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Acquired amegakaryocytic thrombocypenia (AAMT) is an extremely rare hematologic disorder and standard treatment strategy has not been established. We described herein two cases of AAMT who were fully responded to eltrombopag and immunosuppressant. Patient 1 was refractory to steroid, IVIG and recombinant human thrombopoietin (rhTPO). Patient 2 did not respond to high dosage of steroid, IVIG, rhTPO and rituximab. Moreover, his AAMT progressed to aplastic anemia in 5 months. Both patients took eltrombopag and immunosuppressant, then they achieved long-term remission without obvious side effects. Our findings suggest that this combination can be a valuable alternative in AAMT.

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Competing Interests: Declaration of interests We declare no competing interests.

Background: Immune thrombocytopenia (ITP) is an autoimmune disorder of variable origin that results in bleeding and decreased platelet count. Autoimmune abnormalities have been described in patients with malignancies including non-Hodgkin's lymphoma but are rarely described in patients with Hodgkin's lymphoma., Objectives: To describe an unusual presentation of Hodgkin's lymphoma in an unusual age and alarm pediatricians of the challenging diagnosis., Methods: We present two cases that highlight an unusual clinical presentation of childhood Hodgkin's lymphoma occurring at an atypical age., Results: Over a 4-year period, two children aged 5 and 6 years were admitted for suspected ITP, both had cervical lymphadenopathy. Bone marrow examination showed no evidence of tumor or fibrosis. Biopsy of the lymph node was possible only after administration of intravenous immunoglobulins and normalization of the platelet count. Platelet counts increased after initiation of chemotherapy., Conclusions: The identification of the clinical presentation of ITP as a possible presentation of Hodgkin's lymphoma is important to facilitate timely diagnosis and management.

Adult-onset Still's disease (AOSD) sometimes demonstrates hematologic disorder, whereas acquired amegakaryocytic thrombocytopenia (AAT) involvement is extremely rare. We herein report a 67-year-old woman with relapse of AOSD who concomitantly developed AAT. Thrombocytopenia along with high disease activity of AOSD was resistant to high-dose prednisolone, even in combination with methotrexate and tacrolimus. However, alternative treatment with cyclosporine after administering tocilizumab resulted in the improvement of thrombocytopenia, ultimately demonstrating that combination therapy based on suppressing the intractable disease activity of AOSD and subsequently adding a reliable immunosuppressant was required to achieve remission.

Acquired amegakaryocytic thrombocytopenia (AAMT) is a rare disease that causes severe bleeding. The pathogenesis and treatment of AAMT have not yet been defined. We report the case of a 60-year-old woman diagnosed with AAMT, who presented with severe thrombocytopenia, gastroin-testinal bleeding, and significantly reduced bone marrow megakaryocytes. The patient was treated with methylprednisolone, cyclosporin, and intravenous immunoglobulin. After 2 weeks of treatment, her platelet count started to increase, and her bone marrow megakaryocyte count had normalized 3 months after diagnosis. At the time of diagnosis, the patient was seropositive for anti-c-mpl antibody but was seen to be seronegative once the platelet count recovered. In contrast, anti-c-mpl antibodies were not detected in the serum of 3 patients with idiopathic thrombocytopenic purpura. This case study suggests that anti-c-mpl antibody plays an important role in the development of AAMT, and that intensive immunosuppressive treatment is required for autoantibody clearance and recovery of megakaryocyte count., (© 2019 S. Karger AG, Basel.)

Rationale: Two clinical cases are reported of envenomation by the nose-horned viper (Vipera ammodytes ammodytes) venom of a 9-year-old boy and of an 84-year-old woman., Patient Concerns: Both patients had been bitten on their extremities by such a snake in August near Split, a town in southern Croatia., Diagnoses: Clinical manifestation of envenomation was severe in the case of the boy, being characterized by a severe coagulopathy. This was only just apparent in the case of the elderly woman, who suffered extensive local edema and hematoma at the site of the bite, together with a neurotoxic effect-bilateral ptosis. This was the first occasion of thrombocytopenic purpura being observed in patients envenomed by nose-horned viper venom. This unexpected clinical finding was characterized by an unusually profound thrombocytopenia of 5 and 10 × 10/L platelets of the respective patients on their admission to the hospital, together with purpura, observed on the face and thorax of both individuals. In the most serious cases, such pathology can be life threatening if not promptly recognized and treated., Interventions: The patients recovered quickly on receiving the specific antivenom along with all the usual supportive treatments., Outcomes: No serious sequels were noticed at the moment of discharge., Lessons: Our finding constitutes an important message to clinicians to consider the possibility of such complications in the case of nose-horned viper envenomation.

Competing Interests: Competing interests: None declared.

Acquired amegakaryocytic thrombocytopenic purpura (AATP) is a rare hematological disorder characterized by severe thrombocytopenia and a complete or near-to complete absence of megakaryocytes in the bone marrow, while granulopoiesis, as well as erythropoiesis are usually preserved. Although autoimmune mechanisms are believed to be causative, the exact underlying pathogenesis is not known. To date, only few cases have been reported and management of this disease remains controversial with immunosuppression being the treatment modality of choice in the majority of patients. In this article, we report a case of newly acquired AATP without an associated autoimmune disease, refractory to corticoids, intravenous immunoglobulin (IVIG) and second-generation TPO (thrombopoietin) agonists, which have recently been approved for the treatment of thrombocytopenia. Finally, in accordance with other reports, disease progression into aplastic anemia has occurred.

Objective: Corticosteroids generally result in short-lasting neuropsychiatric symptoms following cessation, but the following case highlights an unusually long-lasting course of symptoms in a patient following near immediate cessation of medication, despite medication management and electroconvulsive therapy. The case presentation will be followed by a discussion of the presentation, treatment, and management of steroid-induced neuropsychiatric symptoms., Methods: The patient was followed from symptom onset to resolution., Results: The patient's symptom course was unusually long and required a long course of multimodal therapy., Conclusions: Corticosteroids are commonly used medications both in a wide variety of medical settings, and despite this, their neuropsychiatric effects are poorly understood. The affective and behavioral symptoms, in particular mania and psychosis, can be unpredictable and challenging to treat as in our patient, who developed a long-lasting psychotic episode on high-dose steroids despite discontinuation and treatment of nearly six months. This was despite having tolerated steroids multiple times in the past., (© The Author(s) 2015.)

Eltrombopag is an orally bioavailable thrombopoietin receptor agonist approved for the treatment of thrombocytopenia associated with chronic immune (idiopathic) thrombocytopenic purpura and chronic hepatitis C virus (HCV) infection. This study evaluated the potential drug-drug interactions between eltrombopag and the HCV protease inhibitors boceprevir and telaprevir. In this open-label, 3-period, single-sequence, and crossover study, 56 healthy adult subjects were randomized 1:1 to cohort 1 (boceprevir) or 2 (telaprevir). The dosing was as follows: period 1, single 200-mg dose of eltrombopag; period 2, 800 mg boceprevir or 750 mg telaprevir every 8 hours (q8h) for 10 days; and period 3, single 200-mg dose of eltrombopag with either 800 mg boceprevir or 750 mg telaprevir q8h (3 doses). All doses were administered with food, and eltrombopag was administered specifically with low-calcium food. There was a 3-day washout between periods 1 and 2 and no washout between periods 2 and 3. Serial pharmacokinetic samples were collected for 72 h in periods 1 and 3 and for 8 h in period 2. The coadministration of eltrombopag increased the rate of boceprevir absorption, resulting in a 20% increase in the maximum concentration in plasma (Cmax), a 1-h-earlier time to Cmax (Tmax) for boceprevir, a 32% decrease in the concentration at the end of the dosing interval (Cτ), and no change in the area under the concentration-time curve over the dosing interval (AUC0-τ). The coadministration of eltrombopag did not alter telaprevir pharmacokinetics, and the coadministration of boceprevir or telaprevir did not alter eltrombopag pharmacokinetics. Dysgeusia, headache, and somnolence occurred in ≥2 subjects. One subject withdrew because of nausea, headache, dizziness, sinus pressure, and vomiting. There were no severe or serious adverse events. Dose adjustment is not required when eltrombopag is coadministered with boceprevir or telaprevir given the lack of clinically significant pharmacokinetic interaction., (Copyright © 2014, American Society for Microbiology. All Rights Reserved.)

Purpose of Review: Therapeutic plasma exchange (TPE) is a useful adjunct in the management of antibody-mediated disorders. The indications for TPE now include the perioperative setting. This review updates the anesthesiologist on the relevant clinical indications and precautions of plasma exchange., Recent Findings: Although still considered experimental, TPE for heparin-induced thrombocytopenia for urgent cardiac surgery is the most promising recent advance., Summary: Plasmapheresis, or TPE, removes monoclonal antibodies, immune complexes and paraproteins. The utility of TPE in the perioperative period has recently become more apparent. Antibody-mediated disorders are associated with postoperative morbidity and mortality and are treated with TPE. Indications for TPE for cardiac surgery include heparin-induced thrombocytopenia, thrombotic thrombocytopenia purpura and antiphospholipid syndrome. Other indications for perioperative TPE are typically related to immunomodulation during solid-organ transplant. Immunomodulation, primarily with immunosuppressive medications and TPE, of a previously allosensitized recipient pretransplant increases the likelihood of a successful match. TPE is also useful in the management of intentional and inadvertent ABO incompatible recipients and is essential in the treatment of hyperacute rejection. TPE will likely be more utilized in the future and understanding the essentials of the procedure will facilitate the perioperative management of antibody-mediated disorders.

Immune thrombocytopenic purpura (ITP) complicates 1-2/10 000 pregnancies and accounts for 5% of cases of pregnancy-associated thrombocytopenia. Corticosteroids and intravenous immunoglobulin remain the first-line therapy in pregnancy, and a majority of pregnant women respond to this conventional therapy. Other cytotoxic and immunosuppressive agents used for treatment in non-pregnant patients, for example, danazol, cyclophosphamide, vinca alkaloids and azathioprine, are potential teratogens and cannot be administered during pregnancy. For pregnant women with ITP who fail to respond to medical management and are at a significant risk of haemorrhage due to thrombocytopenia, splenectomy may be considered as an option. We report two cases of splenectomy during pregnancy for refractory ITP. In one patient, it was carried out at 24 weeks, and in the second patient it was carried out during the caesarean section. Splenectomy as a second-line option in cases of refractory severe ITP in pregnancy is discussed.

We present a case of HIV-related thrombocytopenic purpura (HIV-ITP) successfully treated with high-dose dexamethasone and antiretroviral therapy (ART). Although high-dose dexamethasone is regarded as the first-line therapy in adult patients with non-HIV ITP, there is limited information on treatment of HIV-ITP and long-term prednisone therapy is considered the standard therapy. High-dose dexamethasone is preferable to conventional long-term prednisone therapy, because of fewer side effects mainly due to shorter steroid use. The ART helps achieve long-term remission for HIV-ITP, although this therapy lacks an immediate effect. In our patient, administration of high-dose dexamethasone resulted in rapid rise in platelet count and ART maintained long-term remission of HIV-ITP. The combination therapy is potentially suitable strategy for the treatment of patients with HIV-ITP and severe thrombocytopenia or bleeding.

Acquired amegakaryocytic thrombocytopenic purpura (AATP) is a rare disorder characterized by severe thrombocytopenia associated with total absence or a selective decrease in bone marrow megakaryocytes. A 67-year-old male presented with a 2-month bleeding tendency. He was referred to our hospital because of severe thrombocytopenia. Bone marrow biopsy showed complete absence of megakaryocytes without dysplasia in cells of the myeloid and erythroid lineages. AATP was diagnosed. In addition, mild normocytic normochromic anemia and reticulocytosis were also observed and haptoglobin was below the detectable level. Coombs-negative autoimmune hemolytic anemia (AIHA) was diagnosed based on the high titer of RBC-bound IgG and negative direct and indirect coombs test results. He was first treated with cyclosporine 200 mg per day and subsequently with prednisolone but only slight temporary improvement was achieved. Administration of eight doses of rituximab 375 mg/m(2) per week ameliorated both thrombocytopenia and anemia. AATP should be considered in the differential diagnosis of thrombocytopenia, and immunosuppressive therapy is a potential first-line treatment. This is the first case report of AATP accompanied by AIHA successfully treated with rituximab.

Some thrombopoietin receptor-agonists (TPR-As) have been developed and shown to be highly effective in the treatment of immune thrombocytopenic purpura (ITP). Soluble cytotoxic T-lymphocyte-associated antigen 4 (sCTLA-4) can modulate and terminate the immune response. Several reports have shown that sCTLA-4 levels are elevated in patients with some autoimmune disorders. However, sCTLA-4 levels have not previously been investigated in TPR-A exposed patients with ITP. We investigated the levels of transforming growth factor (TGF) β(1) and sCTLA-4 in ITP patients to determine the clinical association with TGFβ(1) and sCTLA-4 in TPR-A-exposed patients with ITP. Thirty-seven ITP patients were divided into 2 groups (TPR-A-exposed: 13 patients; unexposed: 24 patients). Doses of eltrombopag ranging from 12.5mg to 50mg were administered daily, and biochemical data obtained before and after eltrombopag administration were compared. Eltrombopag therapy significantly increased sCTLA-4 and TGFβ(1) levels relative to baseline in patients with ITP. In addition, plasma TGFβ(1) was positively correlated with platelet counts and sCTLA-4 in patients with ITP in the eltrombopag-exposed group. However, no significant change in the detection rate for anti-glycoprotein antibody was observed before and 24 weeks after eltrombopag treatment. These results suggest that eltrombopag can partially modulate some immune responses by TGFβ(1) and sCTLA-4, but it does not induce immune tolerance by 24 weeks after treatment., (Copyright © 2012 Elsevier Ltd. All rights reserved.)

Acquired amegakaryocytic thrombocytopenia is an unusual hematological disorder characterized by thrombocytopenia along with markedly diminished megakaryocytes in the bone marrow. There is no uniform consensus on the treatment and the case reports in pediatric age group are few. The authors report a child with this condition who responded to cyclosporine alone.

A 6-year-old girl, who had received corticosteroid and cyclosporine on the diagnosis of interstitial pneumonitis related to juvenile dermatomyositis, developed severe thrombocytopenia. Her thrombocytopenia was resistant to repeated intravenous immunoglobulin administration and methylprednisolone pulse therapy. After additional treatment with mycophenolate mofetil (MMF), instead of cyclosporine, the thrombocytopenia improved, facilitating a reduction in the dose of corticosteroid without exacerbation of the interstitial pneumonitis. We propose MMF as effective option in the treatment of immune thrombocytopenic purpura with autoimmune disease.

We conducted a retrospective survey to assess prescription practice, response rates to rituximab, and the predictive indicators for a response to rituximab therapy in patients with primary or secondary immune thrombocytopenic purpura (ITP). Data were collected retrospectively from 40 consecutive patients with ITP attending our hospital: 29 (72.5%) had primary ITP and 11 (27.5%) had secondary ITP. Rituximab was given either as four weekly injections (375 mg/m(2)) or two injections of 1,000 mg given 2 weeks apart in 30 and 10 patients, respectively. The primary objective was to evaluate overall (OR) and complete response (CR) to rituximab therapy for ITP. OR was excellent and rapid and similar when secondary ITP was excluded from the analysis: OR was achieved in 28 (71.8%) patients and CR in 22 (56.4%). But, at >6 months, of the 28 responders, only 10 of 22 of the evaluable responses (45.5%) were sustained. In addition, except for the nonsignificant occurrence of antinuclear antibodies, no clinical or biological factors were predictive for OR or CR after the rituximab therapy. Twelve patients received a second course of rituximab. Overall, rituximab therapy achieved a response in two thirds of the patients, but the responders exhibited a high rate of early relapses, with no obvious difference according to the regimen of administration or rituximab dose.

Thrombotic thrombocytopenic purpura (TTP) is an autoimmune disorder characterised by fever, microangiopathic haemolytic anaemia, renal insufficiency, and thrombocytopenia. Neurological involvement, a prominent component of TTP, is characterised by a variety of brain lesions which include reversible cerebral oedema or magnetic resonance imaging (MRI) features of reversible posterior leukoencephalopathy syndrome (RPLS). TTP is frequently associated with deficiency of the von Willebrand factor-cleaving protease, ADAMTS13.Here, we report a case of TTP with severe acute encephalopathy. Posterior leukoencephalopathy and brainstem oedema with triventricular hydrocephalus were observed on MRI. The low activity of ADAMTS13 was not observed and ADAMTS-13 antibodies were absent. Neurological symptoms and patient's condition were completely resolved by plasma exchange therapy in addition to high dose of methylprednisolone.

Serum sickness may occur in patients treated with chimeric monoclonal antibody. Rituximab, an anti-CD20 chimeric monoclonal antibody, is used with increasing frequency in chronic immune thrombocytopenic purpura (ITP). Rituximab is relatively safe; however, serum sickness is reported in 1-20% of patients, more commonly among those with autoimmune conditions. We describe a case of serum sickness in a patient with ITP and review the literature of rituximab-induced serum sickness., (© 2011 The Authors. Internal Medicine Journal © 2011 Royal Australasian College of Physicians.)

Background: The etiology of thrombocytopenia during infancy and early childhood may be different from that of older children, because young children frequently receive vaccines. The following study was performed to understand whether there was a causal relationship between vaccinations and thrombocytopenia., Methods: We retrospectively studied, through chart review, the relationship between vaccination and thrombocytopenic purpura in 20 children with thrombocytopenia (platelet count < 150 x 10³/mm³) under the age of 3 years who were hospitalized between 1989 and 2010. Cases with a history of infectious symptoms/signs between vaccination and the occurrence of thrombocytopenia were excluded. Thrombocytopenia cases not diagnosed as idiopathic thrombocytopenic purpura but as post-vaccination thrombocytopenic purpura should have a similar vaccination-to-thrombocytopenia interval as reported in Western journals, but which should not be more than 9 weeks after vaccination., Results: Of the 20 cases of thrombocytopenic purpura, 12 followed vaccination and 8 were considered idiopathic. Of the 12 post-vaccination cases, 5 occurred after the second dose of hepatitis B virus vaccine at 1 month of age, 4 occurred after the first dose of diphtheria-tetanus-acellular pertussis-containing vaccine at 2-3 months of age, 2 occurred after the first dose of measles-mumps-rubella vaccine at 16 months of age, and 1 occurred after the first dose of varicella vaccine at 14 months of age. One of these 12 cases, who also had a marked decrease in hemoglobin level without bleeding, was suspected to have Evans syndrome., Conclusion: Vaccination may be a risk factor for infant thrombocytopenic purpura., (Copyright © 2010 Elsevier. Published by Elsevier B.V. All rights reserved.)

The aim of our study was to evaluate the influence of steroid therapy on serum cystatin C (cysC) concentrations in patients with acute lymphoblastic leukemia (ALL) and idiopthiás thrombocytopenias purpura (ITP). We studied 17 patients with ITP (girls: boys =5:12, mean age: 7.6 yrs, range between 1 to 17 years) and 18 patients with ALL (girls: boys =6:12, mean age: 6.3 yrs, range between 2 to 17 years). CysC and white blood cell count (WBC) in both group of patients were determined before and after 300 mg/m(2) cumulative dose of steroid therapy. Corticosteroids increased the level of cysC in both groups of patients, however significant increase was found only in ITP patients between pre- and posttreatment values (0.96 +/-0.27 mg/L vs. 1.16 +/- 0.3 mg/L, p =0,02). Pretreatment cysC concentrations were within the reference range in patients with ITP but not with ALL and were significantly higher in ALL patients, than in ITP patients (1.23 +/- 1,12 mg/L vs. 0.96 +/- 0.27 mg/L, p =0,02). Pretreatment WBC of ALL patients were significantly higher than of ITP patients (22.58 G/L, min. 3.5 G/L, max. 102.1 G/L vs. 7.46 G/L, min. 4.8 G/L, max. 12.3 G/L, p =0.03). We have found significant correlation between pretreatment cysC and WBC values in ALL patients (p = 0.04). Although the concentration of cysC may be slightly and reversibly influenced by corticosteroid treatment, cysC is sensitive to detect early and moderate deterioration of GFR in children with cancer.

This study was conducted in 20 children (16 males) (mean age 9.2 +/- 4.34y) with immune thrombocytopenic purpura (ITP) to assess the response to anti-D immunoglobulin. Six patients had newly diagnosed ITP, 6 had persistent ITP and 8 had chronic ITP. The overall response rate was 70% (14/20). The median time to response was 3 days (1-13 days). Response to anti-D was not related to age, sex, severity of bleeding, platelet counts at presentation, ABO blood group, or prior steroid or IVIG response.

Aim: To observe the impact of human serum immunoglobulin treatment of refractory immune thrombocytopenic purpura in clinical efficacy., Methods: 220 patients met the diagnostic criteria of refractory immune thrombocytopenic purpura, patients were randomly divided into treatment group and control group, 110 patients in each group. Groups were given prednisolone tablets 1 mg/ (kg x d), 2 times/d, oral, taking for 2 weeks, and than gradually reducing, the maintenance to be suspended; all-trans retinoic acid, each 10 mg, 3 times a day, oral. On this basis, the treatment group increases the employing blood immunoglobulin 400 mg/ (kg x d) infusion qd for 7 days. The two groups are 4 weeks for the course of treatment, a therapeutic effect after treatment., Results: The total effective treatment group and control group were 94.56%, 80.91%, statistically significant differences between two groups (P < 0.05). Treatment serum IL-2, IFN-gamma, IL-4, IL-10, TGF-beta1, significant differences compared to other indicators (p < 0.05). Platelet counts after treatment, the rates of increase, effective hemostasis time of the treatment group compared with the control group, significant difference (p < 0.05)., Conclusion: The impact of human serum immunoglobulin treatment of refractory immune thrombocytopenic purpura significantly shorten the bleeding time, platelet and platelet rise-time return to normal time.

Nocardia infection is a well recognized complication of the immunocompromised hosts. It is mostly a primary pulmonary infection, which may disseminate to other organs. The central nervous system (CNS) involvement of nocardiosis is usually manifested as brain abscesses. We report a 25-year-old male patient who presented with nocardial pneumonia and meningitis without brain abscess. He was diagnosed as immune thrombocytopenic purpura and methyl prednisolone was started 5 weeks previously. Nocardia spp. was obtained from his cerebrospinal fluid culture, but he died at the 7th day of intensive care. Nocardia meningitis is a rare manifestation of systemic disease. Nocardia meningitis should be considered in the differential diagnosis of meningitis with the coexisting nodular pulmonary lesions in the immunocompromised patient and medications other than co-trimoxazole may be required.

Iatrogenic blood diseases are a primary concern for both patients and physicians. Pathology of blood coagulation system associated with pharmacotherapy has been studied in Samara regional Hemostasiological Centre during the last 30 years. A clinical case of secondary iatrogenic thrombopenic purpura is reported in a patient with a history of uncomplicated allergologic disorder. The disease developed after intake of several drugs at non-toxic doses.

We describe the case of a 76-year-old male presenting a thrombocytopenia at the diagnosis of Hodgkin disease. Basing on bone marrow biopsy and evolution, we diagnosed an immune thrombocytopenia and treated with intravenous gammaglobulins. The platelet count normalized in a few days under this therapy. Immune thrombocytopenia purpura (ITP) is a rare complication of Hodgkin disease (HD). It seems to be due to the production of antibodies directed against platelet membrane proteins. The patient's and the lymphoma's characteristics are not predictive for it to happen. The evolution of HD is also not influenced by its occurrence. Various treatments (including corticoids and immunomodulating agents) have been tried with different efficiencies.

Purpose: Predicting the response to splenectomy in children with immune thrombocytopenic purpura (ITP) continues to be a clinical challenge. The purpose of this study is to identify preoperative predictors of outcome for splenectomy in children with ITP., Methods: The charts of 19 children who underwent splenectomy for ITP were retrospectively reviewed. Platelet responses to treatment are categorized as complete response (CR, > or =150,000/microL), partial response (PR, > or =50,000 but <150,000/microL), or nonresponse (NR, <50,000/microL)., Results: After splenectomy, 13 patients (68%) had CR, 3 (16%) had PR, and 3 (16%) had NR. No correlation existed between CR to splenectomy and any of the following: age, platelet count at diagnosis, last platelet count before splenectomy, platelet count on postoperative day 1, or responses to preoperative intravenous immunoglobulin, WinRho, or Rituximab. However, all 7 patients who had NR to a full course of steroids subsequently had CR to splenectomy. Nonresponse to steroid therapy was directly correlated with CR to splenectomy (P = .01, Fisher's Exact test). Furthermore, postsplenectomy platelet counts were inversely related to peak platelet response to steroids (correlation coefficient = -0.68, P = .01)., Conclusions: Preoperative responsiveness to steroid therapy, as measured by peak platelet count, predicts NR to splenectomy for ITP in children, whereas NR to steroid therapy is highly correlated with CR to splenectomy. These findings challenge the widely held notion that steroid responsiveness portends a favorable outcome after splenectomy., (Copyright 2010 Elsevier Inc. All rights reserved.)

We present the case of a nine-year-old boy with hypoplastic left heart syndrome and immune thrombocytopenic purpura who subsequently developed pneumatosis intestinails with a benign clinical examination. While benign pneumatosis intestinails is a well-known clinical entity, the alarming radiographic findings set off a cascade of clinical angst among many providers. This case reminds physicians to correlate the ancillary study results with the patient's clinical presentation and stability., (Copyright 2009 Society of Hospital Medicine.)

Objective: The aim of this work was to estimate the cost-effectiveness of intravenous immunoglobulin (IVIg) compared with oral prednisone as a treatment for Canadian adults with persistent chronic immune thrombocytopenic purpura (ITP)., Methods: The lifetime costs and effectiveness of IVIg and prednisone were estimated from the perspective of a publicly funded health care system in Canada, using a Markov model that was developed based on a systematic clinical and economic review and recommendations of clinical experts in Canada. Transition probabilities (ie, point estimates and 95% CIs) were estimated from the studies identified in a systematic literature review using a random-effect meta-analysis; point estimates were weighted-mean values from the meta-analysis. No published studies directly estimate the utility weight for patients with relapsed or refractory ITP; therefore, a value of 0.76 was used, based on the mean of the utilities for thrombocytopenia without major bleeding or hemorrhagic stroke. Costs and incremental cost-effectiveness ratios were reported as year-2007 Can $., Results: The incremental costs and quality-adjusted life-years (QALYs) of IVIg versus prednisone were Can $8080 and 0.0071, respectively, resulting in an incremental cost-effectiveness ratio of Can $1.13 million/ QALY in the base-case analysis. The probability of IVIg being cost-effective was 0 if the maximum willingness-to-pay (WTP) value for an additional QALY was below Can $40,000. The probability that IVIg would be cost-effective was only 20%, even if the WTP increased to Can $100,000. The expected value of perfect information (EVPI) and expected value of partial perfect information (EVPPI) were 0 if the WTP was less than Can $30,000. If WTP increased to Can $100,000, the EVPI was Can $1700, and the EVPPI was Can $1010 for utility weights of relapse/refractory states, Can $136 for initial response rates of the treatments, and Can $6 for first-year relapse rates for the treatments., Conclusion: Based on the current available clinical evidence, this model analysis of hypothetical patients suggests that IVIg may not be a cost-effective option for adults with persistent chronic ITP in Canada.