Kasabach-Merritt syndrome

Case contributed by Dr Vincent Tatco

Presentation

Erythematous mass over the right chest extending to the right upper arm with markedly decreased platelet count

Patient Data

Age: 5 days
Gender: Female
Modality: X-ray

There is a soft tissue density mass involving the right hemithorax and right upper extremity.  The liver shadow is enlarged.   

Modality: CT

A large hypervascular mass encompassing the right cervical region extending to the right supraclavicular area, shoulder, and arm down to the anterior, lateral and posterior chest wall.  The mass measures approximately 9.0 x 8.5 x 9.8 cm in anteroposterior, transverse and craniocaudal dimensions.  The mass is devoid of calcifications or phleboliths.  

Oedema of the overlying skin and subcutaneous tissues is observed with associated prominent and tortuous superficial vessels.  The right pectoralis, latissimus dorsi, serratus anterior, intercostal, deltoid and rotator cuff muscles are not delineated from the mass lesion.  The features of the mass are compatible with kaposiform hemangioendothelioma.

Hepatomegaly is also demonstrated. 

Case Discussion

Kasabach-Merritt syndrome (KMS) is characterized by giant vascular tumors and severe thrombocytopenia, which may result in life-threatening multi-organ haemorrhage. The thrombocytopenia associated with vascular lesions is caused by a localized consumptive coagulopathy. The vascular lesion triggers an intravascular coagulation with platelet trapping and consequent thrombocytopenia, as well as activation and consumption of coagulation factors.  

KMS is associated with kaposiform hemangioendothelioma in over 90% of cases and uncommonly with infantile and congenital haemangioma. Kaposiform hemangioendothelioma is typically a solitary tumor which appear in the soft tissues of the limbs, head and neck or retroperitoneum. This vascular tumor is seen in infants less than 2 years of age, although cases have been reported in adults. It can cause serious problems because of local growth, cardiac failure or the associated Kasabach-Merritt phenomenon.

PlayAdd to Share

Case Information

rID: 42186
Case created: 10th Jan 2016
Last edited: 27th May 2016
System: Paediatrics
Inclusion in quiz mode: Included

Updating… Please wait.
Loadinganimation

Alert accept

Error Unable to process the form. Check for errors and try again.

Alert accept Thank you for updating your details.