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Although most statin-related myopathy is self-limited, in rare cases statins trigger autoimmune myopathy, which requires control with immunosuppressive therapy. Three patients with this disorder were treated with intravenous immunoglobulin as initial therapy, with some benefit.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune disorder of the peripheral nervous system. This article highlights our current understanding of the condition along with its phenotypic variants that are encountered in clinical practice. The diagnostic evaluation of CIDP includes laboratory studies to detect associated medical conditions and electrodiagnostic studies to assess for demyelination. Current treatment options include corticosteroids, plasma exchange, and intravenous immune globulin, along with alternative therapies that may be used as corticosteroid-sparing agents or for treatment-refractory cases. Approximately 85% to 90% of patients eventually improve or stabilize with treatment, and the long-term prognosis of CIDP is favorable.

In Canada, intravenous immune globulin (IVIg) products are licensed for six disease indications, however it has been demonstrated that patients with a number of other conditions also benefit from IVIg. Here we report the routine clinical use of Octagam(®) 10 % across three Canadian institutions. A total of 135 patients were treated with Octagam(®), for conditions represented by five distinct indication groups. The results of this review indicate that Octagam(®) has been well adopted and is prescribed to Canadian patients similar to other IVIg products. In alignment with current practices, 85 % of Octagam's utilization was classified as appropriate based on Canadian IVIg guidelines.

Focal segmental glomerulosclerosis (FSGS) causes glomerular lesions that can progress to end-stage renal disease. It is suspected to be caused by a circulating factor that is amenable to plasmapheresis removal and exhibits a risk for recurrence in the renal allograft. We present two patients with FSGS recurrence in their allograft kidneys diagnosed by biopsy after significant proteinuria developed in the posttransplant setting. Treatment with therapeutic plasma exchange induced long-term remission in both patients. Spot urine protein:creatinine ratios were monitored and treatment was continued until a target of

La maladie lymphoproliférative post-greffe (MLP) est une complication grave chez les greffés (d’organes solides ou de cellules souches hématopoïétiques) immunosupprimés suite à l'infection par le virus Epstein-Barr (VEB). En l’absence d'une réponse efficace des lymphocytes T cytotoxiques, les cellules B infectées par le VEB peuvent proliférer et donner lieu à la MLP. Dans le cas des receveurs de greffe immunosupprimés, les cellules B infectées par le VEB de façon lytique, produisent activement de nouveaux virions. Ces derniers infectent les cellules B voisines, entraînant leur expansion polyclonale. La gp350, une protéine du cycle lytique située dans l'enveloppe virale, joue un rôle important dans l'infection par le VEB. Elle interagit avec le récepteur CD21 exprimée à la surface des cellules B pour permettre l’entrée du virus. Ainsi, des anticorps neutralisants anti-gp350 sont considérés être des acteurs clés dans le blocage de l'infection, empêchant ainsi le développement de la MLP. L'effet protecteur des immunoglobulines intraveineuses (IgIV) à titre prophylactique contre le VEB et la MLP chez les greffés de cellules souches hématopoïétiques n’est pas clairement démontré. Par conséquent, le premier objectif de cette thèse a proposé d'évaluer l'efficacité des IgIV contre l'infection par le VEB et la MLP chez les receveurs de cellules souches hématopoïétiques. Le deuxième objectif a proposé de déterminer, en utilisant la technique ELISpot, si la présence d'une réponse forte des lymphocytes T contre l'antigène précoce BMLF1 du cycle lytique du VEB pourrait constituer un marqueur de protection contre la MLP chez les greffés de cellules souches hématopoïétiques. Les résultats ont montré d'une part que, si les IgIV peuvent neutraliser efficacement l'infection par le VEB in vitro, ils ne protègent pas efficacement les patients greffés contre l'infection par le VEB in vivo. D'autre part, l'étude de la réponse des lymphocytes T contre des antigènes du VEB a démontré que les cellules T de certains patients sont capables de reconnaître l'antigène lytique BMLF1. Cette réponse spécifique des lymphocytes T peut s’avérer un bon marqueur de la protection contre la MLP. Les résultats de cette thèse démontrent que l’infection lytique au VEB joue un rôle fondamental dans le développement de la MLP. Les données indiquent également que la présence d'une réponse spécifique des lymphocytes T contre un antigène du cycle lytique du VEB peut constituer un bon marqueur de la protection contre la MLP. Cependant, le traitement des patients recevant des greffes de cellules souches hématopoïétiques avec les IgIV n’apparaît pas efficace dans la prévention de la MLP., Post-transplant lymphoproliferative disease (PTLD) is a serious complication in immunosuppressed transplant patients following infection by the Epstein-Barr virus (EBV). In the absence of an efficient cytotoxic T-lymphocyte response, EBV-infected B cells can expand in number and give rise to PTLD. In the immunosuppressed setting, EBV infected B cells are postulated to be lytically activated, such that the virions released can infect bystander B cells, resulting in polyclonal B cell expansion. Because gp350, a lytic-cycle protein located in the viral envelope, plays an important role in EBV infection, anti-gp350 neutralizing antibodies are thought to be a key player in blocking infection, hence preventing the development of PTLD. The use of intravenous immune globulin (IVIG) as a prophylactic against opportunistic infections (including EBV) for patients receiving hematopoietic stem cell transplantation calls into question the effectiveness of IVIG to inhibit or temper EBV infection in this patient group. Therefore the first objective of this thesis proposed to evaluate the efficacy of IVIG against EBV infection and PTLD in hematopoietic stem cell recipients. The second objective proposed to determine, using the ELISpot technique, whether the presence of a strong T cell response against the EBV lytic-cycle antigen BMLF1 could constitute a marker for protection against PTLD in hematopoietic stem cell transplant recipients. The results revealed firstly that, while IVIG can efficiently neutralize EBV infection in vitro, it does not effectively protect transplant patients against EBV infection in vivo. Secondly, the study of the T-cell response against EBV antigens demonstrated that T cells from certain patients are able to recognize the BMLF1 lytic-cycle antigen. This specific T-cell response may prove to be a good marker of protection against PTLD. In summary, the results of this thesis demonstrate that EBV lytic infection plays a fundamental role in PTLD development. The data also indicate that the presence of a specific T-cell response against an EBV lytic-cycle antigen may constitute a good marker of protection against PTLD. However, IVIG treatment of patients receiving hematopoietic stem cell grafts does not appear effective in preventing PTLD.

The author reviews the many proposed mechanisms by which intravenous immune globulin may exert its antiinflammatory and autoimmunity-inhibiting clinical effects. No single mechanism can explain its activity in diseases with diverse pathophysiological pathways.

250 Background: Hematologic malignancies effect both humoral and cell-mediated immunity. We hypothesize that for patients with Chronic Lymphocytic Leukemia (CLL), Non-Hodgkins Lymphoma (NHL), and Multiple Myeloma (MM) outcomes improve with adherence to National Comprehensive Cancer Network (NCCN) guidelines for intravenous immunoglobulin (IVIG).Our objective is to understand resource allocation and implementation of IVIG in outpatient and inpatient settings. We identified a cohort of patients with hypogammaglobulinemia for assessing incidence of sepsis, outcomes, and resource use. We initiated this project by undertaking a large descriptive study of current IVIG use. Methods: A retrospective Institutional Review Board (IRB) approved data capture was conducted covering 2016-2018. Inclusion criteria involved those on an active chemotherapy plan, age > 18 years and diagnosis of CLL, NHL, or MM. IgA deficiency, anaphylaxis to IVIG, planned chemotherapy, inherited immunodeficiency, and thymic deficiency were excluded. We considered patients to be suitable for IVIG if IgG was < 500 mg/dL for CLL and MM and < 400 mg/dL in NHL. Outcomes, number of admissions, sepsis, ICU care, infectious etiology, and monthly IgG levels were examined. Results: A preliminary i2b2 data capture identified a cohort of 563 patients that yielded 12% with bacteremia, 21% with sepsis, and 23% with pneumonia of which 87% were admitted. 28% received IVIG during the 2-year period. Of the 563, 77% were hospitalized and 21% required ICU care. 54% of ICU patients received inpatient IVIG. All influenza and parainfluenza cases were inpatient. Final data analysis will yield greater detail in comparing inpatient to outpatient IVIG use. Conclusions: Large academic institutions appear to have significant variability in use of IVIG in patients with lymphopenia and hematologic malignancies. With this data, we plan to assess for patient-level outcomes based on adherence to NCCN guidelines as a way to enhance Physician Quality Reporting System (PQRS) standards by prioritizing outpatient use of IVIG.

Recurrent embryo implantation failure is a disorder with potentially dreadful physiological and psychological manifestations for those who are affected. Embryo implantation and formation of a functional placenta are processes that require a plethora of regulatory mechanisms involving both maternal and embryonic cells. In the ensuing overview, an attempt is made to understand (1) the pathophysiology of recurrent unexplained implantation failure (2) the available evidence for the two popular treatment modalities, i.e., intravenous immune globulin and intralipid, (3) the limitation of current available data, (4) the pathophysiology and immunomodulatory treatment options for recurrent implantation failure to address the pathological, psychological, and financial distress, and associated impact on couple's quality of life.

P>In children, one-third of immune thrombocytopenic purpura (ITP) patients follow a chronic course. The present study investigated whether treatment with intravenous immunoglobulin (IVIG) at the time of diagnosis of ITP is of prognostic significance, using data from 1984 children entered in Registry I of the Intercontinental Cooperative ITP Study Group. A matched pairs analysis compared children with thrombocytopenia (platelet count = 50 x 10(9)/l at that time point, the former group was less often treated with IVIG than with steroids (P = 0 center dot 02). Prospective studies are required to further explore this potential effect of IVIG.

Long-term treatment of immune-mediated polyneuropathies remains difficult. For acute polyneuritis, or Guillain-Barre syndrome, the established standard therapy utilizes high doses of polyvalent intravenous immunoglobulins (IVIG). A recently published randomized placebo-controlled study of patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) showed IVIG to be clinically effective also for this disorder in both short and long term. This survey presents data of this so-called ICE study ("Intravenous immune globulin for the treatment of chronic inflammatory demyelinating polyradiculoneuropathy"). It also discusses the value of IVIG in the treatment of immune-mediated polyneuropathies.

Despite publication of guidelines for the prevention and management of hyperbilirubinemia in term and late-preterm newborn infants, kernicterus, although rare, continues to occur. Guidelines written for use in one country may not always be universally appropriate. Bearing this in mind, a committee appointed by the Israel Neonatal Society has formulated a set of guidelines, based on those of the American Academy of Pediatrics (2004), but adapted to the realities of the Israeli scene. The guidelines include methods of surveillance of jaundice, prediction of jaundice, assessment of risk factors, discharge planning and post-discharge follow-up, in addition to therapeutic guidelines including indications for phototherapy, exchange transfusion and the use of intravenous immune globulin. Availability of these guidelines to the international community may offer direction to physicians of other countries who may be setting up guidelines for use in their own communities.

Background. Splenectomy is an effective procedure for children and adults with severe or refractory idiopathic thrombocytopenic purpura (ITP). Data regarding pediatric patients are limited. Procedure. Sixty-eight Intercontinental Childhood ITP Study Group (ICIS) investigators from 57 institutions in 25 countries participated in a splenectomy registry. Data from 153 patients were submitted, of whom 134 had a splenectomy and were analyzed. Results. The median age at splenectomy was 11.8 (2.7-20.7) years. The median postsplenectomy follow-up was 2.0 (0.1-4.5) years. Pre-splenectomy vaccination was not administered in 21 children (15.7%). Open and laparoscopic splenectomy procedures were performed in 67 and 65 evaluable children, respectively. Surgical technique was not reported in two children. Overall immediate platelet response to splenectomy was achieved in 113 patients (86.3%). Eighty percent of responders maintained their status of response during the following 4 years. Older age, longer duration of ITP, and male gender correlated with a complete response. Post-splenectomy sepsis was reported in seven patients without lethal outcome, although sepsis might be differently defined at participating institutions. Conclusions. Splenectomy is effective in children with ITP. Management varies greatly in different institutions. These Registry data may serve as a basis for future clinical trials to assess the indication and timing of splenectomy. Pediatr Blood Cancer 2007;49:829834. (c) 2006 Wiley-Liss, Inc.

In Canada, intravenous immune globulin (IVIG) use has increased by 115% over the past 7 to 8 years. Given this increased usage, Canadian Blood Services and the National Advisory Committee on Blood and Blood Products for Canada identified the need to develop and disseminate evidence-based guidelines to facilitate appropriate IVIG use. As a result, guidelines for IVIG use in hematologic and neurologic conditions have been developed and are published in this supplement of Transfusion Medicine Reviews. This commentary provides a brief description of the process used to develop these guidelines and includes a summary of the recommendations for IVIG use in the various conditions evaluated.

Purpose The use of intravenous immune globulin (IVIG) in the management of streptococcal toxic shock syndrome (STSS) and Clostridium difficile infection (CDI) is reviewed. Summary IVIG has a wide range of uses in clinical practice, including STSS and CDI. It is an attractive option for these two infections because both infections are toxin mediated, and IVIG may contain antibodies that neutralize these toxins. For STSS and CDI, IVIG is often considered for use in critically ill patients who are not responding to traditional therapies. Several encouraging case reports and retrospective chart reviews have been published, highlighting the potential benefit of IVIG in such patients. However, its definitive role remains unclear, mainly due to the lack of high-level evidence. Data supporting its use have been extrapolated from retrospective chart reviews and case reports in which profound heterogeneity in patient populations and treatment modalities exist. The use of IVIG must be weighed carefully because it is not a benign product. As with the use of IVIG for STSS, the role of IVIG for CDI is unclear. Nonetheless, IVIG may serve as a useful adjunct therapy for patients suffering from severe complicated CDI (shock, ileus, or megacolon) who do not respond to conventional treatment. Adverse reactions to IVIG are mild and transitory and occur during or immediately after drug infusion. Conclusion Although randomized, controlled trials supporting the use of IVIG for STSS and CDI are lacking, IVIG may be considered a last-line adjunct therapy in those patients for whom the clinical benefit outweighs the potential adverse effects of therapy.

Background: There are differences between Japan and the United States regarding recommended timing of live virus vaccinations after treatment of Kawasaki disease patients with intravenous immune globulin (IVIG): 6 months in Japan and 11 months in the U.S. The prevalence of antibodies to these vaccines using either vaccination schedule remains undetermined. Objective: The present study aimed to evaluate the effectiveness of the live virus vaccination schedule for Kawasaki disease recommended in Japan. Methods: This was a prospective observational study. Kawasaki disease patients aged 6 months and older without past history of or vaccination against measles, rubella, varicella-zoster (VZ), or mumps were enrolled. The children were vaccinated against measles, rubella, VZ, and mumps 6 months after IVIG. Serologic tests for IgG-class specific antibodies to each vaccine virus were performed prior to IVIG; 2 days, 3 months, and 6 months after IVIG, and 3 months after vaccination. The primary outcome was seroprevalence of positive antibodies, which was defined as serum concentration more than 4 IU/mL. Results: A total of 24 children (mean month age 16.8 ± 2.7 at vaccinations, 70.8% male) were enrolled. The rate of measles, rubella, VZ, and mumps seropositivity was 12.5% (3/24), 0% (0/24), 12.5% (3/24), and 0% (0/24), respectively, just before vaccination. The rate increased to 91.7% (22/24), 87.5% (21/24), 20.8% (5/24), and 8.3% (2/24), respectively, 3 months after vaccination. There were no serious adverse events. Conclusions: Use of the Japanese vaccination schedule led to extremely low seroprevalence of VZ and mumps antibodies but acceptable seroprevalence of measles and rubella antibodies. This study is ongoing and more cases (up to 30, the target sample size) are needed before the appropriateness of the timing of vaccination in Japan can be discussed.

Background Immune thrombocytopenic purpura (ITP) is the most prevalent cause of thrombocytopenia in children. Despite the importance of ITP in children under 2-years old, only a few publications are available in the literature.ITP usually presents itself as isolated thrombocytopenia and mucocutaneous bleeding. Materials and Methods This study was conducted on 187 under 2-year-old children diagnosed with ITP and treated at Dr. Sheikh Hospital from 2004 to 2011.In this retrospective study, clinical symptoms, laboratory findings, history of viral infections, vaccination history, and treatment efficacy in children under 2-years old with ITP were investigated.Patients were followed for one year after being discharged from the hospital. Results The risk of the disease developing into chronic form was higher in older children (0.001). ITP in children under 3-months old was significantly associated with vaccination (p=0.007). There was no significant differences between male and female patients in regards to newly diagnosed ITP, persistent, and chronic disease status (p = 0.21). No significant difference in bleeding symptoms was observed between patients under 3-months old and 3 to 24-months old (p=0.18). Conclusion Infantile ITP respond favorably to treatment. The risk of the disease developing into chronic form is higher in 3-to-24-month-old children compared to under-three-month olds.

Background/aims Few studies have addressed whether there are differences in clinical efficacy between intravenous methylprednisolone (methyl-Pd) and intravenous immunoglobulin (IVIg) use. Methods We retrospectively compared platelet responses and toxicities associated with these two treatments in adult patients with immune thrombocytopenia. Patients received intravenous methyl-Pd therapy followed by oral prednisolone (Pd) from 1993 to 2002 and IVIg together with oral Pd from 2003 to 2008. Results Early response and maintenance of the response were assessed at 7 days and 6 months after treatment, respectively. Of the 87 patients enrolled, 77 (88.5%) were eligible for analysis. Early responses occurred in 30 of 39 patients (76.9%) receiving methyl-Pd versus 33 of 38 patients (86.6%) receiving IVIg (p = 0.187). The response was maintained in 28 patients (71.8%) in the methyl-Pd arm and in 23 patients (60.5%) in the IVIg arm (p = 0.187). The time to a complete response in the IVIg arm (6 days; range, 1 to 35) was shorter than that in the methyl-Pd arm (13.5 days; range, 2 to 29) (p = 0.002). Side effects were mild and tolerable in both arms. Five years after initiating treatment, 7 of 18 patients (38.9%) and five of 14 patients (35.7%) were still maintaining a response in the methyl-Pd and IVIg arms, respectively. Conclusion These results indicate that neither the early response rate nor the long-term outcome differed between the methyl-Pd and IVIg treatments. However, IVIg induced a complete response more rapidly than did methyl-Pd.

Purpose To develop and implement a standardized intravenous immune globulin (IVIG) order set for pediatric patients in a community hospital. Methods Standardized orders are used in many hospitals to help guide the medication ordering process. WakeMed Health and Hospitals in Raleigh, North Carolina, developed and implemented a standardized pediatric IVIG order set. The IVIG orders were designed and approved by a multidisciplinary team. All standing orders at WakeMed are maintained by the Clinical Analysis Department and reviewed every 3 years by the pharmacy and medical staff. The standardized IVIG order set must be completed anf signed by the attending physician for all pediatric IVIG orders. Standardization of the IVIG orders helps promote accurate physician medication ordering, provides safe administration guidelines, and minimized the risk of medication errors and adverse events. Results As a result of the implementation of standardized IVIG orders, the hospital has decreased the potential for adverse events and medication errors in the pediatric population and has eliminated product waste. Hospital pharmacists should encourage the development of standardized orders for pediatric patients receiving immune globulin.

The authors report a patient with Guillain-Barré syndrome who developed acute hypertension, reversible posterior leukoencephalopathy syndrome, ischemic and hemorrhagic strokes, and reversible cerebral arterial vasoconstriction shortly after initiating intravenous immune globulin therapy. Possible interrelationships and mechanisms of these complications are discussed.

Guillain-Barré syndrome is an immune-mediated polyneuropathy that frequently presents with progressive muscle weakness. Hyponatremia has recently been described as a feature of this condition, generally appearing over the course of the illness and following the diagnosis of this demyelinating process. We report a case of Guillain-Barré syndrome presenting with severe hyponatremia that is further exacerbated by intravenous immune globulin therapy. Awareness should be raised for consideration of both Guillain-Barré syndrome and its treatment with intravenous immune globulin therapy as the cause of hyponatremia.

Background Data on the short-term risk of disease progression in HIV-1-infected children are needed to address the question of when to begin combination antiretroviral therapy. We estimated 12-month risks of progression to AIDS and death, by age and most recent measurement of CD4 T-cell percentage (CD4%) or viral load, in children receiving no antiretroviral therapy or zidovudine monotherapy only.Methods We undertook a meta-analysis of individual longitudinal data for 3941 children from eight cohort studies and nine randomised trials in Europe and the USA. Estimates of risk were derived from parametric survival models.Findings 997 AIDS-defining events were recorded over 7297 person-years of follow-up in the analysis of CD4%, and 284 events over 2282 person-years in the viral load analysis, corresponding to 568 deaths (9087 person-years) and 129 deaths (2816 person-years), respectively. In children older than 2 years, risk of death increased sharply when CD4% was less than about 10%, or 15% for risk of AIDS, with a low and fairly stable risk at greater CD4%. Children younger than 2 years had worse outlook than older children with the same CD4%. Risk of progression increased when viral load exceeded about 10(5) copies per mL, although this association was more gradual compared with CD4%. Both markers had independent predictive value for disease progression; CD4% was the stronger predictor.Interpretation This information is important for paediatricians making decisions, and for researchers designing trials, about when to initiate or restart antiretroviral therapy.

Antecedentes: La púrpura trombocitopénica inmune es un trastorno autoinmune caracterizado por un descenso de la cifra de plaquetas acompañado habitualmente de diátesis hemorrágica. Las formas crónicas son aquellas en las que la trombocitopenia persiste a los 6 meses del diagnóstico y las formas recurrentes son las que, tras un período de normalidad, experimentan un descenso en la cifra de plaquetas Objetivos: Valorar la evolución, así como la respuesta al tratamiento, de los pacientes afectados de púrpura trombocitopénica inmune crónica persistente y recurrente Métodos: Estudio retrospectivo y descriptivo de los pacientes asistidos en consultas externas en un período de 3 años, desde enero de 1999 hasta diciembre de 2001 Resultados: De 38 pacientes afectados de púrpura trombocitopénica inmune crónica, 16 (42 %) correspondieron a formas crónicas y 22 (58 %) se consideraron formas recurrentes. No se encontraron diferencias significativas entre ambos grupos en cuanto a sexo, edad al diagnóstico, tiempo de seguimiento, infección viral previa, así como presencia de anticuerpos antiplaquetarios. En las formas recurrentes, el tratamiento médico más eficaz fue la gammaglobulina por vía intravenosa (77 % de respuestas favorables), pero la duración de la respuesta fue corta (media, 22,1 semanas). El 63 % de las formas crónicas persistentes obtuvieron una remisión completa mediante esplenectomía. Seis pacientes de ambos grupos, tratados con gammaglobulina anti-D, obtuvieron resultados favorables. El 4,5 % de las formas recurrentes y el 31,5 % de las persistentes remitieron de forma espontánea durante el período de estudio Conclusiones: Con los resultados obtenidos y en nuestra experiencia, el tratamiento más eficaz en las formas recurrentes fue la gammaglobulina intravenosa, pero ningún tratamiento consiguió respuestas duraderas a largo plazo. En las formas persistentes la esplenectomía sería una alternativa eficaz en situaciones de riesgo hemorrágico, mientras que una conducta expectante parece la mejor opción cuando éste no exista. Aunque el número de pacientes tratados es limitado es de señalar el elevado número de respuestas favorables obtenidas con la gammaglobulina anti-D : Background: Immune thrombocytopenic purpura (ITP) is characterized by a drop in platelet count usually accompanied by hemorrhagic diathesis. In chronic forms the platelet count remains low for six months after diagnosis and in recurrent forms the drop in platelet count appears after a period of normality Objectives: To asses outcome and treatment response in patients with chronic or recurrent ITP Methods: We performed a retrospective, descriptive study of patients attended in the pediatric hematology outpatient clinic between January 1999 and December 2001 Results: Of 38 patients with chronic ITP, 16 (42 %) presented chronic forms and 22 (58%) presented recurrent forms. No significant differences were found between the two groups in age, sex, diagnosis, duration of follow-up, previous viral infection, or antiplatelet antibodies. In recurrent forms, the most effective treatment was intravenous immune gamma-globulin (77 % favorable responses) but response time was short (mean: 22.1 weeks). Splenectomy produced complete remission in 63 % of the chronic forms. Good results were obtained in six patients from both groups treated with intravenous anti-D immune globulin. During the study period, 4.5% of patients with recurrent forms and 31.5% of those with chronic forms showed spontaneous remission without treatment Conclusions: In our experience, the most effective treatment for recurrent forms of ITP was intravenous immune globulin, but none of the treatments achieved long-term responses. In chronic forms, splenectomy is an effective alternative when the risk of hemorrhage is high, while a watchful attitude seems to be the best option when this risk is absent. Although the number of patients treated with intravenous anti-D immune globulin was low, good results were achieved

The use of intravenous immune globulin (IVIg) has increased significantly in the past decade, benefiting a wide variety of immune diseases. Seven different formulations of IVIg are now licensed in the United States. Although all contain pooled IgG, there are differences in their production and composition that affect their efficacy, tolerability, and side-effect profile. Important variables include concentration, volume, osmolality, sodium, and sugar content. This article reviews what is known about the composition and properties of the various IVIg formulations that might affect the therapeutic outcome.

This study was conducted to verify whether the response to high-dose intravenous immune globulin (IVIG) was related to the effect of splenectomy in chronic idiopathic thrombocytopenic purpura (ITP) patients. A total of 79 patients over 16 years of age were enrolled in this study. The response to the treatment was classified on the basis of the platelet count as no response (NR, 150 x 10(9)/l). The response was evaluated after the infusion of high-dose IVIG, within 2 weeks after splenectomy (immediate response), and during a follow-up period of more than 6 months after splenectomy (sustained response), respectively. 58 patients (73.4%) showed responses (CR or IR) to high-dose IVIG. After splenectomy, immediate responses were observed in 73 patients (92%). The response to high-dose IVIG had no relationship with the immediate response to splenectomy (P = 0.333). A follow-up evaluation was possible with 58 patients; 6 patients with NR in immediate responses did not show any response during the follow-up period, and 17 patients relapsed within 6 months after immediate responses, so 35 patients (60.3%) had sustained responses. Responders to IVIG had significantly higher sustained response rates to splenectomy than non-responders (62% vs. 38%, P = 0.001). These results indicate that the response to high-dose IVIG could be a valuable factor predicting the sustained response to splenectomy in chronic ITP patients.

Reports of the true incidence of adverse reactions to intravenous γ-globulin (IVIG) therapy are limited by the lack of studies documenting reactions in a large number of patients. The objective was to describe the frequency of serious side effects to intravenous immune globulin therapy at a major tertiary medical center between 1991 and 1995. A central pharmacy registry of a tertiary medical center was searched for all patients and doses of intravenous immune globulin therapy given in both the inpatient and outpatient setting. Records of patients were reviewed for specific details regarding an adverse reaction. Acute renal failure occurred in 2 of 194 (1%) patients and aseptic meningitis occurred in 4 of 194 (2%) patients. Renal failure occurred in 2 of 1637 (0.1%) of infusions and aseptic meningitis occurred in 5 of 1637 (0.3%) of infusions. Both cases of renal failure were adults receiving high doses (2 g/kg) of IVIG. Of the 4 patients who developed aseptic meningitis, 3 were adults and 1 was a child. A...

The clinical challenge lies in recognizing cases not fully meeting the syndrome's diagnostic criteria and those that strongly resemble a variety of infectious and reactive disorders. Prompt treatment with high-dose intravenous immune globulin in combination with aspirin can significantly reduce the frequency and severity of cardiovascular complications.

The purpose of this feature is to heighten awareness of specific adverse drug reactions (ADRs), to discuss methods of prevention, and to promote reporting of ADRs to the FDA's medWatch program (1-800-FDA-1088). If you have reported an interesting preventable ADR to medWatch, please consider sharing the account with our readers.

The Guillain–Barre syndrome is the most common cause of acute flaccid paralysis. Currently, 5% of patients die and 10% are left with severe motor sequelae at one year. Multidisciplinary teams, trained to specific treatments, are required to manage these patients. Oral and intravenous steroid treatment of GBS has been disappointing. Two large randomized clinical trials comparing plasma exchange (PE) to standard supportive treatment have shown a short-term and a one-year benefit of PE. Appropriate number of exchanges and indications of PE are now more precisely known. Patients with mild forms of the disease (able to walk) should receive two PEs, while a further two exchanges should be done in case of deterioration or in advanced forms (loss of walking ability, mechanical ventilation). A greater number of exchanges does not appeared beneficial. More recently, two randomized trials produced some evidence that intravenous immune globulin (IVIg, 0.4 g/kg daily for five days) and PE had equivalent efficiency in advanced forms. The combination of PE with IVIg did not yield a significant advantage, but did increase cost and risk. In advanced forms, the choice between PE and IVIg depends on the contraindications of each treatment.

Many trials have been done on steroid-resistant atopic dermatitis. Recently, intravenous immune globulin (i.v.IG) was reported to be effective in the treatment of steroid-dependent atopic dermatitis. The aim of this study was to clarify whether i.v.IG therapy is effective in steroid-resistant atopic dermatitis. Forty-one steroid-resistant atopic dermatitis patients were tested in this study. Patients who weighed less than 30 kg were administered 500 mg/kg of i.v.IG. Patients who weighed 30 kg or more were administered 15 g of i.v.IG. Patient evaluations and laboratory tests with peripheral bloods such as eosinophil percentages and serum IgE levels were performed at days 0, 1, 7, and 21. In the present study, patients who responded to i.v.IG therapy were classified as Group A. Twelve patients who showed transient effects with lower clinical significance were classified as Group B (29.3%). Remaining 12 patients (29.3%) in Group C showed no improvement at all. Serum IgE levels and blood eosinophil percentages were markedly decreased in Group A. I.v.IG therapy may be recommended in the treatment of atopic dermatitis with extremely high serum IgE levels.

The alloimmunized pregnancy can result in fetal and newborn mortality due to fetal anemia. Control of fetal anemia has not been possible until recently, and management consists of following the degree of fetal anemia during gestation until intrauterine transfusion is feasible to support the fetus until delivery. Cordocentesis and intrauterine transfusion have potential complications that have been well documented. Control of fetal anemia via immune modulation utilizing plasmapheresis and intravenous immune globulin administration has been attempted alone and in combination with varying results. We present a case report of an Rh(D) alloimmunized pregnancy, in which successful management consisted of initial therapeutic plasmapheresis (TPE) followed by intravenous immunoglobulin (IVIG) administration until delivery at 37 weeks gestation without the need for intrauterine transfusion.

In a case series involving 10 patients with recalcitrant recurrent pemphigus vulgaris, a combination of rituximab plus intravenous immune globulin induced complete remission for 10 years or more, with no further therapy.