Hemangioma-Thrombocytopenia Syndrome Associated with Microangiopathic Hemolytic Anemia

Propp, Richard P.; Scharfman, William B.

doi:10.1182/blood.v28.5.623.623

Cited by 53 publications

(11 citation statements)

References 14 publications

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“…Vascular malformations are sometimes accompanied by a systemic disorder of haemostasis with a latent or manifest haemorrhagic diathesis as a result. Such a disorder has been demonstrated in haemangioma (7,12,13,14,15,19,21,23). It may also occur in Osler's disease or hereditary haemorrhagic telangiectasia (17).…”

Section: Introductionmentioning

confidence: 82%

Osler's Disease With Impaired Adhesion and Aggregation of Platelets

Larsson

1974

Journal of Internal Medicine

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In the investigation of haemostasis in a 37year-old woman with Osler's disease the Ivy bleeding time proved to be prolonged. Platelet adhesiveness, measured both by Hellem's and Salzman's methods, was decreased and platelet aggregation was clearly reduced after addition of ADP, but only slightly after collagen and epinephrine. Platelet factor 3 activity was normal, a s was clot retraction. The therapeutic consequences of the platelet defect, including the risk of using drugs affecting platelet function, are stressed. It is concluded that in cases of Osler's disease the possibility of functional disorders of platelets should be borne in mind.

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Section: Introductionmentioning

confidence: 82%

Osler's Disease With Impaired Adhesion and Aggregation of Platelets

Larsson

1974

Journal of Internal Medicine

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“…This continuous process of clotting-lysis produces a "defibrination" of the circulating blood, which is believed to protect the individual from developing a disseminated intravascular coagulopathy [2]. The development of erythrocyte abnormalities consistent with microangiopathic hemolytic anemia has also been described in this syndrome [7,8], presumably resulting from mechanical trauma sustained by the erythrocytes circulating through the tumor's abnormal, partially thrombosed vascular channels.…”

Section: Discussionmentioning

confidence: 99%

Antifibrinolytic therapy in the management of the kasabach merritt syndrome

et al. 1988

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The Kasabach Merritt syndrome consists of thrombocytopenia, microangiopathic hemolytic anemia, and a localized consumption coagulopathy that develops within the abnormal vascular channels of a hemangioma. In general, these patients demonstrate only mild abnormalities of screening clotting tests, but they can potentially develop life-threatening complications. We present a patient who developed a severe anemia that was refractory to erythrocyte transfusions. Treatment with epsilon-aminocaproic acid to inhibit fibrinolysis and cryoprecipitate to replenish his deficient circulating fibrinogen interrupted the cycle of his systemic coagulopathy and enabled us to transfuse him to a normal hematocrit.

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“…Although their patient also had prolongation of the coagulation time, the bleeding in their case and in subsequent cases was usually due to thrombocytopenia. Other patients were reported who had hypofibrinogenemia and low levels of factors V and VIII and prothrombin [61,91,190,231]. In infants, the hemangioma may subside spontaneously; otherwise, attempts should be made to treat it by x-radiation or surgical removal.…”

Section: Cardiovascular Systemmentioning

confidence: 99%