Biomaterial Database

Thrombotic thrombocytopenic purpura unresponsive to plasma infusion and plasma exchange, but responsive to splenectomy. 1986

A D Sturgess, and B H Chong

A 60-yr-old female presented with typical thrombotic thrombocytopenic purpura (TTP). She remained in coma with frequent seizures for 1 wk, with persisting severe thrombocytopenia and microangiopathic haemolytic anaemia, despite treatment with prednisolone, plasma exchange, fresh frozen plasma, sulphinpyrazone and dipyridamole. Splenectomy induced haematological improvement within 1 d, there was cessation of fitting after 2 d, and full neurological recovery ensued over 3 wk. Laboratory studies did not reveal the presence of a platelet-aggregating factor (PAF), stated to be present in some two-thirds of cases. While plasma exchange and plasma infusion are beneficial in many cases, splenectomy appears still to be of value in unresponsive disease.

UI	MeSH Term	Description	Entries
D008875	Middle Aged	An adult aged 45 - 64 years.	Middle Age
D010949	Plasma	The residual portion of BLOOD that is left after removal of BLOOD CELLS by CENTRIFUGATION without prior BLOOD COAGULATION.	Blood Plasma,Fresh Frozen Plasma,Blood Plasmas,Fresh Frozen Plasmas,Frozen Plasma, Fresh,Frozen Plasmas, Fresh,Plasma, Blood,Plasma, Fresh Frozen,Plasmas,Plasmas, Blood,Plasmas, Fresh Frozen
D010951	Plasma Exchange	Removal of plasma and replacement with various fluids, e.g., fresh frozen plasma, plasma protein fractions (PPF), albumin preparations, dextran solutions, saline. Used in treatment of autoimmune diseases, immune complex diseases, diseases of excess plasma factors, and other conditions.	Exchange, Plasma,Exchanges, Plasma,Plasma Exchanges
D011697	Purpura, Thrombotic Thrombocytopenic	An acquired, congenital, or familial disorder caused by PLATELET AGGREGATION with THROMBOSIS in terminal arterioles and capillaries. Clinical features include THROMBOCYTOPENIA; HEMOLYTIC ANEMIA; AZOTEMIA; FEVER; and thrombotic microangiopathy. The classical form also includes neurological symptoms and end-organ damage, such as RENAL FAILURE. Mutations in the ADAMTS13 PROTEIN gene have been identified in familial cases.	Moschkowitz Disease,Purpura, Thrombotic Thrombopenic,Thrombotic Thrombocytopenic Purpura, Congenital,Thrombotic Thrombocytopenic Purpura, Familial,Congenital Thrombotic Thrombocytopenic Purpura,Familial Thrombotic Thrombocytopenia Purpura,Familial Thrombotic Thrombocytopenic Purpura,Microangiopathic Hemolytic Anemia, Congenital,Moschcowitz Disease,Schulman-Upshaw Syndrome,Thrombotic Microangiopathy, Familial,Thrombotic Thrombocytopenic Purpura,Upshaw Factor, Deficiency of,Upshaw-Schulman Syndrome,Familial Thrombotic Microangiopathy,Microangiopathy, Familial Thrombotic,Schulman Upshaw Syndrome,Thrombocytopenic Purpura, Thrombotic,Thrombopenic Purpura, Thrombotic,Thrombotic Thrombopenic Purpura,Upshaw Schulman Syndrome
D001803	Blood Transfusion	The introduction of whole blood or blood component directly into the blood stream. (Dorland, 27th ed)	Blood Transfusions,Transfusion, Blood,Transfusions, Blood
D003131	Combined Modality Therapy	The treatment of a disease or condition by several different means simultaneously or sequentially. Chemoimmunotherapy, RADIOIMMUNOTHERAPY, chemoradiotherapy, cryochemotherapy, and SALVAGE THERAPY are seen most frequently, but their combinations with each other and surgery are also used.	Multimodal Treatment,Therapy, Combined Modality,Combined Modality Therapies,Modality Therapies, Combined,Modality Therapy, Combined,Multimodal Treatments,Therapies, Combined Modality,Treatment, Multimodal,Treatments, Multimodal
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D013156	Splenectomy	Surgical procedure involving either partial or entire removal of the spleen.	Splenectomies
D013997	Time Factors	Elements of limited time intervals, contributing to particular results or situations.	Time Series,Factor, Time,Time Factor