[Thrombotic thrombocytopenic purpura: report of seven cases].
From May 1999 to January 2002 we observed 7 patients (4 females and 3 males, median age 55 years, range 31-81 years) with thrombotic thrombocytopenic purpura (TTP). Six patients has been previously undiagnosed and 1 patient was at second relapse. Trigger factors of TTP were identified in 6 patients: ticlopidine treatment (2 patients); an acute cutaneous infection episode immediately before the features of TTP (1 patient); presence of devices: orthodontic (1 patient) and intrauterine contraceptive (1 patient), Mycoplasma urealyticum vaginal infection (1 patient). In all the 7 patients the clinical status was mainly related to the hemolytic anemia, thrombocytopenia and neurological events. One of these patients presented with hemolytic-uremic syndrome with acute renal failure and macrohematuria at onset, another one showed a systemic exanthema post-infection-like. Six out of 7 patients presented with different neurological events: headache, confusion, focal neurological failure. All the 7 patients were promptly treated with plasma-exchange and cryosupernatant plasma infusion. In addition they received prednisone 25-50 mg/day. All the 7 patients achieved a complete remission after plasma-exchange, one relapsed 3 months later and was treated with plasma-exchange again. All the patients are in complete remission with a median follow-up of 36.3 months (range 20-62 months). From these cases we suggest: 1) clinicians should take in mind the suspicion of TTP in every patient with hemolytic, negative direct Coombs test, anemia, thrombocytopenia, high level of lactate dehydrogenase; 2) the treatment of choice is plasma-exchange; 3) the response of treatment is good if therapy is promptly and aggressively administered; 4) the possible role of a trigger factor for removing it and to prevent relapses.
Anselmi
Elisa
E
Divisione di Medicina Oncologica ed Ematologia, Ospedale "Guglielmo da Saliceto" di Piacenza.
Arcari
Annalisa
A
Bernuzzi
Patrizia
P
Civardi
Giuseppe
G
Moroni
Carlo Filippo
CF
Vallisa
Daniele
D
Bertè
Raffaella
R
Lazzaro
Antonio
A
Cavanna
Luigi
L
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010951:Plasma Exchange / Q000379:methods; D011241:Prednisone / Q000627:therapeutic use; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology / Q000628:therapy*; D012189:Retrospective Studies; D016896:Treatment Outcome
D000893:Anti-Inflammatory Agents; D011241:Prednisone