Infants with Kasabach-Merritt syndrome do not have “true” hemangiomas,☆☆

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Abstract

Objective: In 1940 Kasabach and Merritt described an infant with a vascular anomaly, extensive purpura, and thrombocytopenia; they called his lesion “capillary hemangioma.” Hemangioma is a benign tumor that grows in infancy and is characterized by proliferation of endothelial cells and regression during childhood. Although Kasabach-Merritt syndrome (KMS) is frequently mentioned as a possible complication of hemangioma, our experience suggests that the anatomic vascular lesion underlying the thrombocytopenia is not a “true,” classic, involuting type of hemangioma of infancy and childhood.

Study design: We reviewed the clinical and hemostasis data and the response to treatment in 22 cases of KMS, and we analyzed the biopsy specimens of 15 of them.

Results: Clinically none of the 22 patients had classic hemangioma. There was no female preponderance. All patients had severe thrombocytopenia (lowest platelet count = 3000/mm3) and consumption of fibrinogen. Histologically, none had the typical “capillary,” involuting type of hemangioma of infancy: they exhibited either a tufted angioma or a kaposiform hemangioendothelioma pattern; all specimens also contained numerous abnormal lymphatic-like vessels; lymphatic malformation was the major component in two patients. The infants exhibited a heterogeneous response to a number of therapeutic regimens, as noted in other reports. Severe morbidity was present; three of our patients died, and one had leg amputation. “Residua” were, in fact, residual vascular neoplasia, variable in duration, and not a stable fibrofatty residuum, as in classic involuted hemangioma; only the hematologic phenomenon was “cured” after a period of years.

Conclusions: KMS is a distinctive disease of infancy, but the underlying vascular lesion is not a “true,” classic, involuting type of hemangioma of infancy. This is a different vascular tumor with a resemblance pathologically to either tufted angioma or kaposiform hemangioendothelioma in association with lymphatic-like vessels. Whether the underlying lesion in KMS is a single anatomic entity or heterogeneous cannot be definitely concluded from this study. We need a better understanding of the pathogenesis of KMS to improve our therapeutic management. (J Pediatr 1997;130:631-40)

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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    Reprint requests: Odile Enjolras, MD, Hôpital Tarnier (CHU Cochin), 89 rue d'Assas, 75006 Paris, France.

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