A patient with a large venous malformation of the trunk or limb may experience intralesional thrombosis which can result in a consumptive coagulopathy.
Thrombosis in the malformation is associated with acute pain.
The risk of thrombosis may be increased by blood stasis in large malformations, hypercoagulable state and/or trauma. Women are more often affected than men.
Features of ongoing thrombosis:
(1) elevated D-dimer
(2) decrease in platelet count
(3) decrease in fibrinogen
(4) decrease in von Willebrand factor
Extensive thrombosis can result in disseminated intravascular coagulopathy (DIC) comparable to the Kasabach-Merritt phenomenon (see above). These patients may experience a clinical bleeding tendency.
Therapy with low molecular weight heparin can reduce pain and prevent the thrombosis causing the consumptive process..
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