: To present our long-term experience regarding the use of chemotherapy plus low-dose involved-field radiotherapy (IFRT) for clinical Stage I-IIA Hodgkin''s lymphoma.Methods and materials: We analyzed the data of 368 patients. Of these, 66 received mechlorethamine, vincristine, procarbazine, and prednisone (MOPP) and 302 received doxorubicin (or epirubicin), bleomycin, vinblastine, and dacarbazine [A(E)BVD]. Patients with complete remission or very good partial remission were scheduled for low-dose IFRT (≤3200 cGy).Results: The 10-year failure-free survival (FFS) and overall survival (OS) rate was 85% and 86%, respectively. A(E)BVD-treated patients had superior 10-year FFS and OS rates compared with MOPP-treated patients (87% vs. 75%, p = 0.009; and 93% vs. 71%, p = 0.0004, respectively). Only 10 of 41 relapses had any infield (irradiated) component. Of the complete responders/very good partial responders treated with low-dose IFRT, those who received <2800 cGy had inferior FFS but similar OS as those who received 2800–3200 cGy. Adverse prognostic factors for FFS included age ≥45 years, leukocytosis ≥10 × 109/L, and extranodal extension. Secondary acute leukemia developed after MOPP with or without salvage therapy (n = 6) or after ABVD plus salvage therapy (n = 2). None of the nine secondary solid tumors developed within the RT fields.Conclusion: IFRT at a dose of 2800–3000 cGy is highly effective in clinical Stage I-IIA HL patients who achieved a complete response or very good partial response with A(E)BVD. The long-term toxicity with respect to secondary malignancies appears to be acceptable. [Copyright &y& Elsevier]

Kyrtsonis M-C, Vassilakopoulos TP, Siakantaris MP, Kokoris SI, Gribabis DA, Dimopoulou MN, Angelopoulou MK, Pangalis GA. Serum syndecan-1, basic fibroblast growth factor and osteoprotegerin in myeloma patients at diagnosis and during the course of the disease. Eur J Haematol 2004: 72: 252–258. © Blackwell Munksgaard 2004. Neovascularisation and bone resorption are related to myeloma disease activity. To investigate the possible prognostic importance of serum syndecan-1, basic fibroblast growth factor (bFGF) and osteoprotegerin (OPG) levels, the relationship between them, with parameters of disease activity and the effect of treatment on their levels. Twenty-seven patients were studied from diagnosis and an additional five from remission, for a median follow-up of 40 months. Twenty-three patients received chemotherapy plus bisphosphonates and nine only bisphosphonates. Sera from 11 healthy individuals (HI) were used as controls. Cytokines were determined by commercially available enzyme-linked immunosorbent assays (ELISA) kits. In HI, median syndecan-1 was 40 ng/mL (28–75), bFGF 8 pg/mL (7–30), OPG 35 pg/mL (4–100). Pretreatment median serum syndecan-1 was 177.5 ng/mL (34–3500), bFGF 11.5 pg/mL (8–65) and OPG 100 pg/mL (4–1000). Pretreatment syndecan-1, bFGF and OPG serum levels were increased in patients compared with HI ( P = 0.001, 0.03 and 0.01, respectively). Syndecan-1 and bFGF levels were correlated with stage ( P = 0.004 and 0.03, respectively). Both syndecan-1 and OPG levels were correlated with β2M ( P = 0.04 and 0.01, respectively). Patients with elevated syndecan-1 and bFGF serum levels had shorter survival than patients with normal levels ( P = 0.01 and 0.05, respectively). After chemotherapy syndecan-1 and OPG levels were found to be decreased in responders and syndecan-1 level was reduced in patients receiving bisphosphonates alone. Pretreatment syndecan-1, bFGF and OPG levels were found to be increased at diagnosis. Syndecan-1 and OPG fluctuated according to MM activity. Elevated serum syndecan-1 and bFGF levels predicted short survival. [ABSTRACT FROM AUTHOR]

Abstract: Objectives : To investigate the prognosis of patients with Hodgkin's lymphoma (HL) who relapse following a complete remission (CR) achieved by chemotherapy with or without radiotherapy (CT±RT), and to identify prognostic factors for freedom from second progression (FF 2 P). Methods : We analyzed the prognostic significance of the initial CT regimen (4 vs. 7–8 drugs), treatment-free interval (TFI), and demographic, clinical, and laboratory factors at the time of relapse and diagnosis, in 113 patients with HL, who relapsed after a CR achieved by CT±RT. Results : Conventional salvage CT±RT was administered in 107 patients, while six received RT only. The 5-yr FF 2 P was 24%, while the 10-yr survival after relapse (O 2 S) was 39% and was not afffected by the initial CT regimen. Multivariate analysis revealed that extranodal disease at relapse ( P <0.001), TFI<6 month ( P <0.001), >=5 involved sites at diagnosis ( P =0.04) and anemia at relapse ( P =0.03) were independent predictors of FF 2 P. 55% of patients had 0 or 1 of these adverse prognostic factors. The 5-yr FF 2 P of patients with 0, 1 or 2 adverse factors was 58%, 34% and 5% ( P <0.0001). The corresponding rates for 10-yr O 2 S were 68%, 51% and 25%, respectively ( P =0.002). Conclusions: Our data confirmed the significance of TFI and extranodal relapse and demonstrated a potential role for anemia at relapse and number of involved sites at diagnosis, for the prognosis of patients with HL relapsing after CT±RT. The combination of these prognostic factors defines a sizeable subgroup of patients with favorable outcome following conventional salvage therapy. [ABSTRACT FROM AUTHOR]

Abstract: Objectives: To describe the clinical, immunophenotypic and molecular features, as well as the clinical course of patients with unusual presentation of mantle cell lymphoma (MCL) purely located to the spleen. Patients and methods: We describe seven patients presented with splenomegaly and a leukemic picture without lymphadenopathy, fulfilling the diagnostic criteria of MCL. In addition to clinical and pathologic features, patients were studied with respect to surface immunophenotype, including adhesion molecule profile, immunohistochemical expression of cyclin-D1 and bcl-1 rearrangement by polymerase chain reaction. Results: Four patients were male and three female. The median palpable spleen size was 15 cm. A preliminary diagnosis of MCL was made, based on blood cell morphology and immunophenotype. All patients underwent splenectomy for therapeutic purposes. Studies done in blood and splenic lymphocytes revealed the following: 7/7 patients were CD19/CD5, CD20 and CD38 positive; CD10 negative and 6/7 CD23 negative. The adhesion molecule expression pattern was consistent in all patients: L-Selectin and CD11c were negative, CD11α and CD18 weakly positive and CD54 strongly positive. The median spleen weight was 1775 g. Histology disclosed a cytologic and architectural pattern consistent with MCL. Cyclin-D1 was positive in 6/6 studied patients. Bcl-1 rearrangement was found in 5/7 patients. Splenectomy was applied as the sole treatment and was beneficial in all patients, with median blood values as following: prior to splenectomy, Ht 29.5%, platelets 110 × 109 /l, lymphoma cells 5.0 × 109 /L, and at 6 months post-splenectomy, Ht 43%, platelets 311 × 109 /l and lymphoma cells 3.0 × 109 /L. Of the seven patients, two developed progressive disease 11 and 26 months post-splenectomy. The remaining five are in improving clinical and hematological condition without chemotherapy at a median follow... [ABSTRACT FROM AUTHOR]

Pulmonary involvement in Waldenström’s macroglobulinaemia (WM) occurs in 3–5% of cases, but lung involvement without bone marrow infiltration is extremely rare. We report 2 patients who presented with bilateral consolidations on chest X-ray and non-specific symptoms and were treated for a long period of time for pulmonary infections until the diagnosis was made by open lung biopsy. Both patients presented high monoclonal IgM in the serum and one also had blood lymphoplasmacytosis. Trephine bone biopsy and bone marrow smears were normal and there was no other site of involvement. Along with the presentation of our patients, we review the literature, discuss some of the possible underlying mechanisms and raise the attention of clinicians to this rare manifestation of the disease.Copyright © 2001 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

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.