Kasabach–Merritt syndrome

Under the Kasabach -Merritt syndrome ( Kasabach -Merritt phenomenon ) is a rare but a special form of haemangiomas ( Riesenhämangiomen ) associated with a consumptive coagulopathy (disseminated intravascular coagulation ). When coagulation is a clotting disorder in which there is a consumption of coagulation factors and platelets (thrombocytes). The latter leads to a lack of blood platelets, a so-called thrombocytopenia.

Appearance

It offer large hemangiomas that extend, for example, over a whole limb. It takes place thrombosis lead to thrombocytopenia and consumptive coagulopathy with bleeding tendency. The pathogenesis of hemangiomas is unknown. Form back the hemangiomas, so also decreases the tendency to bleed. Therapeutically comes the treatment by laser surgery, interventional radiology for the purposes of embolization or medical therapy with interferon or steroids into consideration. The thrombocytopenia may transfusions, the concurrent fibrinolysis are treated by Fibrinolysehemmer.

History

The Kasabach -Merritt syndrome was first described in 1940 by H. Kasabach and K. Merritt syndrome than with consumption coagulopathy and hemangiomas. Only in 1997 it was recognized that it is the hemangiomas characteristically occurring for the syndrome is not common hemangiomas of children, but an aggressive form of Riesenhämangiomen. Not quite correct is sometimes used as a synonym of the term hemangioma - thrombocytopenia syndrome today.

Swell

• Schrier, S.: Extrinsic nonimmune hemolytic anemia due to mechanical damage: Fragmentation hemolysis and hypersplenism. UpToDate v15.3, 2007
• Leiber, The clinical syndromes: syndromes, sequences, and symptom complexes. 8th edition 1996, Urban and Schwarzenberg, ISBN 3-541-01707-4 and ISBN 3-541-01708-2