Idiopathic Immune Thrombocytopenia Research Articles

Long Non-Coding RNA RP11-252C15.1 Is a Potential Biomarker of Prognosis and Hallmark for Leukemogenesis in Children with B-Cell Precursor Acute Lymphoblastic Leukemia

Introduction: Improved understanding of the prognostic biomarkers associated with childhood acute lymphoblastic leukemia (ALL) is needed for accurate risk group stratification. This study aimed to identify potential long non-coding RNA (lncRNA) markers and evaluate their prognostic value in children with ALL. Methods: We selected 50 children with newly diagnosed ALL and 20 age-matched patients with idiopathic immune thrombocytopenia (controls). RNA sequencing was performed to identify differentially expressed lncRNAs between the ALL and control groups. Correlation analysis was performed to determine the relationships between candidate lncRNAs, clinical features, and the risk of leukemogenesis. Results: A total of 1,019 differentially expressed lncRNAs were identified between the ALL and control groups. Reverse transcriptase (RT-qPCR) revealed that lncRNA RP11-252C15.1 and lncRNA RP11-701P16.2 were significantly upregulated in patients with ALL. Furthermore, correlation analysis showed that lncRNA RP11-252C15.1 and lncRNA RP11-701P16.2 represent potential predictors of leukemogenesis; however, only lncRNA RP11-252C15.1 was associated with clinical features and outcome in children with B-cell precursor ALL (BCP-ALL). In vitro experiments confirmed that lncRNA RP11-252C15.1 was significantly overexpressed in BCP-ALL cell lines and promoted proliferation and repressed apoptosis in MHH-CALL-3 cells. Conclusion: lncRNA RP11-252C15.1 is a potential oncogene in BCP-ALL pathogenesis and a prognostic biomarker in children with BCP-ALL.

CT-514 Acute Cardiac Tamponade Following Adult Allogeneic Hematopoietic Transplantation

Large pericardial effusions (LPEs) occur in <1% of the adult population. Early onset (<100 days from allogeneic hematopoietic transplantation (allo-SCT)) LPEs are related to cardiac toxicity of conditioning regimen while late LPEs (>100 from allo-SCT) are chronic GvHD related. The major determinant of acute pericardial tamponade (ACT) is the rapidity with which fluid accumulates. We herein document 4 adult patients with early LPEs that led to ACT. Interestingly, 3 of these also manifested idiopathic immune thrombocytopenia (ITP), an association that has not been previously described. Median age of 3 females and 1 male was 46 years (range 19-58). All patients received high-dose cyclophosphamide either as part of conditioning or post-transplant GvHD prevention (conditioning comprised: busulfan, fludarabine and tiotepa and post-transplantation cyclophosphamide =1, cyclophosphamide, total body irradiation and alemtuzumab =2 and cyclophosphamide and antithymocyte globulin =1). Donors comprised: matched unrelated donor =2, matched sibling donor =1 and haploidentical donor =1. Engraftment occurred on days +20, +30, +12, and +21. All four patients developed ACT at <100 days from allo-SCT on days +1, +16, + 22, and +60 after allo-SCT. ACT manifested as acute, new, or worsening shortness of breath. At ACT, all 4 were negative for CMV infection and acute GvHD (aGVHD). Pericardial fluid was serous in 2 and serosanguinous in 2 patients. Laboratory analysis did not support bacterial or viral infections and rheumatologic profile was negative. ACT was managed by cardiologist who performed either pericardiocentesis with pericardial drain or pericardial window. A cumulative total of 1700 ml, 1500 ml, 1500 ml and 300 ml pericardial fluid was removed. LPEs reoccurred in 2 patients; one with ACT physiology required pericardial window. Additionally, 3 patients developed ITP after development of ACT. The median time to ITP was day+30 (30, 30 and 75 days) from engraftment. Bone marrow biopsies in patients who developed ITP revealed adequate megakaryocytes. ITP responded promptly to steroids, thrombopoietin receptor agonists and IVIG. Prompt cardiac evaluation with doppler echocardiogram facilitates lifesaving management of ACT. Early onset LPEs in our patients was not associated with aGVHD; it is more likely to be associated with cardiac toxicity of cyclophosphamide. Association with ITP may reflect shared pathogenesis.

Cytopenias in CAR-T Cell Therapy and Use of Transfusion Resources in Adult Patients with Lymphoproliferative Disorders

BACKGROUND AND OBJECTIVESCD19-directed chimeric antigen receptor (CAR)-T-cell therapy is associated with early cytopenia in approx. 80% of patients and late cytopenia in 40% of patients. To date, the transfusion profile of patients undergoing these therapies has not been described. Our objectives are: •To define the transfusion profile of CAR-T therapy in our setting.•To evaluate clinical variables related to the intensity and duration of cytopenias.METHODOLOGYThis is an observational, prospective, single centre study. Adult patients with lymphoproliferative disorders and acute lymphoblastic leukaemia proposed for CAR-T therapy from August 2019 to March 2021 were included.RESULTS68 patients were included in the CAR-T cell therapy program. Table 1 describes patients characteristics. Forty-seven received CART therapy, apheresis was not performed in 7 cases (progression n=6, withdrawal of consent n=1), product was not infused in 9 patients due to disease progression and 5 patients are awaiting infusion.Cytokine release syndrome (CRS) occurred in 79% of patients, 25% developed grade≥2 and median time to onset was 2 days (IQR, 1-4 days). Immune effector cell-associated neurotoxicity syndrome (ICANS) occurred in 33% of patients, 16% were grade≥3 and median time to onset was 7 days (IQR, 6-8 days). Median duration of hospital admission was 14 days (IQR, 12 to 21 days).None of the 47 infused patients had neutropenia or anemia in the transfusion range prior to lymphodepletion. 94% (44/47) developed grade ≥3 neutropenia, with a median duration of 14.5 days (IQR, 9.75 to 36 days). G-CSF was administered to 21 patients, with a median time on G-CSF of 115 days (IQR, 27-258 days). A total of 239 red blood cells units (RBC) have been transfused, median 3.5 RBC/patient (IQR, 0 to 7.5 RBC / patient). 70% of this consumption occurred in the first 30 days after infusion.Before CAR T cell infusion, 2 patients had a platelet count <20x10 9/L, and 3 between 30 and 50x10 9/L, mean platelets (SD) prior to lymphodepletion was 153 (+/-106) x10 9/L. Grade ≥3 thrombocytopenia occurred in 53% of patients, median time to reach platelets > 20x10 9/L was 17 days (IQR, 4-54 days) and > 50x10 9/L was 39 days (IQR, 20-79 days). 62% of patients received ≤2 platelet concentrates (PC). However, 21% had long-term thrombocytopenia (> 30 days). This group received a median of 15 PC (IQR, 12-27). The amount of PC used was 198, 52% were administered in the first 30 days. Eltrombopag was indicated in 9 of the 25 patients with grade ≥3 thrombocytopenia, doses never exceeded 75 mg/day. No serious bleeding (WHO grade ≥2) was observed.Factors related to severe thrombocytopenia were female (74% vs 42%, p=0.036), CRS (61% vs 0%, p=0.019), ICANS (80% vs 43%, p=0.020) and use of steroids (92% vs 67%, p=0.003). We found no relationship with underlying disease or status, prior treatment lines, prior autologous transplantation, CAR-T product, duration of lymphopenia, time and duration of CRS or ICANS.With a median follow-up of 167 days (IQR, 73-303 days), 26% of patients died, causes of death were infections n=2 and disease progression n=10. Three months after the procedure, there were 30 evaluable patients for response and 64% remained in remission (47% CR, 17% PR).CONCLUSIONSA high percentage of patients receiving CAR T cell therapy present long-term cytopenias. Reserves of irradiated blood components must be foreseen. Development of CRS and ICANS could be prognostic factors for the development of long-term grade ≥3 thrombocytopenia. Studies are needed on the use of thrombopoietin receptor agonists to reduce the duration of thrombocytopenia, although effective doses could be higher than those used in idiopathic immune thrombocytopenia. [Display omitted] DisclosuresReguera: Janssen, Kite/Gilead, Novartis: Speakers Bureau; BMS-Celgene, Novartis: Membership on an entity's Board of Directors or advisory committees. Perez-Simon: JANSSEN, TAKEDA, PFIZER, JAZZ, BMS, AMGEN, GILEAD: Other: honorarium or budget for research projects and/or participation in advisory boards and / or learning activities and / or conferences.

Detection and dynamics of anti-platelet antibodies in thrombocytopenic dogs with and without idiopathic immune thrombocytopenia.

BackgroundAntiplatelet antibodies are detected in multiple diseases including primary immune thrombocytopenia (ITP). Dynamics of how these antibodies change over time in ITP is unknown in dogs.Hypothesis/ObjectivesAntiplatelet antibodies (APA) will be detected in thrombocytopenic dogs with multiple etiologies and dynamics of APA in dogs with ITP can be used to evaluate response to treatment and relapse. Determine APA at the time of diagnosis in thrombocytopenic dogs and serially in primary ITP dogs.AnimalsSeventy‐nine thrombocytopenic dogs and 28 primary ITP dogs.MethodsDirect flow cytometry was performed in thrombocytopenic dogs at initial evaluation and serially in suspected primary ITP dogs. In primary ITP dogs, a 2‐tailed Fisher's exact test was performed comparing survival to discharge between dogs with and without melena and to relate response to treatment and relapse to changes in APA and platelet count (repeated measures analysis, Spearman correlation).ResultsTwenty percent (16/79) of thrombocytopenic non‐ITP dogs with infectious, neoplastic, or other diseases and all primary ITP dogs were positive for APA. Melena at initial evaluation was associated with decreased survival to discharge (odds ratio 0.06; P = .01). Persistence of APA was not associated with response to treatment, but recurrence of antibodies was associated with relapse (odds ratio 205.0; P < .01). There was no difference in percentage of APA or platelet count at initial diagnosis between dogs that did or did not respond to treatment.Conclusions and Clinical ImportanceSerial monitoring of APA in dogs with primary ITP appeared beneficial for determining relapse of disease.

A Meta-Analysis on the Efficacy of Thrombopoietin (TPO) Agonists in Reducing the Need of Platelet Transfusion before Procedures in Chronic Liver Disease Patients

Introduction: Chronic liver disease (CLD) results in thrombocytopenia [1]. Depression of thrombopoietin (TPO) levels in CLD leads to thrombocytopenia. Thrombocytopenia increases the risk of bleeding in patients undergoing invasive diagnostic or therapeutic procedures [2]. TPO agonists have been depicted to be safe and efficacious in the treatment of thrombocytopenia due to idiopathic immune thrombocytopenia and aplastic anemia. Several studies have evaluated the use of TPO agonist versus placebo for pre-procedural treatment of thrombocytopenic patients with chronic liver disease. These studies were of limited sample sizes. We conducted a meta-analysis to determine if TPO agonists reduce the need of platelet transfusions before procedures in chronic liver disease patients. Methods: We performed a computerized search of Medline, Cochrane and Google Scholar databases through March 2019 for studies evaluating the efficacy of thrombopoietin (TPO) agonists versus placebo in the treatment of thrombocytopenia of chronic liver disease patients undergoing procedures. We screened 295 studies and only 5 of them met our inclusion and exclusion criteria. Inclusion criteria were studies with adults &gt;18 years old, English literature, randomized controlled trials utilizing a TPO agonists in chronic liver disease patients. Exclusion criteria were studies with patients &lt;18 years old, improper study design (e.g. abstract, commentary, editorials) and literature without available data. The primary study outcomes were proportion of patients responding to TPO agonists in comparison to placebo (an increase of 20000 of platelet count /ul and a value of more than 50000 count/ul). Our secondary outcomes were evaluating the proportion of patients not requiring platelet transfusion and evaluating the percentage of patients who developed a portal venous thrombosis (PVT) on study. Statistical analyses were calculated using Cumulative Meta-Analysis software (version 3.0) Results: The final analysis included 5 studies with a total of 1098 patients. Please see Image 1 for Individual Trial data. The proportion of responders for TPO agonists odds ratio was 0.060 (0.038-0.095) with P value &lt;0.0001. The proportion of patients not requiring transfusion on TPO agonist odds ratio was 0.118 (0.077-0.182) with P value &lt;0.0001. The portal vein thrombosis odds ratio while on TPO agonist was 0.631 (0.175-2.268) with P value of 0.480. All 5 trials demonstrated that TPO agonists compared to placebo significantly increased the platelet count and reduced the need of platelet transfusion pre-procedural with a P-value of &lt;0.0001. The risk of developing PVT in patients receiving TPO agonists was not significantly increased when compared to those receiving placebo. Conclusion: Our analysis shows that TPO-agonists provide significant benefit compared to placebo in regard to increasing platelet count and reducing the need for platelet transfusions. The risk of developing PVT in patients receiving TPO agonists was not significantly increased when compared to those receiving placebo. More trials are needed to validate the comparison for PVT outcome. Figure Disclosures Jaglal: NOVARTIS: Consultancy.

Levels of alloantibodies to human platelet antigen in relation to blood dyscrasia occurring at various trimesters of pregnancy

Context Thrombocytopenia is major complication in complicated and uncomplicated pregnancies. Aims The aim of this study was to determine the incidence of thrombocytopenia in pregnancy and determine possible immune thrombocytopenia correlates with blood dyscrasia involving other cellular components of blood. Settings and design The study was an institution-based cross sectional study. Participants and methods The full blood count of 200 women attending an antenatal center was determined. Sera of 73 (36.5%) thrombocytopenic women defined by a platelet count less than 80×10 9 /l and inadequate platelet in the peripheral blood film were evaluated for circulating antiplatelet antibody directed against platelet membrane glycoprotein GPIIb/llla. Statistical analysis Pearson and Kendall's tau-b correlation analysis was carried out on data using the SPSS (PASW version 18) statistical package. Results The antibody was detected in two (3%), 13 (17%), and 44 (60%) of the subset of patients in the first, second, and third trimester, respectively. Fourteen (20%) of the women had no detectable antiplatelet antibody. The idiopathic immune thrombocytopenia observed in the patients was associated with anemia and leukopenia in four (9%) patients in the third trimester of pregnancy. A significant correlation was observed between thrombocytopenia and the red cell count, hemoglobin concentration, and packed cell volume of the women. Irregular attendance at an antenatal clinic and nonutilization of hematinic were evident in groups of thrombocytopenic and anemic women. There was a negative correlation between the occurrence of antiplatelet antibody and the severity of thrombocytopenia. Conclusion There is a need to distinguish immune thrombocytopenia from gestational thrombocytopenia and assess other blood parameters in pregnancy for a proper clinical intervention.

Thrombocytopenia in pregnancy - pathogenesis and diagnostic approach.

Thrombocytopenia (TP) affects 7-10% of pregnant women. It occurs 4 times more frequently in pregnancy than in the non-pregnant women population. Women with thrombocytopenia in pregnancy are a heterogeneous and poorly known group. There are several possible causes of thrombocytopenia in pregnancy. The most common are: gestational thrombocytopenia (GE) (60-75%), preeclampsia (PE) and HELLP (hemolysis, elevated liver enzymes, low platelets) syndrome associated TP (21%), and idiopathic immune thrombocytopenia (ITP) (3-10%). Although thrombocytopenia diagnosed in pregnancy in most cases has a mild course, it has also been reported to be associated with a higher rate of preterm birth and premature detachment of the placenta. Some cases of severe thrombocytopenia with systemic involvement are associated with high risk of serious perinatal complications and require early diagnosis, careful clinical monitoring and medical treatment. The differential diagnosis and proper assessment of clinical risk of TP during pregnancy may be of great concern. The article discusses these issues, focusing on pathophysiology of TP in pregnancy.

Current treatment options for primary immune thrombocytopenia

Traditional treatment of primary (idiopathic) immune thrombocytopenia (ITP) predominantly consists of immune suppression and/or modulation. In addition, many treated patients develop severe adverse effects, and approximately a third of patients do not respond. Two of the newly developed thrombopoietin-receptor agonists, romiplostim and eltrombopag, are now available for the treatment of ITP. Both drugs have been shown to increase the production of platelets in a dose-dependent manner, and to compensate, at least partly, for thrombocytopenia in the majority of ITP patients. The reported adverse effects are predominantly mild, although serious and long-term side effects cannot be excluded. Nevertheless, these drugs are increasingly used in the treatment of patients with thrombocytopenias. Thrombopoietin-receptor agonists do not appear to stop either the production of autoantibodies or the accelerated platelet destruction observed in ITP. Thus, the need for a specific therapy is essential, and the ultimate solution is to clarify and halt the mechanism(s) that lead to the development of ITP.

Hematologic Adverse Effects of Clopidogrel

Clopidogrel is used as a frontline antiplatelet agent in patients with coronary artery disease, cerebrovascular disease, and peripheral vascular disease. Hematologic complications and bleeding have been the most feared outcome of antithrombotic and antiplatelet agents. Among the thienopyridines, clopidogrel is considered to be a safer alternative to ticlopidine due to its decreased incidence of hematologic adverse effects. Although thrombotic thrombocytopenia purpura is the most reported hematologic adverse effect of clopidogrel; neutropenia, acquired hemophilia, isolated thrombocytopenia or idiopathic immune thrombocytopenia, and thrombotic thrombocytopenia purpura with hemolytic uremic syndrome are other rare yet recognized hematologic adverse effects of clopidogrel. Patients treated with clopidogrel should be carefully monitored for hematologic adverse effects especially in the first 2 to 3 months after initiation of therapy. Early recognition and prompt initiation of treatment can be life saving in patients who have hematologic adverse effects to clopidogrel. We have drafted this review by performing literature search using Medline, Pubmed, and EMBASE search engine with relevant search words for all reported hematologic adverse effects and manifestations of clopidogrel and their management.

Glycocalicin in the diagnosis and management of immune thrombocytopenia.

We studied glycocalicin (GC), expressed as plasma GC concentration and as GC index (ratio to platelet count), in 129 thrombocytopenic patients (platelet count < 100 x 10(9)/l) and 60 sex- and age-matched controls. Seventy-two patients had idiopathic immune thrombocytopenia, 32 secondary immune thrombocytopenia, 8 microangiopathic thrombocytopenia and 17 thrombocytopenia secondary to bone marrow aplasia. Patients with immune thrombocytopenia (ITP) were also subclassified, according to their clinical behaviour, as having active disease or being in spontaneous or therapy-induced partial remission. A significant correlation was found between glycocalicin levels and platelet count both in normals and in patients with bone marrow aplasia (r = 0.75). ITP patients showed a GC index significantly higher than controls (6.02+/-7.87 vs. 0.9+/-0.2, p<0.001). When ITP patients with similar platelet count (30-50 x 10(9)/l) were studied, the mean level of GC and the GC index were significantly higher in those patients with active disease than in those in remission (0.97+/-0.38 vs. 0.58+/-0.17 microg/ml, p <0.05; 6.41+/-2.64 vs. 3.44+/-0.94, p<0.05, respectively). A longitudinal study performed in 10 patients with different subtypes of ITP suggested a positive correlation between GC index and the activity of the disease. The GC value and GC index were significantly higher in patients with microangiopathic thrombocytopenia than in controls (1.44+/-0.73 vs. 0.8+/-0.16 microg/ml, p < 0.01; and 18.77+/-22.23 vs. 0.9+/-0.2, p<0.001, respectively). The GC value was significantly lower in bone marrow failure (0.15+/-0.04 microg/ml, p<0.01) compared to controls, while no difference was observed in the GC index. Our data confirm that the GC index is helpful in differentiating thrombocytopenia due to increased platelet destruction from the one due to impaired production. In addition, the assay has been proven useful in the differential diagnosis of different ITP subtypes and their follow-up.

Failure of pulse high-dose dexamethasone in chronic idiopathic immune thrombocytopenia

Idiopathic thrombocytopenic purpura (ITP) is a disorder characterized by increased platelet destruction in the setting of normal megakaryopoiesis. Approximately 20% of patients with ITP are refractory to corticosteroids and splenectomy. Recently, pulse high-dose dexamethasone was reported to be effective in the treatment of chronic ITP in adult patients. We treated 9 patients with severe chronic ITP with monthly high-dose dexamethasone. None of the 9 patients responded with a sustained increase in platelet count. Five of these patients were unable to tolerate the regimen. The failure of high-dose dexamethasone in our hands contrasts with the good results of an earlier publication and suggests that there could be a subset of responders who will require better identification.

Failure of pulse high‐dose dexamethasone in chronic idiopathic immune thrombocytopenia

Idiopathic thrombocytopenic purpura (ITP) is a disorder characterized by increased platelet destruction in the setting of normal megakaryopoiesis. Approximately 20% of patients with ITP are refractory to corticosteroids and splenectomy. Recently, pulse high-dose dexamethasone was reported to be effective in the treatment of chronic ITP in adult patients. We treated 9 patients with severe chronic ITP with monthly high-dose dexamethasone. None of the 9 patients responded with a sustained increase in platelet count. Five of these patients were unable to tolerate the regimen. The failure of high-dose dexamethasone in our hands contrasts with the good results of an earlier publication and suggests that there could be a subset of responders who will require better identification. Am. J. Hematol. 54:267–270, 1997. © 1997 Wiley-Liss, Inc.

Prospective evaluation of the clinical usefulness of an antigen- specific assay (MAIPA) in idiopathic thrombocytopenic purpura and other immune thrombocytopenias

The diagnosis of idiopathic immune thrombocytopenia remains a clinical diagnosis based on the exclusion of other causes of immune and nonimmune thrombocytopenia. Measurement of platelet-associated Ig (PAIg), while sensitive, is nonspecific for the diagnosis of immune thrombocytopenia. Published experience of antigen capture assays (including monoclonal antibody immobilization of platelet antigens or MAIPA) suggest a high sensitivity and specificity (70% to 80%) in selected groups of patients. In a prospective evaluation of 158 patients with thrombocytopenia from all causes, we report a sensitivity of 51% and specificity of 80% for direct MAIPA assays. MAIPA was considerably better in discriminating immune from nonimmune thrombocytopenia than two assays of PAIgG. Antiplatelet antibodies detected by MAIPA were more frequently directed against the glycoprotein (GP) IIb/IIIa than the GP Ib/IX complex. Our experience suggests that MAIPA assays are useful in the laboratory assessment of thrombocytopenia, should be performed before therapy, and that some patients with ‘nonimmune’ thrombocytopenia may have genuine antiplatelet antibodies.

Prospective evaluation of the clinical usefulness of an antigen- specific assay (MAIPA) in idiopathic thrombocytopenic purpura and other immune thrombocytopenias

The diagnosis of idiopathic immune thrombocytopenia remains a clinical diagnosis based on the exclusion of other causes of immune and nonimmune thrombocytopenia. Measurement of platelet-associated Ig (PAIg), while sensitive, is nonspecific for the diagnosis of immune thrombocytopenia. Published experience of antigen capture assays (including monoclonal antibody immobilization of platelet antigens or MAIPA) suggest a high sensitivity and specificity (70% to 80%) in selected groups of patients. In a prospective evaluation of 158 patients with thrombocytopenia from all causes, we report a sensitivity of 51% and specificity of 80% for direct MAIPA assays. MAIPA was considerably better in discriminating immune from nonimmune thrombocytopenia than two assays of PAIgG. Antiplatelet antibodies detected by MAIPA were more frequently directed against the glycoprotein (GP) IIb/IIIa than the GP Ib/IX complex. Our experience suggests that MAIPA assays are useful in the laboratory assessment of thrombocytopenia, should be performed before therapy, and that some patients with 'nonimmune' thrombocytopenia may have genuine antiplatelet antibodies.

Antiplatelet antibody testing in thrombocytopenic pregnant women

OBJECTIVE: The purpose of the study was to attempt to distinguish pregnant women with gestational thrombocytopenia from those with idiopathic immune thrombocytopenia by eight different platelet antibody assays. STUDY DESIGN: Sera from pregnant women with presumed gestational thrombocytopenia ( n = 160) and idiopathic immune thrombocytopenia ( n = 90) were prospectively tested for indirect and platelet-associated immunoglobulins G and M and complement C3, as well as for serotonin release. After the results were analyzed, a subset of patients were subsequently analyzed for circulating antiplatelet antibody directed against platelet membrane glycoprotein GPIIb/IIIa. RESULTS: Indirect immunoglobulin G was significantly greater in the 85 women with idiopathic immune thrombocytopenia than in the 129 women with gestational thrombocytopenia ( p < 0.001). Platelet-associated immunoglobulin G was elevated in the majority of women, both those with gestational thrombocytopenia and those with idiopathic immune thrombocytopenia. There were also no statistically significant differences in the values for platelet-associated C3 or indirect immunoglobulin M and C3. Levels of platelet-associated immunoglobulin M showed a tendency to be higher in women with gestational thrombocytopenia ( p = 0.04), as did the values in the serotonin release assay ( p = 0.06). CONCLUSION: Our data demonstrate that patients with gestational thrombocytopenia had surprisingly high levels of platelet-associated immunoglobulin despite mild thrombocytopenia. Comparison of a relatively large number of patients with idiopathic immune thrombocytopenia and gestational thrombocytopenia indicates that women with idiopathic immune thrombocytopenia cannot be distinguished from those with gestational thrombocytopenia by means of one or more of the prototypic platelet antiglobulin tests currently in use. Our preliminary data with glycoprotein-specific assays indicate that they may be more useful. (AM J OBSTET GYNECOL 1996;174:1014-8.)

The effect of a single course of alpha-2B-interferon in patients with HIV-related and chronic idiopathic immune thrombocytopenia.

13 patients with HIV-related immune thrombocytopenia (HIV-ITP) and 10 patients with chronic idiopathic thrombocytopenic purpura (C-ITP) were treated with a single course of alpha-2b-Interferon (IFN 3 x 10(6) IU subcutaneously for 12 d). The patients had platelet counts lower than 40 x 10(9)/L and thrombocytopenia persisting for over 1 year (range 1-22 years); 7 patients were refractory to previous conventional therapy, 5 were responsive, and 11 had not been previously treated. The response to IFN was complete in 8 patients (platelets > 100 x 10(9)/L), partial in 7 (platelets 50-100 x 10(9)/L); 8 patients showed no response. The treatment with IFN was stopped after 4 d in one patient due to a fall in platelet count. The maximal platelet count (median peak 116 +/- 55 SD x 10(9)/L platelets) was obtained after 13.7 +/- 2.98 d and the improvement in platelet count was maintained for 22.8 +/- 8.6 d. No difference in platelets response was observed between HIV-ITP and C-ITP. The response to IFN seems to be related to the one obtained with previous treatments. Indeed 80% of the patients who were responsive to previous steroids, high dose immunoglobulins or azidothymidine (HIV-ITP) showed a complete or partial response while only 43% of the refractory patients showed a partial response; the positive response rate in previously untreated patients was 73%.

Letter to the editor: Recombinant interferon alpha‐2B in refractory idiopathic immune thrombocytopenia

Letter to the editor: Recombinant interferon alpha‐2B in refractory idiopathic immune thrombocytopenia

Improvement of Platelet Counts in Steroid-Unresponsive Idiopathic Immune Thrombocytopenic Purpura After Short-Course Therapy With Recombinant α 2b Interferon

In 13 patients with severe steroid-refractory idiopathic immune thrombocytopenia (ITP), a short course of recombinant alpha 2b interferon (IFN), given at a dose of 3 MU for 12 doses, caused a significant increase in platelet count in 11 patients. The rise in platelet count occurred following completion of the short course of IFN in 10 patients and occurred during therapy in one patient. Three patients showed an increase to normal platelet counts within 14 days of discontinuing the drug, eight showed a partial response, with a platelet count increase from 30 to 100 x 10(9)/L, and two patients showed minimal response. One complete responder relapsed at 5 months from initial response, and a further course of alpha 2b IFN caused a second prompt response with a rise of platelet count to supranormal levels. Short-course alpha 2b IFN can be recommended as a therapy for severe ITP. Responses are seen in splenectomized and nonsplenectomized subjects, and thrombocytopenia is not exacerbated during treatment.

Improvement of platelet counts in steroid-unresponsive idiopathic immune thrombocytopenic purpura after short-course therapy with recombinant alpha 2b interferon [see comments

In 13 patients with severe steroid-refractory idiopathic immune thrombocytopenia (ITP), a short course of recombinant alpha 2b interferon (IFN), given at a dose of 3 MU for 12 doses, caused a significant increase in platelet count in 11 patients. The rise in platelet count occurred following completion of the short course of IFN in 10 patients and occurred during therapy in one patient. Three patients showed an increase to normal platelet counts within 14 days of discontinuing the drug, eight showed a partial response, with a platelet count increase from 30 to 100 x 10(9)/L, and two patients showed minimal response. One complete responder relapsed at 5 months from initial response, and a further course of alpha 2b IFN caused a second prompt response with a rise of platelet count to supranormal levels. Short-course alpha 2b IFN can be recommended as a therapy for severe ITP. Responses are seen in splenectomized and nonsplenectomized subjects, and thrombocytopenia is not exacerbated during treatment.

Emergency caesarian section and symptomatic immune thrombocytopenic purpura

A 24-year-old woman presented in labour with profound purpuric bleeding due to idiopathic immune thrombocytopenia. She was assessed as requiring immediate caesarean section for cephalopelvic disproportion and foetal distress. In view of her platelet count of 21 x 10(9)/L she was prepared with intravenous methylprednisolone, isovolaemic plasma exchange, and infusion of 400 mg/kg of gammaglobulin and 5 x 10(11) allogeneic platelets. Six hours after commencing the procedure, at a time when her platelet count was 97 x 11(9)/L, she went uneventful operation and with no further therapy this level subsequently reached 175 x 10(9)/L, at which time an uneventful operation was performed. The mother and her normal, full-term infant have been discharged; both are well and at follow-up have normal platelet counts. This experience illustrates that major surgery can safely be undertaken in severely thrombocytopenic patients, even as an emergency procedure, using this regimen.