Blood Res 2018; 53(4): 267  https://doi.org/10.5045/br.2018.53.4.267
Clonal villous lymphocytes and plasma cells in splenic marginal zone lymphoma with plasmacytic differentiation
João Tadeu Damian Souto Filho1,2*, Rodrigo Aires de Morais2, Ana Laura Oliveira Silveira1, Arthur Pires Lacerda1, Isabela Peçanha Bogado Fassbender1, João Marcos Marcelino Chaves Ribeiro1, and Luiza Reis de Sales1

1Faculdade de Medicina de Campos, Hospital Escola Álvaro Alvim, Campos dos Goytacazes, Rio de Janeiro, Brazil.

2Instituto Federal de Educação, Ciência e Tecnologia Fluminense, Campos dos Goytacazes, Rio de Janeiro, Brazil.

Correspondence to: Correspondence to João Tadeu Damian Souto Filho, M.D., Faculdade de Medicina de Campos, Hospital Escola Álvaro Alvim, Rua Barão da Lagoa Dourada, 320/1202, Campos dos Goytacazes, Rio de Janeiro 28035-212, Brazil, drjoaotadeu@yahoo.com.br
Received: March 14, 2018; Revised: April 15, 2018; Accepted: May 10, 2018; Published online: December 17, 2018.
© The Korean Journal of Hematology. All rights reserved.

cc This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0)which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
 

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A 42-year-old man presented with low fever, excessive sweating, weight loss, and splenomegaly. His total leukocyte count, hemoglobin level, and platelet count were 2.1×103/L, 10.8 g/dL, and 92×109/L, respectively. Serum protein electrophoresis demonstrated a monoclonal spike and immunofixation confirmed an IgG-κ monoclonal protein. Peripheral blood smear revealed 11% small to medium-sized lymphocytes with oval nuclei, mature chromatin, inconspicuous nucleoli, and circumferential cytoplasmatic short villous projections (A, B; Wright-Giemsa stain, ×1,000). Peripheral blood flow cytometric immunophenotyping revealed a clonal B-cell population expressing cluster of differentiation (CD) 19+++, CD20+++, CD22+++, CD79b++, CD38+, FMC7++, and CD11c++ with κ light chain. A bone marrow (BM) aspirate smear showed increased plasma cell count (16%) with focal clustering, moderately large and ovoid shape, round and eccentrically placed nuclei, and strong basophilic cytoplasm with a broad perinuclear clear zone without any villous projections (C; Wright-Giemsa stain, ×1,000). No villous lymphocytes were detected. BM flow cytometric immunophenotyping revealed two clonal populations with restricted κ light chain expression: a clonal B-cell population phenotypically similar to peripheral blood, and a clonal plasma cell population that expressed CD19+++, CD38+++, CD138weak, CD45weak, CD56, and CD117. BM biopsy showed nodular interstitial lymphoid involvement and scattered plasma cells. Splenic marginal zone lymphoma with plasmacytic differentiation and villous lymphocytes was diagnosed. Plasmacytic differentiation may occur in almost all low-grade B-cell lymphoproliferative disorders, varying from being rare (mantle cell lymphomas) to being uniformly present (lymphoplasmacytic lymphoma). Distinguishing between myeloma and lymphomas with marked plasmacytic differentiation may be challenging.



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