title: Thrombotic thrombocytopenic purpura in a case of scleroderma renal crisis treated with twice-daily therapeutic plasma exchange.
abstract: We report the rare complication of thrombotic thrombocytopenic purpura (TTP) in an elderly patient with the uncommon scleroderma renal crisis (SRC) at the advanced age of 85 years. Initially, she was treated by standard daily one and a half plasma volume therapeutic plasma exchange (TPE), in combination with steroid therapy. Due to an unsatisfactory clinical response, she was consequently, treated by an intensive twice-daily one and a half plasma volume TPE regimen. Although there was an increase in the platelet count, a 7-day course of the intensive regimen did not elicit further propitious laboratory test results or a clinical improvement and the patient expired secondary to the pulmonary and cardiac conditions related to SRC. Intensive TPE administration has been advocated in few specific patients who are refractory to standard daily treatment. The unfavorable outcome in this case may be attributed to the multiple complications associated with her primary disorder. In conclusion, we observed intensive twice-daily TPE to increase temporarily the platelet count, but not to be of benefit in a patient with SRC and multiple organ failure. The two TPE regimens and outcomes are compared and the pathogenesis of TTP and scleroderma are discussed.
authors.affiliation: Department of Pathology and Laboratory Medicine, American University of Beirut Medical Center, Lebanon. ek01@aub.edu.lb
authors.forename: E M
authors.initials: EM
authors.lastname: Kfoury Baz
authors.forename: R A
authors.initials: RA
authors.lastname: Mahfouz
authors.forename: A F
authors.initials: AF
authors.lastname: Masri
authors.forename: G W
authors.initials: GW
authors.lastname: Jamaleddine
mesh_terms: D058186:Acute Kidney Injury / Q000209:etiology* / Q000628:therapy*; D000368:Aged; D000369:Aged, 80 and over; D004638:Emergency Treatment / Q000379:methods; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D010951:Plasma Exchange / Q000379:methods*; D011697:Purpura, Thrombotic Thrombocytopenic / Q000209:etiology* / Q000628:therapy*; D018570:Risk Assessment; D012595:Scleroderma, Systemic / Q000150:complications* / Q000628:therapy; D016896:Treatment Outcome