SPLENECTOMY IN IDIOPATHIC
THROMBOCYTOPENIC PURPURA (ITP).

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EFFECTIVE, YES, BUT FOR HOW LONG?

40th Annual American Society of Hematology Meeting - Abstract #717 - Sunday, December 6, 1998 - Hall C, 5:00-6:30 Posterboard 101-II, PLATELET DISORDERS II

A. Rovó, D. Penchasky, J. Korin, I. Santos, L. Celebrín, E. Rosenfeld

Hospitales J.M. Ramos Mejía, Italiano, Militar, Posadas, P. Bancario and Consultorio de Estudios Hematológicos, Buenos Aires, Argentina

Purpose: More than two thirds of ITP patients (p) usually achieved satisfactory remissions after splenectomy (Sx), however, long term follow up of this early responses has been seldom reported. The objective of this study was to examine the results immediately, at 1 and 5 years after Sx in ITP patients. Methods: we analized 68 p with a clinical diagnosis of ITP, who underwent Sx. They were divided in two groups: A: Sx performed during the first year since diagnosis, and B: Sx after the first year. Group A: N = 33, mean age 33 years (y), number of patients under 15 years: 4. Female/male ratio 2.5. Mean time before Sx: 7 months (less than 2 months: 7p). The decision to perform Sx was done because of: refractoriness 64%, idem + bleeding 9% and bleeding 27%. Group B: N = 35, mean age: 24 y, 11 p were under 15 y. Female/male ratio 2.5, mean time pre-Sx: 63 months. Sx indications: refractory ITP 71%, idem + bleeding 20% and bleeding alone 9%. Results: Group A: Complete Response (CR) 58%, Partial Response (PR) 15%, No Response (NR) 27%, at 1 year: CR 36%, at 5 y CR was 12%. During follow-up only 3% of the p developed other illness associated to immune thrombocytopenia. Group B: CR 69%, PR 14%, NR 17%. At the first year CR51%, but fell at 5 years to 23%. 14% of patients developed secondary immune thrombocytopenia. 64% of the splenectomized patients who reached CR needed some other therapeutic measures after Sx. Conclusions: Sx resulted in immediate CR (63%) in this group of patients. Nevertheless, during a follow-up period of 5 years a loss of efectiveness of this procedure was progressively noted, resulting in 64% of patients needing complementary treatments. Those in which Sx was performed early since diagnosis, showed no advantage of increasing or maintaining a CR.

Stasi, et al.: Long-Term Observation of 208 Adults with Chronic Idiopathic Thrombocytopenic Purpura. 98: 436-442. The American Journal of Medicine.

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 - 78) at the time of diagnosis, and 51 years (range 19 to 78) 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 50x19**9 platelets per liter received initial treatment with prednisone (PDN) at a dosage of 1 mg/kg of body weight for 1 month. Refractory or elapsed cases underwent splenectomy and/or other therapeutic modalities. In 87 patients with greater than 50x10**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 sustain 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.

N Engl J Med 1997 May 22;336(21):1494-1498 High-dose intravenous immune globulin and the response to splenectomy in patients with idiopathic thrombocytopenic purpura.

Law C, Marcaccio M, Tam P, Heddle N, Kelton JG

Department of Surgery, McMaster University and the Hamilton Health Sciences Corporation, ON, Canada.

BACKGROUND: High-dose intravenous immune globulin produces a temporary rise in the platelet count in patients with idiopathic thrombocytopenic purpura. Splenectomy may also be effective, but it is not possible to predict which patients will have a good response. We hypothesized that the response to intravenous immune globulin predicts the response to splenectomy. METHODS: We studied retrospectively 30 patients with idiopathic thrombocytopenic purpura who had first been treated with immune globulin and then undergone splenectomy. The responses to the two treatments were classified on the basis of the platelet count as poor (<50,000 per cubic millimeter), good (50,000 to 150,000 per cubic millimeter), or excellent (>150,000 per cubic millimeter). RESULTS: All nine patients who had poor responses to intravenous immune globulin also had poor responses to splenectomy at one year. Of the 21 patients with good or excellent responses to intravenous immune globulin, 19 had good or excellent responses to splenectomy. CONCLUSIONS: Patients with idiopathic thrombocytopenic purpura who have good or excellent responses to intravenous immune globulin are likely to have good or excellent responses to splenectomy, whereas patients who have poor responses to intravenous immune globulin are unlikely to have good or excellent responses to splenectomy.

Comments: Comment in: N Engl J Med 1997 Oct 9;337(15):1087-8; discussion 1089 Comment in: N Engl J Med 1997 Oct 9;337(15):1088-9

Br J Haematol 1997 Jun;97(3):547-550 The site of destruction of autologous 111In-labelled platelets and the efficiency of splenectomy in children and adults with idiopathic thrombocytopenic purpura: a study of 578 patients with 268 splenectomies.

Najean Y, Rain JD, Billotey C

Department of Nuclear Medicine, Hospital Saint-Louis, Paris, France.

The indication for splenectomy in chronic idiopathic thrombocytopenic purpura (ITP) remains a controversial subject. The mortality rate of persistent thrombocytopenia is very low, except in severe cases. Conversely, the risks of splenectomy are significant (in the present series, morbidity: 4.1% mortality: 1.4%), with a success rate of only 60-75%. It is therefore useful to define a parameter able to predict the efficacy or failure of splenectomy. An analysis of 578 cases of chronic ITP, where the site of platelet destruction has been determined, is presented. 268 of these cases had been splenectomized. When platelet destruction was splenic, 96% of subjects aged 5-30 years and 91% of cases over the age of 30 years obtained a remission. Conversely, when platelet destruction was hepatic or diffuse, failure or incomplete results were observed in 92% of cases. The site of platelet destruction therefore constitutes a parameter which can help the clinician to make the decision to perform splenectomy.