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Immunological Dysfunction and Persistent Lymphadenopathy in Patients with Classic Hemophilia
Author(s) -
Tsoukas C,
Gervais F,
Fuks A,
Guttmann R D,
Strawczynski H,
Shuster J,
Gold P
Publication year - 1984
Publication title -
scandinavian journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0036-553X
DOI - 10.1111/j.1600-0609.1984.tb02591.x
Subject(s) - medicine , cryoprecipitate , asymptomatic , lymph node , follicular hyperplasia , immunology , gastroenterology , immune system , platelet
Thirty‐four asymptomatic heterosexual adults with severe classic hemophilia without inhibitors receiving more than 40,000 units of factor VIII concentrate per year were studied and compared to 10 heterosexuals receiving cryoprecipitate and to 22 age matched controls. Anergy was noted in 68 percent of the patients treated with factor VIII concentrates, 57 percent of those treated with cryoprecipitate and 5 percent of the controls. The lyophilized factor VIII treated group had a significant elevation of serum IgG, decreased mitogen responses, high nonstimulated background activity, and altered T cell subsets with an increase in total T lymphocytes, T suppressor cells and decreased Th/Ts ratios. Four of the patients with cellular immune dysfunction developed fatigue, night seats, dyspnea, weight loss, neuropathic pain and rashes. They had diffuse, peripheral, non‐tender lymphadenopathy. Two had splenomegaly. All four were anergic, had inverted Th/Ts ratios, with an increase in suppressor cells. Three had lymph node biopsies which demonstrated reactive follicular hyperplasia. All had elevated serial titers to EBV. The systemic symptoms resolved as these titers returned towards normal. The lymphadenopathy is persistent although all four patients have gained weight and feel well. The persistent lymphadenopathy syndrome in hemophilia is a distinct entity which occurs in a subset of hemophiliacs with severe cellular immune dysfunction.