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العنوان
MICROANGIOPATHIC HAEMOLYTIC ANEMIA UPDATE.
الناشر
Ain Shams University. Faculty of Medicine. Clinical and Chemical Pathology Department ,
المؤلف
Fahmy, Marwa Mohamed Farid.
هيئة الاعداد
مشرف / Mervat Abd El-Hameed El-Feky
مشرف / Basima Mahmoud Mohamed Ahmed
مشرف / Fahmy, Marwa Mohamed Farid.
مشرف / Mervat Abd El-Hameed
تاريخ النشر
2006
عدد الصفحات
88p.
اللغة
الإنجليزية
الدرجة
ماجستير
التخصص
الطب (متفرقات)
تاريخ الإجازة
1/1/2006
مكان الإجازة
جامعة عين شمس - كلية الطب - Clinical and Chemical Pathology
الفهرس
Only 14 pages are availabe for public view

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from 156

Abstract

Microangiopathic hemolytic anemia (MAHA) refers to group of clinical disorders characterized by fragmentation of red blood cells within the circulatory system as they pass through the platelet fibrin mesh present in microthrombi which are deposited in capillaries and arterioles leading to intravascular haemolysis.

MAHA can be classified into primary type as thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) or secondary type as disseminated intravascular coagulopathy (DIC), preeclampsia, HELLP syndrome, malignant tumor, chemotherapy, radiotherapy, renal abnormalities, bone marrow transplantation, vasculities, prosthetic heart valves and congenital vascular malformations.

TTP is a rare multi-system disease characterised by the pentad of MAHA, thrombocytopenia, renal dysfunction, fever and neurologic changes. Two main categories of TTP exist; congenital and acquired. Congenital TTP is mainly due to mutations in the gene ADAMTS-13, which encodes for a metalloprotease of the ADAMTS family, which specifically degrades von Willebrand factor (vWF). There are two forms of acquired TTP: idiopathic and secondary TTP. The idiopathic form, is associated with low levels of ADAMTS-13, is the result of the formation of autoantibodies to the metalloprotease. Secondary form is activated by triggering agents such as infection, medications (cyclosporine), malignancy, connective tissue disorder, pregnancy and post bone marrow transplant.