Table 2.
Is TTP associated with other disorders?
Condition | Bukowski (61) | Current evidence |
---|---|---|
Pregnancy | Insufficient data to account for an association of TTP and pregnancy | Most cases of thrombotic microangiopathy are due to HELLP syndrome or atypical HUS. |
Hereditary TTP or pre-existing autoimmune TTP may exacerbate during pregnancy. | ||
De novo cases of acute TTP during pregnancy are probably coincidental. | ||
Lupus and related connective tissue disorders | Patients with positive serological studies for lupus should be suspected of having a connective tissue disorder presenting as a TTP-like disorder. | ANA or other autoantibodies, mostly of low titers, may be present in some cases of TTP. |
Most cases of thrombotic microangiopathy in patients with active connective tissue disorders do not have profound ADAMTS13 deficiency and inhibitors of the enzyme are not detectable. | ||
Malignancy | The association of TTP and a malignancy was coincidental | Chemotherapeutic agents and embolism of metastasizing cancer cells, but not ADAMTS13 inhibitors, account for most cases of thrombotic microangiopathy in patients with underlying malignancies. |
Drugs | A definitive cause-and-effect relationship is lacking in most cases of drug-induced TTP. | Only ticlopidine has been demonstrated to be associated with ADAMTS13 inhibitors. |
Infection or Vaccination | A clear association of TTP with infection or recent vaccination is not recognized. | Infections may exacerbate pre-existing TTP. |
Only HIV infection has been associated with TTP. |
ANA: antinuclear antibodies; HELLP: hemolysis with elevated liver enzymes and low platelets; HUS: hemolytic uremic syndrome; TTP; thrombotic thrombocytopenic purpura.