Secondary thrombotic microangiopathy with generalized purpura in a young woman

Abstract

A 22-year-old woman with no previous medical history presented with shock and extensive purpura spots after a sudden rash on both upper limbs and trunk (Figure 1A–C). Blood tests revealed multiple organ failure, increased inflammatory response, and coagulation abnormalities. We started antibiotics for septic shock with purpura fulminans; however, pancytopenia with hemolytic anemia and renal failure worsened. We diagnosed thrombotic microangiopathy (TMA) including thrombotic thrombocytopenic purpura (TTP) and initiated plasma exchange (PE) on Day 3. Five PE rounds dramatically improved her condition; however, dry necrosis remained in both hands. We amputated the necrotic tissue 18 days after admission, from the distal end of the basal phalanx of the second to fifth finger. She was discharged on Day 60. The trunk healed without scarring; however, scars were left on the upper limbs (Figure 1D–F). Biopsy findings from the abdomen and left knee indicated TMA. Culture and immunological tests, including ADAMTS13 activity, were negative. She had fever, cold symptoms, and arthritis 2 days before admission, leading to viral infection-induced secondary TMA diagnosis. Secondary TMA rarely presents with extensive purpura, although its involvement in infection-related TMA is reported.^{1, 2} PE should be aggressively performed for secondary TMA, especially when caused by infection.

The authors declare no conflicts of interest.

Approval of the research protocol: None.

Informed consent: Informed consent was obtained from the patient and parents.

Registry and the registration no. of the study/trial: None.

Animal studies: None.