Thrombotic Thrombocytopenic Purpura Associated with Systemic Lupus Erythematosus and Antiphospholipid Syndrome: A Case Report

The occurrence of thrombotic thrombocytopenic purpura (TTP) in patients with systemic lupus erythematosus (SLE) is rare TTP frequence. In pregnancy women is increased. TTP with simultaneous SLE, and antiphospholipid syndrome (APS) occurring in the same patient is extremely rare.

We described a 41-year-old Chinese female with TTP in her third trimester (33⁺⁴ gestational weeks) of pregnancy who suffered from SLE. and APS at the same time. The clinical features and positive laboratory abnormalities included fever, repeated abortion in first trimester, palpitation, dyspnea, orthopnea, hypertension (180/113mmHg), proteinuria, renal dysfunction, thrombocytopenia (platelets count 12×10⁹/L), hemolytic anemia (Hb 67g/L), positive Coombs test, positive lupus test, positive antinuclear antibody, anti-Sm antibody, anti-insulin antibody, anti-ENA antibody and anti-RNP antibody. The level of plasma ADAMTS13 activity was below 5%, and ADAMTS13 inhibitor was present in the patient's plasma. This patient was treated with a combination of corticosteroids and plasma infusion and plasmapheresis. At 33⁺⁵ weeks of gestation she delivered a healthy infant via cesarean section. The patient improved quickly with these therapies.

This is a rare TTP case with simultaneous SLE, APS and preeclampsia. Her overlaping clinical manifestations and laboratory abnormalities made the diagnosis very difficult. These data indicate that plasma therapy and corticosteroids can improve the prognosis of TTP associated with SLE and APS. In addition, the termination of pregnancy may be also helpful for controlling the development of the disease.

No relevant conflicts of interest to declare.