Blastic transformation of splenic lymphoma with villous lymphocytes after a well-controlled chronic phase of more than 10 years.
A 30-year-old Japanese man with splenomegaly and lymphocytosis was examined in 1985. Blood analysis revealed that some of the lymphocytes had short-surface villi with polar distribution. The cells showed Ig lambda+, CD5+, CD11c+, CD19+, CD22+, CD23+, CD24+, FMC7+ phenotype. A small M peak was detected in the serum. Splenic lymphoma with villous lymphocytes (SLVL) was diagnosed on the basis of these findings. Remission was induced and was maintained with low-dose chlorambucil for more than 10 years. In 1996, the patient developed splenomegaly and lymphadenopathy with "B" symptoms and a high serum lactase dehydrogenase (LDH) level. Large blastoid cells with prominent nucleoli were observed in the bone marrow; later, a small number appeared in the peripheral blood. The bone marrow cells showed a complex chromosomal abnormality involving del(7)(q32). Southern blot analysis of immunoglobulin gene rearrangements in SLVL cells that had been cryopreserved in 1986 and of bone marrow cells in 1996 showed 2 rearranged bands in each cell sample; 1 band showed identical sizes in the 2 samples, and the other showed different sizes. These findings suggest that the blastoid cells were derived from SLVL cells through transformation. After this transformation, the disease followed a highly aggressive course. Various chemotherapeutic agents had little effect, and the patient died 3 months later.
Kuwayama
M
M
Department of Hematology and Oncology, Osaka University Medical School, Japan.
Machii
T
T
Yamaguchi
M
M
Yamaguti
K
K
Kitani
T
T
Kanakura
Y
Y
D000328:Adult; D015703:Antigens, CD / Q000097:blood; D000970:Antineoplastic Agents / Q000627:therapeutic use; D001854:Bone Marrow Cells / Q000276:immunology / Q000473:pathology; D019070:Cell Lineage; D002869:Chromosome Aberrations; D025063:Chromosome Disorders; D002880:Chromosomes, Human, Pair 11; D002883:Chromosomes, Human, Pair 14; D017809:Fatal Outcome; D006801:Humans; D007564:Japan; D008213:Lymphocyte Activation; D008214:Lymphocytes / Q000276:immunology / Q000473:pathology; D008223:Lymphoma / Q000188:drug therapy / Q000235:genetics / Q000473:pathology*; D008297:Male; D013160:Splenic Neoplasms / Q000188:drug therapy / Q000235:genetics / Q000473:pathology*; D014178:Translocation, Genetic
D015703:Antigens, CD; D000970:Antineoplastic Agents