Tufted Angioma with Kasabach–Merritt Phenomenon ( 2 фото )

A full-term baby boy was transferred to a quaternary care hospital 1 day after birth for evaluation of a vascular lesion on his back. On physical examination, a violaceous, firm, hypertrichotic plaque, measuring 10 cm by 5 cm, was seen on the right side of the back (Panel A). Histopathological testing of a punch-biopsy specimen showed numerous vascular lobules throughout the dermis that were composed of spindle-shaped cells in a “cannonball” pattern. Magnetic resonance imaging of the back revealed a large, noninfiltrative vascular lesion extending from the right lateral to left medial back. Laboratory studies were notable for a platelet count of 51,000 per microliter (reference range, 114,000 to 295,000), a fibrinogen level of 153 mg per deciliter (reference range, 200 to 400), and a d-dimer level of 9.56 μg per milliliter (reference value, <0.50). A diagnosis of tufted angioma with the Kasabach–Merritt phenomenon was made. Tufted angiomas are benign, rare, cutaneous vascular tumors that appear in infancy. Tufted angiomas — as well as a more aggressive vascular tumor called kaposiform hemangioendothelioma — can be associated with the Kasabach–Merritt phenomenon, a consumptive coagulopathy and thrombocytopenia that results from platelet sequestration and destruction within the tumor. Treatment with sirolimus was initiated. At 2 months of follow-up, the tumor had regressed (Panel B) and hematologic variables had normalized.
Sarah Servattalab, M.D. and Pierre-Olivier Grenier, M.D.Author Info & Affiliations
Published May 24, 2025
N Engl J Med 2025;392: e51
DOI: 10.1056/NEJMicm2414170
VOL. 392 NO. 20


DeepL Переводчик

Крепкого здоровья!