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Review
. 2017 Mar 16:10:1179545X17699849.
doi: 10.1177/1179545X17699849. eCollection 2017.

Kasabach-Merritt Phenomenon: Classic Presentation and Management Options

Priya Mahajan  1 , Judith Margolin  1 , Ionela Iacobas  1
Affiliations
Review

Kasabach-Merritt Phenomenon: Classic Presentation and Management Options

Priya Mahajan et al. Clin Med Insights Blood Disord. .

Abstract

Kasabach-Merritt phenomenon (KMP) is a rare consumptive coagulopathy associated with specific vascular tumors, kaposiform hemangioendothelioma, and tufted angioma. Kasabach-Merritt phenomenon, characterized by profound thrombocytopenia, hypofibrinogenemia, elevated fibrin split products, and rapid tumor growth, can be life-threatening. Severe symptomatic anemia may also be present. With prompt diagnosis and management, KMP can resolve and vascular tumors have been shown to regress. This review highlights the clinical presentation, histopathology, management, and treatment of KMP associated with kaposiform hemangioendothelioma, and less frequently tufted angioma. A classic clinical case is described to illustrate the presentation and our management of a patient with KMP.

Keywords: Kasabach-Merritt phenomenon (KMP); coagulopathy; kaposiform hemangioendothelioma (KHE); sirolimus; thrombocytopenia.

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Conflict of interest statement

DECLARATION OF CONFLICTING INTEREST: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Figures

Figure 1.
Figure 1.
Classic clinical case of Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon (KMP) diagnosed in a 5-week-old patient. Diagnosis, response to blood product transfusion, and resolution of KMP with sirolimus + steroids therapy. (A) Platelet counts (normal: 150-450 × 103/µL), (B) hemoglobin (normal: 9.5-13.5 g/dL), (C) fibrinogen (normal: 220-440 mg/dL), and (D) d-dimer (normal: <0.4 µg/mL).
Figure 2.
Figure 2.
Classic clinical case of Kaposiform hemangioendothelioma with Kasabach-Merritt phenomenon (KMP) diagnosed in a 5-week-old patient. Extension of cutaneous involvement. Photos presented with mother’s permission. (A) At the peak of activity of KMP (day 4)—patient with purpura, edema, irregular margins, and extensive size. (B) After 3 weeks of treatment, the cutaneous component improved significantly. (C) After 2 months of therapy with sirolimus and steroids, complete resolution of the cutaneous component was achieved.
See this image and copyright information in PMC

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