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Kasabach-Merritt Phenomenon: Classic Presentation and Management Options
Priya Mahajan1, Judith Margolin1, Ionela Iacobas1
1Department of Pediatrics, Baylor College of Medicine, Vascular Anomalies Center at Texas Children's Cancer and Hematology Centers, Texas Children's Hospital, Houston, TX, USA.
Clinical Medicine Insights. Blood Disorders
|June 6, 2017
Summary
Kasabach-Merritt phenomenon (KMP), a rare coagulopathy linked to vascular tumors, presents with low platelets and bleeding risks. Prompt diagnosis and management lead to tumor regression and KMP resolution.
Area of Science:
- Hematology
- Pediatric Oncology
- Vascular Biology
Background:
- Kasabach-Merritt phenomenon (KMP) is a rare, life-threatening consumptive coagulopathy.
- It is primarily associated with kaposiform hemangioendothelioma and tufted angioma.
- KMP presents with severe thrombocytopenia, hypofibrinogenemia, and elevated fibrin split products.
Purpose of the Study:
- To review the clinical presentation, histopathology, management, and treatment of KMP.
- To highlight KMP associated with kaposiform hemangioendothelioma and tufted angioma.
- To illustrate KMP management through a classic clinical case.
Main Methods:
- Literature review of KMP, kaposiform hemangioendothelioma, and tufted angioma.
- Analysis of clinical presentation and diagnostic criteria.
- Discussion of current treatment strategies and outcomes.
Main Results:
- KMP is characterized by profound thrombocytopenia, hypofibrinogenemia, and elevated fibrin split products.
- Rapid tumor growth and severe anemia can be present.
- Prompt diagnosis and management are crucial for resolution and tumor regression.
Conclusions:
- KMP requires timely diagnosis and multidisciplinary management.
- Treatment strategies aim to control coagulopathy and induce tumor regression.
- Effective management can lead to favorable outcomes for patients with KMP.
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