TAFRO syndrome

Orpha code: 457077OMIM code:

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
-

No additional description.

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