Multiagent induction and maintenance therapy for patients with refractory immune thrombocytopenic purpura (ITP)

Patients with severe immune thrombocytopenic purpura (ITP) may require an acute increase in the platelet count for surgery or ongoing hemorrhage as well as long-term maintenance treatment. Certain of these patients may be refractory to steroids, intravenous anti-D, intravenous immunoglobulin (IVIG), and splenectomy. Therefore, acute platelet increases were studied in 35 patients completely unresponsive to IVIG or high-dose steroid treatment. Because of their lack of response to either or both single agents, these patients were administered a 3- or 4-drug combination including IVIG 1 g/kg, intravenous methylprednisolone 30 mg/kg, Vinca alkaloids (VCR 0.03 mg/kg), and/or intravenous anti-D (50-75 microg/kg). Subsequent maintenance therapy with the oral combination of danazol (10-15 mg/kg) and azathioprine (2 mg/kg) was given to 18 of the 35 patients. Seventy-one percent of the patients responded to the intravenous combination treatment with acute platelet increases of at least 20 x 10(9)/L to a level greater than 30 x 10(9)/L. Two thirds of the patients given maintenance therapy achieved stable platelet counts greater than 50 x 10(9)/L without other treatments. One patient developed an ileus, but otherwise there was little toxicity of combination treatment. Combination chemotherapy is a useful approach for patients with ITP refractory to conventional treatments both for acute induction and for long-term maintenance therapy.     

Lambert-Eaton myasthenic syndrome associated with idiopathic thrombocytopenic purpura and diffuse panbronchiolitis: long-term remission after a course of intravenous immunoglobulin combined with low-dose prednisolone.

We report a case of Lambert-Eaton myasthenic syndrome (LEMS) associated with idiopathic thrombocytopenic purpura (ITP) and diffuse panbronchiolitis (DPB). An extensive search for malignancy yielded negative results. Interestingly, ITP and DPB developed simultaneously when the patient suffered from myasthenic symptoms. This is the first report in the Japanese or English literature of an association of LEMS, ITP, and DPB. The use of cholinesterase blocker alone did not improve the myasthenic symptoms, and the symptoms and signs relapsed with the tapering of prednisolone (PSL) dosage. However, after administration of immunoglobulin (IVIG) (0.4 g/kg/day x 5 days), low-dose PSL (20 mg/day) alleviated the LEMS and ITP, and the diseases have remained in remission for 8 months without additional IVIG. We suspect that there is a synergistic relationship between IVIG and PSL.     

Effects of maintenance treatment after high-dose intravenous gamma-globulin for idiopathic thrombocytopenic purpura]

High-dose intravenous immunoglobulin (IVIG) for idiopathic thrombocytopenic purpura (ITP) produces a dramatic and substantial increase in platelet count, but the increased count tends to return rapidly to its pretreatment level. We studied the effects of immunosuppressive treatment aimed at the maintenance of platelet counts following the IVIG administration in ITP. Thirty-five patients with ITP were treated with IVIG, and then thirty-two of them with an immunosuppressant (azathioprine) and a glucocorticoid (prednisolone). After IVIG, the platelet count increased significantly. With immunosuppressive therapy after IVIG, most patients had a tendency to maintain the counts. In particular, this maintaining effect was remarkable in those patients who had been responsive to the standard prednisolone therapy while non-responders to the prior prednisolone failed to maintain the counts. When prednisolone was given after IVIG, the effect of maintaining platelet counts was dose-dependent. The treatment with azathioprine and prednisolone after IVIG appears to be effective in maintenance of platelet counts.     

Use of the novel thrombopoietin receptor-agonist romiplostim, in combination with steroids and immunoglobulins for the increase of platelets prior to splenectomy, in refractory immune thrombocytopenia: a case report

This case report describes a patient with relapsed primary immune thrombocytopenic purpura (ITP), in which splenectomy was not possible due to the persistence of a low platelet count despite treatment with corticosteroids, intravenous immunoglobulins (IVIG) and platelet transfusion treatment. As an attempt to increase platelet count prior to performing splenectomy, the thrombopoietin receptor agonist, romiplostim, was administered in combination with steroids and IVIG. A single administration of romiplostim was found to be markedly effective, allowing a rapid and notable platelet increase, required for a well tolerated splenectomy. This case confirms the potent activity of romiplostim in ITP, and indicates that patients with recurrent primary ITP who are unresponsive to conventional immunosuppressive therapy may benefit from the addition of a short course of romiplostim.     

Comparison of anti-D immunoglobulin, methylprednisolone, or intravenous immunoglobulin therapy in newly diagnosed pediatric immune thrombocytopenic purpura

This study aimed to evaluate the efficacy, cost, and effects of anti-D immunoglobulin (anti-D Ig), methylprednisolone, or intravenous immunoglobulin (IVIG) therapy on the development of chronic disease in children who are Rh-positive with diagnosed immune thrombocytopenic purpura (ITP). Children with newly diagnosed ITP and platelet count <20,000/mm³ were prospectively randomized to treatment with anti-D Ig (50 μg/kg), methylprednisolone (2 mg/kg/day), or IVIG (0.4 g/kg/day, 5 days). Sixty children with a mean age of 6.7 years were divided into three equal groups. No difference was observed between platelet counts before treatment and on day 3 of treatment. However, platelet counts at day 7 were lower in the methylprednisolone group than in the IVIG group (P = 0.03). In the anti-D Ig group, hemoglobin and hematocrit levels were significantly lower at the end of treatment (P < 0.05). Chronic ITP developed in 30 % of the anti-D Ig group, 35 % of the methylprednisolone group, and 25 % of the IVIG group, but no significant difference was noted among the groups. The cost analysis revealed that the mean cost of IVIG was 7.4 times higher than anti-D Ig and 10.9 times higher than methylprednisolone. In the treatment of ITP in childhood, one 50 μg/kg dose of anti-D Ig has similar effects to IVIG and methylprednisolone. Among patients who were treated with anti-D Ig, serious anemia was not observed, and the cost of treatment was less than that of IVIG treatment.     

Childhood acute immune thrombocytopenic purpura: 20 years later

Childhood acute immune thrombocytopenic purpura (ITP) is a typically benign, self-limiting illness usually occurring after an infectious disease. Most affected children have platelet counts < 20 x 10 (9)/L at presentation and are at small, but definite risk for an intracranial hemorrhage. This feared complication occurs in < 1% of all children with acute ITP. There is consensus that a bone marrow aspirate should be performed in children with acute ITP and atypical features (e.g., hepatosplenomegaly), and most physicians continue to recommend this investigation before corticosteroids are administered. Issues such as hospitalization versus observation at home, and treatment versus no treatment continue to be debated; there is consensus, however, that children with extreme thrombocytopenia (platelet counts < 10 x 10 (9)/L) and/or clinically significant hemorrhage merit treatment with a regimen known to rapidly increase the circulating platelet count. Candidate regimens include high-dose intravenous (IV)/oral corticosteroids (>/= 4 mg/kg/day of prednisone or an equivalent corticosteroid preparation), IV immunoglobulin (IG; 0.8 to 1.0 g/kg once) or IV anti-D (75 microg/kg once) for Rhesus-positive patients. For those rare children with organ- or life-threatening hemorrhage (e.g., intracranial hemorrhage) multimodality therapy including platelet transfusion, IV high-dose methylprednisone (30 mg/kg, maximum 1 g) and IVIG (1 g/kg) is indicated with consideration of emergency splenectomy. Future prospective trials should include outcome measures other than the platelet count alone (e.g., bleeding scores) and health-related quality-of-life assessments. Key questions that remain to be addressed in children with acute ITP include the need for bone marrow aspiration in typical cases if corticosteroid therapy is planned, the role of hospitalization, and most important, the unresolved issue of treatment versus no treatment, especially in patients with typical features and mild clinical bleeding symptoms.     

Efficacy and safety of dapsone as a second-line treatment in non-splenectomized adults with immune thrombocytopenic purpura

In adults with immune thrombocytopenic purpura (ITP), steroids are usually proposed as first-line therapy, but long-term complete responses are obtained in no more than 20% of patients. For the remaining patients, splenectomy is considered the treatment of choice, with reported “cure” rates from 60–70%. However, the inherent risks of surgery and sepsis after splenectomy without a guarantee of success justify the search for strategies aimed to avoid splenectomy. Here we retrospectively evaluated the results of dapsone treatment in ITP patients that failed first-line therapy with steroids. These patients received dapsone 100 mg/day for a minimum of 30 days before splenectomy was considered. Efficacy was defined as a sustained rise in platelet counts (>50 × 10⁹/l) clearly attributed to dapsone treatment. Among 52 steroid-dependent or refractory patients, dapsone resulted in sustained increases in platelet counts in 44.2% of patients, after a median follow-up of 21.10 months after treatment initiation. The long-term efficacy of dapsone in this setting is further corroborated by the observation that none of the “responding” patients required splenectomy in the follow-up, compared to 69.0% of the “non-responding” patients. Dapsone-related adverse events were mild and promptly reversed by treatment withdrawal. The results of our retrospective analysis suggest that dapsone is a safe and effective second-line agent for steroid-dependent or refractory ITP patients. Because of its well-known safety profile and low cost compared to other potential second-line treatments for ITP, a trial course of dapsone should be viewed as an attractive option before splenectomy in steroid-dependent of refractory adult ITP patients.     

Intravenous gammaglobulin (Gaminume) for treatment of chronic idiopathic thrombocytopenic purpura (ITP): a two-year follow-up

Thirteen subjects 5-20 years of age with the chronic, autoimmune form of idiopathic thrombocytopenic purpura (ITP) were given intravenous gammaglobulin (Gamimune; Cutter Biological, Berkeley, CA) in a dose of 400 mg/kg per day for 5 consecutive days. Two of the 13 children had undergone splenectomy; the other 11 had not. Eight of these 13 children had also received corticosteroid therapy with no sustained increase in platelet counts. Six of 13 children had a good or excellent response to the first 5 day course of gammaglobulin therapy, and one had a fair response. The peak platelet count occurred within 7 days of the start of therapy except in one patient, whose platelet count peaked on day 12. Six of seven patients who initially responded to Gamimune required booster doses to maintain platelet counts at a safe level. All children had marked increases in serum IgG following Gamimune except one (who had undergone splenectomy for chronic ITP), who had high baseline levels of immunoglobulin G (IgG). No untoward reactions necessitating cessation of therapy were encountered during this study. The most common side effect observed was headache. During the first year of follow-up after Gamimune, three of seven initial responders became refractory to Gamimune therapy. Two of these three refractory subjects later underwent splenectomy with excellent response. The third refractory patient who was splenectomized prior to gammaglobulin therapy had spontaneous remission of his ITP 5 months after the last dose of Gamimune. Three of the four other initial responders have continued to do well and have maintained platelet counts above 40,000/mm3 (one without booster). The fourth subject dropped out of the study. Thus our observations indicate that Gamimune is an effective form of treatment for some children with chronic ITP, and can be considered as an alternative to splenectomy or as a potential therapeutic modality in those who have failed to respond to splenectomy.     

Efficacy and safety of dapsone as a second-line treatment in non-splenectomized adults with immune thrombocytopenic purpura

In adults with immune thrombocytopenic purpura (ITP), steroids are usually proposed as first-line therapy, but long-term complete responses are obtained in no more than 20% of patients. For the remaining patients, splenectomy is considered the treatment of choice, with reported "cure" rates from 60-70%. However, the inherent risks of surgery and sepsis after splenectomy without a guarantee of success justify the search for strategies aimed to avoid splenectomy. Here we retrospectively evaluated the results of dapsone treatment in ITP patients that failed first-line therapy with steroids. These patients received dapsone 100 mg/day for a minimum of 30 days before splenectomy was considered. Efficacy was defined as a sustained rise in platelet counts (>50 x 10(9)/l) clearly attributed to dapsone treatment. Among 52 steroid-dependent or refractory patients, dapsone resulted in sustained increases in platelet counts in 44.2% of patients, after a median follow-up of 21.10 months after treatment initiation. The long-term efficacy of dapsone in this setting is further corroborated by the observation that none of the "responding" patients required splenectomy in the follow-up, compared to 69.0% of the "non-responding" patients. Dapsone-related adverse events were mild and promptly reversed by treatment withdrawal. The results of our retrospective analysis suggest that dapsone is a safe and effective second-line agent for steroid-dependent or refractory ITP patients. Because of its well-known safety profile and low cost compared to other potential second-line treatments for ITP, a trial course of dapsone should be viewed as an attractive option before splenectomy in steroid-dependent of refractory adult ITP patients.     

Randomized trial of anti-D immunoglobulin versus low-dose intravenous immunoglobulin in the treatment of childhood chronic idiopathic thrombocytopenic purpura

BACKGROUND: Chronic idiopathic (immune) thrombocytopenic purpura (ITP) develops in approximately 20% of children with acute ITP. Six years ago, low-dose intravenous immunoglobulin (IVIG) treatment of childhood ITP was started at the Pediatric Hematology Unit, Ain Shams University, while intravenous anti-D has been introduced in Egypt in 2001. OBJECTIVES: To assess the efficacy and safety of intravenous anti-D compared to low-dose IVIG in the treatment of children with chronic ITP. PATIENTS AND METHODS: This randomized trial comprised 34 patients with chronic ITP (18 boys and 16 girls) with recurrent bleeding episodes. Median age of the patients was 6.5 years, duration of thrombocytopenia was > 6 months, and platelet count (PC) was < 30 x 10(9)/l (30 K). The patient cohort was divided into two subgroups: group A comprised 18 patients treated with anti-D in a dose of 50 microg/kg i.v. initially, and in 12 of them repeated doses (50 microg/kg) were given every 4 weeks, and group B consisted of 16 children who received IVIG in a dose of 250 mg/kg for 2 consecutive days. Bleeding manifestations, complete blood cell and reticulocyte counts were assessed at baseline and 3, 7, 14 and 28 days after infusion. RESULTS: Clinically, more than 80% of the patients (82.3%) showed good control of bleeding. On day 3, 33.3% of group A versus 37.5% of group B, and on day 7: 66.6% of group A versus 75% of group B patients demonstrated a good response (PC > 50 K and/or doubling of baseline PC). On days 14 and 21, no significant changes in PCs were observed between both groups. However, only 11.1% of group A and 12.5% of group B patients could maintain PC > 100 K on day 28, while 38.8 versus 37.5% of group A and group B, respectively, still had PC > or = double the initial count. The peak response to anti-D was noticed 7 and 14 days following infusion and to IVIG on days 3 and 7. Repeated doses of anti-D could maintain PC > 50 K (or > double the baseline PC) in 75% of patients 1 week after infusion, and in 60% of them by day 28, with good control of bleeding. Splenectomy was postponed and/or avoided in 4 (33.3%) patients on anti-D maintenance therapy who experienced recurrent severe bleeding episodes before starting therapy. The safety of anti-D was judged by the degree of intravascular hemolysis. The mean hemoglobin decrease was 0.8 +/- 0.4 g/dl; in 61.1% of patients the Hb level dropped but none of them experienced a drop of more than 3 g/dl or required transfusion. CONCLUSION: Both single intravenous anti-D and low-dose IVIG effectively increased PC in children with chronic ITP at risk of bleeding or those with previous bleeding episodes. Repeated doses of anti-D could maintain PC above the critical values or double baseline counts in nearly two thirds of the patients showing good control of bleeding and may serve as an alternative to splenectomy in these patients.     

A pilot study of rhuIL-11 treatment of refractory ITP

The objective of this research was to determine whether rhuIL-11 is an effective treatment in patients with refractory immune thrombocytopenic purpura (ITP). Platelet production is decreased in certain cases of refractory ITP. IL-11 stimulates megakaryocytopoiesis in vitro and was licensed for its clinical effects to ameliorate chemotherapy-induced thrombocytopenia. A pilot study was initiated, intending to enroll 12 patients with ITP. These patients were to receive rhuIL-11 (Neumega) at a dose of 50 microg/kg subcutaneously daily for 21 consecutive days and be observed afterward for 21 additional days. CBC with platelets were obtained twice weekly with visits and physical examinations weekly. The study was terminated after 7 patients were enrolled because of toxicity and lack of efficacy. All 7 patients had had ITP for >9 years and had failed splenectomy, intravenous gammaglobulin, corticosteroids, and a variety of other treatments. The patients at entry all had platelet counts <20,000/microl; 5 of 7 had counts <10,000/microl. The maximal median increase for any day of the study was 6,000/microl. No patient achieved a count of 30,000/microl, and only 3 patients achieved (once each) a platelet count >20,000/microl. Substantial toxicity was seen. The nadir hemoglobin decrease was a mean of 2 g/dl. rhuIL-11 was not effective at increasing the platelet count in any of these patients with refractory ITP. Toxicity was substantial. The lack of platelet response to rhuIL-11 in this study does not exclude the possibility of better effects at other doses and/or in less refractory patients.     

Cyclic thrombocytopenia with chronic thyroiditis and ankylosing spondylitis]

A 58-year-old man with cyclic thrombocytopenia who was initially diagnosed as idiopathic thrombocytopenic purpura (ITP), concomitant with chronic thyroiditis and ankylosing spondylitis, was reported. Serum level of T 3 (0.48 ng/ml) and T 4 (2.1 micrograms/ml) were both subnormal and that of TSH (257.1 microU/ml) was markedly elevated. Thyroid test (6400X) and microsome test (6400X) was both positive, but anti-nuclear antibodies were negative. Radiographic findings of lumbar spine showed the typical "bamboo spine" and HLA B 27 was positive. Therapies for ITP, such as adrenocorticosteroids including steroid pulse therapy, high-dose intravenous gamma-globulin, danazol, slow infusion of vinca alkaloids and splenectomy, were only effective transiently. After these therapies platelet counts began to fluctuate from 0.4 X 10(10)/L to 34.4 X 10(10/L, therefore the diagnosis of cyclic thrombocytopenia was done. Interestingly low-dose methotrexate (MTX) was effective, and the cyclic fluctuation of platelet counts disappeared. These observations in this case were very suggestive of the pathogenesis of cyclic thrombocytopenia and mechanisms of cyclic change of platelet counts.     

Refractory chronic immune thrombocytopenic purpura in a child with acute lymphoblastic leukemia

Immune thrombocytopenic purpura (ITP) has been associated with several hematologic malignancies such as Hodgkin and non-Hodgkin lymphomas and chronic lymphocytic leukemia, but it is rare in children with acute lymphoblastic leukemia (ALL). Here, we report a 7-year-old girl with chronic ITP during early intensive phase of chemotherapy for ALL. She underwent splenectomy because thrombocytopenia had persisted even after treatment with intravenous immunoglobulin (IVIG), steroids, vincristine, rituximab, and anti-D antibody. After splenectomy, her platelet count had recovered, and maintenance therapy could be resumed with a support of IVIG. To our knowledge, this is the first child case of chronic ITP during chemotherapy for ALL and splenectomy was effective in this patient.     

Poor response to prednisolone of idiopathic thrombocytopenia with human T-lymphotropic virus type I infection

We describe the unique clinical characteristics of patients with idiopathic thrombocytopenic purpura (ITP) who are infected by human T-lymphotropic virus type I (HTLV-I). Thirty-seven patients with ITP were examined in the present study: 10 patients had HTLV-I infection, and the remaining 27 did not. The mean age of the group with HTLV-I infection was significantly older than that of the group without infection (57.8 +/- 14.0 and 42.4 +/- 20.1, P = 0.022). The difference in mean platelet counts at diagnosis between the two groups was not significant, 29 x 10(9)/L and 21 x 10(9)/L, respectively. The levels of platelet associated IgG, red blood cell count, white blood cell count, bone marrow cell count, and megakaryocyte count did not show any significant difference. Nine patients in the group with HTLV-I infection were treated with prednisolone (1 mg/kg, daily oral). Only 3 of them responded to the therapy (one complete response [CR] and two partial responses [PR]). However, 17 of 22 patients not infected with HTLV-I were treated with prednisolone successfully: 14 patients achieved CR, and 3 patients achieved PR. There was a significant difference in response to prednisolone between the two groups (P = 0.034). Two patients with the infection and one patient without the infection achieved CR with splenectomy. These results suggest that HTLV-I infection may cause immune thrombocytopenia by a different mechanism than classical ITP; HTLV-I may modify the clinical features of ITP through an unknown immune pathway.     

Long-term outcomes in adults with chronic ITP after splenectomy failure

Adult chronic immune thrombocytopenic purpura (ITP) is an autoimmune disorder manifested by thrombocytopenia from the effects of antiplatelet autoantibodies and T lymphocyte-mediated platelet cytotoxicity. Multiple studies show that corticosteroid treatment and splenectomy, alone or together, increase platelet counts to safe levels in 60% to 70% of patients. However, there is little information on the outcomes of ITP patients refractory to splenectomy. We studied 114 patients with ITP for whom splenectomy failed and who required additional therapy; long-term follow-up was available on 105 (92%) patients. Seventy-five (71.4%) patients attained stable partial (platelet count greater than 30 x 10(9)/L) or complete (normal platelet count) remission; 51 patients remained in remission after therapy was discontinued, whereas 24 patients required continued treatment. Median time to remission after splenectomy failure was 46 months (range, 1-437 months). Median remission durations were 60 months (range, 10-212 months) for patients off therapy and 48 months (range, 2-167 months) for patients on therapy. Thirty (29.6%) patients remained unresponsive to treatment. Thirty-two patients died, 17 (15.7%) of ITP (bleeding, 11 patients; therapy complications, 6 patients) and 15 (13.9%) of unrelated causes. We conclude that most patients with refractory ITP attain stable remission, though on average this occurs slowly. However, a subpopulation with severe, resistant disease experiences significant morbidity and mortality.     

High-dose dexamethasone for splenectomy in patients with idiopathic thrombocytopenic purpura.

High-dose intravenous immune globulin (IV IgG) is currently the treatment of choice for patients with idiopathic thrombocytopenic purpura (ITP) who undergo splenectomy; however, this treatment is extremely expensive. We report on 13 ITP patients with severe thrombocytopenia (<20 x 10(9)/l) who were prepared for laparoscopic splenectomy with a 4-day oral course of high-dose (40 mg/day) dexamethasone (DEX). Four patients had an excellent response with platelet counts that increased to above 150 x 10(9)/l. Seven patients had a good response with a platelet count that increased to between 50 and 150 x 10(9)/l (median 121 x 10(9)/l). Two patients were resistant both to DEX and IV IgG. The operation was uneventful in all the patients, including the 2 who had resistant ITP and were operated on while their platelet count was very low (5 x 10(9)/l). Thus, high-dose DEX, which is an easy, effective and inexpensive treatment, is recommended for the preparation of ITP patients prior to splenectomy.     

5- to 16-year follow-up following splenectomy in chronic immune thrombocytopenic purpura in children

The long-term outcome after splenectomy in children with chronic immune thrombocytopenic purpura (ITP) has not been widely analyzed. We reviewed the medical records of 288 children and adolescents with chronic ITP between 1980 and 1996: 112 were splenectomized; 59 were steroid resistant and 42 were steroid dependent, and 11 were managed with repeated courses of intravenous immunoglobulin (IVIG). All had platelet counts (PCs) <30 x 10(9)/l with frequent bleeding episodes or persistent thrombocytopenia <10 x 10(9)/l. Ninety-eight patients (88%) were evaluated; 58 (60%) patients had never received immunotherapy for ITP following splenectomy. At 5 years, 44 (45%) remained in complete response (CR) and 34 (35%) in partial response (PR). In multivariate analysis, steroid-resistant patients were more likely to relapse after an initial CR (RR 5.2). CONCLUSION: The long-term CR was 45%; 60% had stable PCs >30 x 10(9)/l not requiring therapy. Most postsplenectomy relapses occurred during the 1st year. Initial response to steroids and IVIG prior to splenectomy was a predictor of long-term response to splenectomy.     

Helicobacter pylori eradication based upon a drug sensitivity test effectively increased platelet count in a patient with refractory idiopathic thrombocytopenic purpura

We report on case of a 52-year-old male with refractory idiopathic thrombocytopenic purpura. Treatment with prednisolone, vincristine, azathioprine, colchicine, danazol, diaphenylsulfone, and splenectomy were tried but all were ineffective and platelet counts mostly stayed below 5,000/microliter. We finally tried eradicating Helicobacter pylori (HP) with the standard combination of amoxicillin (1,500 mg), clarithromycin (400 mg), and lansoprazole (60 mg) for 7 days, but it failed. We therefore gave the patient a second eradication therapy based upon a drug sensitivity test using HP obtained from his gastric mucosa. According to the drug sensitivity test, we treated him with minocycline (200 mg), levofloxacin (600 mg), and lansoprazole (60 mg) for 7 days. The platelet counts increased gradually and reached to 30,000/microliter after the eradication, and the patient was spared extended hospitalization.     

Plasma soluble interleukin-2 receptor levels in patients with idiopathic thrombocytopenic purpura

Soluble interleukin-2 receptor (sIL-2R) was measured in the plasma of 31 patients with idiopathic thrombocytopenic purpura (ITP) and 22 normal controls. When thrombocytopenia persisted longer than 6 months, the diagnosis of chronic ITP was made. Twenty patients had acute ITP, 11 patients had chronic ITP, and all patients received high-dose methylprednisolone (HDMP) (30 mg/kg/d for 3 days, 20 mg/kg/d for 4 days). The sIL-2R levels of the patients were determined before being giving HDMP and 14 days after the end of HDMP therapy. Platelet counts were determined before administration of HDMP, one day after the end of HDMP therapy, and once every 28 days for 7 months thereafter. There was not a significant difference between the mean pre-treatment plasma sIL-2R levels of both acute and chronic ITP groups (P > 0.05), and these were higher than that of the control group (P < 0.001). The mean post-treatment sIL-2R level of the chronic ITP group was significantly higher than those of both the control and post-treatment acute ITP groups (P < 0.001). There were negative correlations between the plasma sIL-2R levels and platelet counts of both group patients in the pre-treatment period and between post-treatment sIL-2R levels and platelet counts in chronic ITP group (P < 0.05). We think that there was a good correlation between prognosis of ITP and sIL-2R levels after HDMP therapy, and platelet counts in patients with ITP are linked to sIL-2R levels. Am. J. Hematol. 57:119–123, 1998. © 1998 Wiley-Liss, Inc.     

Intravenous gammaglobulin in refractory immune thrombocytopenic purpura: efficacy with or without concomitant steroid therapy

Two patients with immune thrombocytopenic purpura (ITP) refractory to conventional management with steroids and splenectomy were treated with 5-day courses of intravenous gamma globulin (Gamimune-Cutter), 400 mg/kg/day. Improvement was observed in both cases even while steroids were being tapered. In each case when thrombocytopenia subsequently recurred or worsened, further therapy with IV gamma globulin without concomitant steroids was followed by definite increase in platelet numbers. These observations confirm that the Cutter preparation of IV gamma globulin is efficacious in the management of refractory ITP and suggest that in this clinical setting response to IV gamma globulins can occur without concomitant administration of corticosteroids.