Hemangioma-thrombocytopenia syndrome; a disseminated intravascular coagulopathy

Lang, P. G.

doi:10.1001/archderm.111.1.105

Cited by 8 publications

(2 citation statements)

References 0 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…4 The process seems to be localised in the tumour rather than generalised, as suggested by previous studies, but it may be exacerbated by surgery or biopsies, extending into a more disseminated process, manifesting as further thrombocytopenia, anaemia or developing venous thrombosis. 5 In the few reported adult cases, the presenting symptoms include abdominal pain, increased mass size, disseminated intravascular coagulopathy, bleeding, fractures and high output cardiac failure. Owing to the rarity of this syndrome, no clear guidelines for management are available.…”

Section: Discussionmentioning

confidence: 99%

Massive liver haemangioma causing Kasabach–Merritt syndrome in an adult

Alimoradi

Sabra

El-Helou

et al. 2020

annals

View full text Add to dashboard Cite

Liver haemangiomas are common, but their size very rarely exceeds 40cm. Most people with liver haemangiomas are asymptomatic, and diagnosis is usually made incidentally during imaging for other complaints. When a liver haemangioma is symptomatic or produces complications, surgical intervention may be warranted. Kasabach–Merritt syndrome is an uncommon complication reported in certain rare vascular tumours in children, with only a few cases reported in adults. The syndrome describes a consumptive coagulopathy initiated within a vascular tumour, mainly tufted angiomas and kaposiform haemangioendotheliomas and, less commonly, giant haemangiomas. The process can extend beyond the tumour and become disseminated in certain cases due to trauma or surgery. The definitive treatment for giant liver haemangiomas can include arterial embolisation, surgical excision, hepatectomy or even liver transplantation. We report the case of a 32-year-old woman with a 42 × 32 × 27cm (18,870ml) liver haemangioma associated with Kasabach–Merritt syndrome. The diagnosis was challenging, even with proper imaging, owing to the rarity of the condition. It was achieved with an exploratory laparotomy with biopsy.

show abstract

Section: Discussionmentioning

confidence: 99%

Massive liver haemangioma causing Kasabach–Merritt syndrome in an adult

Alimoradi

Sabra

El-Helou

et al. 2020

annals

View full text Add to dashboard Cite

show abstract

“…Vascular lesions such as hemangiomas have been frequently associated with hemostasis dysfunction and this led us to examine coagulative and fibrinolytic parameters in fps MF mice [31–37]. Intrinsic and extrinsic coagulation were measured using APTT and PT assays, respectively.…”

Section: Hemostasis Defects In Fpsmf Micementioning

confidence: 99%

Hemostatic and hematological abnormalities in gain‐of‐function fps/fes transgenic mice are associated with the angiogenic phenotype

Sangrar

Senis

Samis

et al. 2004

Journal of Thrombosis and Haemostasis

View full text Add to dashboard Cite

show abstract

Antifibrinolytic therapy in the management of the kasabach merritt syndrome

et al. 1988

View full text Add to dashboard Cite

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.

show abstract

Hemangioma-thrombocytopenia syndrome; a disseminated intravascular coagulopathy

Cited by 8 publications

References 0 publications

Massive liver haemangioma causing Kasabach–Merritt syndrome in an adult

Massive liver haemangioma causing Kasabach–Merritt syndrome in an adult

Hemostatic and hematological abnormalities in gain‐of‐function fps/fes transgenic mice are associated with the angiogenic phenotype

Antifibrinolytic therapy in the management of the kasabach merritt syndrome

Contact Info

Product

Resources

About