Relationship between hairy cell leukemia variant and splenic lymphoma with villous lymphocytes: Presentation of a new concept

Tsieh Sun, Klaus Dittmar, Prasad Koduru, Myron Susin, Saul Teichberg, Judith Brody

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14 Scopus citations

Abstract

An unusual case of low-grade B-cell lymphoproliferative disorder with peripheral lymphocytosis and splenomegaly followed for 4 1/2 years is reported. During this period, the phenotype of the tumor cells in the blood changed from that of hairy cell leukemia (HCL)/chronic lymphocytic leukemia (CLL) to HCL/prolymphocytic leukemia (PLL), to PLL. The lymphoid population in the blood showed a mixture of hairy cells, villous lymphocytes, small lymphocytes, and prolymphocytes, corresponding to the phenotypes at various stages. Although relatively specific markers for CLL, HCL, and PLL, such as CD5, CD11c, CD22, CD25, and FMC-7, were positive at various stages, all these markers have also been demonstrated in a large study series of splenic lymphoma with villous lymphocytes (SLVL). In addition, the histologic pattern of the bone marrow biopsy and splenectomy - specimen were not typical for HCL. This case can therefore be classified either as HCL variant or as SLVL. As SLVL assumes various cytologic and histologic patterns, which overlap with different lymphoproliferative disorders, especially HCL variants, this entity appears to represent a heterogeneous group of lymphomas/leukemias that may evolve into each other. The absence of activation of c-myc and bcl-2 oncogenes as well as mutation of p53 tumor suppressor gene, together with the presence of only one single rearranged band for both heavy chain and K light chain genes in our case suggest that these morphologically different lymphoid tumors may belong to the same family.

Original languageEnglish (US)
Pages (from-to)282-288
Number of pages7
JournalAmerican Journal of Hematology
Volume51
Issue number4
DOIs
Publication statusPublished - Apr 1996

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Keywords

  • Hairy cell leukemia
  • Immunophenotyping
  • Molecular biology
  • Splenic lymphoma with villous lymphocytes
  • Subclassification

ASJC Scopus subject areas

  • Hematology

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