Splenic marginal zone lymphoma presenting as myelofibrosis associated with bone marrow involvement of lymphoma cells which secrete a large amount of TGF-� (original) (raw)
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Splenic Marginal Zone Lymphoma In Bone Marrow:A Case Report And Review Of The Literature
2020
Marjinal zon lenfomalar (MZL), sekonder lenfoid folikullerin marjinal bolgesinde B lenfositlerinden kaynaklanan nadir gorulen dusuk dereceli B lenfomalaridir. Noduler Hodgkin lenfomalarinin (NHL) %1'inden azini olustururlar. Dunya Saglik Orgutu'nun siniflamasina gore marjinal bolge lenfomalar uc gruba ayrilir: ekstranodal MALT lenfoma, dalak MZL (SMZL) ve nodal MZL. Her uc grupta da kromozomal, genetik ve immunfenotibik benzerlikler vardir. SMZL genellikle kemik iligi ve dalak hilar lenf dugumleri tutan bir lenfomadir. Dalak periferik kan ve bazen karaciger infiltrasyon gosterir, ve genellikle yasamin altinci on yilinda ortaya cikar. Tedavi icin standart bir yaklasim yoktur. Hastalarin ucte ikisi tani sirasinda asemptomatiktir ve hastalarin onemli bir kismi basarili bir sekilde splenektomi ile tedavi edilir.
Unusual Course of Splenic Marginal Zone Lymphoma
World Journal of Oncology, 2013
A 53-year-old woman was diagnosed with splenic marginal zone lymphoma by pathological examination on left submandibular lymph node and bone marrow biopsies and markedly enlarged spleen. Four cycles of Rituximab chemotherapy were given. Seven months after finishing Rituximab chemotherapy, she developed left upper extremity swelling without evidence of deep venous thrombosis. Repeat PET/CT scan demonstrated multiple left axillary lymph nodes extending to left retroclavicular region and a new lymph node posterior to the left scapula. Biopsy of the lymph node demonstrated marginal zone lymhoma pattern with increased numbers of large cells, but not outright diffuse large B-cell lymphoma. Despite resuming rituximab, patient had persistent leukocytosis and severe anemia. Restaging PET/CT showed 3 new left anterior cervical lymph nodes and 1 new right axillary lymph node. Spleen has further enlarged. R-CHOP chemotherapy was started, which improved leukocytosis. After 4 cycles of R-CHOP, PET/CT showed new metabolic activity within right inguinal and abdominal lymph nodes. Patient was given one cycle of Bendamustine. She developed a possible "hematoma" in right medial elbow. However, MRI study revealed a subcutaneous deposit of the lymphoma. Patient needs consistently blood transfusion and she deteriorated quickly. Our patient had an aggressive course of splenic marginal zone lymphoma, not responding to four trials of chemotherapy although SMZL is well-known to be an indolent low grade lymphoma. This case report emphasizes the importance to individualize the treatment in SMZL patients and repeat bone marrow biopsy if the disease recurs.
A Case Report on Splenic Marginal Zone Lymphoma
Journal of Pharmaceutical Research International, 2021
Background: Out of the various malignant tumours originating from the lymphatic hematopoietic system, lymphoma is one such important entity. It is divided into Non-Hodgkin’s Lymphoma (NHL) and Hodgkin Lymphoma (HL) depending on its cell source. A very rare type of malignant variant of lymphoma is the primary splenic lymphoma, involving exclusively the spleen and splenic hilar lymph nodes. Moreover, splenic marginal zone lymphoma (SMZL) is even more infrequent. SMZL is an uncommon chronic B lymphocyte proliferative disease, which only accounts for about 1–2% of all non-Hodgkin’s lymphoma. The mean age of SMZL incidence is about 65 years. There is no known significant gender predominance. A quarter of patients with early diagnosed SMZL have known to have vague symptoms like abdominal pain and distention; and other patients may be accompanied by loss of weight, malaise, cachexia, splenomegaly, or other manifestations. Conclusion: Although, a good prognostic outcome is what is usually e...
Progression to Large B-Cell Lymphoma in Splenic Marginal Zone Lymphoma
The American Journal of Surgical Pathology, 2001
Splenic marginal zone lymphoma (SMZL) is considered to be an indolent extranodal B-cell lymphoma. Despite its low aggressivity, histologic progression has been described in sporadic reports, although the frequency, characteristics, and underlying molecular abnormalities of this phenomenon are largely unknown. We review here the clinical, morphologic, immunohistochemical, and molecular features of a series of 12 SMZL cases that showed progression to large B-cell lymphoma (LBCL). The most frequent location of secondary LBCL was in peripheral lymph node. This occurred between 12 and 85 months after diagnosis of SMZL. However, in two cases LBCL was diagnosed at the initial stage of the disease (one spleen tumoral nodule and one hilar lymph node). The histologic and immunophenotypic features of these cases were similar to those of transformed LBCL at other sites. In four cases the immunoglobulin heavy chain gene polymerase chain study revealed the same rearrangement pattern in both primary and secondary tumors, thereby confirming their identity and excluding the possibility of a second malignancy. As is the case with other low-grade lymphoproliferative disorders, SMZL may undergo high-grade transformation. These 12 cases represent 13% of our series of SMZL with adequate follow-up. The incidence of large cell transformation in SMZL seems to be lower than in follicular lymphoma (25-60%) and mantle cell lymphoma (11-39%), although it is similar to the frequency of transformation in B-chronic lymphocytic lymphoma/small lymphocytic lym-phoma (1-10%). The mean proliferative index (MIB1 staining) in initial SMZL specimens of cases with LBCL transformation was 28.6%, higher than that of MIB1 staining in the overall SMZL series (21.8%), although not statistically significantly so. p53 or p16 INK4a inactivation in this series was observed in only one case, in contrast with the situation observed in chronic lymphocytic leukemia, follicular lymphoma, and mantle cell lymphoma. It seems that progression in SMZL is mainly independent of p53 or p16 INK4a inactivation. The frequency of the 7q deletion in this series was 3 of 7 (42%). 7q loss may play an alternative role in the inactivation of the p53 and p16 INK4a pathway, thereby favoring tumoral progression.
factors in a series of 60 patients Splenic marginal zone lymphoma: clinical characteristics and prognostic http://bloodjournal.hematologylibrary.org/content/100/5/1648.full.html Updated information and services can be found at: (4217 articles) Neoplasia (3716 articles) Clinical Trials and Observations Articles on similar topics can be found in the following Blood collections http://bloodjournal.hematologylibrary.org/site/misc/rights.xhtml#repub\_requests Information about reproducing this article in parts or in its entirety may be found online at: http://bloodjournal.hematologylibrary.org/site/misc/rights.xhtml#reprints Information about ordering reprints may be found online at: http://bloodjournal.hematologylibrary.org/site/subscriptions/index.xhtml Information about subscriptions and ASH membership may be found online at: A precise description of clinical features at presentation and analysis of clinical and biologic prognostic factors in splenic marginal zone lymphoma (SMZL) are still lacking. Here we describe the clinical and biologic features of a series of 60 SMZL patients diagnosed after splenectomy. Analysis for overall survival (OS), failurefree survival (FFS), and the probability of obtaining a response was performed using univariate and multivariate tests. The median age of the patient was 63 years (range, 35-84 years). Performance status according to the Eastern Cooperative Oncology Group (ECOG scale) was 0 ؍ 16%, 1 ؍ 58%, and 2 ؍ 25%. Of the 60 patients, 53 (86.6%) were at Ann Arbor stage IV. All 60 patients received splenectomies, 29 of 60 also received chemotherapy, and 2 received spleen radiotherapy. A complete response (CR) was achieved by 38.3% of patients, and a partial response (PR) was achieved by 55%. Mean OS of the series was 103 months (range, 2-164 months); mean FFS was 40 months (range, 3-164 months). At 5 years from diagnosis, 39 patients (65%) were alive. Patients dying from the disease had a relatively aggressive clinical course, with a short survival (17.5 months [range, 2-72 months]). Significant prognostic factors in multivariate analysis were (1) (for OS and FFS) lack of response to therapy (CR versus noncomplete response [nCR]) and involvement of nonhematopoietic sites, and (2) (for the probability of obtaining CR) bone marrow involvement. Chemotherapy did not influence OS or FFS. p53 overexpression predicted a shorter OS in the univariate analysis. These data confirm the relative indolence of this disease, indicating the existence of a subset of more aggressive cases, which should stimulate the search for predictive biologic factors and alternative therapies. (Blood. 2002;100:1648-1654)
Splenic Marginal Zone Cell Lymphoma: Case Report
SAS Journal of Surgery, 2020
Case Report Splenic marginal zone cell lymphoma (LZMS) is a very rare B lymphoma; Representing 2% of all NHL, this lymphoma invades the spleen, perisplenic nodes and frequently the marrow, which can be a source of diagnostic traps. Patient aged 65, hospitalized for the exploration of a splenic tumor mass confirmed by a computed tomography (CT) scan. The biological assessment finds an inflammatory SD + high LDH. A splenectomy was performed. The microscopic study of the operating room showed a diffuse lymphomatous proliferation with small cells, with labeling by CD20. CD 5 and CD 43 are negative. The diagnosis of LZMS was accepted. LZMS affects the subject over the age of 50, usually characterized by the presence of a large splenomegaly without lymphadenopathy. The hemogram shows in three quarters of the cases the inconsistent presence of villous lymphocytes. The diagnosis is essentially anatomopathological, it shows a constant nodular or sometimes diffuse attack of the white pulp of the splenic parenchyma. The tumor cells are small, expressing the B lymphoid markers: CD19, CD20, CD22, CD79. They are negative for CD5, CD10, cyclinde D1 and CD43. No specific cytogenetic abnormality of LZMS was identified. It is an indolent lymphoma, the treatment of which has not yet been codified, depends on prognostic factors. Death is linked to the risk of transformation to large cell lymphoma.
Splenectomy--a therapeutic option in splenic marginal zone cell lymphoma
Romanian journal of internal medicine = Revue roumaine de médecine interne, 2009
We present the case of a 65 years old male, admitted in the Hematology Department of the Universitary Emergency Hospital Bucharest, complaining of physical asthenia and weight loss; periodical medical examination has revealed splenomegaly and leucocytosis with lymphocytosis, persistent for the past 3 years. The clinical and paraclinical exam demonstrated splenomegaly (21 cm in diameter on computer tomography scan), hepatomegaly and generalized lymphadenopathies. The laboratory tests confirmed leucocytosis with lymphocytosis--a clonal population of B lymphocytes CD20+ CD19+ CD23+/- CD79b+(low), CD43+ FMC7+ CD5+ CD38+ ZAP70+ cyclin D1-. Lymph node and bone marrow biopsy together with flowcytometry established the diagnosis of Malignant non-Hodgkin Lymphoma--Atypical Splenic Marginal Zone B-cell lymphoma (aberrant expression of CD5) stage IVB, with leukemic picture, complicated with autoimmune hemolytic anemia with highly positive Coombs' tests. We performed therapeutic splenectomy...
British Journal of Haematology, 2003
We studied 86 bone marrow biopsies (BMB) from 58 patients presenting with primary splenic marginal zone lymphoma (PSMZL). In 42 patients, a splenectomy was performed which enabled a histopathological diagnosis. In these patients, 44 biopsies were carried out before, and 25 after, splenectomy. In 16 recently observed patients, 17 BMB led to PSMZL diagnosis, and these patients were treated without splenectomy. Seven different patterns of infiltrates were recognized: intravascular, interstitial, nodular, massive, plasmacytic mimicking myeloma and transformation into large B-cell lymphoma (DLBCL). The association of an intravascular infiltrate and nodules with a germinal centre and/or a marginal zone favoured a diagnosis of MZL. Immunohistochemistry demonstrated the expression of B cell-associated antigens and, in 40% of the patients, a monotypic lymphoplasmacytic cell component. These patients often presented a serum M component and autoimmune disorders. In the past, such cases have been diagnosed as lymphoplasmacytic lymphoma. BM involvement was present in all patients. Successive biopsies showed progression and, after chemotherapy, a slight decrease in infiltrates. Transformation into DLBCL occurred in 11 of 34 patients. The patterns described are not specific for PSMZL and occur also in primary nodal MZL and, more rarely, in MALT-type lymphoma.