Biomaterial Database

Coagulopathy associated with hematin treatment for acute intermittent porphyria. 1981

D L Morris, and M D Dudley, and R D Pearson

Hematin has been shown to be effective therapy for acute intermittent porphyria. Few complications have been found. We cared for a patient who developed a markedly prolonged prothrombin time, partial thromboplastin time, thrombocytopenia, mild hypofibrinogenemia, mild elevation of fibrin split products, and a 10% fall in hematocrit while receiving hematin. No other cause for the coagulopathy could be found. The abnormal coagulation variables returned to normal when hematin was discontinued. Patients receiving hematin for acute intermittent porphyria should be closely observed for signs of coagulopathy.

UI	MeSH Term	Description	Entries
D011164	Porphyrias	A diverse group of metabolic diseases characterized by errors in the biosynthetic pathway of HEME in the LIVER, the BONE MARROW, or both. They are classified by the deficiency of specific enzymes, the tissue site of enzyme defect, or the clinical features that include neurological (acute) or cutaneous (skin lesions). Porphyrias can be hereditary or acquired as a result of toxicity to the hepatic or erythropoietic marrow tissues.	Porphyria,Porphyrin Disorder,Disorder, Porphyrin,Disorders, Porphyrin,Porphyrin Disorders
D012008	Recurrence	The return of a sign, symptom, or disease after a remission.	Recrudescence,Relapse,Recrudescences,Recurrences,Relapses
D001778	Blood Coagulation Disorders	Hemorrhagic and thrombotic disorders that occur as a consequence of abnormalities in blood coagulation due to a variety of factors such as COAGULATION PROTEIN DISORDERS; BLOOD PLATELET DISORDERS; BLOOD PROTEIN DISORDERS or nutritional conditions.	Coagulation Disorders, Blood,Disorders, Blood Coagulation,Blood Coagulation Disorder,Coagulation Disorder, Blood,Disorder, Blood Coagulation
D005260	Female		Females
D006418	Heme	The color-furnishing portion of hemoglobin. It is found free in tissues and as the prosthetic group in many hemeproteins.	Ferroprotoporphyrin,Protoheme,Haem,Heme b,Protoheme IX
D006427	Hemin	Chloro(7,12-diethenyl-3,8,13,17-tetramethyl-21H,23H-porphine-2,18-dipropanoato(4-)-N(21),N(22),N(23),N(24)) ferrate(2-) dihydrogen.	Ferriprotoporphyrin,Hematin,Alkaline Hematin D-575,Chlorohemin,Ferrihaem,Ferriheme Chloride,Ferriprotoporphyrin IX,Ferriprotoporphyrin IX Chloride,Panhematin,Protohemin,Protohemin IX,Alkaline Hematin D 575,Chloride, Ferriheme,Chloride, Ferriprotoporphyrin IX,Hematin D-575, Alkaline
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000208	Acute Disease	Disease having a short and relatively severe course.	Acute Diseases,Disease, Acute,Diseases, Acute
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults