Lethal End of Spectrum of Clots-Thrombotic Storm

Table 1

Diagnostic criteria of thrombotic storm reproduced with permission from Kitchens et al.

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Thrombotic Storm Diagnostic Criteria

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Typically encountered characteristics

Younger age plus 2 or more of the following criteria:

Acute, 2 or more arterial or venous thromboemboli, with

or without thrombotic microangiopathy, in a

compressed period of time (1-2 weeks) yet may recur

from time to time over years.

Unusual location^†

Progressive/recent unexplained recurrence

Refractory to acute therapy or atypical response to

therapy

Exacerbated by inadequate or interrupted treatment (eg,

subtherapeutic anticoagulation)

Frequently preceded by an initiating event (“trigger”)^‡

Characteristics usually not encountered

Cancer (excluding minor skin cancers)

Myocardial infarction in the setting of advanced coronary

artery disease

Cocaine use associated with symptom onset

Expected thrombotic complications associated with

intravascular devices

Known paroxysmal nocturnal hemoglobinuria or

myeloproliferative disorder

Multi-trauma/severe trauma (eg, multiple limb injury)

Premorbid clinical status before development of thrombotic

complications

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microangiopathy defined as microvascular thrombosis (arteriole, venule, capillary) on tissue pathology. ^†Unusual locations would include thromboembolic complications other than pulmonary embolism, lower extremity deep venous thrombosis, myocardial infarction, and stroke such as thrombosis of hepatic, cerebral, portal, or renal veins, skin (purpura fulminans), and adrenal glands. ^‡Such as pregnancy, surgery, trauma, infections, and inflammatory states.