Clinicopathologic and prognostic features of chronic idiopathic thrombocytopenic purpura in adult Chinese patients: an analysis of 220 cases

Abstract

To determine the clinicopathologic and prognostic features of chronic idiopathic thrombocytopenic purpura (ITP) in adult Chinese patients, we conducted a retrospective analysis of 220 patients seen at a single center over a 40-year period. The female-to-male ratio was 4:1, with a mean age of 42.1 +/- 1.3 years, a mean platelet count of 33.7 +/- 2.3x10(9)/l, and a mean follow-up of 116 +/- 7 months. Initial steroid treatment was required in 142 patients, 67 of whom (47.2%) achieved complete remission (CR). At 470 months, 46% patients remained in CR. Splenectomy was performed in 37 patients: in 23 patients due to primary steroid refractoriness and in 7 patients due to disease relapse following initial CR with steroids. In seven patients, data on response to steroids prior to splenectomy were not available. Splenectomy for steroid nonresponders resulted in an inferior CR rate (13 of 23, 56%) as compared with that for relapses after steroid treatment (7 of 7, 100%) (P<0.05). Compared with patients with negative antinuclear antibody (ANA), those who were ANA positive had similar responses to steroids, but significantly shorter remission after splenectomy (P<0.01). In conclusion, Chinese patients with ITP could maintain long-term remission after steroid therapy and splenectomy. In addition, primary steroid refractoriness and positive ANA were bad prognostic factors of the subsequent response to splenectomy.

Prognostic factors for positive immune thrombocytopenic purpura outcome after laparoscopic splenectomy

“…Different guidelines indicate steroids (prednisolone or dexamethasone) as the first step treatment for ITP patients. Despite their high initial response rate (50-90%), curative response is achieved only in 10-30% of patients [9][10][11][12][13]. Three studies analyzed the response rate of high-dose dexamethasone (30 -40 mg/day orally for four days per month) as the first-step treatment.…”

“…On the other hand, the most of patients relapse after stopping the steroid usage. Osteoporosis, hypercorticism, diabetes mellitus, infections are side effects due to prolonged steroid use [9][10][11][12][13]. Intravenous (IV) immunoglobulin (IG) can be used in combination with steroids or as monotherapy for ITP treatment.…”

Resolution of chronic idiopathic thrombocytopenia purpura following syngeneic peripheral blood progenitor transplant

“…Major review articles do not include bone marrow or PBPT as a treatment choice in ITP. [1][2][3][4] Successful allogeneic BMT has been reported in cases of autoimmune diseases including immune hemolytic anemia, rheumatoid arthritis, psoriasis, SLE, ulcerative colitis and autoimmune hepatitis. 7 There has been a case report of successful resolution of immune thrombocytopenia and anemia in a patient with Evans syndrome who received an allogeneic cord blood transplant.…”

“…The clinical syndromes of ITP are distinct between children and adults: Childhood ITP characteristically is acute in onset and resolves spontaneously in most cases within 6 months, 1 whereas adult ITP typically has an insidious onset and rarely resolves spontaneously. [2][3][4][5] The incidence of ITP in children is estimated to be approximately 46 new cases per million per year. Patients who fail to maintain a normal platelet count after conventional therapies are labeled as chronic refractory ITP patients.…”

“…Treatment modalities include glucocorticoids, splenectomy, intravenous immunoglobulin (IVIG), anti-Rh (D) immune globulin, azathioprine, cyclophosphamide, vinca alkaloids, danazol, vitamin C, combination chemotherapy, cyclosporine, dapsone and interferon-␣-2b. [1][2][3][4][5] Recent information suggests that the anti-CD20 antibody, rituximab may be beneficial. 6 To date, limited information exists on the use of hematopoietic progenitor cell transplantation as a treatment for this disease.…”