Intravenous immunoglobulin in the treatment of human immunodeficiency virus-related thrombocytopenia.

Fourteen patients with sexually transmitted human immunodeficiency virus (HIV)-related immune thrombocytopenia were treated with intravenous gammaglobulin (IVIG). The patients were treated with a uniform program consisting of 1 g/kg of IVIG on day 1 and day 2, followed by 1 g/kg on day 15. Most patients had pretreatment bleeding symptoms, which included petechiae, spontaneous and traumatic ecchymoses, gum bleeding, and epistaxis. Median baseline platelet count was 17,000/mm3 (range 3-61,000/mm3). After the infusion of the IGIV, all patients had a resolution of their bleeding by day 8. The median maximum platelet count achieved with the IGIV was 220,000/mm3 (range 76-426,000/mm3). No patient achieved either a sustained complete or partial remission after the conclusion of the IVIG therapy. Toxicities were minimal with the majority being headache and nausea. In conclusion, patients with sexually transmitted HIV infection and immune thrombocytopenia respond favorably to IVIG. This treatment should be considered as first-line therapy for patients with HIV-related immune thrombocytopenia who require immediate but temporary increase in their platelet count, attributable to symptoms or signs of clinical bleeding or because of the need for an invasive procedure.     

小剂量利妥昔单抗治疗原发性干燥综合征继发血小板减少四例疗效观察

目的 初步评价小剂量利妥昔单抗治疗原发性干燥综合征(pSS)继发血小板减少的疗效与安全性.方法 4例pSS继发血小板减少患者,2例为难治性血小板减少,2例为糖皮质激素依赖性血小板减少,静脉滴注利妥昔单抗100 mg,每周1次,共2次,同时联合泼尼松1～2 mg·kg-1·d-1治疗.观察血小板和外周血B细胞的变化.结果 4例患者治疗前血小板水平为(3 ～39)×109/L,小剂量利妥昔单抗治疗后,血小板于1～2周内上升,3～8周内恢复至(107～241)×109/L,维持缓解27 ～52周.12周内泼尼松减为3.75 ～7.50 mg/d并维持.1例患者于第27周时复发,血小板降至47×109/L,再次静脉滴点利妥昔单抗100 mg,4周后血小板升至81 x 109/L.4例患者外周血B细胞降至(0.007 ～0.010)×109/L,但未达清除状态.输注过程中均无严重不良反应发生.结论 小剂量利妥昔单抗可用于治疗pSS继发血小板减少,减少糖皮质激素用量,部分清除B细胞.     

Severe aplastic anemia remarkably improved by a treatment with antilymphocyte globulin, high-dose methylprednisolone and danazol

Sixteen-years-old female with severe aplastic anemia received a therapy combined with antilymphocyte globulin (ALG), high-dose methylprednisolone (m-PSL) and danazol. At the hospitalization, hematological examination demonstrated as follows; reticulocyte 21,000/microliters, granulocyte 350/microliters, platelet 10,000/microliters and hypocellular bone marrow. Treatment schedule were 1) m-PSL 1,000 mg (day 1-4), 500 mg (5-8)--then tapered. 2) ALG lg/day (day 4-8) 3) danazol 600 mg/day. During ALG administration, leukocytopenia and thrombocytopenia appeared but thereafter hematological recovery was obtained and the patient was free from supportive care. She developed mild diabetes mellitus and moderate liver dysfunction, nevertheless, both of which were controlled. At 3 months after the beginning of the treatment, hematological examination demonstrated as follows; reticulocyte 236,000/microliters, granulocyte 1,900/microliters, platelet 56,000/microliters and normocellular bone marrow. Although this immunosuppressive therapy was remarkably effective to this patient, immunological relation to the onset of aplastic anemia was not demonstrated in in vitro examination. This combined therapy seems to be effective one for patients with severe aplastic anemia.     

Effect of splenectomy for management of thrombocytopenia associated with systemic lupus erythematosus: a case report

A 51-year-old female with systemic lupus erythematosus (SLE) was admitted in November 1987 because of general fatigue and muscular weakness. She was treated with prednisolone (PSL) 30 mg and azathioprine (AZP) 50 mg after failure in the management of thrombocytopenia by PSL 15 mg. She exhibited no splenomegaly. Muscular atrophy and weakness were seen in the proximal muscles. Her platelet count was 44,000/microliters. A bone marrow aspiration revealed an increase in megakaryocytes. The blood chemistry revealed a normal CPK level and an elevated LDH level, indicating a presence of steroid myopathy. A splenectomy was performed after an increase of platelet count by giving gamma-globulin 400 mg/kg for 5 days. The platelet count rose to 368,000/microliters on the 46th postoperative day. She was treated with PSL 5 mg and AZP 50 mg as postsplenectomy therapy. The splenectomy did not adversely affect other aspects of SLE, in particular, renal function. She had no major complications in the postoperative period. Her platelet count reached a plateau 4 months later and revealed 115,000/microliters 18 months postoperatively.     

Intermittent cyclophosphamide for the treatment of autoimmune thrombocytopenia in systemic lupus erythematosus

STUDY OBJECTIVE: To determine the effect of monthly intravenous cyclophosphamide therapy in patients with systemic lupus erythematosus and autoimmune thrombocytopenia. DESIGN: Uncontrolled, retrospective clinical study. SETTING: Government referral-based research hospital. PATIENTS: Seven patients with systemic lupus erythematosus and 2 or more months of thrombocytopenia refractory to or requiring excessive doses of corticosteroids. Two patients had also failed to respond to splenectomy and repeated intravenous methylprednisolone infusions. Six patients had severe active renal disease at the time of treatment. INTERVENTIONS: Cyclophosphamide, 0.75 to 1.0 g/m2 body surface area, was given intravenously every month for at least 4 months. Prednisone dose ranged between 0.5 to 1.0 mg/kg.d. MEASUREMENTS AND MAIN RESULTS: All seven patients had normal platelet counts within 2 to 18 weeks after cyclophosphamide treatment (one to four doses). Prednisone was tapered to 0.25 mg/kg on alternate days in all patients. All six patients had significant improvement in their renal disease and lupus serologies. Cyclophosphamide was discontinued after four to six doses in five patients. Four patients maintained normal platelet counts on low dose, alternate-day prednisone for a mean of 5.6 years of follow-up. Two patients had recurrence of thrombocytopenia 1 to 3 years after discontinuing cyclophosphamide. CONCLUSIONS: Monthly intravenous cyclophosphamide is potentially useful for the management of autoimmune thrombocytopenia in patients with systemic lupus erythematosus who are refractory to or dependent on unacceptably high doses of corticosteroids, or are experiencing side effects of conventional medical or surgical treatment.     

Treatment of severe immune thrombocytopenia associated with systemic lupus erythematosus: 59 cases

OBJECTIVE: To evaluate the response to treatment in a large cohort of patients with systemic lupus erythematosus (SLE) associated with autoimmune thrombocytopenia. METHODS: Response to treatment was assessed retrospectively in 59 patients with SLE, either definite (n = 44) or incomplete (n = 15), associated with frank autoimmune thrombocytopenia (defined as platelet count < 50 x 10(9)/l). Response to treatment was classified as complete (CR: platelet count > 150 x 10(9)/l), partial (PR: platelet count > 50 x 10/l), or failure (FR) in the other cases. RESULTS: Oral prednisone alone was used in 50 of the 59 patients (mean initial dose 1 mg/kg body weight/day). A response was obtained in 80% of cases (CR in 28. PR in 12) but only 11 (22%) had a sustained response (CR, n = 7; PR, n = 4). In contrast, combined treatment with prednisone and either danazol (n = 18) or hydroxychloroquine (n = 11) resulted in 50% (7 CR, 2 PR) and 64% (4 CR, 3 PR) longterm responses, respectively, allowing prednisone to be withdrawn or the dose tapered below 0.2 mg/kg body weight/day. High dose methylprednisolone pulses (n = 10) and intravenous immunoglobulin (IVIG) (n = 31) resulted in positive responses in 60% (4 CR, 2 PR) and 65% (12 CR, 8 PR) of cases, respectively, but the response was transient in each case. Splenectomy (n = 17) resulted in 65% longterm responses (10 CR, 1 PR). Only 2 longterm partial responses were obtained with the 22 immunosuppressant-containing regimens administered to 14 patients. At the end of the study. a response was observed in 52 (88%) patients [CR: 36 (61%), PR: 16 (27%)], mainly as a result of splenectomy or combined treatment with prednisone and either danazol or hydroxychloroquine. CONCLUSION: Longterm remission was obtained in the majority of patients. The major treatments inducing remission were splenectomy and prednisone combined with danazol or hydroxychloroquine.     

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.     

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.     

High-dose intravenous gammaglobulin (IVG) in neonatal immune thrombocytopenia

Recent reports suggest that intravenous gammaglobulin (IVG) may be an effective treatment modality in patients with immune thrombocytopenia (ITP). Two newborns with isoimmune thrombocytopenia secondary to HLA-A2 and PLA1 platelet antigen incompatibilities with their respective mothers and two newborns with thrombocytopenia secondary to maternal ITP were treated with IVG 400 mg/kg/day x 5 days. One patient was exposed to steroids in utero; only one mother was thrombocytopenic at the time of delivery. All patients were severely thrombocytopenic on day 1 of treatment with mean platelet count of 5.7 x 10(9)/L. All had petechiae and positive quaiac stools, and patients with isoimmune thrombocytopenia had CT scan evidence of intracranial bleeds. The mean platelet count after 24 hr was 26.7 x 10(9)/L and the average platelet increase was 21 x 10(9)/L and 33 x 10(9)/L at 24 and 48 hr, respectively. The two cases with isoimmune thrombocytopenia had sustained platelet increases; the two cases secondary to maternal ITP had transient platelet elevations. IVG can rapidly elevate the platelet count in these patients, especially those with severe bleeding manifestations.     

Combination therapy with low-dose cyclosporin A, azathiopurine, and prednisolone for a child with refractory chronic idiopathic thrombocytopenic purpura

We report on a boy with refractory chronic idiopathic thrombocytopenic purpura (ITP) successfully treated with combination therapy composed of low-dose cyclosporin A (CsA), azathiopurine, and prednisolone. The patient was diagnosed as having ITP at 5 years of age, and received high-dose intravenous immunoglobulin (IVIG), followed by oral prednisolone, intravenous pulsed dexamethasone, oral cepharantin, and intermittent IVIG therapies. Because there were no or only transient responses to these medical therapies over 2 years, he was splenectomized. However, 3 months after the splenectomy, his platelet counts fell to below 10 x 10(3)/microl accompanied by wet purpura. We resumed low-dose intermittent IVIG treatment for 1 year without sustained efficacy. We then started combination therapy with CsA (2.5 mg/kg/day), azathiopurine (1.7 mg/kg/day), and prednisolone (0.8 mg/kg/day). Complete remission was achieved within 2 weeks and the platelet counts remained > 50 x 10(3)/microl even after tapering off the prednisolone and azathiopurine at 6 and 12 months, respectively and have moreover remained normal for more than 10 months after completion of 2 years of CsA treatment. There were no adverse events during the therapeutic course. This is the first pediatric case of ITP treated with CsA in Japan. Such combination therapy may be promising and tolerable for childhood ITP with splenectomy failure.     

Alloimmune thrombocytopenia in the fetus and newborn.

Alloimmune thrombocytopenia is an interesting and challenging disease. Identification in the fetus and newborn by screening remains to be clarified. The primary clinical criterion for neonatal diagnosis appears to be a neonatal platelet count of <50 x 10(9)/L. Treatment of the neonate can be accomplished with intravenous immunoglobulin (IVIG) +/- steroids or with matched platelet transfusion. Cranial ultrasonography is important. Testing can be performed on the parents and requires a highly experienced laboratory. If an affected fetus is identified, based on a previous affected neonate and a homozygous father, antenatal management is needed. Studies have been completed that inform the still controversial decision. IVIG remains the basis of therapy but appears to require a higher dose (2 g/kg/week) and/or the addition of 1 mg/kg of prednisone in the highest risk cases, those with antenatal intracranial hemorrhage.     

Oral high-dose methylprednisolone and intravenous immunoglobulin treatments in adult chronic idiopathic thrombocytopenic purpura

Ten adult patient of chronic idiopathic thrombocytopenic purpura (CITP) used oral prednisone and were treated with seven doses of oral high-dose methylprednisolone (30 mg/kg). Nine of ten patients responded after HDMP treatment (plt > 150 × 10⁹/L). Two patients having 8 and 10 years of history achieved long-term remission after first HDMP treatment. One unresponsive and one responsive patients did not accept IVIG treatment as second therapy and lost the follow-up. The remaining six patients received IVIG (0.5 mg/kg for 5 days) as second therapy after 3 months. Platelet count increased above 150 × 10⁹/L in 4 patients and between 60–80 × 10⁹/L in 2 patients. The peak platelet counts of both therapy users were higher under HDMP than IVIG therapy (252 ± 110.4 vs 174.2 ± 83.7 × 10⁹/L), but the difference was not significant. Responses were transient and returned to pretreatment values at 14–30 days, excluding long-term remission of 2 (2/10) patients after HDMP treatment. No side effect was observed. Thus, oral HDMP appears a good initial therapy for continuous remission in a small ratio of patients and a good security for emergency situations and prior to surgery in adult CITP patients. Am. J. Hematol. 56:191–192, 1997. © 1997 Wiley-Liss, Inc.     

Superior effect of intravenous anti-D compared with IV gammaglobulin in the treatment of HIV-thrombocytopenia: results of a small, randomized prospective comparison

This small, prospective, randomized study compared increases in platelet counts and duration of response after intravenous gammaglobulin (IVIG) and IV anti-D in patients with HIV-related thrombocytopenia (HIV-TP). Nine Rh+, nonsplenectomized HIV-positive patients with thrombocytopenia were treated sequentially, in random order, with IVIG and IV anti-D in a cross over design, receiving each therapy for 3 months. Peak platelet counts and duration of effect after each treatment were compared. In addition, viral load measurements and CD4 counts were followed serially, as well as thrombopoietin levels. IV anti-D resulted in a mean peak platelet count of 77 x 10(9)/L compared to only 29 x 10(9)/L after IVIG (P = 0.07). The mean duration of response was significantly longer in patients treated with anti-D (41 days) compared to IVIG (19 days, P = 0.01). No consistent changes were seen in the CD4 counts or viral load measurements as a result of either therapy. Thrombopoietin levels were normal in all patients despite often severe thrombocytopenia. Anti-D was more efficacious than IVIG for the treatment of HIV-TP, confirming and extending previous results. Anti-D should be the first line therapy in HIV-positive, Rh+ patients, when antiretroviral agents are not indicated, not effective, or there is an urgent need to increase the platelet count.     

Mechanisms of thrombocytopenia in patients with lymphoproliferative diseases.

Different factors are involved in the development of thrombocytopenia in patients with lymphoproliferative disorders. Significant correlation was detected between the number of megakaryocytes in bone marrow and platelet count (r = 0.485, p = 0.002, n = 37) and significant difference between the number of megakaryocyte in patients with normal platelet count (> 200,000/microliters) and patients with marked thrombocytopenia (platelet count < 100,000/microliters). All patients in the latter group (n = 15) had a relatively low number of megakaryocytes. Low but significant reverse correlation was found between the level of platelet-associated IgG (PA-IgG) and platelet count (r = -0.249, p = 0.024, n = 82) and significant difference between the mean levels of PA-IgG in the groups of patients with platelet count > 200,000/microliters and < 100,000/microliters. PA-IgG were increased in 46% of patients in the total group and in 65% of patients with platelet count < 100,000/microliters. The correlation between platelet count and PA-IgG was about 2 times higher in splenectomized (r = -0.549, p = 0.005, n = 24) than nonsplenectomized patients. All splenectomized patients with platelet count < 100,000/microliters (n = 8) had a significant increase in PA-IgG. Serum antibodies were detected in only 7% of tested patients. This group was characterized by severe thrombocytopenia (in 6 of 10 patients--platelet count < 50,000/microliters) and a high incidence of haemorrhages (in 5 of 10 patients). Thus the depression of platelet production was suggested to be the basic cause of thrombocytopenia in lymphoproliferative disorders. Involvement of immune mechanisms was revealed in a large number of patients and correlated with a deeper and more complicated thrombocytopenia.     

Oral cyclic megadose methylprednisolone therapy for chronic immune thrombocytopenic purpura in childhood

The objectives of this study were to investigate the effectiveness of oral megadose methylprednisolone (OMMP) therapy in children with chronic immune thrombocytopenic purpura (ITP). Twenty-two patients were given oral methylprednisolone daily for 7 d (30 mg/kg for 3 d and then 20 mg/kg for 4 d). OMMP therapy was repeated once per month if the platelet count was less than 20,000/mm3 at the 30th day of therapy, for up to six courses. The number of platelets of all patients increased gradually during the OMMP therapy, with a peak number at the 7th day, then decreased until the 14th day, and remained relatively stable until 12 months. During the study no patient had a platelet count less than 20,000/mm3 at the 3rd day and 50,000/mm3 at the 7th day. Although the number of platelets was gradually decreased between the 7th and 14th days, it remained above 100,000/mm3 for at least 12 months in the nine patients, and above 20,000/mm3 in the four patients. None of these 13 patients required hospitalization or therapy during the follow-up period. All of the patients tolerated the medication well. None of them reported side-effects that were severe enough to discontinue therapy. We conclude that OMMP therapy is a safe, easy and effective therapy in children with refractory chronic ITP, and it may provide long-term remission in about two thirds of the patients.     

Cyclosporin therapy for idiopathic thrombocytopenic purpura

Nine adult patients with chronic idiopathic thrombocytopenic purpura (ITP) were treated with cyclosporin. Their platelet counts were all below 5 x 10(4)/microliters. It was administered orally at 5 mg/kg/day for 8 weeks. In one patient, the platelet count increased over 10 x 10(4)/microliters in 4 patients it did over 5 x 10(4)/microliters. Gingival hyperplasia was observed in one patient. Renal dysfunction was not observed in any patients. The elevation of PAIgG declined during the period of treatment. These results suggest that this therapy may be useful in refractory idiopathic thrombocytopenic purpura.     

Effects of intravenous immunoglobulin on platelet count and antiplatelet antibody disposition in a rat model of immune thrombocytopenia

Experiments were conducted to investigate the effects of intravenous immunoglobulin (IVIG) in a rat model of immune thrombocytopenia (ITP). Rats were pretreated with 0 to 2 g/kg IVIG and then challenged with an antiplatelet antibody (7E3, 8 mg/kg). IVIG effects on 7E3-induced thrombocytopenia and on 7E3 pharmacokinetics were determined. IVIG pretreatment led to significant changes in the degree and time-course of 7E3-induced thrombocytopenia (P =.031). Nadir percent platelet counts were 121% to 279% greater in animals treated with IVIG (0.4-2 g/kg) than in animals receiving 7E3 alone. IVIG treatment also led to dose-dependent increases in 7E3 clearance (P <.001), with more than 2-fold increases in 7E3 clearance seen following the highest dose of IVIG. In vitro experiments showed that IVIG effects on platelet count are not likely due to anti-idiotypic inhibition of 7E3-platelet binding and that IVIG did not directly bind to 7E3. Consequently, IVIG-7E3 binding cannot explain the increase of 7E3 clearance following IVIG treatment. We propose that the observed increase in 7E3 clearance with IVIG therapy is due to saturation of the FcRn salvage receptor for IgG. The importance of the effect of IVIG on 7E3 clearance to the prevention of thrombocytopenia in these animals is unclear at present; nonetheless, these data provide experimental support for a new mechanism of IVIG action in ITP (ie, IVIG-mediated increases in antiplatelet antibody elimination). This model of ITP will be useful for further investigations of IVIG mechanism of action and for development of new therapies for ITP.     

Treatment for Churg-Strauss syndrome: induction of remission and efficacy of intravenous immunoglobulin therapy.

Churg-Strauss syndrome (CSS) is characterized by the presence of asthma, eosinophilia, and small-vessel vasculitis with granuloma. It is a distinct entity, as determined from all classifications of systemic vasculitis. The poor prognostic factors in CSS are renal insufficiency, cardiomyopathy, severe gastrointestinal (GI) tract, and central nervous systems (CNS) involvement. The initial management of CSS should include a high dose of a corticosteroid: prednisone at 1 mg/kg/day or its equivalent for methylprednisolone with tapering over 6 months. In patients with severe or rapidly progressing CSS, the administration of methylprednisolone pulse at 1 g/body/day for 3 days is recommended. When corticosteroid therapy does not induce remission, or when patients have poor prognostic factors, immunosuppressive cytotoxic therapy is indicated. However, some patients with severe CSS often show resistance to conventional treatment. We think that IVIG therapy is a hopeful candidate for second-line treatment for CSS patients, particularly in the case of neuropathy and/or cardiomyopathy, which are resistant to conventional therapy. However, there is not much evidence supporting the effectiveness of IVIG in CSS, and the mechanisms underlying the action of IVIG remain unclear. Now we are performing clinical trials of IVIG therapy for CSS patients who are resistant to conventional treatment, through a nationwide double-blinded placebo-controlled study in Japan.     

Treatment of autoimmune neonatal thrombocytopenia with high-dose intravenous immunoglobulins

During the neonatal period, 6 infants born to mothers with auto-immune thrombocytopenia purpura were diagnosed as having severe thrombopenia (platelet counts below 30 X 10(9)/l). High doses of intravenous immunoglobulins (IVIG) (0.4 g/kg/injection for 2 infants and 1 g/kg/injection for 4 infants) were administered as of the first week of life. Short-term efficacy was good in all cases (platelet counts over 50 X 10(9)/l, 2-10 days after the first injection). During the second week of life, 5 newborns had relapses which were rapidly reversed by one or several IVIG injections. The thrombopenia was cured between days 19 (shortest) and 41 (longest) and, more over, no side effects were observed. High doses of IVIG seem to be an effective and well-tolerated treatment for neonatal auto-immune thrombopenia, and they can be recommended over classical treatments (corticotherapy, exchange transfusion, platelet transfusion) as a first line of attack.     

Treatment of platelet alloimmunization with intravenous immunoglobulin in a child with aplastic anemia

A 9-year-old girl with severe refractory aplastic anemia had become refractory to platelet transfusion due to platelet alloimmunization. As a result, a huge right parieto-temporo-occipital cerebral hematoma and a subarachnoid hemorrhage subsequently occurred. Intravenous immunoglobulin (IVIG) (Green Cross, Taiwan) at a dose of 0.4 g/kg/day for 5 days, followed by another 3 doses during the following 5 days, and combined with large-dose platelet transfusions effectively controlled the bleeding by elevating the platelet count to above 100 × 10³/mm³. The patient soon recovered with only a mild sequela. Two months later, because of multiple caries and a periodontal abscess, dental extraction needed to be performed. IVIG (0.4 g/kg/day × 6 days) enabled surgery to proceed by elevating the platelet count to above 55 × 10³/mm³, and no bleeding complications occurred during or after the procedure. The potential benefit of high-dose IVIG in modulating platelet alloimmunization is a result of increasing the survival of transfused platelets. Thus, this therapy is recommended when patients with platelet alloimmunization have critical bleeding episodes or undergo surgical procedures. © 1995 Wiley-Liss, Inc.