TY - JOUR
T1 - Relationship between hairy cell leukemia variant and splenic lymphoma with villous lymphocytes
T2 - Presentation of a new concept
AU - Sun, Tsieh
AU - Dittmar, Klaus
AU - Koduru, Prasad
AU - Susin, Myron
AU - Teichberg, Saul
AU - Brody, Judith
N1 - Copyright:
Copyright 2007 Elsevier B.V., All rights reserved.
PY - 1996/4
Y1 - 1996/4
N2 - 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.
AB - 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.
KW - Hairy cell leukemia
KW - Immunophenotyping
KW - Molecular biology
KW - Splenic lymphoma with villous lymphocytes
KW - Subclassification
UR - http://www.scopus.com/inward/record.url?scp=0029993370&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0029993370&partnerID=8YFLogxK
U2 - 10.1002/(SICI)1096-8652(199604)51:4<282::AID-AJH6>3.0.CO;2-S
DO - 10.1002/(SICI)1096-8652(199604)51:4<282::AID-AJH6>3.0.CO;2-S
M3 - Article
C2 - 8602628
AN - SCOPUS:0029993370
SN - 0361-8609
VL - 51
SP - 282
EP - 288
JO - American Journal of Hematology
JF - American Journal of Hematology
IS - 4
ER -