Kaposiform Hemangioendothelioma with Kasabach–Merritt Phenomenon
| Autor: | Mrinal Kanti Das, Norbu Sherpa, Supratim Datta, Akshay Rana, Emilee Das, Soumyadeep Sarkar, Sananda Pati |
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| Rok vydání: | 2021 |
| Předmět: |
Vincristine
medicine.medical_specialty business.industry Kasabach-Merritt Phenomenon Microangiopathic hemolytic anemia Phalanx medicine.disease Surgery Sepsis 03 medical and health sciences 0302 clinical medicine Kaposiform Hemangioendothelioma 030225 pediatrics Consumptive Coagulopathy Pediatrics Perinatology and Child Health Medicine Fresh frozen plasma business 030217 neurology & neurosurgery medicine.drug |
| Zdroj: | Indian Journal of Pediatrics. 88:1142-1144 |
| ISSN: | 0973-7693 0019-5456 |
| DOI: | 10.1007/s12098-021-03817-2 |
| Popis: | A 3-y-3-mo old male child presented with massive hypertrophy and bluish-purple discoloration of the left upper limb and adjacent chest wall of 3 mo duration. There was no h/o fever, weight loss, painful large joint swelling, or any bleeding manifestations. He had spindle like nonprogressive, painless swelling of all fingers of the left hand since infancy. The child was moribund with microangiopathic hemolytic anemia, thrombocytopenia, and consumptive coagulopathy without sepsis. He received multiple transfusions of fresh frozen plasma (FFP), platelets, and packed RBC. Paradoxical worsening of symptoms with platelet transfusions and radiological evidences led to the diagnosis of a very rare congenital multifocal vascular tumor, kaposiform hemangioendothelioma (KHE) with Kasabach-Merritt phenomenon (KMP). The index case of KHE was multifocal with cutaneous lesions, osteolytic bony lesions of all phalanx and metacarpals of the left hand, and intrathoracic extension. It was successfully managed with a combination of steroid, vincristine and sirolimus. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
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