Background Chronic idiopathic thrombocytopenic purpura is adestructive thrombocytopenia caused by an autoantibody. About80 percent of patients with chronic idiopathic thrombocytopenicpurpura have remissions after either corticosteroid therapyor splenectomy. Some patients with resistant disease respondto other agents, but a substantial group are refractory to therapy.
Methods We used combination chemotherapy to treat 10 patientswith refractory immune thrombocytopenia. An average of 6.8 (range,3 to 10) previous therapies, including corticosteroids and splenectomy,had been unsuccessful in these patients. The patients receivedfrom three to eight cycles of therapy consisting of cyclophosphamideand prednisone combined with either vincristine (one patient),vincristine and procarbazine (four patients), or etoposide (sixpatients, including one patient who received four cycles eachcontaining procarbazine and etoposide).
Results Among the 10 patients, 6 had complete responses (plateletcount, >180,000 per cubic millimeter); of these, 4 patientshad responses that persisted for more than 11, 30, 54, or 126months, 1 had a relapse 9 months after therapy but had a remissionwith further therapy and remained in remission for 48 monthsbefore dying of an unrelated illness, and another relapsed justbefore her fifth course of therapy. Two patients had partialresponses (platelet count, >50,000 per cubic millimeter);the platelet counts in one remained stable for more than ninemonths after the end of therapy, and the other patient relapsed.The remaining two patients had no response. Complete responseswere associated with a disappearance or marked decrease in thelevel of platelet-associated autoantibody.
Conclusions Combination chemotherapy is beneficial in some patientsin whom immune thrombocytopenia is refractory to corticosteroidsand splenectomy.
Source Information
From the Department of Molecular and Experimental Medicine, the Scripps Research Institute, 10666 N. Torrey Pines Rd., La Jolla, CA 92037, where reprint requests should be addressed to Dr. McMillan.
Immunization of Adults
Rothstein E. P., Bernstein H. H., Long S. S., Gluckman S. J., Krause D. S., Gardner P., Schaffner W.
Extract |
Full Text
N Engl J Med 1993;
329:1046-1048, Sep 30, 1993.
Correspondence
This article has been cited by other articles:
Burzynski, J.
(2009). New options after first-line therapy for chronic immune thrombocytopenic purpura. Am J Health Syst Pharm
66: S11-S21
[Abstract][Full Text]
Ruggeri, M., Fortuna, S., Rodeghiero, F.
(2008). Heterogeneity of terminology and clinical definitions in adult idiopathic thrombocytopenic purpura: a critical appraisal from a systematic review of the literature. haematol
93: 98-103
[Abstract][Full Text]
Boruchov, D. M., Gururangan, S., Driscoll, M. C., Bussel, J. B.
(2007). Multiagent induction and maintenance therapy for patients with refractory immune thrombocytopenic purpura (ITP). Blood
110: 3526-3531
[Abstract][Full Text]
Cines, D. B., Bussel, J. B.
(2005). How I treat idiopathic thrombocytopenic purpura (ITP). Blood
106: 2244-2251
[Full Text]
Stasi, R., Provan, D.
(2004). Management of Immune Thrombocytopenic Purpura in Adults. Mayo Clin Proc.
79: 504-522
[Abstract]
Vesely, S. K., Perdue, J. J., Rizvi, M. A., Terrell, D. R., George, J. N.
(2004). Management of Adult Patients with Persistent Idiopathic Thrombocytopenic Purpura Following Splenectomy: A Systematic Review. ANN INTERN MED
140: 112-120
[Abstract][Full Text]
Cines, D. B., Bussel, J. B., McMillan, R. B., Zehnder, J. L.
(2004). Congenital and Acquired Thrombocytopenia. ASH Education Book
2004: 390-406
[Abstract][Full Text]
Kuwana, M., Okazaki, Y., Kaburaki, J., Kawakami, Y., Ikeda, Y.
(2002). Spleen Is a Primary Site for Activation of Platelet-Reactive T and B Cells in Patients with Immune Thrombocytopenic Purpura. J. Immunol.
168: 3675-3682
[Abstract][Full Text]
Cines, D. B., Blanchette, V. S.
(2002). Immune Thrombocytopenic Purpura. NEJM
346: 995-1008
[Full Text]
Stasi, R., Pagano, A., Stipa, E., Amadori, S.
(2001). Rituximab chimeric anti-CD20 monoclonal antibody treatment for adults with chronic idiopathic thrombocytopenic purpura. Blood
98: 952-957
[Abstract][Full Text]
McMillan, R.
(2001). Long-Term Outcomes after Treatment for Refractory Immune Thrombocytopenic Purpura. NEJM
344: 1402-1403
[Full Text]
McCrae, K. R., Bussel, J. B., Mannucci, P. M., Remuzzi, G., Cines, D. B.
(2001). Platelets: An Update on Diagnosis and Management of Thrombocytopenic Disorders. ASH Education Book
2001: 282-305
[Abstract][Full Text]
Bussel, J. B., Kunicki, T. J., Michelson, A. D.
(2000). Platelets: New Understanding of Platelet Glycoproteins and Their Role in Disease. ASH Education Book
2000: 222-240
[Abstract][Full Text]
Werth, V. P.
(1997). Pulse Intravenous Cyclophosphamide for Treatment of Autoimmune Blistering Disease: Is There an Advantage Over Oral Routes?. Arch Dermatol
133: 229-230
[Abstract]
George, J. N., El-Harake, M. A., Raskob, G. E.
(1994). Chronic Idiopathic Thrombocytopenic Purpura. NEJM
331: 1207-1211
[Full Text]
Andersen, J. C.
(1994). Response of Resistant Idiopathic Thrombocytopenic Purpura to Pulsed High-Dose Dexamethasone Therapy. NEJM
330: 1560-1564
[Abstract][Full Text]
Kao, N. L., Richmond, G. W., Figueroa, M., McMillan, R.
(1993). Combination Chemotherapy for ITP. NEJM
329: 1046-1046
[Full Text]