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Indian Journal of Cancer, Vol. 48, No. 3, July-September, 2011, pp. 370-371 Letter to Editor Mantle cell lymphoma, blastoid variant presenting as acute leukemia - A rare case report S Shukla, S Singh, V Puri, G Buxi Department of Pathology, Lady Hardinge Medical College, New Delhi, India Code Number: cn11097 PMID: 21921343 DOI: 10.4103/0019-509X.84917 Sir, Blastoid mantle cell lymphoma (B-MCL) is a rare and clinically more aggressive variant than typical mantle cell lymphoma (MCL). The chromosomal translocation t(11:14)(q13;q32), which results in an overexpression of PRAD1/cyclin D1 in mRNA transcript, is highly characteristic of MCL and B-MCL. [1] A 40-year-old male presented with a four-month history of abdominal fullness and generalized lymphadenopathy. Abdominal examination showed hepatosplenomegaly (liver 2 cm and spleen 3 cm below the costal margin). Blood count showed leucocytosis with WBC count 51×10 9 /μL (53% blasts), hemoglobin 8.9 g/dL with normal red cell indices, platelet count 4.1×10 9 /μL. These blasts were 2-3 times the size of small mature lymphocytes with scant agranular cytoplasm. Nucleus was large with irregular nuclear contour, fine chromatin and 1-2 prominent nucleoli [Figure - 1]. Bone marrow aspiration showed 70% blasts having similar morphology as above. Bone marrow biopsy showed diffuse effacement of architecture by these blasts. On immunohistochemistry, cyclin D1 showed uniform positivity in these blasts [Figure - 1], [inset]. Flow cytometry studies of bone marrow shows presence of large population of cells with cell surface immunoglobulin (sIg), kappa light chain restriction. This cell population showed expression of pan leukocyte marker CD45, CD19, CD22, CD20, CD79B, FMC7 and CD5 [Figure - 2]. It was negative for CD23, CD10 and terminal deoxy nucleotidyl transferase (TdT). Fine needle aspiration (FNA) cytology from cervical lymph nodes showed cellular smears with presence of monomorphic blastoid lymphoid cells. Based on cytomorphology and flow cytometric analysis and immunohistochemistry, a final diagnosis. Leukemic presentation of B-MCL is an exceedingly uncommon event. We have not come across any such case from Indian literature. In case of leukemic B-MCL, the differentiation from acute lymphoblastic leukemia, B cell prolymphocytic leukemia (B-PLL) and leukemic phase of large cell lymphoma is required. Cells of acute lymphoblastic leukemia lack sIg and cyclin D1 expression and express TdT, while those of B-MCL express sIg but not TdT. Large cell lymphoma cells can rarely express CD5 in 10% of cases. [2] These can be distinguished from B-MCL by the absence of Cyclin D1 expression. B-PLL may be very difficult to differentiate from B-MCL on the morphological grounds and immunophenotyping, however t(11;14) translocation and cyclin D1 expression is not seen in B-PLL. [3] Hence it is difficult to diagnose patients with B-MCL correctly without immunophenotyping. Immunohistochemistry and cytogenetic analysis can substantiate the diagnosis. MCL and B-MCL is incurable with standard chemotherapy. Response rates to single-agent and combination chemotherapy range from 40% to 70% but generally are short-lived, with progression in 6 to 18 months. [4] Rituximab plus thalidomide are found to be beneficial in few patients. [5] References
Copyright 2011 - Indian Journal of Cancer The following images related to this document are available:Photo images[cn11097f2.jpg] [cn11097f1.jpg] |
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