Differential diagnosis of coagulopathy in vascular anomalies
It is important to be certain of the diagnosis as clinically the
differential diagnosis of a vascular lesion with coagulopathy may
overlap with other conditions (Table 1). Kaposiform lymphangiomatosis
(KLA) is a very rare lymphatic disorder that usually presents with
multifocal or diffuse involvement of the mediastinum, lungs, bones and
abdomen. These patients can have very severe thrombocytopenia with
coagulopathy similar to KMP18-20. They do not
typically respond to corticosteroids. They can be differentiated from
KHE by the presence of a somatic activating NRAS mutation which
have not been shown in KHE21. Both KLA and KHE have
elevation of angiopoetin-2 (Ang-2)22 but this may be a
helpful biomarker to distinguish from other vascular lesions. Another
vascular disorder that may present with bleeding and thrombocytopenia is
multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT). MLT,
however, does not usually have the findings of intravascular
coagulopathy (hypofibrinogenemia, elevated d-dimer, prolonged PT/aPTT)
and bleeding typically occurs directly from the lesions present in the
gastrointestinal tract or lungs23,24. Some congenital
hemangiomas may also present with a mild consumptive coagulopathy, but
it is typically not as severe, and the congenital hemangiomas do not
continue to enlarge after birth as the KHE with KMP typically
do25. Large venous malformations may also have
evidence of LIC presenting with very elevated d-dimer and, when severe,
thrombocytopenia and hypofibrinogenemia26,27. These
lesions should be differentiated from KHE with imaging findings.