Congenital thrombotic thrombocytopenic purpura

Orpha code: 93583OMIM code: 274150

Definition

A hereditary form of thrombotic thrombocytopenic purpura (TTP) characterized by profound peripheral thrombocytopenia, microangiopathic hemolytic anemia (MAHA) and single or multiple organ failure of variable severity.

Disease data
Classification

Clinical subtype

Synonyms
Congenital ADAMTS-13 deficiency
Rodzinna TTP
Wrodzona TTP
Wrodzony Niedobór ADAMTS-13
Zespół Upshawa i Schulmana
Congenital TTP
Familial TTP
Upshaw-Schulman syndrome
ORPHA code
93583
OMIM code
274150
ICD10 code
M31.1
ICD11 code
-

No additional description.

Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl