Translate this page into:
Kaposiform haemangioendothelioma with Kasabach - Merritt phenomenon
*For correspondence: hematpgi@gmail.com
-
Received: ,
This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.
This article was originally published by Wolters Kluwer - Medknow and was migrated to Scientific Scholar after the change of Publisher.
A 16 yr old male child†, having painless left upper limb swelling since early childhood, presented to the department of Internal Medicine, Postgraduate Institute of Medical Education & Research, Chandigarh, India, in August 2017, with sudden increase in swelling and bleeding after blunt trauma. He had a firm violaceous nodular swelling of the left upper limb extending to the left axilla, scapular and mammary areas (Figure A-C). Investigations revealed thrombocytopaenia (51,000/mm3) and coagulopathy [prothrombin time (23.2 sec), activated partial thromboplastin time (43 sec), fibrinogen (0.3 g/l) and D-dimer levels (2250 ng/ml)], suggesting Kasabach-Merritt phenomenon. Magnetic resonance imaging showed multiple poorly demarcated intramuscular lobulated soft tissue masses consistent with Kaposiform haemangioendothelioma (Figure C and D). Bleeding was controlled by compression dressing along with platelet and fresh frozen plasma transfusion. A mild decrease in swelling was noticed with sirolimus (2 mg/day) after one year follow up. Kaposiform haemangioendothelioma, an uncommon vascular tumour, may rarely present with bleeding due to the development of Kasabach-Merritt phenomenon; sirolimus monotherapy may be effective in non-resectable cases.
- (A and B) Kaposiform haemangioendothelioma involving the left upper limb left scapular region and the left mammary area. (C) Coronal T2-weighted image showing multiple lobulated soft tissue masses which are hyperintense relative to muscle. (D) Coronal T1-weighted image showing heterogeneous isointense signal.
Conflicts of Interest: None.
