       Document 0012
 DOCN  M9580012
 TI    Long-term observation of 208 adults with chronic idiopathic
       thrombocytopenic purpura.
 DT    9506
 AU    Stasi R; Stipa E; Masi M; Cecconi M; Scimo MT; Oliva F; Sciarra A;
       Perrotti AP; Adomo G; Amadori S; et al; Department of Hematology,
       University of Rome Tor Vergata, S.; Eugenio Hospital, Italy.
 SO    Am J Med. 1995 May;98(5):436-42. Unique Identifier : AIDSLINE
       MED/95250795
 AB    PURPOSE: To define response to therapy and ultimate outcome of adults
       with idiopathic thrombocytopenic purpura (ITP). PATIENTS AND METHODS: We
       retrospectively analyzed patients with ITP diagnosed between 1978 and
       1988, and reexamined them between June 1992 and March 1993. Data from
       208 cases were collected. Median patient age was 44 years (range 14 to
       78) at the time of diagnosis, and 51 years (range 19 to 86) at
       reexamination. Length of follow-up ranged from 48 to 151 months (median
       92) and was longer than 10 years in 26 patients (12.5%). Reexamination
       included a careful interview, physical examination, complete blood
       count, screening for HIV infection, determination of platelet-bound IgG,
       and, in persistently thrombocytopenic patients, autoimmunity markers and
       routine laboratory investigations. RESULTS: A total of 121 patients with
       fewer than 50 x 10(9) platelets per liter received an initial treatment
       with prednisone (PDN) at a dosage of 1 mg/kg of body weight for 1 month.
       Refractory or relapsed cases underwent splenectomy and/or other
       therapeutic modalities. In 87 patients with greater than 50 x 10(9)
       platelets per liter, no therapy was scheduled. An initial complete
       response to PDN was observed in 38.8% cases. A sustained complete
       remission (CR) lasting more than 6 months with no maintenance therapy
       was attained in 18.7%. At the time of last follow-up only 11 of these
       patients remained in CR. Sixty-three patients underwent splenectomy.
       Forty-seven (74.6%) had a CR, with 41 achieving a prolonged recovery (>
       6 months). Twelve other cases attained a sustained partial remission.
       Long-lasting recoveries were observed in 7 other cases following
       alternative treatments. Spontaneous remissions occurred in 8 of 87
       untreated cases after observation periods of 6 months or more. Eleven
       deaths were recorded (6 women and 5 men, median age 73), but only 5 were
       attributable to thrombocytopenia. At last control, 43 patients were in
       complete remission and free from therapy, and 52 were still on therapy.
       Four thrombocytopenic patients had laboratory features and a clinical
       history consistent with an autoimmune disease. CONCLUSIONS: This
       analysis of ITP in adults suggests that splenectomy remains the most
       effective treatment. The majority of patients who undergo splenectomy
       can have a CR for many years, while only a minority of those who do not
       have this therapeutic modality or fail it are likely to attain similar
       results. The long-term prognosis of ITP is benign even in refractory
       cases. Spontaneous remissions can be observed in a significant
       percentage of untreated patients (about 9%). The development of overt
       autoimmune diseases is relatively uncommon. Particular attention should
       be given to the management of ITP in the elderly, where bleeding
       episodes of the central nervous system tend to occur more frequently.
 DE    Adolescence  Adult  Age Distribution  Aged  Chronic Disease
       Disease-Free Survival  Female  Follow-Up Studies  Human  Male  Middle
       Age  Platelet Count  Prednisone/THERAPEUTIC USE  Prognosis  Purpura,
       Thrombocytopenic, Idiopathic/*BLOOD/*THERAPY  Retrospective Studies  Sex
       Distribution  Splenectomy  Support, Non-U.S. Gov't  Treatment Outcome
       CLINICAL TRIAL  JOURNAL ARTICLE

       SOURCE: National Library of Medicine.  NOTICE: This material may be
       protected by Copyright Law (Title 17, U.S.Code).

