Immune Thrombocytopenic Purpura as a Hemorrhagic Versus Thrombotic Disease: An Updated Insight into Pathophysiological Mechanisms.

Immune thrombocytopenic purpura (ITP) is a blood disorder characterized by a low platelet count of (less than 100 × 109/L). ITP is an organ-specific autoimmune disease in which the platelets and their precursors become targets of a dysfunctional immune system. This interaction leads to a decrease in platelet number and, subsequently, to a bleeding disorder that can become clinically significant with hemorrhages in skin, on the mucous membrane, or even intracranial hemorrhagic events. If ITP was initially considered a hemorrhagic disease, more recent studies suggest that ITP has an increased risk of thrombosis. In this review, we provide current insights into the primary ITP physiopathology and their consequences, with special consideration on hemorrhagic and thrombotic events. The autoimmune response in ITP involves both the innate and adaptive immune systems, comprising both humoral and cell-mediated immune responses. Thrombosis in ITP is related to the pathophysiology of the disease (young hyperactive platelets, platelets microparticles, rebalanced hemostasis, complement activation, endothelial activation, antiphospholipid antibodies, and inhibition of natural anticoagulants), ITP treatment, and other comorbidities that altogether contribute to the occurrence of thrombosis. Physicians need to be vigilant in the early diagnosis of thrombotic events and then institute proper treatment (antiaggregant, anticoagulant) along with ITP-targeted therapy. In this review, we provide current insights into the primary ITP physiopathology and their consequences, with special consideration on hemorrhagic and thrombotic events. The accumulated evidence has identified multiple pathophysiological mechanisms with specific genetic predispositions, particularly associated with environmental conditions.

Efficacy and safety of thrombopoietin receptor agonists in children with chronic immune thrombocytopenic purpura: meta-analysis.

Immune thrombocytopenic purpura (ITP) is an autoimmune disorder presenting with low platelet count <100 × 109/L. The condition affects both adults and children. Thrombopoietin receptor agonists (TPO-RAs) are second-line of therapy that includes Romiplostim and Eltrombopag, which stimulate the production of normally functioning platelets. Although the biological effect of these drugs is well established, there has not been a meta-analysis in children. To estimate the efficacy and safety of Romiplostim and Eltrombopag, we performed a systematic review and meta-analysis in children with chronic ITP. Systematic literature search was conducted in the following database: PubMed, EMBASE, and Cochrane Central Register of Controlled Trials (CENTRAL). Review Manager 5.3 for Windows was used to analyze the data. Five randomized controlled trials with total of 261 pediatric patients from 1-17 years of age were included. The efficacy and safety analysis showed TPO-RA groups were superior over placebo, and there was no difference in adverse event occurrence between TPO-RA (Romiplostim and Eltrombopag) and placebo groups. The efficacy and safety of Eltrombopag did not differ significantly from those of Romiplostim. Both drugs were effective in treatment of children with chronic ITP. Our findings extend the currently available data on ITP treatment and is helpful for pediatric health providers and for the design of future clinical trials.

Immune thrombocytopenic purpura presenting in a patient after renal transplant for diabetic nephropathy.

BACKGROUND: Immune thrombocytopenic purpura (ITP) is primarily characterized by immune-mediated destruction of platelets in circulation. Major treatment options range from careful observation, steroids, immunosuppressive medications, immunoglobulins to splenectomy. Interestingly and rarely, ITP has also been reported after solid organ transplantation in patients receiving immunosuppressive medications. While the incidence of new onset ITP after solid organ transplant is comparatively well documented, new onset ITP after renal transplant has only been reported in two patients. Both these patients underwent renal transplant for underlying Immunoglobulin-A (IgA) nephropathy and were treated effectively with steroids. We present successful management of the first reported case of new-onset ITP presenting after renal transplant in a patient with underlying diabetic nephropathy. The case report discusses the potential management strategies in such a novel scenario aiming simultaneously for a well-functioning renal graft, adequate hemostasis, minimum therapy- related morbidity and least cost implications for the patient. CASE PRESENTATION: A 43-year-old male with hypertension and diabetes mellitus (DM), complicated by nephropathy and retinopathy, underwent pre-emptive living related renal transplant by donation from his 33-year-old wife. His immediate post-transplant period was unremarkable. Six months after the transplant, he presented with isolated thrombocytopenia. An extensive workup revealed no clinical or laboratory evidence of unusual substance intake, infection, hemolysis, microangiopathy, autoimmune disease or hematological malignancy. Eight months after the transplant, while the patient was maintained on steroids, cellcept and tacrolimus, his platelet count dipped to 13,000/microL and he had an episode of mild epistaxis. He was administered steroids in line with the adult ITP management protocol. Steroids were well tolerated, and platelet counts showed a good response to therapy. Steroids were then successfully tapered over the next ten weeks with steady and acceptable platelet counts and graft function. CONCLUSIONS: The case report discusses the diagnostic considerations and successful management of new-onset post-renal transplant ITP. It also highlights the various therapeutic options available in the medical armamentarium including shuffling of immunosuppressive drugs, rituximab, thrombopoietin receptor agonists (TPO's) and splenectomy for their potential use in complicated scenarios like relapsing, or steroid-refractory post renal transplant ITP.

Importance of immature platelet fraction as predictor of immune thrombocytopenic purpura.

BACKGROUND AND OBJECTIVE: Immune thrombocytopenic purpura (ITP) is a clinical syndrome in which a decreased number of circulating platelets (thrombocytopenia) manifests as a bleeding tendency, easy bruising (purpura) or extravasation of blood from capillaries into skin and mucous membranes (petechiae). The diagnosis of ITP can be made clinically on the basis of symptoms, we need to see if ITP can be confirmed in patients by quantification of residual RNA containing immature platelets (megakaryocytic mass) or immature platelets fraction (IPF) using automated hematology analyzers (Sysmex XE-2100). METHODS: In order to check the efficacy of IPF% parameter of Sysmex XE-2100 a total of 231 patients of thrombocytopenia were included in this study. Complete blood count (CBC) was estimated. The data was statistically analyzed by SPSS version 17. RESULTS: About 62 patients were diagnosed as ITP and 169 patients were diagnosed as non ITP on the basis of clinical history. The mean IPF % value of ITP patients was 16.39% and the IPF % value of Non ITP patients was ~7.69% respectively. There was no significant difference in IPF% values with respect to time between sampling and acquisition of complete blood count. The diagnostic sensitivity of IPF% as biomarker for ITP and non-ITP was 85.71% (95%CI: 84.04% to 85.96%) and 41.76% (95% CI: 39.87% to 43.65%). CONCLUSION: The mean IPF % value by Sysmex XE-2100 can be used to predict ITP.

Intracerebral Hemorrhage in a Patient With Newly Diagnosed Immune Thrombocytopenic Purpura Precipitated by a Viral Illness.

Intracerebral hemorrhage (ICH) is a rare and severe complication of immune thrombocytopenic purpura (ITP) that can be spontaneous. Viral illnesses, other infections, autoimmune disorders, and medications can cause ITP. ITP causes a significant decrease in platelet levels, increasing bleeding risk. ITP can be treated by steroids, intravenous immunoglobulin, plasmapheresis, platelet transfusion, biological agents, and splenectomy. ICH treatment involves the treatment of underlying ITP, as well as any neuro-interventional procedures needed. In this case report, we look at the presenting symptoms and treatment course of an interesting case of ICH in a patient who developed ITP after a viral upper respiratory infection.

Refractory immune thrombocytopenic purpura (ITP) after accessory splenectomy: A case report and literature review.

Immune thrombocytopenic purpura (ITP) is an autoimmune disorder characterized by a platelet count of less than 100 x 109 /L, resulting from antibody-mediated platelet destruction. Treatment for ITP typically involves steroids, and intravenous immunoglobulins (IVIG) can be added. Splenectomy is performed in cases with refractory ITP. Rituximab can suppress immunity but has limited efficacy in ITP cases. Herein, we present a rare case of a 30-year-old female who was first diagnosed with ITP and underwent a splenectomy two years later. However, seven years after surgery, she was presented with symptoms of ITP. A splenic scan showed an accessory spleen in the spleen bed, for which she underwent accessory spleen removal surgery. Her laboratory tests three days post-operation showed a rise in platelet count and hence was discharged a few days later. The patient had recurrent attacks of ITP even after the removal of the normal and accessory spleen, suggesting that accessory spleen removal may not always be an effective treatment for ITP. The patient eventually died. While splenectomy is a common treatment for ITP, it may not always be effective in all cases, and other treatments such as bone marrow transplantation may be necessary.

Immune thrombocytopenic purpura in primary biliary cholangitis and localized cutaneous systemic sclerosis: case report and literature review.

Primary biliary cholangitis (PBC) is a chronic progressive cholestatic liver disease of uncertain etiology. Although PBC is frequently complicated by Sjögren's syndrome and chronic thyroiditis, it can also be associated with a variety of other autoimmune disorders. We herein describe a rare case of immune thrombocytopenic purpura (ITP) coexistence with PBC and localized cutaneous systemic sclerosis (LcSSc). A 47-year-old woman with PBC and LcSSc who was positive for antiphospholipid antibody experienced a rapid decrease in platelet count to 1.8 × 104/µL during follow-up. After clinical findings ruled out thrombocytopenia from cirrhosis, a diagnosis of ITP was made following bone marrow examination. Her human leukocyte antigen (HLA) type was HLA-DPB1*05:01, which has been associated with disease susceptibility to PBC and LcSSc, but not to ITP. A careful review of similar reports suggested that in PBC, other collagen disease complications, positive antinuclear antibody, and positive antiphospholipid antibody may all support a diagnosis of ITP. Clinicians should be vigilant for ITP when rapid thrombocytopenia is observed during the course of PBC.

Inpatient Rheumatology Consultation Prompted by Positive Autoantibodies in Patients Receiving Intravenous Immunoglobulin Therapy: A Case Series and Literature Review.

Intravenous immunoglobulin (IVIG) is a therapeutic preparation used in the treatment of multiple diseases. Autoimmune testing with antinuclear antibody (ANA) screening is often obtained for some of these conditions, but only after initiation of IVIG treatment. This can present a diagnostic dilemma in hospitalized patients and may trigger a rheumatology consultation. We describe our consultative inpatient two-year experience with five such patients and review the pertinent literature. A retrospective chart review of rheumatology inpatient consultations between 6-2018 and 6-2020 at our academic tertiary care hospital for post-IVIG positive serologies was performed. A pertinent literature review was performed. Five patients had a positive ANA and other autoantibodies detected in their serum after they received IVIG for non-rheumatological conditions. None of these patients met the criteria for a connective tissue disease. The literature review identified a total of 58 patients from case reports and case series, several of whom tested positive for ANA and other antibodies after receiving IVIG. Studies assessing specific IVIG products detected multiple autoantibodies in the donor pool. Autoimmune testing is initiated on inpatients receiving IVIG for non-rheumatological conditions. If an autoantibody ANA screen is positive, a rheumatology consultation may be requested. In the absence of pre-IVIG antibody tests it is difficult to interpret post-IVIG-positive antibodies. Whether such positive antibodies are of clinicopathological significance is determined by clinical judgment and time.

Sarcoidosis-Associated Immune Thrombocytopenic Purpura and Focal Segmental Glomerulosclerosis.

Sarcoidosis is well known for heterogeneity of its presentation and multisystem organ involvement. It commonly involves respiratory tract, skin, eyes, and lymph nodes, as well as hematologic and renal systems. While anemia and lymphopenia are the most common hematologic abnormalities seen in sarcoidosis, immune thrombocytopenic purpura (ITP) is considered rare. Renal abnormalities, although infrequent, are usually more likely to involve tubules rather than glomeruli. In this report, we present a case of sarcoidosis-associated ITP and focal segmental glomerulosclerosis (FSGS), refractory to first-line therapy, but successfully treated with Rituximab and thrombopoietin-receptor agonist.

Blockage of CD72 reduces B cell proliferation in immune thrombocytopenic purpura, involving interleukin 1 and macrophage migration inhibitory factor secretion.

AIM: This study aims to explore the expression and role of CD72 in B lymphocytes in immune thrombocytopenic purpura (ITP). METHODS: The expression level of CD72 in B lymphocytes was detected by flow cytometry in 18 ITP patients and 19 controls of healthy donor or iron-deficiency anemia patients. B cell proliferation was determined by 5-bromo-2'-deoxyuridine incorporation (BrdU) in the culture of 17 ITP patients' and 11 controls' peripheral mononuclear cells (PMNCs). The secretion levels of antibodies against human platelet antigens (HPA), as well as B cell proliferation-related cytokine interleukin 1(IL-1) and macrophage migration inhibitory factor (MIF) in culture supernatants were measured by ELISA. RESULTS: CD72 was significantly increased in B cells of newly diagnosed or persistent ITP compared with ITP in remission. B cell proliferation in culture with CD72 antibody addition was significantly decreased both in ITP patients and in controls compared with isotype antibody addition. CD72 antibody did not significantly alter HPA antibody level in ITP patients. CD72 antibody increased IL-1 and MIF levels in ITP patients' cell culture supernatant but not in controls. CONCLUSION: CD72 expression elevation accompanies the active status of ITP. In vitro addition of CD72 antibody has a negative impact on B cell proliferation. The function of CD72 in B cell proliferation in ITP may be related to IL-1 and MIF secretion.

A Case of Cytomegalovirus Infection With Severe Thrombocytopenia in an Infant Who Was Successfully Managed With Antiviral Therapy.

Cytomegalovirus (CMV) is a member of the Herpesviridae family of DNA viruses. It is one of the major infectious causes that induce thrombocytopenia. We herein report a case of CMV infection in an infant complicated with severe thrombocytopenia that was successfully managed by antiviral treatment. A three-month-old girl presented with generalized petechial lesions in the limbs, trunk, and eyelids, discovered by chance during a vaccination consultation in an apyretic context. Blood examinations demonstrated thrombocytopenia at 26,000/mm3. She was diagnosed with immune thrombocytopenic purpura. Intravenous immunoglobulin was administered thrice and corticosteroid therapy at a dose of 2 mg/kg/day was started. The evolution during her hospitalization was marked by the increase to 373,000/mm3. A week later, the platelet had decreased again to 8000/mm3. Positive serology and high CMV DNA detected in serum by real-time quantitative polymerase chain reaction confirmed the diagnosis of CMV infection. In consideration of the severe thrombocytopenia, antiviral therapy with ganciclovir 5 mg/kg/12 hours was initiated. The platelet counts increased with decreasing CMV loads. She was discharged home after clinical stabilization with a close follow-up over one year.

Prevalence of Cytomegalovirus (CMV) and Epstein-Barr Virus (EBV) Subclinical Infection in Patients with Acute Immune Thrombocytopenic Purpura (ITP).

Background: Immune thrombocytopenic purpura (ITP) defined as a bleeding disorder in which the number and production of platelets reduced by the immune system; however, the destruction of peripheral blood platelets also occurs. Although its exact etiology and pathogenesis not already know, several studies have shown that Epstein-Barr virus (EBV) and cytomegalovirus (CMV) known as possible causative agents of ITP. This investigation aims to evaluate the presence of CMV and EBV in two groups of case and control by polymerase chain reaction (PCR). Materials and Methods: we considered the presence of CMV and EBV in 48 acute ITP patients and 48 healthy people. Study participants were recruited from Ahvaz Shafa Hospital between 2017 and 2018 and the presence of two viruses was investigated by (PCR). Results: Out of 48 acute ITP patients, the CMV DNA was detected from the blood of 12 (25%) patients and the EBV DNA from the blood of 2 (4.2%) other patients. In addition, only one patient was (2.1%) co-infected with CMV and EBV. In contrast, in 48 healthy subjects, 3 (6.6%) had CMV and none of the control group was infected with EBV. Conclusion: Due to the presence of both EBV and CMV in the acute ITP patients in Ahvaz, they can be considered as factors in the progression of this disease. Therefore, consideration of the methods of elimination and treatment of these two viruses in these patients may be used as a treatment strategy in ITP patients in the future.

COVID-19 induced immune thrombocytopenic purpura: case report.

Immune thrombocytopenic purpura (ITP) is an autoimmune state of decreased platelets caused by antibody or T-cell mediated destruction of platelets through the reticuloendothelial system and impairment of their production. Symptoms of ITP include bleeding usually from nose or gums, easy bruising, petechiae commonly of lower extremities, menorrhagia, hematuria, hematemesis, hematochezia and most dreadful, intra cranial hemorrhage. Molecular mimicry between viral antigens and host platelet antigens forming cross-reactive anti-platelet autoantibodies may lead to increased platelet clearance in ITP associated with viral infections. One of the many viruses associated with this is the Coronavirus disease 2019 (COVID-19). It has caused a devastating pandemic. It can activate innate and adaptive immune responses. It has numerous signs and symptoms including but not limited to dyspnea, fever, cough, fatigue, myalgias, loss of taste and smell. It leads to diseases such as pneumonia, acute respiratory distress syndrome, thrombosis and cardiomyopathy. Hematologic manifestations include thrombocytopenia and more commonly lymphopenia. Treatment includes steroids, immune globulin, romiplostim, eltrombopag, rituximab or splenectomy. Contact sports should be avoided due to risk of intra cranial bleeding with head impact. Nonsteroidal anti-inflammatory drugs (NSAIDs) and aspirin should be used with caution since they impair platelet function. We discuss a patient with COVID-19 who developed thrombocytopenia thought to be due to ITP. Not much is known about the association between the two. It is important to keep this differential in mind when taking care of patients with COVID-19 who develop thrombocytopenia.

Dengue hemorrhagic fever as a rare cause of chronic immune thrombocytopenic purpura-a pediatric case report.

BACKGROUND: Dengue is a common mosquito-borne infection in tropical countries. Dengue incidence in Sri Lanka is generally showing a rising trend. Both chronic immune thrombocytopenia purpura (ITP) children and chronic ITP triggered by dengue fever in the pediatric age group are rarely reported. This unusual presentation is a diagnostic challenge to clinicians. The authors have reported a pediatric patient who presented with chronic ITP following recovery from dengue hemorrhagic fever. CASE PRESENTATION: A 14-year-old previously healthy boy was initially managed as for dengue hemorrhagic fever. Following initial detection of persistent thrombocytopenia at 2 weeks post-discharge, his parents defaulted follow-up for 1 year as he remained asymptomatic. However, 1 year after initial admission, the child re-presented with ecchymotic patches and a platelet count of 30 × 103/cumm. Review of serial blood counts performed during previous hospital admission and by his parents themselves revealed persistent thrombocytopenia over preceding 12 months. Subsequently, the child had an in-depth evaluation. The diagnosis of ITP was confirmed by ruling out differential diagnosis and he was managed as for chronic ITP. His platelet counts showed good response to oral corticosteroids and he is currently being followed up at the pediatric hematology clinic. CONCLUSION: While reporting, a 14-year-old boy who developed chronic ITP following dengue hemorrhagic fever, this report highlights importance of frequent monitoring of blood counts to accurately detect and manage critical phase of dengue fever. The report also highlights the value of monitoring platelet counts in post-recovery phase to ensure they have normalized.

Prediction of response to first-line therapy with ITP by flow cytometric analysis of bone marrow lymphocyte phenotypes.

In the present study, we analyzed phenotypes of cells in the lymphocyte region of bone marrow in 68 patients with primary immune thrombocytopenia (ITP) to determine whether cellular phenotype predicts response to first-line therapy (corticosteroids or corticosteroids plus intravenous immunoglobulin). In 52 newly diagnosed ITP patients, an abnormal CD4:CD8 ratio (CD4/CD8 ratio < 0.4 and 2.3 < CD4/CD8 ratio) was noted in 22 patients in the responder group, whereas all non-responder and control individuals showed normal CD4:CD8 ratio (p < 0.001). The absolute number of CD19+ cells in patients with 0.4 ≤ CD4/CD8 ratio ≤ 2.3 or 2.3 < CD4/CD8 ratio was higher than that in other groups. (p = 0.016). In 16 chronic ITP patients, the absolute number of NK cells in the responder group was lower than those in the non-responder and control groups (p = 0.032). An abnormal CD4:CD8 ratio was noted in all patients in the responder group, whereas all patients in non-responder and control groups showed normal CD4:CD8 ratio (p < 0.001). The present results indicate that CD4:CD8 ratio, B cells, and NK cells contribute to the prediction of therapeutic outcomes of ITP patients.

Treatments for Primary Immune Thrombocytopenia: A Review.

Immune thrombocytopenic purpura (ITP) is an autoimmune condition that affects nearly 1:10,000 people in the world. It is traditionally defined by a platelet count of less than 100 x 109L, but treatment typically depends on symptomology rather than on the platelet count itself. For primary idiopathic ITP, corticosteroids have been the standard first-line of treatment for symptomatic patients, with the addition of intravenous immune globulin (IVIG) or Rho(D) immune globulin (anti-RhD) for steroid-resistant cases. In cases of refractory or non-responsive ITP, second-line therapy includes splenectomy or rituximab, a monoclonal antibody against the CD20 antigen (anti-CD20). In patients who continue to have severe thrombocytopenia and symptomatic bleeding despite first- and second-line treatments, the diagnosis of "chronic refractory ITP" is appropriate, and third-line treatments are evaluated. This manuscript describes the efficacy of different treatment options for primary ITP and introduces the reader to various third-line options that are emerging as a means of treating chronic refractory ITP.

Helicobacter pylori: An Underrated Cause of Immune Thrombocytopenic Purpura. A Comprehensive Review.

Idiopathic thrombocytopenic purpura (ITP) is the autoimmune-mediated destruction of platelets. ITP is a diagnosis of exclusion after other identifiable etiologies have been ruled out. After the first report by Gasbarrini et al. (1998) showing rising platelet counts in ITP patients following Helicobacter pylori (HP) eradication therapy, there is growing evidence that highlights the role of HP in triggering ITP. However, the exact pathophysiology of HP-associated ITP is still unclear, but many theories have been implicated in this regard. According to various reports, the postulated mechanisms for the role of HP in cITP include molecular mimicry, increased plasmacytoid dendritic cell numbers, phagocytic perturbation, and variable host immune response to HP virulence factors. One famous theory suggested molecular mimicry between platelet surface antigen and bacterial virulence factor, i.e. cytotoxin-associated gene A (CagA). It is thought that a chronic inflammatory response following an HP infection induces the host autoantibodies' response against CagA, which cross-reacts with platelet surface glycoproteins; therefore, it may accelerate platelet destruction in the host reticuloendothelial system. However, further studies are mandated to better understand the causal link between ITP and HP and study the role of biogeography. Nowadays, it is recommended that every patient with ITP should undergo HP diagnostic testing and triple therapy should be administered in all those candidates who test positive for HP infection. In our review, there were a few pregnant female ITP patients who took HP eradication therapy mainly after 20 weeks of gestation without maternal or fetal worst outcomes. However, large-scale studies are advisable to study the adverse fetal outcomes following triple therapy use.

Expression, purification and biological activity assessment of romiplostim biosimilar peptibody.

BACKGROUND: Romiplostim is a peptibody analogue of thrombopoietin (TPO) which regulates platelet production. This molecule consists of two main parts: Peptide sequences which like wild type TPO, mimics stimulation of TPO receptor and IgG1Fc, (Peptide + Antibody = Peptibody). This drug is used in treatment of chronic Immune Thrombocytopenic Purpura (ITP). METHODS: In this project E. coli bacteria were transformed by a construct harboring peptibody fusion gene. This construct consisted of two repeated peptide sequences which have fused to Carboxyl group of IgG1Fc. Designed construct in E. coli host resulted in protein expression in cytoplasm as inclusion body. The inclusion bodies were separated, washed and after denaturation and solubilization, in the last stage the desired peptibodies were refolded and purified. The resulting peptibodies were characterized by SDS-PAGE and Western immunoblotting. The bioactivity were assessed in vivo using subcutaneous injection in mice. RESULTS: Results showed accurate molecules were produced and purified. Also, in vivo experiment showed significant increment (more than two fold) of platelets compared to control group. CONCLUSION: In this study laboratory scale production of recombinant romiplostim showed proper in-vivo bioactivity. This new approach in expression and purification of this recently introduced thrombopoietin receptor agonist drug may be followed by scale up of its production to response the chronic ITP patient's demand.

Therapeutic Efficacy of a Single Dose of Anti-D Immunoglobulin 50 microgram/kg in Childhood Acute Immune Thrombocytopenic Purpura

BACKGROUND: To investigate the efficacy and adverse effects of a single dose of anti-D immunoglobulin (50 microgram/kg) in Korean children with acute immune thrombocytopenic purpura (ITP). METHODS: We serially evaluated the platelet count in 21 acute ITP patients that were treated with anti-D immunoglobulin (50 microgram/kg) to determine how many patients achieved platelet counts over the levels of 20 x 10(3)/mm3, 50 x 10(3)/mm3 and 100 x 10(3)/mm3 after the infusion of anti-D immunoglobulin. In addition, constitutional symptoms were monitored and changes in the hemoglobin levels were serially evaluated. RESULTS: By three days after treatment, 90.5% of the patients had achieved a platelet count over 20 x 10(3)/mm3. At seven days after treatment, 66.7% of patients achieved a platelet count of 50 x 10(3)/mm3. In addition, at seven days after treatment 61.9% of patients achieved a platelet count of 100 x 10(3)/mm3. Constitutional adverse symptoms were observed 61.9% of patients, and the symptoms diminished spontaneously without any severe sequelaes. The decline of hemoglobin concentration after treatment recovered to the initial level after two weeks. CONCLUSION: A single dose of anti-D immunoglobulin (50 microgram/kg)was effective in Korean children with acute ITP to raise the platelet count rapidly. The adverse effects of anti-D immunoglobulin, including hemolytic anemia, were not severe to prevent the use of the anti-D.

Long-term Follow-up of Laparoscopic Splenectomy in Patients with Immune Thrombocytopenic Purpura

Laparoscopic splenectomy (LS) has been reserved for intractable and relapsing immune thrombocytopenic purpura (ITP) despite medical treatment. With further experiences of LS in ITP, we investigated long term outcomes of LS, especially newly developed morbidities, and tried to find predictive factors for favorable outcomes. From August 1994 to December 2004, fifty-nine patients whose follow-up period was more than 12 months after LS were investigated. After a long-term follow-up (median 54 months, range 12.5-129 months), a complete response (CR) was found in 28 patients (47.5%), partial response in 24 (40.7%), and no response in 7 (11.9%). The relapse rate during follow-up periods was 15.2%. The rapid response group (p=0.017), in which the platelet count increased more than twice of the preoperative platelet count within 7 days after LS, relapsing after medical treatment (p=0.02), and the satisfactory group as the initial result of LS (p=0.001) were significant for predicting CR in univariate analysis, but only the initial satisfactory group was an independent predictive factor for CR in multivariate analysis (p=0.036, relative risk=6419; 95% CI, 1.171-35.190). Infections were the most frequent morbidities during the follow-up period, which were treated well without mortality. LS is a safe and effective treatment modality for ITP. Active referral to surgery might be required, considering complications and treatment results related to long-term use of steroid-based medications.