Infants with Kasabach-Merritt syndrome do not have “true” hemangiomas☆,☆☆
Section snippets
METHODS
This study concerns 22 infants with KMS; they were accrued in the following way: (1) 14 cases from Paris, France, were referred to us from 1980 to 1996, either for diagnosis, evaluation, advice on treatment, or clinical and biologic follow-up; (2) 3 cases from Nijmegen, in the Netherlands, were treated in a pediatric surgery unit from 1990 to 1996; and (3) 5 cases were treated at the University of California, San Francisco, in the United States, during a period of 6 years. Clinical data and
RESULTS
Of our 22 patients, 11 were boys and 11 were girls. KMS was superficial in 16 patients and superficial, visceral, or both in 6. Lesions involved the lower limb (6 patients); upper limb or shoulder (5); trunk (4); face bilaterally (1); neck and airway (1); face, neck, and airway (2); face, neck, airway, and mediastinum (1); and mediastinum (2). The Table outlines the main characteristics of the patients. Clinically, none of the cases had a classic hemangioma. They had had, since birth, an area
DISCUSSION
Kasabach and Merritt 1 described a rare and distinctive syndrome. Typically the patient is an infant who, at birth or soon after, suddenly acquires a huge inflammatory, bruising reddish or purple mass, in association with a severe thrombocytopenia and ecchymoses spreading to other areas. 6, 7 Thrombocytopenia is constant and severe. Profound thrombocytopenia is the sine qua non of KMS, but consumption of fibrinogen and coagulation factors can be associated. When a cutaneous lesion was noted
ADDENDUM
Since the article was accepted for publication, patient 22 had a biopsy of the residual lesion. There were features of TA, as well as spindle cells and increased lymphatic-like vessels.
Acknowledgements
We thank the following pathologists for providing the histopathologic specimens: from France: Dr. M. Leibowitch (deceased; Paris) and Drs. P. Bruneval (Paris), P. Josset (Paris), P. Bioulac-Sage (Bordeaux), P. Bureau (Nantes), J. P. Leroy (Brest), R. Loire (Lyon), and P. Y. Paquet (Valenciennes); from San Francisco, Calif.: Drs. T. H. McCalmont and W. Rosenau; and from Nijmegen, The Netherlands: Drs. Kubat and Boerman.
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