Adult idiopathic thrombocytopaenic purpura: therapy strategy. Analysis of 30 cases (1991-1995) with a brief review of the literature
Keywords:
adult ediopathic thrombocytoprenic purpura, therapy, responses, evolutionAbstract
Background: We analysed epidemiological data,therapies, clinical efficacy and evolution of patientes with adult idiopathic thrombocytopaenic purpura (ITP).
Patients and methods: A retrospective study of patients with an established diagnosis of ITP, referred to our lnternal Medical Department between 1991 and 1995, was carried out.
Dejinitlons of therapy efficacy and evolution are as follows.· complete remisson platelets > 150000/mm3; continuous complete remission: platelets >150000/mm3 for at least 2 months without therapy partial remission: platelets >50000< 150000/mm3; without therapy response<50000/mm3.
Results: Between 1991 and 1995, 30 patients were admit ted with IPT ( 23female; 7male; median ages: 49,5 years). Corticosteroid therapy was given to 30 patients, 9 achived complete remission; 4 of those were in continuous complete remission; 15 patients had partial remission and 6 failed to achieve a response. 14 patients received gammaglobins intra-venously; 1 achived complete remission; 9 had partial remission and 6 had no response. Only 4 patients were splenectomized; 2 achived complete remission; other were lost for to follow-up.
Four patients were treated with azothioprine; 3 achived partial remission and 1 patient had no response. Danazol was used in 2 patients with no beneftcial effect.. Median follow-up for 24 months revealed: 13 patients had multiple relapses, 4 achieved continuous complete remission and 1 died; 12 patients were lost to follow-up.
Conclusions: Corticosteroid therapy was the most effective therapy in ITP, with 30% of pattents achieving complete remisston in our study.
ln spite of the many different treatment regimes available, 43% of patients had a chronic disease evolution, in accord with the international ITP disease statistics.
Downloads
References
George JN, El-Harake M, Aster RH. Thrombocytopenic dueto enhanced platelet destruction by immunologic mechanis ms. ln: Beutler E, Lichtman MA, Coller BS, Kipps TJ. Williams Hematology 5th ed. New York. Me Graw-Hill 1995: 1315- 1355.
Defino SM, Lacchant NA, Kirshner JJ, et al. Adult Idopathic Thrombocytopenic Purpura. Clinical Findings and Response to Therapy. Am J Med 1980; 69: 430-442.
Bithell TC. Thrombocytopenia caused by immunologic platelet destruction: Idiopathic Thromboc.ytopenic Purpura(ITP), drug-inducecl thromhocytopenia, and miscelaneous. ln Lee GR, Bithell TC, Foerster J, Ahens JW, Lukens JN. Wintrobe'S Clinicai Hematology 9th ed. Philadelphia: Lea and Febiger 1993: 1329-1355.
GeorgeJN, El Harake M, Raskoh GE. Chronic Idiopathic Thrombocytopenic Purpura. N Engl J Med 1994; 331: 1207-1211.
Cartilazzo S, Finazzi G, Buelli M, Maltini A, Viero P, Barhin T. High risk of severe bleeding in aged patients with chronic idiopathic thrombocytopenic purpura. Blood 1991; 77:31-33.
Fujisawa K, Tani P, O' Toole TE, Ginsberg MH, Me Millan R. Different specificities of platelet associated and plasma autoantibodies to platelet GPIIh-IIa in patients with Chronic Immune Thrombocytopenic Purpura. Blood 1992; 79: 1441-1446.
Kitchins CS, Pendergast JF. Human thrombocytopenia associa ted with strutural abnormalities of the endothelium that are ameliorated by glucocorticosteroid administration. Blood 1986; 67:203-206.
Gernsheimer T, Stratton J, Bailem PJ, Slichter SJ. N Engl JMed 1989; 320: 974-980.
Fujisawa K, Tani P, Pino L, McMillan R. The effect of therapy on platelet- associated autoantibody ln Chronic Immune Thromhocytopenic Purpura. Blood 1993; 81: 2872-2877.
Lefor AT, Melvin WS, Bailey RW, Flowers JL. La poroscopicsplenectomy in the management of lmmune Thrombocytopenia Purpura. Surgery 1993;114: 613-618.
McMillan R. Therapy for adults with Refractory lmmune Thrombocytopenia Purpura. Ann Intern Med 1997; 126: 307-314.
Anclerson JC. Response of resistant Idiopathic Thrombocytopenia Purpura to pulse high-dose dexamethasone therapy. N Engl J Med 1994; 330:1560-1564.
Caulier MT, Rose C, Roussel MT, Huart C, Bauters F, Fenaux P. Pulsecl high-dose dexamethasone in refractory chronic idiopathic thrombocytopenic purpura: a report on 10 cases. Br J Haematol 1995; 91: 447-449.
Ahn YS, Harrington WJ, Mylvaganam R, Alleu LM, Pall LM. Slow infusion of vinca alkaloida in the treatment of Idiopathic Thrombocytopenic Purpura. Ann lntern Med 1984;100:192-196
Ahn YS, Harrington W:J, Simon SR, Mylvaganam R, Pall LM, So AG. Danazol for the treatment of Idiopathic Thromhocytopenic Purpura. N Engl J Med 1983; 308: 1396-1399
Schreiher AD, Chien P, Tomaski A, Ciner DB. Effect of danazol in Immune Thromhocytopenic Purpura. N Engl J Med 1987; 316: 503-508.
Strother SV, Zukerman KS, Lo Buglio AF. Colchicine therapy for Refractory Idiopathic Thromhoytopenic Purpura. Arch Intern Med 1984; 144; 2198-2200.
Hernandez F, Linares M, Colomina P, Pastor E, Cervero A, Perez A et al. Dapsone for Refractory Idiopathic Thromhocytopenic Purpura. Br J Haematol 1995; 90: 473-475.
Quiguandon I, Fenaux P, Caulier MT, Pagniez D. Re-evaluation of the role of azathioprine in the treatment of adult of chronic Idiopathic Thromhocytopenic Purpura a report on 53 cases. Br J Haematol 1990; 74:223-228.
Laros RKJ, Penner JA. "Refractory" Thromhocytopenic Purpu ra treated successfully with cyclophosphamide. JAMA 1971; 215: 445-449.
Snyder HW, Cochran S, Balint JP, Bertrnm JH el al. Experience with protein A- immunoabsortion in treatment- resistant adult Immune Trombocytopenic Purpura. Blood 1992; 79: 2237-2245.
Reiner A, Gernsheimer T, Slichter SJ. Pulse cyclophosphamide therapy for refractory autoimmune Tromhocytopenic Purpura. Blood 1995; 85: 351-358.
Figueroa M, GehlsenJ, Hammond D et al. Combination chemeotherapy in refractory Immune Tromhocytopenic Purpura. N EnglJ Med 1993; 328: 1226-1229.
Protor SJ, Jackson G, Carey P, Starka et al. Improvement of platelet counts in steroide-unresponsive Idiopathic Immune Tromhocytopenic Purpura after short course therapy with recombinant a 2h interferon. Blood 1989; 74: 1894-1897.
Kelsey PR, Schofield KP, Geary CG. Refractory Idiopathic Trombocytopenic Purpura (ITP) treated with cyclosporine. Br J Haematol 1985; 60: 197-198.
Bussel JB, Graziano JN, Kimberly RP, Pahwa S, Aledort AI. Intravenous anti- D treatment of Immune Tromhocytopenic Purpura: analysis of efficacy, toxicity and mechanism of effect. Blood 1991; 77: 1884-1893.
Fehr J, Hofmann V, Kappeler U. Transient reversal of thrombocytopenic in lmmune Tromhocytopenic Purpura by high dose intravenous gammaglobulin. N Engl J Med 1982; 306: 1254-1258.
BusselJB, Pham LC, Aledort L, Nachman R. Maintenance treatment of adults with chronic refrectory Immune Trombocytopenic Purpur-J using repeated intravenous infusions of gammaglobulin. Blood 1988; 72: 121-127.
Bussel JB, Kimberly RP, Imman RD, et al. Intravenous gammaglobulin treatment of chronic Idiopathic Trombocytopenic Purpurn. Blood 1983; 62: 480-486.
Baumann MA, Menitove JE, Aster RH, Anderson T. Urgent treatment of idiopathic Tromhocytopenic Purpura with single dose gammaglobulin infusion followed by platelet transfusion. Ann Int Med 1986; 104: 808-809.
Additional Files
Published
How to Cite
Issue
Section
License
This work is licensed under a Creative Commons Attribution 4.0 International License.
Copyright (c) 2023 Medicina Interna