Document 1174 DOCN M94A1174 TI Idiopathic CD4+ T-lymphocytopenia accompanying hypogammaglobulinemia with increased CD5+ B lymphocytes. DT 9412 AU Fujiyama Y; Hodohara K; Yoshida T; Matsubara H; Bamba T; Dept. Int. Med., Shiga Univ. Med., Otsu, Japan. SO Int Conf AIDS. 1994 Aug 7-12;10(2):192 (abstract no. PB0780). Unique Identifier : AIDSLINE ICA10/94371401 AB Idiopathic CD4+ T-lymphocytopenia (ICTL) is an acquired immunodeficiency in which no evidence for HIV type 1 and type 2 infection was found. Here we report a case of ICTL accompanying profound hypogammaglobulinemia with increased CD5+ B lymphocytes. A 36 year old japanese man was refered to our university hospital on June 1993 for hypogammaglobulinemia developed after an episode of acute hepatitis. He had no past history suggesting preceded immunodeficiency nor any risk factor of HIV infection. The serum immunoglobulins levels were decreased from 733 to 467 for IgG, from 108 to 10 for IgA and from 50 to 22 mg/dl for IgM in about one month from the clinical onset of acute hepatitis. Absolute peripheral blood lymphocyte counts was 1632/mm3 and the immunophenotyping studies of peripheral lymphocytes revealed; CD3+ 43%, CD19+ 33%, CD4+ 5%, CD8+ 35%, CD8+CD57+ 30%, CD5+CD20+ 30%, Sm-kappa+ 17%, Sm-lambda+ 2%, CD16+ 26%. He had no evidence of HIV-1, 2 infection, as judged by multiple serologies, syncytium formation assay using MT4 cells, and HIV-1 proviral DNA amplification by PCR. Serologies of HAV, HBV, HCV, CMV, EBV and HTLV-1 were negative, and PCR failed to demonstrate HBV-DNA, HCV-RNA, EBV-DNA, HSV-DNA, HHV6-DNA and CMV-DNA. In vitro proliferative responses of PBMC to PHA and Con A were both remarkably suppressed (1,768 cpm for PHA and 2,055 cpm for Con A, respectively). Bone marrow aspirate was morphologically normal, and rearrangement band of TCRs, Ig heavy and light chains of PBMC was not detected by PCR. Nevertheless, the absolute CD4+ T-cell counts had been remained below 100/mm3 over 8 months of observation, he had been uneventful without any opportunistic infection until February 1994, when Herpes zostor infection developed. Although, any viral activity has not been, so far, demonstrated, the increased CD5+ B lymphocyte might be indicative of autoimmune mechanism induced by some infectious agent causing acute hepatitis. DE Acute Disease Adult Agammaglobulinemia/BLOOD/COMPLICATIONS/*IMMUNOLOGY Antigens, CD/*BLOOD B-Lymphocyte Subsets/*IMMUNOLOGY Bone Marrow/PATHOLOGY Case Report Cells, Cultured Concanavalin A Gene Rearrangement, T-Lymphocyte Genes, Immunoglobulin Hepatitis/BLOOD/COMPLICATIONS/IMMUNOLOGY Human IgA/BLOOD IgM/BLOOD Immunoglobulins, Heavy-Chain/GENETICS Immunoglobulins, Light-Chain/GENETICS Lymphocyte Transformation Male Phytohemagglutinins Polymerase Chain Reaction T-Lymphocytopenia, Idiopathic CD4-Positive/BLOOD/COMPLICATIONS/ *IMMUNOLOGY MEETING ABSTRACT SOURCE: National Library of Medicine. NOTICE: This material may be protected by Copyright Law (Title 17, U.S.Code).