Thrombotic thrombocytopenic purpura (TTP): initial treatment with plasma exchange plus steroids and immunosuppressive agents for relapsing cases.
BACKGROUND
The thrombotic thrombocytopenic purpura (TTP) is a rare disorder with a high mortality rate if untreated or delayed therapy. Whether immediate diagnosis and promptly treated with plasma exchange can change the grave prognosis?


METHODS
Retrospective analysis was performed on clinical characteristics and treatment outcome of 13 patients diagnosed and treated during a 6-year period, from August 1994 to August 2000, in a tertiary care university hospital in Taiwan.


RESULTS
Among the patients, 8 were males and 5 were females, 10 were idiopathic, 2 were ticlopidine, and 1 was SLE-induced; 12 (92.3%) had neurological abnormalities, 11 (84.6%) had ecchymosis, 8 (61.5%) had fever, and 6 (46.2%) had renal impairment (creatinine > or = 1.5 mg/dL) at initial presentation of the syndrome. Excluding the SLE patient, 6 of 10 (60%) had shown antinuclear antibody (ANA) non-specific positive (titer > or = 1:40). All patients were initially treated with plasma exchange plus steroids. Of these 13 patients, 11 (84.6%) achieved complete remission, one had partial remission, and one, which was ticlopidine-induced, had no response and died of a progressive disease complicated with pneumonia. Within a median follow-up period of 31 months, 4 of 11 patients who achieved complete remission relapsed after one week, two weeks, three weeks, and three months, respectively. In the four relapsing patients, three late relapsing patients received FFP infusion, increased steroid dosages, added cyclophosphamide plus vincristine; and one early relapsing patient, relapsing twice, received an additional two courses of plasma exchange and added cyclophosphamide plus vincristine. All of the four patients achieved complete remission again. The patient who had partial remission relapsed early and responded promptly to another course of plasma exchange plus cyclophosphamide and vincristine and achieved complete remission.


CONCLUSIONS
Based on the results in this study, we conclude that plasma exchange plus steroids can effectively treat TTP. For patients with a refractory or relapsing disease, immunosuppressive therapy with cyclophosphamide plus vincristine should be administered as well.
Yang
Chung-Wei
CW
Hsin-Chu Hospital, Department of Health, The Executive Yuan, Taoyuan, Taiwan.
Chen
Yung-Chang
YC
Dunn
Po
P
Chang
Ming-Yang
MY
Fang
Ji-Tseng
JT
Huang
Chiu-Ching
CC
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000743:Anemia, Hemolytic / Q000209:etiology / Q000401:mortality / Q000628:therapy; D001663:Bilirubin / Q000097:blood; D015415:Biomarkers / Q000097:blood; D003131:Combined Modality Therapy; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006454:Hemoglobins / Q000378:metabolism; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008180:Lupus Erythematosus, Systemic / Q000097:blood / Q000150:complications; D008297:Male; D008875:Middle Aged; D010951:Plasma Exchange / Q000009:adverse effects; D010975:Platelet Aggregation Inhibitors / Q000009:adverse effects; D010976:Platelet Count; D011697:Purpura, Thrombotic Thrombocytopenic / Q000209:etiology / Q000401:mortality / Q000628:therapy*; D012008:Recurrence; D017701:Reticulocyte Count; D012189:Retrospective Studies; D012720:Severity of Illness Index; D013256:Steroids / Q000627:therapeutic use; D016019:Survival Analysis; D013624:Taiwan; D013988:Ticlopidine / Q000009:adverse effects; D016896:Treatment Outcome
D015415:Biomarkers; D006454:Hemoglobins; D007166:Immunosuppressive Agents; D010975:Platelet Aggregation Inhibitors; D013256:Steroids; D013988:Ticlopidine; D001663:Bilirubin