A Case of Thrombotic Thrombocytopenic Purpura Refractory to Plasma Exchange

Abstract

We experienced a case of thrombotic thrombocytopenic purpura (TTP) finally relieved after 74 sessions of plasma exchange (PE). The patient was a 56‐year‐old male. In August 1999, he was examined in emergency because of brown urine and a lowered level of consciousness. As TTP was suspected according to the laboratory findings of abnormally high lactate dehydrogenase and total bilirubin, decreased platelet counts, and numerous fragmented erythrocytes, he was admitted to the ICU of our hospital. Immediately after admission, PE was started consecutively. Upon concomitant use of antiplatelet drugs and prostacyclin, the level of platelet counts recovered to 100,000/μl once, but decreased again. Thus, in addition to the PE, prednisolone and vincristine were administrated, which elevated the level of platelet counts to 200,000 to 300,000/μl. Since the erythrocyte fragmentation was noted frequently, PE was continued twice a week. From the 60th day of admission onward, however, his body temperature rose above 40°C with a rapid increase of C‐reactive protein. A blood culture detected methicillin‐resistant Staphylococcus aureus (MRSA) which derived from a left lung abscess. During the course of anti‐MRSA treatment, he presented acute renal failure and acute hepatic dysfunction, but survived because of the combined therapy. He was discharged on the 180th day of admission. These results suggest that a combined therapy of steroid and vincristine is effective to treat TTP refractory to PE, but careful attention should be paid to the complications caused by immunosuppression.

Effect of Plasma Exchange on Hepatocyte Oxidative Stress, Mitochondria Function, and Apoptosis in Patients With Acute Fatty Liver of Pregnancy

“…Most importantly, PE can save time for liver recovery (50,51). PE has been found to decrease bilirubin levels by 40% (bile acid 25%, endotoxin 30%), greatly improving the survival rate of patients with acute liver failure (52,53). The temporizing effect of PE occurs via the removal of circulating endotoxins, replacement of normal coagulation factors and proteins, interruption of coagulopathy, and finally, improvement of hepatic functions.…”

Infection as a cause of early relapse in patients recovering from thrombotic thrombocytopenic purpura

“…Some of the organisms implicated in previous reports include S. aureus and Acinectobacter anitratus [12,15,17]. Creager et al reported two cases in which it was presumed that bacterial infection was responsible for refractoriness of TTP to treatment [18].…”

Vincristine as an Adjunct to Therapeutic Plasma Exchange for Thrombotic Thrombocytopenic Purpura: A Single Institution Experience

“…Vincristine is one of the several adjuncts to TPE. Data on vincristine use in relapsed/refractory TTP are limited to a few non-randomized studies including mostly case reports and small series ( 14 , 20 ). Some evidence regarding vincristine use comes from experiences with Jehovah’s Witnesses, who refuse to be treated with allogeneic blood products due to religious concerns ( 21 , 22 , 23 ).…”

“…Various dosing schedules are applied for vincristine in the treatment of TTP, both in relapsed/refractory patients and in the front-line setting. Standardized regimens include TPE, corticosteroids and vincristine at a dose of 1.4 mg/m 2 on days 1, 4, 7, and 10 in the refractory setting ( 4 ) or 1.5 mg/week for a total of 4 times ( 14 ) or vincristine 1-2 mg up to 2 mg total dose within 24 hours 3 days after the first TPE, followed by 1 mg administered after 1 week as suggested by Mazzei et al ( 24 ). Ziman et al ( 25 ) treated patients at presentation with vincristine 1.4 mg/m 2 (up to 2 mg total dose) after the first TPE.…”

“…Side effects include sensory neuropathy, muscle weakness, paralytic ileus, leukopenia, and transient alopecia ( 1 ). Vincristine has been used in relapsed or refractory TTP, and many studies support a potential role for vincristine in the treatment of TTP ( 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 ).…”