[Adult Autoimmune Thrombocytopenia: Diagnosis and Treatment]

Overview

Journal Wien Klin Wochenschr

Publisher Springer

Specialty General Medicine

Date 2006 Jul 1

PMID 16810482

Citations 1

Authors

Klaus Lechner

Ansgar Weltermann

Ingrid Pabinger

Affiliations

Soon will be listed here.

Abstract

The incidence of AITP is 20-30/million/year. The diagnosis is based on the finding of an isolated thrombocytopenia without other blood abnormalities and absence of a palpable spleen. Additional tests such as bone marrow examination, determination of platelet antibodies and of thrombopoetin are required only in special cases. The usual first line therapy in patients with bleeding tendency and a low platelet count is prednisolone at a dose of 1 mg/kg/day. Patients who have platelet counts of less than 20,000/microl 3-6 months after steroid therapy are candidates for splenectomy, in particular if more than 0.1 mg/kg/day prednisolone is required to keep the patient free of bleedings. Laparoscopic splenectomy has a low mortality (0.2%) and morbidity (10%). The risk of post-splenectomy overwhelming pneumococcal septicaemia can be minimized by preoperative vaccination. Older patients, who have low platelet counts after splenectomy, have a high bleeding risk. The most effective treatment options for these patients are cyclophosphamide, azathioprine and rituximab, but the choice of treatment should be carefully considered, since the risk of adverse effects may be greater than the risk of fatal bleeding.

Citing Articles

Diagnosis and treatment of autoimmune haemolytic anaemias in adults: a clinical review.

Valent P, Lechner K Wien Klin Wochenschr. 2008; 120(5-6):136-51.

PMID: 18365153 DOI: 10.1007/s00508-008-0945-1.

References

Feuillet L, Guedj E, Laksiri N, Philip E, Habib G, Pelletier J . Deep vein thrombosis after intravenous immunoglobulins associated with methylprednisolone. Thromb Haemost. 2004; 92(3):662-5. View

Linares M, Cervero A, Colomina P, Pastor E, Lopez A, Perez A . Chronic idiopathic thrombocytopenic purpura in the elderly. Acta Haematol. 1995; 93(2-4):80-2. DOI: 10.1159/000204116. View

Davoren A, Bussel J, Curtis B, Moghaddam M, Aster R, McFarland J . Prospective evaluation of a new platelet glycoprotein (GP)-specific assay (PakAuto) in the diagnosis of autoimmune thrombocytopenia (AITP). Am J Hematol. 2005; 78(3):193-7. DOI: 10.1002/ajh.20309. View

Godeau B, Durand J, Roudot-Thoraval F, Tenneze A, Oksenhendler E, Kaplanski G . Dapsone for chronic autoimmune thrombocytopenic purpura: a report of 66 cases. Br J Haematol. 1997; 97(2):336-9. DOI: 10.1046/j.1365-2141.1997.412687.x. View

Bierling P, Godeau B . Intravenous immunoglobulin and autoimmune thrombocytopenic purpura: 22 years on. Vox Sang. 2004; 86(1):8-14. DOI: 10.1111/j.0042-9007.2004.00391.x. View

Sakane Y, Wada H, Kazuyo H, Shimura M, Nakasaki T, Katayama N . Increased thrombopoietin levels in idiopathic thrombocytopenic purpura patients with a poor response to steroid therapy. Blood Coagul Fibrinolysis. 1998; 9(4):315-21. DOI: 10.1097/00001721-199806000-00003. View

Saag K, Emkey R, Schnitzer T, Brown J, Hawkins F, Goemaere S . Alendronate for the prevention and treatment of glucocorticoid-induced osteoporosis. Glucocorticoid-Induced Osteoporosis Intervention Study Group. N Engl J Med. 1998; 339(5):292-9. DOI: 10.1056/NEJM199807303390502. View

Zhang W, Ji L, Cao X, Chen Y, He A, Liu J . Mycophenolate mofetil as a treatment for refractory idiopathic thrombocytopenic purpura. Acta Pharmacol Sin. 2005; 26(5):598-602. DOI: 10.1111/j.1745-7254.2005.00088.x. View

Sato R, Murakami K, Watanabe K, Okimoto T, Miyajima H, Ogata M . Effect of Helicobacter pylori eradication on platelet recovery in patients with chronic idiopathic thrombocytopenic purpura. Arch Intern Med. 2004; 164(17):1904-7. DOI: 10.1001/archinte.164.17.1904. View

10.

Cooper N, Stasi R, Cunningham-Rundles S, Feuerstein M, Leonard J, Amadori S . The efficacy and safety of B-cell depletion with anti-CD20 monoclonal antibody in adults with chronic immune thrombocytopenic purpura. Br J Haematol. 2004; 125(2):232-9. DOI: 10.1111/j.1365-2141.2004.04889.x. View

11.

Sailer T, Weltermann A, Zoghlami C, Kyrle P, Lechner K, Pabinger I . Mortality in severe, non aggressively treated adult autoimmune thrombocytopenia. Hematol J. 2003; 4(5):366-9. DOI: 10.1038/sj.thj.6200299. View

12.

Portielje J, Westendorp R, Kluin-Nelemans H, Brand A . Morbidity and mortality in adults with idiopathic thrombocytopenic purpura. Blood. 2001; 97(9):2549-54. DOI: 10.1182/blood.v97.9.2549. View

13.

Kotb R, Pinganaud C, Trichet C, Lambotte O, Dreyfus M, Delfraissy J . Efficacy of mycophenolate mofetil in adult refractory auto-immune cytopenias: a single center preliminary study. Eur J Haematol. 2005; 75(1):60-4. DOI: 10.1111/j.1600-0609.2005.00437.x. View

14.

Schrezenmeier H, Muller H, Gunsilius E, Heimpel H, Seifried E . Anticoagulant-induced pseudothrombocytopenia and pseudoleucocytosis. Thromb Haemost. 1995; 73(3):506-13. View

15.

Vianelli N, Valdre L, Fiacchini M, De Vivo A, Gugliotta L, Catani L . Long-term follow-up of idiopathic thrombocytopenic purpura in 310 patients. Haematologica. 2001; 86(5):504-9. View

16.

Michel M, Chanet V, Galicier L, Ruivard M, Levy Y, Hermine O . Autoimmune thrombocytopenic purpura and common variable immunodeficiency: analysis of 21 cases and review of the literature. Medicine (Baltimore). 2004; 83(4):254-263. DOI: 10.1097/01.md.0000133624.65946.40. View

17.

Pietrabissa A, Moretto C, Antonelli G, Morelli L, Marciano E, Mosca F . Thrombosis in the portal venous system after elective laparoscopic splenectomy. Surg Endosc. 2004; 18(7):1140-3. DOI: 10.1007/s00464-003-9284-5. View

18.

Frederiksen H, Schmidt K . The incidence of idiopathic thrombocytopenic purpura in adults increases with age. Blood. 1999; 94(3):909-13. View

19.

Li J, Yang C, Xia Y, Bertino A, Glaspy J, Roberts M . Thrombocytopenia caused by the development of antibodies to thrombopoietin. Blood. 2001; 98(12):3241-8. DOI: 10.1182/blood.v98.12.3241. View

20.

Damodar S, Viswabandya A, George B, Mathews V, Chandy M, Srivastava A . Dapsone for chronic idiopathic thrombocytopenic purpura in children and adults--a report on 90 patients. Eur J Haematol. 2005; 75(4):328-31. DOI: 10.1111/j.1600-0609.2005.00545.x. View