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CLINICAL IMAGE
Year : 2020  |  Volume : 152  |  Issue : 7  |  Page : 226

Kaposiform haemangioendothelioma with Kasabach - Merritt phenomenon


Department of Internal Medicine, Postgraduate Institute of Medical Education & Research, Chandigarh 160 012, India

Date of Submission20-Nov-2019
Date of Web Publication25-May-2021

Correspondence Address:
Aditya Jandial
Department of Internal Medicine, Postgraduate Institute of Medical Education & Research, Chandigarh 160 012
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijmr.IJMR_2379_19

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How to cite this article:
Jandial A, Malhotra P. Kaposiform haemangioendothelioma with Kasabach - Merritt phenomenon. Indian J Med Res 2020;152, Suppl S1:226

How to cite this URL:
Jandial A, Malhotra P. Kaposiform haemangioendothelioma with Kasabach - Merritt phenomenon. Indian J Med Res [serial online] 2020 [cited 2021 Aug 4];152, Suppl S1:226. Available from: https://www.ijmr.org.in/text.asp?2020/152/7/226/316843

Patient's consent obtained to publish clinical information and images.


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 1]A, [Figure 1]B, [Figure 1]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 1]C and [Figure 1]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.
Figure 1: (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.

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Conflicts of Interest: None.


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