Abstract
Background and ObjectivesThrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are characterized by microangiopathic hemolytic anemia and thrombocytopenia. Interestingly, markedly different survival rates have been reported despite increases in survivability. We studied TTP-HUS 30-day mortality and relapse rates of patients who received TPE at our institution and compared them to published data.Patients and MethodsRetrospective study analyzed 30-day mortality and relapse rates attributed to TTP-HUS from 01/01/2008 to 12/31/2012 and compared them to comparable literature reporting mortality and survival. Studies describing other etiologies for TPE and different mortality time interval were excluded.ResultsFifty-nine patients were analyzed and all were initially treated with TPE and corticosteroids. Eleven patients were classified as not having TTP-HUS due to testing or clinical reassessment which ruled in other etiologies, and 18/59 patients had ADAMTS13 activity <10%. Of remaining patients, 36/48 (75%) were diagnosed as idiopathic and 12/48 (25%) as secondary TTP-HUS. Patients received a mean of 12 TPEs (range 1-42); 42/48 (87.5%) patients had ADAMTS13 activity measured; complete response obtained in 39/48 (81.2%) patients (platelet count >100 x 109/L); partial response in 4/48 (8%); and 5/48 (10.4%) did not have increases in platelet counts (2/5 of these patients died within the study period). Forty percent of patients obtained platelet counts >150 x 109/L. Overall 30-day mortality for our patient cohort was 6.7% (4/59). Comparison of our mortality rate to combined data of five published studies of 16% (92/571) showed a significant difference, p = 0.04. Our relapse rate was 18.6% (11/59) similar to previous reports.ConclusionsWide differences in mortality may be due to grouping of two distinct pathologic entities under TTP-HUS; and presence of confounding factors in the patient populations under study such as co-morbidities, promptness of TPE initiation, delay in diagnosis and therapeutic practice.
Highlights
Thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are rare diseases characterized by microangiopathic hemolytic anemia (MAHA) and thrombocytopenia
Eleven patients were classified as not having TTP-HUS due to testing or clinical reassessment which ruled in other etiologies, and 18/59 patients had ADAMTS13 activity
Patients received a mean of 12 therapeutic plasma exchange (TPE); 42/48 (87.5%) patients had ADAMTS13 activity measured; complete response obtained in 39/48 (81.2%) patients; partial response in 4/48 (8%); and 5/48 (10.4%) did not have increases in platelet counts (2/5 of these patients died within the study period)
Summary
Thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are rare diseases characterized by microangiopathic hemolytic anemia (MAHA) and thrombocytopenia. HUS is characterized by three clinical signs: kidney failure, hemolytic anemia, and thrombocytopenia while TTP has been defined by a diagnostic pentad of thrombocytopenia, hemolytic anemia, fever, neurologic changes and renal compromise [1]. Even though ADAMTS13 differences were originally described, not all patients presenting with the clinical diagnosis of TTP have a deficiency in this metalloprotease leading to the suggestion that it lacks importance in the diagnostic workup of these disease entities [6], and as predictor of clinical response to therapy which should be based mostly on clinical criteria [7]. Thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are characterized by microangiopathic hemolytic anemia and thrombocytopenia. We studied TTP-HUS 30-day mortality and relapse rates of patients who received TPE at our institution and compared them to published data
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