Thrombotic thrombocytopenic purpura in southern Turkey: a single-center experience of 29 cases

Abstract

In this retrospective, nonrandomized study, we describe our experience in the management of 29 consecutive patients with thrombotic thrombocytopenic purpura (TTP) treated with a combined therapy of plasma exchange (PE) and steroids at a single center. We compared the effectiveness of high-dose steroids (20-25 mg/kg methyl prednisolone) as first-line treatment in combination with PE therapy with the combination of standard-dose steroids (1 mg/kg methyl prednisolone) and PE in adult patients with TTP. Clinical, laboratory data and treatment outcomes such as response rate, median time to recovery and survival were evaluated retrospectively. Overall (OR) and complete (CR) response rates were 69 and 52% respectively. Similar response rates were found in patients treated with pulse or conventional dose steroids; however, the median time to response was delayed in the high-dose methyl prednisolone (HDP) group. The median time from the initiation of symptoms to initiation of treatment was approximately 15 days (range: 0-30). Delayed treatment in our patients because of delayed referral to our center resulted in poor response to treatment. In all, four of 14 (27%) complete responders experienced relapses. The predicted relapse rate was 48% at a median of 30 months. All the relapses presented with a combination of thrombocytopenia and microangiopathic hemolytic anemia. This analysis showed that high-dose steroid treatment did not prove to be beneficial for TTP patients as firstline therapy combined with PE. Moreover, pulse steroid interventions might have resulted in delayed responses and our data suggest that initiation of treatment with PE should not be delayed.

Renal Thrombotic Microangiopathies Induced by Severe Hypertension

“…Espinosa et al (9) reported that 13% of patients with systemic TMA showed malignant hypertension. On the other hand, the frequency of intrarenal TMA with malignant hypertension has not been well described (10,11).…”

A dramatic response to rituximab in a patient with resistant thrombotic thrombocytopenic purpura (TTP) who developed acute stroke

“…Adult TTP is considered to be an autoimmune disorder often accompanied by focal, reversible neurologic findings, and seizures [1,3]. Autoantibodies against ADAMTS13 work as inhibitors that induce a deficiency protease.…”

“…Immunomodulatory agents (eg, corticosteroids, vincristine, cyclophosphamide, azathioprine, cyclosporine, and high-dose immunoglobulins) have all been used to treat refractory TTP, all with unreliable effectiveness [1][2][3][5][6][7]. Efficacy of Rituximab for TTP patients by eliminating antibody-producing B lymphocytes has been reported in many previous literatures [4][5][6][7][8][9][10][11].…”

“…Thrombotic thrombocytopenic purpura (TTP) is a rare and potentially fatal disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, renal failure, fever, and neurologic changes [1][2][3]. An unusual increase in vWF caused by decreased amounts of ADAMTS13 due to an antibody against ADAMTS13 inhibition is suggested for the occurence of TTP [2][3][4].…”

Thrombotic Thrombocytopenic Purpura and Hemolytic Uremic Syndrome in 2014—Current Knowledge and Outcomes with Plasma Exchange

“…41,42,44,[47][48][49][50][51] Plasma infusion should only serve as a temporizing measure until plasma exchange therapy can be delivered.…”