scholarly journals CD5-Negative, CD10-Negative Low-Grade B-Cell Lymphoproliferative Disorders of the Spleen

2021 ◽  
Vol 28 (6) ◽  
pp. 5124-5147
Author(s):  
John J. Schmieg ◽  
Jeannie M. Muir ◽  
Nadine S. Aguilera ◽  
Aaron Auerbach
Keyword(s):  
Bone Marrow ◽  
B Cell ◽  
Hairy Cell Leukemia ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Lymphoproliferative Disorders ◽  
Low Grade ◽  
Hairy Cell ◽  
Cell Leukemia ◽  
Red Pulp

CD5-negative, CD10-negative low-grade B-cell lymphoproliferative disorders (CD5-CD10-LPD) of the spleen comprise a fascinating group of indolent, neoplastic, mature B-cell proliferations that are essential to accurately identify but can be difficult to diagnose. They comprise the majority of B-cell LPDs primary to the spleen, commonly presenting with splenomegaly and co-involvement of peripheral blood and bone marrow, but with little to no involvement of lymph nodes. Splenic marginal zone lymphoma is one of the prototypical, best studied, and most frequently encountered CD5-CD10-LPD of the spleen and typically involves white pulp. In contrast, hairy cell leukemia, another well-studied CD5-CD10-LPD of the spleen, involves red pulp, as do the two less common entities comprising so-called splenic B-cell lymphoma/leukemia unclassifiable: splenic diffuse red pulp small B-cell lymphoma and hairy cell leukemia variant. Although not always encountered in the spleen, lymphoplasmacytic lymphoma, a B-cell lymphoproliferative disorder consisting of a dual population of both clonal B-cells and plasma cells and the frequent presence of the MYD88 L265P mutation, is another CD5-CD10-LPD that can be seen in the spleen. Distinction of these different entities is possible through careful evaluation of morphologic, immunophenotypic, cytogenetic, and molecular features, as well as peripheral blood and bone marrow specimens. A firm understanding of this group of low-grade B-cell lymphoproliferative disorders is necessary for accurate diagnosis leading to optimal patient management.

scholarly journals A Case of CD5 Positive Hairy Cell Leukemia withBRAF V600EMutation and Lymph Node Involvement; Unique Case Report and Review of the Literature:

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 17-18
Author(s):  
Sara Javidiparsijani ◽  
Yun Kyong Choi ◽  
Arnaldo A. Arbini
Keyword(s):  
Bone Marrow ◽  
Lymph Node ◽  
B Cell ◽  
Hairy Cell Leukemia ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Lymph Node Involvement ◽  
Hairy Cell ◽  
Cell Leukemia ◽  
Node Involvement

Objective: Hairy cell leukemia (HCL) is distinct indolent neoplasm of small mature B cells accounting for 2% of the lymphoid leukemias. The classic immunophenotypic profile of HCL includes bright expression of B cell surface antigen and immunoglobulins and lack of both CD5 and CD10. The tumor cells are predominantly present in the spleen, peripheral blood and bone marrow. Aberrant expression of CD5 and lymph node involvement are very rare. A case of CD5(+) HCL with a documentedBRAF V600Emutation has not been previously reported. This case is also unique due to lymph node involvement by HCL. To our knowledge, this is the only case report of a documentedBRAF V600Emutated CD5(+) HCL along with lymph node involvement in the literature. Method: We are presenting a 45 years old man with multiple prior medical conditions including diabetes, COPD and interstitial pneomonitis with new onset of B-symptoms and neutropenia/monocytopenia. Peripheral blood flow cytometry revealed 21% CD5(+) CD10(-) monoclonal B cell population. Further workup revealed the neoplastic cells are positive for CD11c, CD103 and CD25. Bone marrow study revealed an extensive involvement of the marrow.By immunohistochemistry, the cells are also positive for CD5, CD20, BRAF and cyclin D1. Cytogenetic studies show that the cells are negative t(11;14) and molecular studies revealedBRAF V600Emutation. Reexamination of the prior wedge lung resection revealed intrathoracic lymph node involvement. Results and conclusion: The most common differential diagnoses of a CD5(+) B cell lymphoma involving the bone marrow is chronic lymphocytic leukemia (CLL) and mantle cell lymphoma. The key into the diagnosis of this case was marked monocytopenia that lead to addition of HCL antibody tubes despite the CD5 positivity and detection of CD25, CD103 and CD11c antigens in the leukemic cells. Bone marrow biopsy show extensive involvement by a CD5(+) cyclin D1(+) B cell lymphoma. The most important differential diagnosis in this case was mantle cell lymphoma. The diagnosis of HCL was confirmed by detection ofBRAF V600Emutation and absence of t (11;14). Retrospective examination of the intrathoracic lymph nodes show one lymph node involvement by hairy cell leukemia cells. The patient was treated with Vemurafenib and his follow up flow cytometry revealed only 0.60% leukemic involvement. Incorporation of clinical data at the time of flow cytometric examination is essential to aid the hematopathologist in having a broader differential diagnoses and addition of appropriate tests that lead to the more accurate diagnosis and treatment. Disclosures No relevant conflicts of interest to declare.

Splenic Diffuse Red Pulp Small B Cell Lymphoma: Transformation To Diffuse Large Cells B Lymphoma

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 5070-5070
Author(s):  
Evandro Dantas Bezerra ◽  
Leila Patricia Fontenele ◽  
Juliana Pereira ◽  
Luis Alberto de Padua Covas Lage ◽  
Felipe Maciel ◽  
...  
Keyword(s):  
B Cell ◽  
Hairy Cell Leukemia ◽  
Clinical Course ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Large Cell ◽  
Complex Karyotype ◽  
Hairy Cell ◽  
Cell Leukemia ◽  
Red Pulp

Abstract Objective Report immunophenotypic and genetic aspects and clinical course of a case of Splenic Diffuse Red Pulp Small B Cell Lymphoma with transformation to diffuse large cell. Introduction Until recently, only Hairy Cell Leukemia was recognized as B chronic lymphoproliferation with primary involvement of the splenic red pulp. The 2008 WHO classification included two new provisional entities: Splenic diffuse red pulp small-B cell lymphoma and Hairy Cell Leukemia variant. These entities are rare, encompassing less than 1% of B chronic lymphoproliferation, characteristically presenting as an indolent clinical course and good control with splenectomy. Case report Female, 29 years old, with non-replicative chronic hepatitis B, and splenomegaly detected 7 years before without evidence of portal hypertension or schistosomiasis. Two months before admission to our hospital, she developed increasing splenomegaly for 39cm, B symptoms and abdominal lymphadenopathy. CBC showed Hb: 8,1g/dl, platelet: 80.000/mm3, WBC: 12.000/mm3 with 85% of abnormal lymphoid cells with immunophenotyping CD45++/CD19+/CD20++/CD25+/CD23dim/CD200dim/FMC7+/CD11c-/CD103- and cykappa restriction. Complex karyotype in PB suggested lymphoproliferative disorder, with strutural abnormalities in 14q32, isocromosomes 8 and 17, deletions 6q and 7q and trisomy 18. FISH studies showed extra copy of MYC and p53 deletion. Bone marrow aspirate showed 7,6% of cells with same phenotype. CT and PET-CT showed splenomegaly of 39cm (SUV: 6.1), hepatomegaly (SUV: 3.2), abdominal lymph node conglomerate (SUV: 6.3), cervical and mediastinal lymphadenopathy with no increase in dimensions (SUV: 3,8). The patient was submitted to splenectomy and histological findings revelead mature lymphoid neoplasm, ranging from small to intermediate cells with few large cells, primary from spleen red pulp and diffusely infiltrating the sinusoidal spaces and secondly the white pulp (CD20+, DBA44+, CD5-, CD10-, CD23-, CD3-, cyclin D1- and Ki67: 40%). The cytogenetic analysis of the spleen showed similar complex karyotype as seen in PB. With the diagnosis of Splenic Diffuse Red Pulp Small B Cell Lymphoma with aggressive transformation to diffuse large cell, she was submitted to 8 cycles of R-CHOEP and is in complete remission, waiting for consolidation with high-dose chemotheraphy followed with peripheral stem cells rescue. Discussion Splenic Diffuse Red Pulp Small B-cell Lymphoma was included as a provisional entity in WHO 2008 classification. The few cases reported show an indolent course with good response to splenectomy. In this case the diagnosis was suspect with clinical course, cytogenetic and flow cytometry PB studies and confirmed after splenectomy when the clinical course of disease changed with B symptoms, spread to peripheral blood with atypical cells and increased proliferative markers, such as LDH. This fact highlights the difficulty in establishing the diagnosis of this entity during the course of prolonged and indolent disease, manifested by splenomegaly oligosymptomatic often neglected by physicians and by the patients themselves. Disclosures: No relevant conflicts of interest to declare.

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

scholarly journals BRAF and MAP2K1 mutations in hairy cell leukemia and splenic marginal zone B-cell lymphoma

2016 ◽  
Vol 11 (1) ◽  
pp. 34-36 ◽  
Author(s):  
I. A. Yakutik ◽  
L. S. Al’-Radi ◽  
H. L. Julhakyan ◽  
B. V. Biderman ◽  
A. B. Sudarikov
Keyword(s):  
B Cell ◽  
Hairy Cell Leukemia ◽  
Marginal Zone ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Hairy Cell ◽  
Cell Leukemia

scholarly journals A Rare Case of a Patient With Hairy Cell Leukemia Developing Blastoid Marginal Zone B-Cell Lymphoma

Cureus ◽  
2021 ◽  
Author(s):  
Yeshanew Teklie ◽  
Stephen Bell ◽  
Precious Idogun ◽  
Madhavi Venigalla
Keyword(s):  
B Cell ◽  
Hairy Cell Leukemia ◽  
Rare Case ◽  
Marginal Zone ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Hairy Cell ◽  
Cell Leukemia

Differential Expression of Enhancer of Zeste Homolog 2 (EZH2) Protein in Low and High Grade B-Cell Non-Hodgkin Lymphomas and Differential Regulation of EZH2 Expression By p-ERK and MYC in High Grade B Cell Lymphomas

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 2660-2660
Author(s):  
Xuejun Tian ◽  
Ali Shahsafaei ◽  
David M. Dorfman
Keyword(s):  
B Cell ◽  
Hairy Cell Leukemia ◽  
Cell Lymphoma ◽  
Low Grade ◽  
High Grade ◽  
Hairy Cell ◽  
Cell Leukemia ◽  
B Cell Lymphomas ◽  
Neoplastic Cells ◽  
Ezh2 Expression

Abstract Background: EZH2, amember of the polycomb protein group, is an important methyltransferase that is over-expressed in various carcinomas, some B and T cell lymphomas, as well as myeloid disorders. We investigated EZH2 expression in the range of low and high grade B cell neoplasms and correlated its expression with that of p-ERK, MYC, and p-STAT3, potential regulators of EZH2 expression, in high grade B cell lymphomas. Methods: Immunohistochemical staining (IHC) for EZH2 was performed on a total of 162 low and high grade B cell non-Hodgkin lymphomas, using formalin fixed, paraffin-embedded tissue [Table 1]. We subsequently performed IHC for p-ERK, p-STAT3, and MYC on high grade B cell lymphomas. Cases were scored for percentage positivity of neoplastic cells using the above antibodies. Table 1. EZH2 expression in low grade and high grade B cell lymphomas Lymphoma type Cases (POS/Total) EZH2 positive % low grade MM 10/12 10 LPL 9/12 5-10 CLL/SLL 15/18 10--15 MCL 8/9 30 MZL 20/21 15-20 FL(1-2) 8/10 20 HCL 13/14 5-10 HCL-V 9/10 30-40 High grade DLBCL 29/33 90 BL 19/19 100 CLL-Richter 5/5 80-90 FL-3 6/6 80 DHL 22/22 90-100 PMLBCL 19/19 60-80 B-ALL 11/11 95-100 Abbreviations: MM - multiple myeloma; LPL - lymphoplasmacytic lymphoma; CLL/SLL - chronic lymphocytic leukemia/small lymphocytic lymphoma; MCL - mantle cell lymphoma; MZL - marginal zone lymphoma; FL(1-2) - follicular lymphoma grade 1-2/3; HCL - hairy cell leukemia; HCL-V - hairy cell leukemia-variant; DLBCL - diffuse large B cell lymphoma; BL - Burkitt lymphoma; CLL-Richter transformation of CLL; FL -3 - follicular lymphoma grade 3/3; DHL - double hit lymphoma; PMLBCL - primary mediastinal large B cell lymphoma; B-ALL - B lymphoblastic leukemia/lymphoma. Results: In low grade lymphomas, 5-40% of neoplastic cells were positive for EZH2, with variable intensity of staining. Of note, there was a significant difference in EZH2 expression in hairy cell leukemia versus hairy cell leukemia-variant (p<0.01). In high grade lymphomas, including those transformed from low grade B cell lymphomas, 70-100% of tumor cells were positive for EZH2 expression, a significant difference in EZH2 expression compared with low grade B cell lymphomas (p<0.01). Among the high grade B cell lymphoma, 40-80% of neoplastic cells in DLBCL (24 cases), were positive for p-ERK expression, but <30% of neoplastic cells were positive for p-ERK expression in BL (15 cases) and DHL (17 cases). In contrast, 80-100% and 50-90% of neoplastic cells were positive for MYC expression in BL (17 cases) and DHL (26 cases), respectively, but only 5-50% of neoplastic cells were positive for MYC expression in DLBCL (30 cases). There were significant differences in both MYC and p-ERK expression in BL and DHL versus DLBCL (p<0.01). None of the high grade B cell lymphomas showed significant p-STAT3 positivity in neoplastic cells. Conclusion: EZH2 expression correlates with tumor grade in B cell neoplasms, and the high level of EZH2 expression in high grade B cell lymphomas suggests that this molecule may function as an oncogenic protein in these neoplasms. Furthermore, our findings show there are different signaling cascades in the regulation of EZH2 expression in different types of high grade B cell lymphomas. The p-ERK signaling cascade, but not MYC expression, plays an important role in high EZH2 expression in DLBCL; while in BL and DHL, high MYC expression, but not p-ERK expression, is associated with increased EZH2 expression, possibly through miRNA regulation. These findings suggested that EZH2 and specific disease-related signaling cascades may serve as therapeutic targets for the treatment of high grade B cell lymphomas. Disclosures No relevant conflicts of interest to declare.

Occurrence of immunoblastic B-cell lymphoma in hairy cell leukemia

Cancer ◽  
1987 ◽  
Vol 59 (6) ◽  
pp. 1161-1164 ◽  
Author(s):  
F. Arnalich ◽  
J. Camacho ◽  
C. Jimenez ◽  
C. Lahoz ◽  
M. Patrón
Keyword(s):  
B Cell ◽  
Hairy Cell Leukemia ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Hairy Cell ◽  
Cell Leukemia
Sign in / Sign up

Export Citation Format

Share Document