Diagnostic and treatment guidelines for thrombotic thrombocytopenic purpura (TTP) 2017 in Japan

Abstract

Thrombotic thrombocytopenic purpura (TTP) can rapidly progress into a life-threatening condition, thus the importance of appropriate diagnosis and treatment cannot be overstated. Until recently, TTP has mainly been diagnosed by clinical findings such as thrombocytopenia and non-immune hemolytic anemia. In addition to these clinical findings, however, reduced activity of a disintegrin-like and metalloprotease with thrombospondin type 1 motif 13 (ADAMTS13) below 10% has been accepted internationally as a diagnostic criterion for TTP. In the present guidelines, we have taken all of these criteria into consideration. TTP is classified as acquired if the patient is positive for anti-ADAMTS13 autoantibodies, and as congenital if ADAMTS13 gene abnormalities are detected. Fresh-frozen plasma (FFP) transfusion is performed in patients with congenital TTP to supplement ADAMTS13. Plasma exchange therapy using FFP is conducted in patients with acquired TTP to supplement ADAMTS13 and remove anti-ADAMTS13 autoantibodies. To suppress autoantibody production, corticosteroid therapy may be administered in conjunction with plasma exchange. Recent reports show that the monoclonal anti-CD-20 antibody rituximab is effective in patients with refractory or relapsed TTP.

ADAMTS13 kinetics after therapeutic plasma exchange and plasma infusion in patients with Upshaw‐Schulman syndrome

“…Nevertheless, the previously described distinct disease course in homozygous c.4143_4144dupA patients is consistent with our practice . In general, plasma infusion (10‐15 mL kg −1 ) is recommended as maintenance prophylactic therapy for USS patients . However, based on our own experiences in patients with more severe disease courses, we prefer TPE.…”

Pregnancy Management and Outcome in ADAMTS13 Deficiency: A Case Report

“…Both of the diseases share similar clinical and laboratory parameter and it is difficult to differentiate. Both associated with thrombocytopenia and microangiopathic changes on peripheral blood smear [8]. Differentiating HELLP syndrome with TTP is occasionally possible when abnormalities persist after delivery [9].…”

A 22‐Year‐Old Woman With Systemic Lupus Erythematosus Presents With Two Damaged Kidneys, One Seizure, No Platelets, and Many Possible Diagnoses

“…The latter has been described in SLE . The diagnosis of TTP is confirmed when the ADAMTS‐13 functional assay is severely decreased to less than 5‐10% of the normal activity with or without detection of an ADAMTS‐13 inhibitor . Interestingly, systemic diseases, including SLE, can moderately lower ADAMTS‐13 activity without causing pathologic TTP .…”

“…TTP classically manifests with a pentad of fever, encephalopathy, MAHA, thrombocytopenia, and acute renal failure. Its pathogenesis is linked to a deficiency of ADAMTS‐13, an enzyme that cleaves von Willebrand factor multimers . The etiology of TTP can be genetic through functional or quantitative deficiencies in ADAMTS‐13 or acquired via formation of anti–ADAMTS‐13 antibodies.…”