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Durable remissions following prolonged plasma exchange in thrombotic thrombocytopenic purpura
Author(s) -
Dawson R. Ben,
Brown James A.,
Mahalati Kathy,
Sapsiri Suchada,
Pearlman Steve,
Gulden Diane,
Bilenki Lois,
Wenk Robert E.
Publication year - 1994
Publication title -
journal of clinical apheresis
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.697
H-Index - 46
eISSN - 1098-1101
pISSN - 0733-2459
DOI - 10.1002/jca.2920090203
Subject(s) - medicine , thrombotic thrombocytopenic purpura , splenectomy , vincristine , gastroenterology , surgery , platelet , plasmapheresis , spontaneous remission , chemotherapy , cyclophosphamide , immunology , spleen , antibody , alternative medicine , pathology
We evaluated the efficacy of prolonged plasma exchange (PEX) for attaining durable remissions in thrombotic thrombocytopenic purpura (TTP). A recent review using steroids or PEX in initial management showed an 80% response rate but produced a relapse rate of 67‐84%. Records of 50 patients starting PEX treatment for TTP/HUS were reviewed to identify and select those whose course of treatment had ended over 1 year earlier, whether or not the result was satisfactory. Records were evaluated for outcome, especially remission associated with treatment by “prolonged” plasma exchange. “Prolonged” was defined as continuing PEX beyond the stage where a normal platelet count was attained and until evidence of hemolysis was “minimal or at least compensated.” If disease activity as judged by the criteria of hemolysis became accelerated or resumed, PEX was increased by volume of FFP (e.g., from 3 to 4 L) or rate (from less than daily to daily). Of 50 consecutive patients treated by PEX for TTP/HUS there were 40 cases after which at least one year had passed since the end of treatment. These 40 patients were evaluated for the results of treatment by PEX. Eight failed to achieve remission, dying in hospital within 1 month of admission. Twenty‐eight achieved remission, sustained for 1 year or more in all. These are the reasons for our enthusiasm about this report. Four achieved remission lasting less than 1 year. Splenectomy was performed to obtain a sustained remission in one patient following administration of three 2 mg doses of vincristine and two relapses. The second was discharged with an adequate platelet count, but had ongoing hemolytic anemia and had to be retreated as an early relapse. The third, a mitomycin‐induced TTP, had an early relapse which responded to additional PEX. The fourth was a late relapse. Our response rate of 80% is not higher than that of recent reviews. However, the relapse rate of 9% with prolonged plasma exchange is three times better than the average relapse rate of 27% from the 20 previous reports in the literature over the same period (Onundarson et al., Arch Int Med 152:761–766, 1992). We attribute this benefit to a strategy of continuing to perform plasma exchange on a tapering, alternating day schedule until disease activity is minimal as defined by criteria of hemolysis. Since relapses are often as devastating as the original episode, the treatment plan described is recommended.