Six cases are presented demonstrating key features of what may be termed thrombotic storm: (1) an underlying hypercoagulable disorder; (2) a provocation to initiate thrombosis; (3) rapid development of new thromboses; (4) response to prompt use of thrombolytic agent or anticoagulant therapy; and (5) remarkable good long-term prognosis if the cycle of thrombosis is interrupted. Continued activation of coagulation by fresh thrombosis is hypothesized as the cause of the syndrome, which may explain its control by anticoagulants. Whereas these unusual patients’ courses most likely represent only an extreme of hypercoagulability and not a new disorder, their characteristic behavior warrants attention.
The underlying hypercoagulable disorder was not always identified although strongly suspected to be present based on underlying clinical behavior.
Recently Kitchens and his group have published an update in the same journal. In their series to date the presence of antiphospholipid antibodies was the most common underlying hypercoagulable state. Those patients resembled catastrophic antiphospholipid syndrome. There appears to be some overlap between catastrophic antiphospholipid syndrome and thrombotic storm. Catastrophic antiphospholipid syndrome may represent a subset of thrombotic storm.
The word “storm” is apt. All storms eventually pass. Thrombotic storm is remarkable in that it remits with the institution of effective anticoagulant treatment.