Kasabach-Merritt syndrome natural history, complications and prognosis

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Kasabach-Merritt syndrome Microchapters

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Overview

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Differentiating Kasabach-Merritt syndrome from other Diseases

Epidemiology and Demographics

Natural History, Complications and Prognosis

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Case #1

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

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Overview

Natural History

Complications

They are at risk of bleeding complications including intracranial hemorrhage. The thrombocytopenia and coagulopathy are managed with platelet transfusions and fresh frozen plasma, although caution is needed due to the risk of fluid overload and heart failure from multiple transfusions. The possibility of disseminated intravascular coagulation, a dangerous and difficult-to-manage condition, is concerning. Anticoagulant and antiplatelet medications can be used after careful assessment of the risks and benefits.

Prognosis

Patients with KMS can be extremely ill and may need intensive care. KMS has a mortality rate of about 30%. For patients that survive the acute disease, supportive care may be required through a gradual recovery.

Furthermore, patients may need care from a dermatologist or plastic surgeon for residual cosmetic lesions. On long-term followup, most patients have skin discoloration and/or mild disfiguration from the dormant tumor.[1]

References

  1. Enjolras O, Mulliken J, Wassef M, Frieden I, Rieu P, Burrows P, Salhi A, Léauté-Labrèze C, Kozakewich H (2000). "Residual lesions after Kasabach-Merritt phenomenon in 41 patients". J Am Acad Dermatol. 42 (2 Pt 1): 225–35. PMID 10642677.

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