Splenic Marginal Zone Lymphoma (SMZL): Clinical Course after Splenectomy. A Single Institution Experience

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 3993-3993
Author(s):  
Susanna S Gaykazyan ◽  
Nalini Janakiraman ◽  
Philip Kuriakose ◽  
Koichi Maeda ◽  
Tareq Hammour
Keyword(s):  
Bone Marrow ◽  
B Cell ◽  
Systemic Therapy ◽  
Marginal Zone ◽  
Clinical Course ◽  
Bone Marrow Involvement ◽  
Marginal Zone Lymphoma ◽  
Low Grade ◽  
Splenic Marginal Zone Lymphoma ◽  
Symptomatic Disease

Abstract SMZL is an indolent B-cell malignancy accounting for 1–2% of chronic lymphoid leukemia found on bone marrow examination and up to 25% of low-grade B-cell neoplasms in splenectomy patients. Aggressive transformation of SMZL rarely occurs. It usually presents as an incidental finding or with symptoms of splenomegaly and anemia. There is still no reliable clinical or biological scoring system for prognostic stratification. We reviewed pathology reports of 41 splenectomized patients at HFHS from 1994 to 2007 and identified 14 patients with splenic marginal zone lymphoma (SMZL). The reasons for splenectomy were symptoms of splenomegaly in all 14 patients, anemia in 13 patients, thrombocytopenia in 12 patients, AIHA in 4 patients, splenic laceration in one patient. We report here the demographics, clinical course and pathology review of these patients. The median age of patients was 77.8 years. There were 7 male and 7 female patients. ECOG performance status was 0–1 in 12(86%), and 2 in 2(14%). Of the 14 patients, 8(57%) were at Ann Arbor stage IV, 1(7%) was at stage III, 4(29%) were at stage II, and 1(7%) at stage I. LDH was above normal in 9(64%) patients B-symptoms were observed in 1(7%). Bone marrow involvement was documented in 8(57%) of the patients. Anemia in 13(93%), thrombocytopenia in 12(86%), AIHA in 4(29%). IPI score was 1–2 in 5(36%), and score 3–4 in 9(64%) of the patients. Median weight of the spleen was 1235 gm. Bone marrow cytogenetics were abnormal in 4(29%) cases. Following splenectomy, cytopenias resolved completely or partially (CR/PR) in 13(93%) patients. Bacterial infections were observed in 4(29%) patients and 2(14%) died of infectious complications. Progressive disease requiring additional systemic therapy was documented in 5(36%) patients. Total of 5(36%) patients died. One secondary to NSLC, 1(7%) of urothelial carcinoma, 1(7%) secondary to hypercalcemia, 2(14%) due to bacterial sepsis. Patients were followed up to 139 months (with median follow-up time of 42 months). The estimated median overall survival (OS) for this group was 116.5 months (9.7 years), the median progression-free survival (PFS) was 91 months (7.6 years). The Kaplan Meier method was used to calculate these estimates. A simple median was calculated for the sample median. In summary, we report the course of 14 patients with SMZL who underwent splenectomy for symptomatic disease. Only 5(36%) required systemic therapy following splenectomy. No death was attributed to progressive SMZL. Overall course was indolent even after splenectomy. Estimated OS was 116.5 months (9.7 years), PFS - 91 month (7.6 years).

DISTINCT CLINICOLABORATORY CHARACTERISTICS of the t(14;19)(q32;q13)-POSITIVE SPLENIC MARGINAL ZONE LYMPHOMA

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 5200-5200
Author(s):  
Hunan Julhakyan ◽  
Aminat Magomedova ◽  
Sergey K Kravchenko ◽  
Tatyana N Obukhova ◽  
Rima Samoylova ◽  
...  
Keyword(s):  
Bone Marrow ◽  
B Cell ◽  
Marginal Zone ◽  
Bone Marrow Involvement ◽  
Marginal Zone Lymphoma ◽  
Research Centre ◽  
Rapid Progression ◽  
Splenic Marginal Zone Lymphoma ◽  
Poor Prognostic Factor ◽  
High Level

Abstract Abstract 5200 Background: Splenic marginal zone lymphoma (SMZL) is a well recognized B-cell neoplasm which is characterized by splenomegaly, bone marrow involvement, immunologically by typical phenotype of marginal zone cells. The most frequent cytogenetic findings are involvement of chromosomes 1, 3, 7(usually deleted in 7q) and 8. The t(14;19)(q32;q13) is a rare cytogenetic abnormality with bcl-3 rearrangement that has been reported in other B-cell lymphomas. Aims: To describe the clinical, morphological, immunophenotypic findings in SMZL associated with t(14;19)(q32;q13). Methods and results: In Hematological Research Centre, Moscow between January 2001 and May 2011 three cases SMZL with t(14;19)(q32;q13) were identified. All patients were males with age 51, 58, 67 y.o. Lymphoma presented with B-symptoms, high level of lactate dehydrogenase (LDH), hepatosplenomegaly and regional lymphadenopathy (enlarge splenic hilar lymphnodes). The hemoglobin was 92 g/l, 110 g/l, 122 g/l. All patients had normal count of leukocytes with an absolute lymphocytosis (lymphocytes count 72 × 109 g/l, 79 × 109 g/l, 83 × 109/l) and thrombocytopenia. Morphological examination of peripheral blood and bone marrow lymphocytes showed that all lymphocytes are atypical with wide cytoplasm and nuclear indentation. In all cases there was nodular type of bone marrow involvement, composed of majority medium sized cells. Immunophenotypic analysis has shown the expression of mature B-cells antigens (CD19, CD20, CD22, FMC7, sIg) and absence of ÑD10, CD23, CD5, CD43, CyclinD1. Two patients were treated with CHOP-regimen without any response. They progressed with spleen enlargement and decreased of thrombocytes counts. All 3 patients undergo splenectomy. Weight of spleen was 1800 g, 2083 g, 2850 g. Splenic section generally show massive nodular pattern (involvement of the white and red pulp) associated with diffuse invasion of the sinuses. In all cases discovered high Ki-67. All patients demonstrated progression after splenectomy during 3–6 months that was characterized by increase of leukocytes count (range 45,4 – 101,8 × 109 /l), high level of LDH, appearance of peripheral and visceral lymph nodes. Considering the increase leucocytes, presence of lymphadenopathy in all cases CHOP, FMC regimen were used. All patients died of disease progression and infectious complications. Time of observation was 21, 30, 34 months. Conclusions: The t(14;19)(q32;q13)-positive SMZL is distinct variant which is characterized by rapid progression after splenectomy, poor responses to chemotherapy and short survival. So t(14;19)(q32;q13) may be regarded as a poor prognostic factor. Disclosures: No relevant conflicts of interest to declare.

Blastic Transformation of Splenic Marginal Zone B-Cell Lymphoma

2000 ◽  
Vol 124 (5) ◽  
pp. 748-752
Author(s):  
Hernani Cualing ◽  
Paul Steele ◽  
David Zellner
Keyword(s):  
Bone Marrow ◽  
B Cell ◽  
Marginal Zone ◽  
B Cell Lymphoma ◽  
Marginal Zone Lymphoma ◽  
Low Grade ◽  
Splenic Marginal Zone Lymphoma ◽  
Moderate Amount ◽  
Blastic Transformation ◽  
One Year

Abstract To our knowledge, blastic transformation of splenic marginal zone lymphoma, a recently characterized low-grade lymphoproliferative disorder, has not been reported previously. In this regard, we report the unique case of a 70-year-old woman whose untreated splenic marginal zone lymphoma underwent blastic transformation 3 years after diagnosis. Her hematologic medical history started in 1988 as thrombocytopenia refractory to steroids associated with atypical lymphoid infiltrate in the bone marrow. She underwent splenectomy in 1989, which revealed splenic marginal zone lymphoma. One year later, the patient developed lymphadenopathy noted in the chest, axillary, abdominal, and retroperitoneal lymph nodes. Because she was asymptomatic, treatment was limited to a conservative supportive regimen. The nodal lymphoma cells had features associated with marginal zone lymphoma and expressed B-cell monotypic κ light chain. She was readmitted for the last time 2 years later with findings of 16% blasts in the peripheral blood and massive infiltration of the bone marrow by large blastoid cells. The blasts showed dispersed chromatin and prominent nucleoli, and possessed a moderate amount of clear cytoplasm. The blasts, like the previous nodal and splenic lymphomas, had a CD20-, CD19-, IgM-positive phenotype, but lacked reactivity for CD5, CD10, and CD23. The patient displayed clinical remission after treatment with vincristine and prednisone, but died of aspiration pneumonia 1 month later. These observations suggest that, similar to the other low-grade lymphoproliferative disorders, an untreated splenic marginal zone lymphoma may undergo high-grade blastic transformation.

CD5+ Extranodal Marginal Zone B-Cell (MALT) Lymphoma:A Low Grade Neoplasm With a Propensity for Bone Marrow Involvement and Relapse

1996 ◽  
Vol 105 (1) ◽  
pp. 31-37 ◽  
Author(s):  
Judith A. Ferry ◽  
Woo-Ick Yang ◽  
Lawrence R. Zukerberg ◽  
Andrew C. Wotherspoon ◽  
Andrew Arnold ◽  
...  
Keyword(s):  
Bone Marrow ◽  
B Cell ◽  
Marginal Zone ◽  
Bone Marrow Involvement ◽  
Low Grade

Absence of Mutations of the Histone Methyltransferase Gene EZH2 In Splenic B-Cell Marginal Zone Lymphoma

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 5086-5086
Author(s):  
Luz Martínez-Avilés ◽  
Marta Salido ◽  
Beatriz Bellosillo ◽  
Vera Adema ◽  
Ana Ferrer ◽  
...  
Keyword(s):  
B Cell ◽  
Marginal Zone ◽  
Mutational Analysis ◽  
Conflicts Of Interest ◽  
Direct Sequencing ◽  
Normal Karyotype ◽  
Marginal Zone Lymphoma ◽  
Low Grade ◽  
Splenic Marginal Zone Lymphoma ◽  
7Q Deletion

Abstract Abstract 5086 Background Splenic marginal zone lymphoma (SMZL) is a rare low-grade B-cell lymphoproliferative disorder with characteristic clinical, cytological, histological and immunophenotypical features. The most common cytogenetic abnormality, present in 30–40% of the patients is the 7q deletion, that extends from 7q21 to 7q36. This aberration may represent a primary pathogenic event in SMZL. Recently, mutations in the EZH2 gene, located at 7q36.1, have been described in different hematological malignancies including B-cell lymphomas. However, the role of the EZH2 gene in SMZL has to be elucidated. Aim To determine the prevalence of EZH2 mutations in a cohort of SMZL patients. Patients and Methods Twenty-nine patients with SMZL were screened for mutations in the EZH2 gene. From the whole cohort, 11 patients presented 7q deletion (three of them as a single anomaly), 11 had a normal karyotype and 7 had other cytogenetic aberrations. The mutational analysis of the EZH2 gene was performed by direct sequencing using primers covering the whole exome of the gene. DNA was extracted from CD19 isolated B-cells from peripheral blood or from total lymphocytes if the percentage of pathologic B-cell was higher than 50%. Results From the whole cohort of 29 SMZL patients, no pathogenic mutations (frameshift or nonsense mutations) were detected in the EZH2 gene in any of the patients analyzed. Five patients harboured the missense mutation D185H in exon 6, that has been previously described as a single nucleotide polymorphism (SNP). Conclusions In conclusion, the EZH2 gene is not mutated in our series of SMZL patients suggesting that this gene is not involved in the pathogeny of this entity. Acknowledgments: Fellowship FI2008 (AGAUR) to LMA, This work was supported (in part) by grants from Instituto de Salud Carlos III FEDER; Red Temática de Investigación Cooperativa en Cáncer (RTICC, FEDER): RD06/0020/0031 and RD07/0020/2004; Ministerio de Sanidad y Consumo (Spain): PI07/0586. Disclosures: No relevant conflicts of interest to declare.

A Phase 2 Study of Fludarabine and Rituximab for the Treatment of Marginal Zone Lymphomas.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 1358-1358 ◽  
Author(s):  
Jennifer R. Brown ◽  
Jonathan Friedberg ◽  
Yang Feng ◽  
Kimberly Phillips ◽  
Jennifer C. Clark ◽  
...  
Keyword(s):  
B Cell ◽  
Marginal Zone ◽  
Marginal Zone Lymphoma ◽  
Chromosome 1 ◽  
Hematologic Toxicity ◽  
Low Grade ◽  
Splenic Marginal Zone Lymphoma ◽  
Phase 2 ◽  
Phase 2 Study ◽  
Grade 3

Abstract The marginal zone lymphomas are a recently defined group of related diseases likely arising from a common cell of origin, the marginal zone B cell. The clinical presentation varies; data on therapy for subtypes other than gastric MALT has been largely limited to retrospective case series. We therefore undertook this prospective phase 2 study of fludarabine 25 mg/m2 for 5 days with rituximab 375 mg/m2 on day 1 for the treatment of marginal zone lymphomas. To be eligible, patients were required to have newly diagnosed or relapsed, histologically confirmed MALT, marginal zone lymphoma, or a CD5/CD10 negative low-grade B cell lymphoproliferative disorder. They could not be candidates for curative local therapy. From 2004 to 2007, 26 patients were enrolled with a median age of 64 (31–84) and a median time from diagnosis to treatment of 1.6 months. This was the initial therapy for 21 of 26 patients (81%). Seven were diagnosed with MALT lymphomas (27%), 12 with nodal marginal zone lymphomas (46%), 3 with splenic marginal zone lymphoma (12%) and 4 with CD5/10 negative low-grade lymphoproliferative disorders (15%). FISH for BCL-6, trisomy 3, MALT1 and chromosome 1 rearrangements was attempted on 18 available tissue biopsies. Of these, four were normal, three showed BCL-6 rearrangement with other abnormalities, four had chromosome 3 abnormalities, two MALT1 rearrangements and one chromosome 1 abnormality. The majority of patients had stage IV disease (18; 69%), with 5 stage 3, 2 stage 2 and 1 stage 1E disease. Of the 23 patients who have completed therapy, 18 completed at least 4 cycles (78%), with 12 patients completing the planned 6 cycles (52%). Nine patients discontinued therapy due to unacceptable toxicity (39%), six for hematologic toxicity, two for grade 3 rash and one for a delayed grade 3 reaction to rituximab. Of 26 patients evaluable for toxicity, 46% developed grade 4 toxicity (solely hematologic), and 35% grade 3 toxicity. Grade 3–4 toxicities included: neutropenia 14 (54%), thrombocytopenia 5 (19%), febrile neutropenia 2 (8%), rash 3 (11%), myositis 1 (4%), allergic reaction 1 (4%). Two delayed opportunistic pneumonias were observed, one Nocardia and one P. jiroveci. The ORR in the 23 patients who have completed therapy and are evaluable for response is 83% (95% CI 61–95%), with 12 patients achieving CR/CRu (52%). Three patients have relapsed. Two patients have died, one due to small cell lung cancer diagnosed after study enrollment, and the other due to urosepsis with bone marrow aplasia. At the median follow-up of 1.8 years, the PFS is 84% (95% CI 68–99%), and OS 94% (95% CI 82–99%). Concurrent fludarabine and rituximab is therefore a highly effective regimen in the treatment of marginal zone lymphoma but one which is complicated by significant hematologic toxicity and allergic hypersensitivity. These toxicities prevented half the patients from completing the planned therapy and were more severe than usually seen in other low-grade lymphomas, emphasizing the need to study marginal zone lymphomas as a separate entity.

scholarly journals VILLOUS LYMPHOCYTES IN BLOOD AND BONE MARROW IN SOME FORMS OF B-CELL LYMPHOID MALIGNANCIES

2020 ◽  
pp. 29-33
Author(s):  
Alyona Polishchuk ◽  
Michael Zavelevich ◽  
Daniil Gluzman
Keyword(s):  
Bone Marrow ◽  
B Cell ◽  
Hairy Cell Leukemia ◽  
Marginal Zone ◽  
Marginal Zone Lymphoma ◽  
Splenic Marginal Zone Lymphoma ◽  
Hairy Cell ◽  
Cell Leukemia ◽  
Trap Activity ◽  
Red Pulp

The cytological and immunocytochemical features of the lymphocytes with villous morphology in peripheral blood and bone marrow in some B-lymphoproliferative disorders were studied. The diagnosis of hairy cell leukemia, a hairy cell leukemia variant, splenic marginal zone lymphoma and splenic diffuse red pulp small B-cell lymphoma was ascertained in accordance with the new revision of the WHO classification (2016). The neoplastic cells of hairy cell leukemia were determined by the presence of high tartrate resistant acid phosphatase (TRAP) activity. Cell surface expression of CD19, CD20 and CD21 antigens was detected. Also, the expression of CD25, CD103 and CD200, and in some cases cyclin D1, was found out. CD5, CD10 and CD23 were not detected. The immunophenotype of cells in splenic marginal zone lymphoma with villous processes also corresponded to the mature B cells. The expression of CD19, CD20 and CD21 was observed in all cases, CD11c – in 50% of patients, CD25 or CD5 – in 10% of patients. In 80% of patients, the pathologic cells did not show TRAP activity. In the bone marrow and peripheral blood cells of patients with diffuse red pulp lymphoma, TRAP activity was not detected. An immunophenotype in the hairy cell leukemia variant was different from those of classic HCL (CD19+CD20+CD22+CD103+CD11c+CD5–CD10–CD23–). Characterized immunophenotypical markers, which have differential diagnostic values in several forms of lymphoid tumors of B cell origin, will be important for the choice of treatment methods and prognosis

Outcomes in Patients with Splenic Marginal Zone Lymphoma or Marginal Zone Leukemia/Lymphoma Treated with Immunotherapy, Chemoimmunotherapy, or Chemotherapy.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 922-922
Author(s):  
Apostolia-Maria Tsimberidou ◽  
Daniel Catovsky ◽  
Ellen Schlette ◽  
Susan O’Brien ◽  
Hagop Kantarjian ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Systemic Therapy ◽  
Marginal Zone ◽  
Statistical Significance ◽  
Univariate Analysis ◽  
Median Number ◽  
Marginal Zone Lymphoma ◽  
Cancer Center ◽  
Splenic Marginal Zone Lymphoma ◽  
Platelet Counts

Abstract Purpose: The optimal management of splenic marginal zone lymphoma (SMZL) or marginal-zone leukemia/lymphoma (MZL) is controversial. We retrospectively assessed the clinical outcome of patients with SMZL/MZL treated with systemic therapy. Patients and Methods: Patients were assessed by the time of their first treatment at U. T. M. D. Anderson Cancer Center, Dept. of Leukemia (5/95 to 10/04). Diagnosis was confirmed in 70 patients by slide review. The indications for treatment were the same as those used for patients with CLL. Results: The median age was 64 years (range, 33–88); and 61% of patients had monoclonal gammopathy. The median number of CD20 molecules/cell was 65.2 x 103 (16–260 x 103). Of the patients who required systemic therapy, 26 were treated with immunotherapy (rituximab, 25; alemtuzumab, 1); 6 with chemoimmunotherapy (CI/T; rituximab combined with a fludarabine-based regimen); and 11 with chemotherapy (C/T). Ten patients had splenectomy, and 17 were in the observation group. The overall response rates were 88% (CR, 31%) in the immunotherapy group, 83% (CR, 17%) in the CI/T group, and 55% (CR, 18%) in the C/T group. The median follow-up was 2.7 years. Patients treated with immunotherapy +/− C/T had higher rates of overall and failure-free survival compared with those treated with C/T. Figure Figure In univariate analysis, the only factors predicting longer survival were age >60 years (p=0.01) and immunotherapy +/− chemotherapy (p=0.04). Seventeen (24%) of 70 patients had other malignancies prior to (n=8) or after (n=8) treatment of SMZL/MZL or both (n=1). Changes in bone marrow and blood counts in patients treated with rituximab (n=25) were compared with those of patients who had splenectomy as initial therapy (n=17, including 7 who had subsequent therapies). Rituximab resulted in the disappearance of a palpable spleen (median size, 6 cm; range 0–20cm) in 23 (92%) patients. Rituximab was superior to splenectomy in normalizing the white blood cell (WBC) counts (p<0.001) and absolute lymphocyte counts (ALC)(p<0.001). Splenectomy resulted in higher platelet counts compared with rituximab, but platelet counts remained within the normal range in all patients treated with rituximab. Hemoglobin levels and bone marrow cellularity did not reach statistical significance, but there was a trend towards a significantly lower proportion of lymphocytes in patients treated with rituximab (p=0.1). Conclusions: Rituximab with or without C/T induces durable remissions and prolongs survival in patients with SMZL/MZL, probably because CD20 molecules/cell are higher in SMZL/MZL than in CLL. Our data demonstrate that rituximab effectively controls SMZL, as evidenced by improvement in WBC and ALC and splenomegaly, and may be the treatment of choice, at least in older SMZL patients with comorbid diseases. Clinical trials of immunotherapy or CI/T are warranted.

scholarly journals Splenic marginal zone lymphoma: from genetics to management

Blood ◽  
2016 ◽  
Vol 127 (17) ◽  
pp. 2072-2081 ◽  
Author(s):  
Luca Arcaini ◽  
Davide Rossi ◽  
Marco Paulli
Keyword(s):  
Bone Marrow ◽  
B Cell ◽  
Marginal Zone ◽  
Treatment Options ◽  
Marginal Zone Lymphoma ◽  
Splenic Marginal Zone Lymphoma ◽  
Virus Eradication ◽  
Marginal Zone B Cells ◽  
Asymptomatic Patients ◽  
Typical Cell

AbstractSplenic marginal zone lymphoma (SMZL) is a rare B-cell malignancy involving the spleen, bone marrow, and frequently the blood. SMZL lymphomagenesis involves antigen and/or superantigen stimulation and molecular deregulation of genes (NOTCH2 and KLF2) involved in the physiological differentiation of spleen marginal zone B cells. Diagnosis requires either spleen histology or, alternatively, the documentation of a typical cell morphology and immunophenotype on blood cells coupled with the detection of intrasinusoidal infiltration by CD20+ cells in the bone marrow. Among B-cell tumors, deletion of 7q and NOTCH2 mutations are almost specific lesions of SMZL, thus representing promising diagnostic biomarkers of this lymphoma. Although the majority of SMZLs show an indolent course with a median survival of approximately 10 years, nearly 30% of patients experience a poor outcome. No randomized trials are reported for SMZL, and few prospective trials are available. A watch-and-wait approach is advisable for asymptomatic patients. Treatment options for symptomatic patients ranges from splenectomy to rituximab alone or combined with chemotherapy. In some geographic areas, a subset of patients with SMZL associates with hepatitis C virus infection, prompting virus eradication as an effective lymphoma treatment. It would be worthwhile to explore deregulated cellular programs of SMZL as therapeutic targets in the future; improved clinical and biological prognostication will be essential for identifying patients who may benefit from novel approaches.

scholarly journals Whole-genome sequencing identifies recurrent somatic NOTCH2 mutations in splenic marginal zone lymphoma

2012 ◽  
Vol 209 (9) ◽  
pp. 1553-1565 ◽  
Author(s):  
Mark J. Kiel ◽  
Thirunavukkarasu Velusamy ◽  
Bryan L. Betz ◽  
Lili Zhao ◽  
Helmut G. Weigelin ◽  
...  
Keyword(s):  
B Cell ◽  
Marginal Zone ◽  
Marginal Zone Lymphoma ◽  
Lymphocytic Leukemia ◽  
Primary Lymphoma ◽  
Reactive Lymphoid Hyperplasia ◽  
Low Grade ◽  
Splenic Marginal Zone Lymphoma ◽  
Whole Genome ◽  
Histological Transformation

Splenic marginal zone lymphoma (SMZL), the most common primary lymphoma of spleen, is poorly understood at the genetic level. In this study, using whole-genome DNA sequencing (WGS) and confirmation by Sanger sequencing, we observed mutations identified in several genes not previously known to be recurrently altered in SMZL. In particular, we identified recurrent somatic gain-of-function mutations in NOTCH2, a gene encoding a protein required for marginal zone B cell development, in 25 of 99 (∼25%) cases of SMZL and in 1 of 19 (∼5%) cases of nonsplenic MZLs. These mutations clustered near the C-terminal proline/glutamate/serine/threonine (PEST)-rich domain, resulting in protein truncation or, rarely, were nonsynonymous substitutions affecting the extracellular heterodimerization domain (HD). NOTCH2 mutations were not present in other B cell lymphomas and leukemias, such as chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL; n = 15), mantle cell lymphoma (MCL; n = 15), low-grade follicular lymphoma (FL; n = 44), hairy cell leukemia (HCL; n = 15), and reactive lymphoid hyperplasia (n = 14). NOTCH2 mutations were associated with adverse clinical outcomes (relapse, histological transformation, and/or death) among SMZL patients (P = 0.002). These results suggest that NOTCH2 mutations play a role in the pathogenesis and progression of SMZL and are associated with a poor prognosis.

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