Rinsho Ketsueki
Online ISSN : 1882-0824
Print ISSN : 0485-1439
ISSN-L : 0485-1439
AIDS with Fever, Giant Splenomegaly and Severe Pancytopenia
Mitsunobu MATSUBARAGohta MASUDAHideo TERADAHaruo NIHKURA
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1988 Volume 29 Issue 5 Pages 748-755

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Abstract

A case of AIDS which presented unusual abnormalities in both clinical manifestations and laboratory examinations is reported.
The patient is a 30-year-old bisexual American male born in New York City. After three years of persistent lymphadenopathy, he developed gouty attacks, fever, and giant splenomegaly, and was admitted to St. Luke's International Hospital in Tokyo on August 23, 1985. White cell count was 2,300/μl with lymphocyte 722/μl, hemoglobin 7.9g/dl, and platelet 36,000/μl. Uric acid was 10.4mg/dl. M-protein of IgG-K type was found in immunoelectrophoresis. Hepatic and renal function tests were within normal limits.
Bone marrow examination revealed total cell count 112,000/μl, with atypical plasma cells, striking hemophagocytosis of histiocytes, an increase of platelet non-producing megakaryocytes, and slight granulocytosis containing toxic granules.
CMV titer rose slightly to x200 (normal range<x100), and VCA-IgG and EBNA showed high titer. Candida albicans was cultured from throat culture and oral cavity.
OKT4/OKT8 ratio decreased to 0.23 and HTLV III-antigen was positive.
During the patient's hospitalization, cervical lymphonodes rapidly enlarged with marked pancytopenia, and hepato-renal disorder appeared. He was transferred to Tokyo Metropolitan Komagame Hospital on September 13th, and travelled back to America on September 24th. Three days later, he died probably due to septic shock during hemodialysis.
Splenomegaly is often reported in cases of AIDS associated with CMV and EBV infections, but giant splenomegaly is not common. However, in this case, neither CMV infection nor EBV infection were definite. Giant splenomegaly and increased histiocytes with marked phagocytosis in bone marrow coupled with severe pancytopenia and hyperuricemia, were thought to be caused by strong reaction to viral infections.
Besides T-cell abnormalities, various dysfunctions of B-cell system are reported. But the monoclonal gammopathy with increased atypical plasma cells in this case was though to be new types of B-cell abnormalities in AIDS.

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© 1988 The Japanese Society of Clinical Hematology
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