Rapidly involuting congenital haemangioma associated with transient thrombocytopenia and coagulopathy: a case series

Abstract

Rapidly involuting congenital haemangioma (RICH) may present with thrombocytopenia, low fibrinogen and elevated fibrin degradation products and D-dimers. Such complications have rarely been reported. We wished to define the clinical characteristics of the thrombocytopenia and coagulopathy associated with RICH, to emphasize the transient nature of this haematological complication and to distinguish these abnormalities from true Kasabach-Merritt phenomenon (KMP). We present a case series of seven patients with large RICH who presented with thrombocytopenia and coagulopathy during the first week of life. Clinical and haematological characteristics were recorded retrospectively. Two of the patients were treated with embolization due to early signs of high-output cardiac failure; four patients received oral corticosteroids in the range of 2 mg kg(-1) daily; one patient did not receive any treatment in the neonatal period, although the tumour was excised at 6 months of age. Two patients with platelet counts lower than 10 x 10(9) L(-1) received a platelet transfusion. There were no bleeding complications and only one patient presented with petechiae. In all seven patients, platelet counts started to increase at > 2 weeks of age and the coagulopathy resolved. We conclude that RICH may present with thrombocytopenia and coagulopathy similar to mild KMP early in the neonatal period. However, in contrast to true KMP, these abnormal laboratory findings are self-limited and are usually not complicated by bleeding problems.

Rapidly involuting congenital haemangioma of the liver

“…The thrombocytopaenia in RICH may be a feature of larger lesions as it has never been reported in RICHs of smaller size 18. This consumptive coagulopathy is distinct from the Kasabach-Merritt syndrome, which is associated with profound thrombocytopaenia,19 fibrinogen consumption and secondary formation of D-dimer 20.…”

Diagnosis and Management of Infantile Hemangioma

“…4 RICHs, in some cases, have been associated with thrombocytopenia but with milder and more transient coagulopathy than that seen in Kasabach-Merritt phenomenon (KMP; see discussion that follows); rarely, they can be associated with congestive heart failure. 5,6 Some RICHs show incomplete involution, and it is possible that RICH and NICH lie at opposite ends of the same clinical spectrum. 7,8 Both subtypes of congenital hemangioma were initially believed to be variants of IH that exhibited prenatal growth until North et al 9 showed that, unlike IH, neither lesion expresses glucose transporter protein isoform 1 (GLUT1).…”

Life-Threatening Hemorrhaging in Neonatal Ulcerated Congenital Hemangioma

“…This could be because these tumors grow extremely rapidly during the later stages of pregnancy. Although very large CHs may be complicated by cardiac failure, thrombocytopenia, or coagulopathy, 13 these lesions are usually benign, and no treatment is recommended. Ulceration is a common, predominantly benign complication in IH and bleeding seems to occur only rarely and most often is moderate.…”