Composite Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma and Mantle Cell Lymphoma; Small Cell Variant: A Real Diagnostic Challenge. Case Presentation and Review of Literature

Abstract

Rare co-existance of disease or pathology Background: Chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) and mantle cell lymphoma (MCL) both have a common origin arising from mature CD5+ B-lymphocytes. Their distinction is crucial since MCL is a considerably more aggressive disease. Composite lymphoma consisting of CLL/SLL and MCL has been rarely reported. This type of composite lymphoma may be under-diagnosed as the 2 neoplasms have many features in common, both morphologically and immunophenotypically. Case Report: We report the case of a 57-year-old male patient who presented with a 4-month history of recurrent abdominal pain and distention with hepatosplenomegaly. Peripheral blood showed a high leukocytes count (46.7×10 3 /uL) with marked lymphocytosis of 35.0×10 3 /uL, mostly small mature-looking, with some showing nuclear irregularities, with approximately 3% prolymphocytes. Immunophenotyping by flow cytometry and immunohistochemistry revealed 2 immunophenotypically distinct abnormal CD5+monotypic B-cell populations. Fluorescence in situ hybridization (FISH) on peripheral blood demonstrated IGH/CCND1 rearrangement consistent with t(11;14) in 65% of cells analyzed. Accordingly, based on compilation of findings from morphology, flow cytometry, immunohistochemistry, and FISH, A diagnosis of composite lymphoma consisting of MCL; small cell variant and CLL/SLL was concluded. Conclusions: We describe a case of composite lymphoma of MCL (small cell variant) and CLL/SLL that emphasizes the crucial role of the multiparametric approach, including vigilant cyto-histopathologic examination, immunophenotyping by flow cytometry and immunohistochemistry, as well as genetic testing, to achieve the correct diagnosis.

Downregulation of Lymphoid enhancer‐binding factor 1 (LEF‐1) expression (by immunohistochemistry and/ flow cytometry) in chronic Lymphocytic Leukemia with atypical immunophenotypic and cytologic features

“…We found out that LEF‐1 is a useful marker in cases of composite lymphomas (CL). In out cohort, we had a case of CL (CLL and Mantle cell lymphoma), 31 LEF‐1 expression by FCM had nicely separated the two different clones and served as an additional interesting tool to differentiate both types of lymphoma (Figure 2D); MCL clone (lambda restricted) and CLL clone (kappa restricted). The LEF‐1 MFI ratio for lambda restricted (MCL) clone was 1.4 which is (< cutoff value) while the LEF‐1 MFI ratio for kappa restricted (CLL) clone is 2.5.…”

Classical and molecular methods in differentiation of mantle cell lymphoma and small lymphocytic lymphoma in composite lymphoma: a case report

“…For V3J4 (R 2 < 0, 05; p > 0, 05)-changes in SOX11 expression are not associated with changes in V3J4 rearrangement marker highly specific for MCL. SOX11 expression is found in over 90% of MCL cases [3,20] and can be used as an MRD monitoring marker, as we previously reported [16].…”

Mature B-Cell Neoplasms with Immunophenotypically Distinct Dual B-Cell Clones: A Case Series

“…A diagnosis of composite lymphoma of CLL and MCL (small cell variant), stage IV, MIPI score 4 was concluded, published case. [10]. Molecular analysis (conducted after publication) supports the presence of more than one B cell clone (Figure 3a,3b& 3c).…”