Systemic infections mimicking thrombotic thrombocytopenic purpura

Abstract

The absence of specific diagnostic criteria, the urgency to begin plasma exchange treatment, and the risk for complications from plasma exchange make the initial evaluation of patients with suspected thrombotic thrombocytopenic purpura (TTP) difficult. Systemic infections may mimic the presenting clinical features of TTP. In the Oklahoma TTP-HUS (hemolytic-uremic syndrome) Registry, 1989–2010, 415 consecutive patients have been clinically diagnosed with their first episode of TTP; in 31 (7%) the presenting clinical features were subsequently attributed to a systemic infection. All 31 patients had diagnostic criteria for TTP; 16 (52%) had the complete “pentad” of microangiopathic hemolytic anemia, thrombocytopenia, neurologic abnormalities, renal failure and fever. Four (16%) of 25 patients who had ADAMTS13 measurements had <10% activity; three patients had a demonstrable ADAMTS13 inhibitor. Compared to 62 patients with severe ADAMTS13 deficiency (<10%) who had no recognized alternative disorders, patients with systemic infections had more frequent fever, coma, renal failure, and the complete “pentad” of clinical features. Seventeen different infectious etiologies were documented. A systematic literature review identified 67 additional patients with a diagnosis of TTP or HUS and also a systemic infection. Among all 98 patients, infections with 41 different bacteria, viruses, and fungi were documented, suggesting that many different systemic infections may mimic the presenting clinical features of TTP. Initial plasma exchange treatment is appropriate in critically ill patients with diagnostic features of TTP, even if a systemic infection is suspected. Continuing evaluation to document a systemic infection is essential to determine the appropriateness of continued plasma exchange.

Diagnosis and Management of Tickborne Rickettsial Diseases: Rocky Mountain Spotted Fever and Other Spotted Fever Group Rickettsioses, Ehrlichioses, and Anaplasmosis — United States

“…For example, RMSF-associated cutaneous necrosis and gangrene might be difficult to distinguish clinically from purpura fulminans associated with meningococcemia (105). RMSF-associated neurologic manifestations, renal failure, and thrombocytopenia have led to confusion with the diagnosis of thrombotic thrombocytopenic purpura (TTP) (106)(107)(108). RMSF-associated vasculitis has been confused with idiopathic, acute vasculitides, such as Kawasaki disease in pediatric patients.…”

Thrombocytopenia in critically ill patients due to vascular microthrombotic disease: pathogenesis based on “two activation theory of the endothelium”

“…It is also associated with thrombocytopenia (i.e., TCIP) and disseminated intravascular microthrombosis (DIT) [28,29]. Other clinical syndromes associated with critical illnesses include SIRS [13,14], ARDS [19,22], MODS [4,6,11,12,14], "DIC" [30][31][32], thrombotic thrombocytopenic purpura (TTP)-like syndrome [33][34][35][36][37][38][39], hepatic coagulopathy, and others [36,40].…”

“…Both almost always occur in critical illnesses (e.g. sepsis/ septic shock, trauma, immunologic and collagen-vascular diseases, and complications of surgery, pregnancy and transplant) [38,60,61]. Pathologically both are characterized by arteriolar and capillary hyaline microthrombi with variable fibroblastic proliferation [49,62].…”

A case of leptospirosis presenting as TTP

“…Clinical studies have demonstrated that thalidomide and lenalidomide each have their own distinct toxicity and efficacy [1][2][3][4] despite sharing an overlapping immunomodulatory profile [5,6]. It is evident that patients previously refractory to the less potent IMiD thalidomide can still respond when treated with lenalidomide [7,8].…”

“…In their recent article, Booth et al describe a number of cases of systemic infection with reduced a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13) activity, whose presenting clinical features mimicked thrombotic thrombocytopenic purpura (TTP) [1]. In the past, a small number of cases of leptospirosis have been reported to be associated with TTP [2][3][4].…”