Rinsho Ketsueki
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
Aggressive Natural Killer Cell Lymphoproliferative Disease of Large Granular Lymphocytes with Leukemia-like Clinical Course in the Teminal Stage
Tetsuya TSUKADAToshiyuki OHNOKouichi MORITAHiroshi OTSUJITakao SEKINEMasakatsu NISHIKAWATohru KOBAYASHIKenkichi KITAShigeru SHIRAKAWAKohji NAMBA
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1991 Volume 32 Issue 2 Pages 162-166

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Abstract
A 20-year-old man was admitted to our clinic with fever elevation up to 39°C for two months, generalized lymphadenopathy and hepatosplenomegaly. Histological examination of right scalene lymph node with HE staining showed T cell lymphoma-like finding. The patient was given vindesine and prednisolone, and there was almost no clinical improvement. Abnormal large granular lymphocyte appeared in peripheral blood and increased up to 17,000/μl in the terminal stage of clinical course. These lymphocytes had abundant pale cytoplasm with rich large azurophilic granules and a large nucleus with a few nucleoli. The phenotype of these cells were as follows: FcγR+, CD2+, CD5-, CD7-, CD3-, CD4-, CD1-, CD8-, sIg-, CD20-, CD11-, CD13-, OKIa+, CD25-, CD16+, Leu7-. These cells did not have the activity of antibody dependent cellular cytotoxicity but had natural killer activity. The gene of T cell receptor (β and γ chain) did not rearranged in these cells. We concluded that the abnormal cells were derived from natural killer cells, which caused aggressive clinical course.
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© 1991 The Japanese Society of Hematology
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