Description of the disease * EnglishPolish Pobierz sekcję do PDF Definition A rare systemic disease characterized by acute or subacute onset of thrombocytopenia, anasarca (edema, pleural effusion, ascites), and systemic inflammation (fever and/or elevated C-reactive protein). Minor diagnostic categories are Castleman's disease-like features on lymph node biopsy, reticulin myelofibrosis and/or increased number of megakaryocytes in bone marrow, progressive renal insufficiency, and mild organomegaly including hepatosplenomegaly and lymphadenopathy. Most patients show elevated levels of serum alkaline phosphatase, while marked polyclonal hypergammopathy is rare. Disease data Classification Disease Synonyms Thrombocytopenia-anasarca-fever-renal insufficiency-organomegaly syndrome Zespół małopłytkowości, uogólnionego obrzęku skóry i tkanki podskórnej, gorączki, niewydolności nerek i organomegalii ORPHA code 457077 OMIM code - ICD10 code M35.8 ICD11 code - *Soruce Extended description of the disease Pobierz sekcję do PDF No additional description. Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl