Background: Kasabach-Merritt phenomenon (KMP) is a rare condition and optimal treatments have not yet been established, especially for cases that are unresponsive to first-line therapy. We retrospectively reviewed 11 KMP cases treated over the past 13 years in our institute.
Observations: With the exception of 1 case, steroids were administered as the first-line therapy. Eight cases required second-line or third-line therapy. The effective salvage therapies include interferon (n=1), radiotherapy (n=1), and chemotherapy (n=5). One case continues to depend upon chemotherapy. Three refractory cases were therapy dependent over 1 year of age, whereas 8 were treated effectively by 6 months of age.
Conclusions: Chemotherapy seems to be the most effective therapy for steroid-resistant KMP cases.
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http://dx.doi.org/10.1097/MPH.0b013e318281558e | DOI Listing |
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 PDFPediatr Dermatol
December 2024
Section of Dermatology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
Infantile hemangiomas (IH) are the most common benign tumors of infancy and progress through recognized stages of evolution including early proliferation, plateau, and involution. Ulceration is a common complication of IHs typically observed during the early proliferative stage characterized by rapid growth. In rare cases, ulceration is the primary clinical manifestation of IHs.
View Article and Find Full Text PDFJ Med Case Rep
December 2024
Shifa International Hospital/Shifa Tameer e Millat University, Islamabad, Pakistan.
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 PDFJ Med Case Rep
December 2024
Department of Surgery, Aga Khan University, Karachi, Pakistan.
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 PDFFront Pediatr
November 2024
[This corrects the article DOI: 10.3389/fped.2021.
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