Rare Inherited Defects of the Complement System in Purpura Fulminans

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[Purpura fulminans].

Purpura Fulminans is an unusual condition, characterized by rapid progression, high systemic toxicity and great mortality. The clinical history of a girl aged 3 years and 6 months is presented. She was hospitalized in an Infectious Diseases Unit, from February 6 to March 3, 1981. Her illness began two days prior to admission, with fever, cpistaxis, moderate postration, violet -coloured lesions ...

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Neonatal Purpura Fulminans

Neonatal purpura fulminans is a rare and life threatening disease that can be inherited or acquired in etiology. It manifests as DIC and extensive subcutaneous thrombosis. The condition is often fatal unless there is prompt diagnosis, and judicious therapy. The most important causes of this condition are infections and congenital deficiency of anticoagulant proteins C and S.In the case of PC (p...

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Purpura Fulminans following Thermal Injury

Purpura fulminans is a rare syndrome of intravascular thrombosis and hemorrhagic infarction of the skin, which is an unusual cutaneous manifestation of disseminated intravascular coagulation. It often occurs in small children and babies due to infection and/or sepsis, rarely in adults in clinic. We report the first case of deadly purpura fulminans following thermal injury in a 64-year-old Chine...

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Purpura fulminans with haemolytic anaemia.

A 10-month-old female infant with purpura fulminans is reported. The patient had clotting abnormality compatible with consumption coagulopathy. Histopathological examination revealed the presence of microthrombi. Heparin was effective in correcting the clotting defects. The infant also suffered from microangiopathic haemolytic anaemia. She died 17 days after the onset of the illness from infect...

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ژورنال

عنوان ژورنال: Blood

سال: 2020

ISSN: 0006-4971,1528-0020

DOI: 10.1182/blood-2020-138729