Cases Of Idiopathic Thrombocytopenic Purpura Research Articles

IDIOPATHIC THROMBOCYTOPENIC PURPURA(ITP) AND ROLE OF A UNANI DRUG IN ITS MANAGEMENT. A SINGLE CASE STUDY

Idiopathic Thrombocytopenic Purpura (ITP) is immune- mediated with involvement of autoantibodies, most often directed against the platelet membrane glycoprotein IIb/IIIa, which sensitise the platlet, resulting in premature removal from the circulation by cells of the reticulo-endothelial system.ITP is a blood disorder that causes a decrease in the number of platelets in the blood. Platelets help stop bleeding. So, a decrease in platelets can result in easy bruising, bleeding gums, and bleeding inside the body. The lower the platelet count, the greater the risk of bleeding. ITP may be acute or chronic. Eventhough, most cases of ITP are self-limiting, but treatment include steroids, other drugs and in resistant cases, splenectomy is the treatment option. Even though classical Unani texts has no mention of any such disease, but this disease can be discussed under the title of bleeding disorders.A single Unani drug was selected in a dosage of 500 mgs twice daily in a patient who was otherwise advised splenectomy for the disease.

Regulatory T cell CD4 ‏&CD25‏ expression and chemokine C-X-C ligand 13 level before and after corticosteroid therapy in pediatric ITP patients

Childhood Idiopathic thrombocytopenic purpura (ITP) is one of the most commons autoimmune bleeding disorders characterized by isolated, immune-mediated low platelet count, the T-follicular helper (Tfh) cells are a subset of effector CD4 (+) T cells, that plays a pivotal role in maintaining self-tolerance, deregulation of Tfh activities has a key role in immune process taking place in ITP in which the production of platelet autoantibodies might be caused by cytokine network dysregulation. The objective of our study was to analyze the relationship of Tfh cells CD4&CD25 and C-X-C ligand 13 (CXCL13) expressions before and after steroid thereby in pediatric ITP. Material and method: the study included 45 pediatric patients with acute ITP and 20 healthy controls; we used flowcytometry to assess percentages of CD4‏ and CD25‏ cells as markers of regulatory T cells, also, the serum level of interleukin- CXCL13 was measured by ELISA at diagnosis and after 4 weeks receiving corticosteroid. Results: the expression of CD4‏ & CD25‏ ‏ markers were significantly reduced in ITP cases, who also showed an elevated CXCL13 level in comparison to controls, however, the level of CXCL13 declined after treatment, the CXCL13 optimum cut off point for predicting ITP response to therapy was determined to be 90 pg/ml with AUC 0.976, Sensitivity 88.89% and Specificity 100% P-value < 0.00. Conclusion: Serum CXCL-13 levels could be used as a significant predictor of response to therapy in ITP patients, CD4&‏CD25 expression has a role in the pathogenesis of childhood acute ITP principally linked to the level of platelet count drop.

Characteristics of functional somatic syndromes and bodily distress syndrome in the general Danish population–The DanFunD study

Childhood Idiopathic thrombocytopenic purpura (ITP) is one of the most commons autoimmune bleeding disorders characterized by isolated, immune-mediated low platelet count, the T-follicular helper (Tfh) cells are a subset of effector CD4 (+) T cells, that plays a pivotal role in maintaining self-tolerance, deregulation of Tfh activities has a key role in immune process taking place in ITP in which the production of platelet autoantibodies might be caused by cytokine network dysregulation. The objective of our study was to analyze the relationship of Tfh cells CD4&CD25 and C-X-C ligand 13 (CXCL13) expressions before and after steroid thereby in pediatric ITP. Material and method: the study included 45 pediatric patients with acute ITP and 20 healthy controls; we used flowcytometry to assess percentages of CD4‏ and CD25‏ cells as markers of regulatory T cells, also, the serum level of interleukin- CXCL13 was measured by ELISA at diagnosis and after 4 weeks receiving corticosteroid. Results: the expression of CD4‏ & CD25‏ ‏ markers were significantly reduced in ITP cases, who also showed an elevated CXCL13 level in comparison to controls, however, the level of CXCL13 declined after treatment, the CXCL13 optimum cut off point for predicting ITP response to therapy was determined to be 90 pg/ml with AUC 0.976, Sensitivity 88.89% and Specificity 100% P-value < 0.00. Conclusion: Serum CXCL-13 levels could be used as a significant predictor of response to therapy in ITP patients, CD4&‏CD25 expression has a role in the pathogenesis of childhood acute ITP principally linked to the level of platelet count drop.

The association between idiopathic thrombocytopenic purpura and cardiovascular disease: a retrospective cohort study

Background Idiopathic thrombocytopenic purpura (ITP) is classically characterized by a transient or persistent decrease of platelet count. Mortality is higher in the ITP population than the general population, with a possible association with increased cardiovascular disease (CVD). Objectives The objective was to assess the strength of the association between ITP and CVD, with a secondary aim to assess the impact of splenectomy on CVD. Methods A population-based retrospective, open cohort study using clinical codes was performed using data from 6591 patients with ITP and 24 275 randomly matched controls (up to 1:4 ratio matched by age, sex, body mass index and smoking status). The main outcome was the risk of CVD, which included ischemic heart disease, stroke, trans-ischemic attack and heart failure. Adjusted incidence rate ratios were calculated using Poisson regression. Results During a median 6-year observation period there was a CVD diagnosis recorded in 392 (5.9%) ITP patients and 1114 (4.5%) control patients. There was an increased risk of developing CVD in the ITP cohort (incidence rate ratio [IRR], 1.38; 95% confidence interval [CI], 1.23-1.55), which remained robust even after a sensitivity analysis only including incident cases of ITP. Findings suggested that patients who had undergone splenectomy were at even further increased risk of developing CVD when compared with the ITP population who had not undergone splenectomy (adjusted IRR, 1.69; 95% CI, 1.22-2.34). Conclusion There is an increased risk of developing CVD in patients with ITP and even further increased risk for those patients with ITP who underwent splenectomy.

Zika virus and autoimmunity. One-step forward.

Zika virus (ZIKV) infection has been associated with the development of Guillain-Barré syndrome (GBS) and idiopathic thrombocytopenic purpura (ITP). Whether ZIKV infection is related to other autoimmune diseases is unknown. Therefore, an association study to evaluate rheumatic and thyroid autoimmunity in patients with ZIKV disease was conducted through a panel of 14 autoantibodies. In addition, a literature review on ZIKV, and GBS and ITP was performed. Our results disclosed a lack of association of rheumatoid and thyroid autoimmunity with ZIKV disease. A total of 272 cases of GBS related to ZIKV were retrieved from the literature, the majority of them being males (54.8%). Electrophysiological findings indicated acute inflammatory demyelinating polyneuropathy as the most frequent subphenotype (75.7%). Up to date, twenty-four cases of ITP in patients with ZIKV disease have been published. Although a few fatal cases have been observed, most of the reported patients responded well to immunomodulatory treatment. A review of the mechanisms incriminated into the development of autoimmune phenomenon in ZIKV disease indicates molecular mimicry as the most plausible one. Nevertheless, more research aimed at deciphering ZIKV disease pathogenesis and its relationship with autoimmunity is warranted.

Thrombocytopenia in patients with melanoma receiving immune checkpoint inhibitor therapy

BackgroundImmune checkpoint inhibitors, including antibodies against programmed death 1 (PD-1) and cytotoxic T-lymphocyte antigen 4 (CTLA-4), are being used with increasing frequency for the treatment of cancer. Immune-related adverse events (irAEs) including colitis, dermatitis, and pneumonitis are well described, but less frequent events are now emerging with larger numbers of patients treated. Herein we describe the incidence and spectrum of thrombocytopenia following immune checkpoint inhibitor therapy and two severe cases of idiopathic thrombocytopenic purpura (ITP).Case presentationsA 47-year-old female with recurrent BRAF mutant positive melanoma received combination anti-PD-1 and anti-CTLA-4. Two weeks later, she presented with mucosal bleeding, petechiae, and thrombocytopenia and was treated with standard therapy for ITP with steroids and intravenous immunoglobulin (IVIG). Her diagnosis was confirmed with bone marrow biopsy, and given the lack of treatment response, she was treated with rituximab. She began to have recovery and stabilization of her platelet count that ultimately allowed her to be retreated with PD-1 inhibition with no further thrombocytopenia. A second patient, a 45-year-old female with a BRAF wild-type melanoma, received anti-PD-1 monotherapy and became thrombocytopenic 43 days later. Three weeks of steroid treatment improved her platelet count, but thrombocytopenia recurred and required additional steroids. She later received anti-CTLA-4 monotherapy and developed severe ITP with intracranial hemorrhage. Her ITP resolved after treatment of prednisone, IVIG, and rituximab and discontinuation of checkpoint inhibition. In a retrospective chart review of 2360 patients with melanoma treated with checkpoint inhibitor therapy, <1% experienced thrombocytopenia following immune checkpoint inhibition, and of these, most had spontaneous resolution and did not require treatment.ConclusionsThrombocytopenia, especially ITP, induced by immune checkpoint inhibitors appears to be an uncommon irAE that is manageable with observation in mild cases and/or standard ITP treatment algorithms. In our series, the majority of patients had mild thrombocytopenia that resolved spontaneously or responded to standard corticosteroid regimens. However, in two severe cases, IVIG and rituximab, in addition to steroids, were required. Checkpoint inhibition was resumed successfully in the first patient but rechallenge was not tolerated by the second patient.

Pneumococcal 13-Valent Conjugate Vaccine (Prevnar 13)-Associated Immune Thrombocytopenic Purpura in a Renal Transplant Recipient: A Case Report

BackgroundPneumococcal 13-valent conjugate vaccine (PCV13) is used for immunization to prevent invasive disease caused by Streptococcus pneumoniae. Rare cases of idiopathic thrombocytopenic purpura (ITP) after vaccination with PCV13 have been reported. MethodsA case of ITP associated with PCV13 administration in a renal allograft recipient is described. A 77-year-old man presented with bruising at insulin injection sites for 1 week. He had end-stage renal disease and received a kidney transplant 22 months previously. Maintenance immunosuppression included tacrolimus and prednisone. ResultsPhysical examination showed normal vital signs and petechial rash in the areas of insulin injections. Laboratory testing resulted in hemoglobin, 11.7 g/dL; white blood cell count, 7700/mm3; and platelet count, 3000/mm3 (baseline, 140,000). Workup for infection, hemolysis, and thrombotic thrombocytopenic purpura was negative. Isolated thrombocytopenia was diagnosed as ITP and was attributed to PCV13 vaccination that he had received 1 month ago. He was treated with methylprednisolone, pooled immunoglobulins, and platelet transfusions. Subsequent platelet counts improved to his baseline of 140,000 over a period of 3 weeks. The PCV13 works by generating a T-helper–cell response. ConclusionsITP may involve antibody production driven by T-helper cells reacting to platelet surface glycoproteins. This case is unique in that it occurred in a patient who was on immunosuppression with a calcineurin inhibitor and corticosteroids.

Spectrum of Disorders Diagnosed By Bone Marrow Aspiration in Pediatric Population

AIMS AND OBJECTIVES – To identify and analyse the most common hematological disorders diagnosed by doing bone marrow aspiration in pediatric patients. MATERIAL AND METHOD – Bone marrow aspiration was done from Manubrium of the Sternum after injecting 2% xylocaine to the part in addition to sedation with diazepam. Bone marrow smears were prepared and stained with Leishman stain along with the simultaneous staining of the peripheral smears. A complete hemogram including Hb%, PCV, Red cell indices, platelet count, total leucocyte count and differential leucocyte count was also done by Automated cell counter. Finally, the bone marrow and peripheral smears were examined manually under oil immersion. CONCLUSION - In this study it was found that the most frequently diagnosed hematological disorders on bone marrow aspiration in children are Anemias followed by Idiopathic Thrombocytopenic Purpura (ITP). Hematological disorders are more common in childhood period as compared to late adulthood. If taken individually then ITP cases outnumber the Anemia cases. Commonest leukemia in children is Acute Lymphoblastic Leukemia (ALL) and it follows the Anemias and ITP.

The Incidence and Characterization of ITP in Prostate Cancer

INTRODUCTION:Idiopathic thrombocytopenic purpura (ITP) is a common autoimmune disorder. The association between ITP and lymphoid neoplasms is well known, but the association of ITP with solid tumors is poorly documented. To date only one case of prostate cancer associated with ITP has been reported in the literature and the association was unclear. The purpose of this study is to estimate the incidence of ITP in men with prostate cancer and to characterize its clinical course.METHODS:We performed a retrospective cohort study using a large multi-institutional database of 3,258,677 US veterans to identify those with and without prostate cancer (ICD-9 code 185). We evaluated them during the years 2006-2011 for the development of ITP using ICD-9 code 287.31. We examined the association of the presence of prostate cancer with incident ITP using Cox proportional hazard analyses, with adjustments for age, race, and presence of systemic lupus erythematosus, lymphomas and rheumatoid arthritis.In order to increase the specificity and sensitivity of our evaluation and to analyze the characteristics associated with ITP in prostate cancer, we then evaluated patients visiting the Memphis Veterans Administration Medical Center (VAMC) between January 2006 and December 2011. We identified 3973 patients with prostate cancer and randomly selected 4801 patients without prostate cancer during the same time period. In both of these cohorts, we performed an in depth chart review of all the patients with thrombocytopenic disorders, expanding the ICD.9 codes to 287.31 (ITP) and 287.5 (Thrombocytopenia NOS). We identified patients with ITP based on the American Society of Hematology criteria for the diagnosis. We then reviewed the ITP identified charts for characteristics of disease.RESULTS:Among 3,258,677 US veterans without a baseline diagnosis of thrombocytopenia, we identified 265,446 patients with prostate cancer and 2,993,231 patients without prostate cancer. In both these cohorts 1,847 patients developed ITP according to ICD.9 code 287.31. The incidence of ITP in patients with prostate cancer was 12.3/100,000 patient years (95%CI: 10.7-14.2) and in patients without prostate cancer was 9.5/100,000 patient years (95% CL: 9.0-10.0). Prostate cancer was associated with a 30% higher risk of incident ITP in unadjusted analyses (hazard ratio, 95%CI: 1.30, 1.12-1.51, p=0.001), but the difference was non-significant after multivariable adjustments (adjusted HR, 95%CI: 1.11, 0.95-1.30, p=0.18). Men with prostate cancer were older than controls (mean age 69 vs 59). Patients with ITP were more likely to have lupus (2.0% vs. 0.2%), lymphomas (7.3% vs. 0.8%) and rheumatoid arthritis (3.6% vs. 1.8%).This analysis provided a broad correlation between ITP and prostate cancer but lacked sensitivity due to the specificity of the single ICD-9 code. In our chart review of 3973 veterans with prostate cancer, we identified six cases of ITP (30/100,000 patient years). Among 4801 veterans without prostate cancer, none developed ITP. These numbers were too small to demonstrate clinical significance. The prostate cancer patients in our local institution were older than the controls (67.6 vs 57.7), similar to the age discrepancy in the large VA database. In none of the patients with ITP, thrombocytopenia was attributable to prior irradiation or chemotherapy. Two patients had severe ITP episodes preceding prostate cancer progression. Four patients had ITP worsening with stable prostate cancer and one patient had stable ITP with prostate cancer progression. Two of the ITP patients never required treatment, three required and responded to steroids and immunosuppressive drugs, and of these one required splenectomy.CONCLUSION:ITP, identified by the ICD-9 code 287.31 in a large multi-institutional survey, occurs more often in patients with prostate cancer, but this association may be due to confounding variables such as age. The true incidence is likely higher than this. At the Memphis VAMC, utilizing broader ICD.9 codes, including both ITP and Thrombocytopenia NOS, followed by an in-depth chart review, there is a strong suggestion of an increased incidence of ITP among prostate cancer patients, but the numbers are too small to be clinically significant. The clinical course of ITP in prostate cancer patients is similar to that seen with other disorders and does not clearly parallel the course of the prostate cancer. DisclosuresNo relevant conflicts of interest to declare.

Application of immunohistochemical staining in detection of Helicobacter pylori infection in patients with idiopathic thrombocytopenic purpura

Objective To analyze the clinical value of immunohistochemical staining of Helicobacter pylori (Hp) in patients with idiopathic thrombocytopenic purpura (ITP). Methods A total of 79 ITP patients were performed gastroscopy and biopsy in gastric mucosa. Hematoxylin and eosin (HE) staining and immunohistochemical staining were performed. Before gastroscopy, 14C-urea breath test was used to detect the Hp infection. Results The positive rates of Hp infection detected by 14C-urea breath test and Hp immunohistochemical staining in 79 cases of ITP were 51.9 % (41/79) and 67.1 % (53/79) respectively, the difference was statistically significant (χ 2 = 71.53, P = 0.023). Conclusions Hp immunohistochemistry staining is clear and accurate,and easy to observe. It is an effective method for detection of Hp infection in ITP patients. Key words: Purpura, thrombocytopenic, idiopathic; Helicobacter pylori; Immunohistochemistry; 14C-urea breath test

Concomitant Immune Thrombocytopenic Purpura and Crohn’s Disease

The coexistence of immune (idiopathic) thrombocytopenic purpura (ITP) and Crohn’s disease (CD) is rare. We performed a review of cases of concomitant ITP and CD in the English and Japanese literature. Among 17 identified cases of concomitant ITP and CD, ITP was initially diagnosed in four cases and CD was initially diagnosed first in six cases. Simultaneous diagnoses were reported in the remaining seven cases. No fatalities were reported in any of the 17 cases. However, resistance or transient responses to standard therapies, such as glucocorticoids or intravenous immunoglobulin (IVIG), and splenectomy for the treatment of ITP were reported in a number of concomitant cases. Moreover, the administration of anti-tumor necrosis factor (TNF)-alpha antibodies was a commonly considered pharmacological therapy in cases of concomitant ITP and CD.

Interpretation of Bone Marrow Trephine Biopsies in Hematological Disorders with Special Emphasis on Morphological Patterns in Lymphomas

Context: Hematological disorders are seen in all age groups. Bone marrow trephine biopsy plays a major role in the diagnosis of underlying causes, in addition to staging and follow-up of hematological malignancies. Aims: To study the trephine biopsies received in the department of clinical pathology with respect to age and sex incidence, to correlate with bone marrow aspirations, with special emphasis to its importance in cases where bone marrow aspiration yields a dry tap. Settings and Design: 105 cases of bone marrow trephine biopsies performed in our institution over a period of one and a half years are included in the study. Methods and Materials: A prospective study of 105 cases of bone marrow trephine biopsies was conducted. The biopsies were fixed in 10% buffered formalin solution and decalcified using 5% concentrated nitric acid for 4-6 hours, followed by routine processing. Serial sections were stained with Hematoxylin and Eosin (H and E), reticulin stains and Immunohistochemical markers in some. Results: The majority of bone marrow biopsies showed reactive marrows (42 cases) followed by normal marrows (12 cases) and hypoplastic marrows (5 cases). Bone marrow biopsy showed involvement by leukemias in 11 cases(10.5%), lymphomas in 8 cases (7.6%),Myeloma in 6 cases(5.7%)and secondary deposits in 2 cases (1.9%). There were 5 cases of Megaloblastic anemia(4.8%), four cases of myelofibrosis (3.8%), two cases of idiopathic thrombocytopenic purpura (ITP)(1.9%) and one case each of aplastic anemia and autoimmune hemolytic anemia(0.9%). Out of 8 cases of Non Hodgkin’s lymphomas - 3(37.5%) showed nodular, 2 diffuse(25%), 1(12.5%) interstitial pattern, 1 interstitial diffuse(12.5%) and 1 para trabecular pattern(12.5%) of marrow infiltration on trephine biopsy section. Nodular, interstitial and para trabecular patterns had good prognosis. Interstitial diffuse had intermediate prognosis and diffuse pattern had bad prognosis. There were 28 cases of dry tap in bon

Coexistence of Primary Biliary Cirrhosis and Immune Thrombocytopenic Purpura

Although autoimmune diseases are often concomitant, the coexistence of primary biliary cirrhosis (PBC) and immune (idiopathic) thrombocytopenic purpura (ITP) is rare. This is a review of the English-language literature regarding concomitant cases of PBC and ITP. Among 17 concomitant cases reported, including four diagnosed with Evans syndrome, which includes ITP symptoms, PBC was diagnosed first in five cases, ITP was diagnosed first in two cases, and both were almost simultaneously diagnosed in remaining 10 cases. Standard pharmacotherapy was ursodeoxycholic acid (UDCA) for PBC and corticosteroids for ITP/Evans syndrome. Among these 17 cases, one fatality was observed although there appears to be no trend for worse outcome compared with either PBC or ITP alone.

口腔出血を呈した特発性血小板減少性紫斑病 (ITP) の2例

口腔出血を呈した特発性血小板減少性紫斑病 (ITP) の2例

Quick Recertification Series

Quick Recertification Series

Role of H. pylori Infection (Serology, PCR) in Chronic Idiopathic Thrombocytopenic Purpura in an Endemic Country: A Case Control Study, Tehran, IRAN

Background: A practical guideline for detection and managements of some common infectious agents in cases with chronic ITP (Idiopathic Thrombocytopenic Purpura) is so important. Objectives: to investigate the role of H. pylori infection in children with chronic ITP in an endemic area. Materials and methods: A case control study done in pediatric ward Rasul Hospital, Tehran, Iran (2009-2010). 51 chronic ITP cases and 25 controls were assessed. H. pylori IgG & IgA ELISA (LDN -Germany) assesses in all cases and controls. All cases undergoing Bone Marrow Aspiration. H. pylori -PCR evaluated (QIAquickP® QIAGEN; Germany). P-value <0.05 was considered statistically significant. Results: cases were between 1- 20 years (mean 13.3.5 ± 7.6 y). Platelet count varied between 5000-1330000 (mean 63621 ± 37369.9) Positive H. pylori- IgA observed in 70% (36/51) of cases and 4% (1/25) of controls; p-value=0.00. H. pylori (IgG) was not significantly difference between cases and controls. [51% (26/51) vs. 32% (8/25), p-value=0.09] .Poor agreement observed between H. pylori –IgA and H. pylori - IgG antibodies and severity of thrombocytopenia in ITP cases (Kappa=-0.11; 0.04). Positive PCR results was % 5.9% (3/51) in ITP cases without significant difference in age between positive and negative PCR results (mean age 9.3 ± 9.7 years vs. 13.5 ± 7.52 years; p-value =0.3) Poor agreement between positive PCR and positivity of IgA (actual agreement=47.062%; p-value =0.5; Kappa=- 0.04), and IgG antibodies (actual agreement=40.91%; p-value =0.6; Kappa=- 0.04 respectively) were observed in ITP cases. Conclusion: We concluded that H. pylori infection (serologically) is high in young Iranian population. In chronic ITP, the H. pylori infection can be considered as an additional disorder which aggravates the main disease. The management of mild-to-moderate chronic ITP in Iranian patients, especially those with a recent onset of disease, should include an investigation for and eradication of infection with H. pylori.

MicroRNA expression in childhood acute granulocytic leukemia and its subtypes

Recent studies have suggested that there is a close relation between microRNA and acute leukemia (AL). The aim of this study was to investigate and better understand the classification and diagnosis of AL as well as pathogenesis and prognosis of this disease. A total of 93 children with AL and and 12 cases of idiopathic thrombocytopenic purpura (as control group) were enrolled in this study. Microarray chip analysis of their bone marrow samples was conducted to evaluate the microRNA profiles. Quantitative real-time PCR was performed for validating the abnormal expression of microRNA. The microRNA expression profiles were different between acute granulocytic leukemia and acute lymphoblastic leukemia and also between the three subtypes (M1, M2 and M3) of acute granulocytic leukemia according to FAB classification based on leukemic cell differentiation. These three subtypes of leukemia could be identified by unsupervised hierarchical cluster analysis of microRNA expression and had specific up-regulation of miR-335, miR-126 and miR-125b, respectively. However, in the M2 and M3 subtypes with positive AML1-ETO and PML-RARα, respectively, which have a better prognosis, the expressions of miR-126 and miR-125b were significantly higher than those with negative AML1-ETO and PML-RARα. Further more, miR-335 and miR-146 were up-regulated in acute granulocytic leukemia observed in this study, which are different from those reported for adult patients. microRNA cascade may serve as new biomarkers for the classification and diagnosis of pediatric AL. It is also suggested that there might be different pathogenesis and prognosis between AL types related to specific expression and regulation of microRNA.

Thrombocytopenia: diagnosis with flow cytometry and antiplatelet antibodies

To identify antiplatelet antibodies by flow cytometry (direct method) in patients with thrombocytopenia. Between January 1997 and March 2004 a total of 15100 patients were referred to the Centro de Hematologia de São Paulo for hematological investigation of several diagnoses (anemia, leukopenia, thrombocytopenia, coagulation abnormalities, adenomegaly, leukemia and others). Of those, 1057 were referred because of thrombocytopenia and were divided into two groups: Group Idiopathic thrombocytopenic purpura, with no identifiable cause; and Group Other thrombocytopenia, which included low normal platelet counts cause to be established, hepatitis C and HIV infection, hypersplenism, EDTA-induced artifacts, laboratory error, and other causes. Flow cytometry immunophenotyping was done in 115 cases to identify platelet autoantibodies (direct method). Of the total number of patients, 1057 (7%) presented low platelet counts, 670 were females (63.4%) and age range of one to 75 years. Of the 115 cases (9.7%) submitted to immunophenotyping, the results were positive in 40% and the test was inconclusive in 5%. Idiopathic thrombocytopenic purpura was found in 52% of patients, more often in women. Hepatitis C virus infection was found in 7% and HIV infection in 1%. Low normal platelet counts were found in 17%, laboratory errors in 6%, and laboratory artifacts in 1% of cases. Platelet autoantibodies were found in 76.9% of all idiopathic thrombocytopenic purpura cases. It was negative in 83.3% of the low normal counts. antiplatelet autoantibodies when present help to diagnose idiopathic thrombocytopenic purpura. When absent, suggest other causes of thrombocytopenia.

Clinical analysis of pregnancy complicated with severe thrombocytopenia

To investigate the etiology and perinatal outcome of pregnancies complicated with extremely severe thrombocytopenia [at least two times of platelets count (PLT) < 10 × 10(9)/L during pregnancy]. Clinical data, including basic information, etiology, management and outcomes of pregnant women with extremely severe thrombocytopenia, admitted to Peking University People's Hospital from January 2004 to March 2009, were retrospectively collected. The management of these cases varied according to different etiology and the symptoms: (1) PLT were maintained > 20 × 10(9)/L and hemoglobulin > 70 g/L in those women without spontaneous bleeding; (2) PLT transfusion would be required when PLT < 10 × 10(9)/L or bleeding occur and RBC would be supplied when hematocrit < 25% and hemoglobulin < 70 g/L; (3) Hemoglobulin should be > 70 g/L and PLT > 30 × 10(9)/L before cesarean section or delivery; (4) Predinisone and/or intravenous immunoglobulin G (IVIG) would be given in women complicated with idiopathic thrombocytopenic purpura (ITP) when PLT < (20 - 30) × 10(9)/L or bleeding. PLT would be given if all the above management were failed, or PLT < 10 × 10(9)/L, or bleeding. Women without bleeding would be closely monitored and delivery would be planned. (1) Twenty-six cases were identified among 9302 deliveries during the study period (0.28%), with an average of maternal age of 29. Seventeen were diagnosed before conception and 9 during pregnancy. Among the 26 women, half received regular prenatal check in our hospital and the average gestations at diagnosis was 24 weeks and the other half without regular prenatal visits and the average gestations at diagnosis was 32 weeks. Etiology was identified in 24 out of the 26 women, including 14 (54%) ITP, 5 myelodysplastic syndrome (MDS), 4 chronic aplastic anaemia (CAA) and 1 systemic lupus erythematosus (SLE). (2) MANAGEMENT: All of the 26 women received blood products. Among the 14 ITP cases, 6 received predinisone and IVIG and 8 only took predinisone. Nine of the 26 patients (35%) had pregnant complications, among which 6 (6/9) were preeclampsia. The overall average gestation at delivery was 36 weeks. Only 2 delivered vaginally with the average blood loss of 83 ml and 23 cesarean sections were performed with the average blood loss of 410 ml. (3) Perinatal outcomes: There were 26 perinatal babies, among which 1 died intrauterine and 25 were born alive (12 preterm infants). The average birth weight was 2877 g. Neonatal severe thrombocytopenia presented in 2 newborns whose mother complicated with ITP. The main cause of extremely severe thrombocytopenia during pregnancy is ITP, managed mainly by predinisone and IVIG, followed by CAA and MDS, which may require supportive treatment. Pregnancy complicated with extremely severe thrombocytopenia is not an indication of termination. Better maternal and fetal outcomes can be achieved through proper treatment based on the etiology, intensive care in prevention and management of complications and cesarean section.

A Randomized, Double-Blind, Placebo-Controlled Phase 1/2 Study to Determine the Safety and Efficacy of Romiplostim in Children with Chronic Immune (Idiopathic) Thrombocytopenic Purpura (ITP).

Abstract 680 Introduction: ITP is an autoimmune disorder characterized by thrombocytopenia due to accelerated destruction as well as suboptimal platelet production. Childhood ITP is most commonly an acute illness; however, chronic ITP (duration > 6 months) develops in 20%–30% of ITP cases. Romiplostim, a peptibody protein designed to increase platelet production, is approved for treating chronic ITP in adults. The objective of this study was to evaluate the safety and efficacy of romiplostim in the treatment of thrombocytopenia in children with chronic ITP. Patients and Methods: ITP patients aged 12 months to 20 × 109/L above baseline for 2 consecutive weeks without rescue therapy at any point during the treatment period). Treatment was initiated at 1 μg/kg once weekly by subcutaneous injection. The dose was adjusted in 2 μg/kg increments every two weeks, to a maximum dose of 10 μg/kg/week based on weekly platelet counts. The incidence of adverse events (AEs) during the 12-week treatment period and the number of patients achieving platelet counts >50 × 109/L for 2 consecutive weeks during the treatment period, or achieving an increase in platelet count >20 × 109/L above baseline for 2 consecutive weeks during the treatment period was recorded. Results: A total of 22 (romiplostim, 17; placebo, 5) patients were randomized; 16 (73%) were boys and 6 (27%) were girls. Eight patients had undergone splenectomy. The mean age was 9.5 (SD: 5.1) years, with 4 subjects aged 12 months - Conclusion: Treatment with romiplostim appeared to be well tolerated in pediatric ITP patients, with no new safety concerns observed in this study as compared to adults with chronic ITP. Romiplostim was effective in treating thrombocytopenia in children with chronic ITP. Disclosures: Buchanan:Amgen Inc.: Research Funding. Off Label Use: Use of romiplostim, a thrombopoietin mimetic, in treatment of thrombocytopenia in pediatric ITP patients. . Bomgaars:Novartis: Research Funding. Bussel:Eisai, Inc: Research Funding; Sysmex: Research Funding; Shionogi: Membership on an entity9s Board of Directors or advisory committees; Ligand: Membership on an entity9s Board of Directors or advisory committees, Research Funding; Immunomedics: Research Funding; Amgen: Equity Ownership, Membership on an entity9s Board of Directors or advisory committees, Research Funding; GlaxoSmithKline: Equity Ownership, Membership on an entity9s Board of Directors or advisory committees, Research Funding; Cangene: Research Funding; Genzyme: Research Funding; Scienta: Speakers Bureau. Nie:Amgen Inc.: Employment, Equity Ownership. Eisen:Amgen Inc.: Employment, Equity Ownership. Berger:Amgen Inc.: Employment, Equity Ownership.