TMA and Infections, A Vicious Circle

Abstract

Background: Thrombotic Microangiopathies (TMA) are rare but serious disease with a pattern of damage that can occur in smallest blood vessels inside many vital organs.

Case History: Female, 62y.o, entered ER due to worsening asthenia, thrombocytopenia and anemia on laboratory tests. Confirmed hemolytic anemia (increased LDH, indirect bilirubin, rare schistocytes on peripheral smear) anti-ADAMTS13 activity assay is performed, which is almost suppressed (< 5%) confirming diagnosis Thrombotic Thrombocitopenic Purpura (TTP). Plasma exchange, caplacizumab and steroids (1mg/kg body weight) were started. During hospitalization, due to increased inflammation indices during an episode of recrudescence (thrombocytopenia and undetectable ADAMTS13 activity) laboratory tests were performed, confirming diagnosis of septic state secondary to Salmonella typhi infection, complicated by septic arthritis. Rituximab and antibacterial therapy were started, achieving resolution of the septic state, but due to the persistence of thrombocytopenia (with normal ADAMTS13 activity) and finding of hepatosplenomegaly, was documented reactivation of Cytomegalovirus infections, therefore therapy with Ganciclovir was started, with resolution of the infectious state and TTP.

Discussion: Bacterial/viral infections are commonly observed amongst TMA hospitalized patients and may act as a trigger (including recrudescences and relapses) or a consequence of immunosoppressive therapy; timely diagnosis and most appropriate treatment can help resolve PTT.