Kasabach-Merritt Syndrome (KMS) originally referred to a hemangioma associated with life-threatening thrombocytopenia and a consumption coagulopathy. Treatment includes surgery, steroids, and alpha interferon. However response may be unsatisfactory and mortality rates of 10-37% have been reported. We report a case of a 4-month-old male whose disease had not responded to steroids and alpha interferon. He required ventilation, and his condition was deteriorating. It was thought that he would probably die of his condition. He was treated with 9 Gy in three fractions weekly of radiotherapy (RT). Clinical response was good and he is well and free from progression 4 years and 7 months after RT. A review of the literature on the role of RT in the management of hemangiomas is presented.

Download full-text PDF

Source
http://dx.doi.org/10.1002/pbc.20767DOI Listing

Publication Analysis

Top Keywords

kasabach-merritt syndrome
8
steroids alpha
8
alpha interferon
8
radiotherapy life-threatening
4
life-threatening mediastinal
4
mediastinal hemangioma
4
hemangioma kasabach-merritt
4
syndrome kasabach-merritt
4
syndrome kms
4
kms originally
4

Similar Publications

Neonatal Cutaneous Vascular Anomalies.

Neoreviews

January 2025

Vascular Anomalies Center, Division of Pediatric Surgery, Children's Hospital Colorado, Aurora, Colorado.

Vascular anomalies are broadly classified into 2 categories: vascular tumors and vascular malformations. Vascular anomalies frequently present as cutaneous lesions in infants. This review summarizes vascular anomalies that most commonly present as dermatologic lesions in the neonatal period, with a focus on the clinical findings, pathophysiology and histology, relevant radiographic findings, and management of common vascular anomalies such as infantile hemangiomas, congenital hemangiomas, and Kaposiform hemangioendothelioma, along with vascular malformations, including capillary, lymphatic, venous, and arteriovenous malformations.

View Article and Find Full Text PDF

Background: Angiosarcoma is a rapidly proliferating vascular tumor that originates in endothelial cells of vessels. Rarely, it can be associated with consumptive coagulopathy due to disseminated intravascular coagulation eventually leading to thrombocytopenia and microangiopathic hemolytic anemia. This specific manifestation is termed Kasabach-Merritt syndrome.

View Article and Find Full Text PDF

Background: Kaposiform hemangioendothelioma is a rare vascular tumor primarily occurring in infants and children. The most common sites for kaposiform hemangioendothelioma are extremities, with very few cases of abdominal kaposiform hemangioendothelioma reported in neonates. Making a diagnosis of Kaposiform hemangioendothelioma can be challenging when the patient presents with generalized symptoms such as bilious vomiting and constipation that can be attributed to other more common causes of intestinal obstruction.

View Article and Find Full Text PDF

Hemostasis and thrombosis risks and management in vascular anomalies.

Hematology Am Soc Hematol Educ Program

December 2024

Pediatric Hematology-Oncology, University of Arkansas for Medical Sciences, Little Rock, AR.

The role of the hematologist in the management of vascular anomalies is evolving. Several vascular tumors and malformations are associated with complex coagulation derangements. Kaposiform hemangioendothelioma or tufted angiomas may present with a consumptive coagulopathy known as the Kasabach-Merritt phenomenon (KMP).

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!