Patients Receiving Immunoglobulin Replacement Research Articles

Infectious Complications in Primary Immunodeficiency Patients Receiving Immunoglobulin Replacement Therapy: 5 Years Experience

Infectious Complications in Primary Immunodeficiency Patients Receiving Immunoglobulin Replacement Therapy: 5 Years Experience

Vaccination against respiratory tract pathogens in primary immune deficiency patients receiving immunoglobulin replacement therapy.

Inborn errors of immunity (IEI) increase morbidity and mortality risks, particularly from respiratory tract infections. Hence, vaccination becomes pivotal for IEI patients. This study aims to examine the vaccination and respiratory tract infection rates in a diverse IEI patient cohort undergoing immunoglobulin replacement therapy (IGRT). We retrospectively evaluated IEI patients on IGRT at a tertiary care center. Data on vaccinations and respiratory infections were extracted from medical records. : The study included 33 patients (mean age= 37.7 ± 11.4 years; 17 male). The most common clinical phenotype in our cohort was primary antibody deficiencies (90.9%). Only two patients had a genetic diagnosis, both of whom were brothers diagnosed with Wiskott-Aldrich syndrome (WAS). Almost half (48.5%) of our patients had bronchiectasis and 81.8% were on prophylactic antibiotics. All patients with IEI included in the study were regularly receiving IGRT. The vaccination rate of patients against respiratory tract infections was 42.4%, 57.6%, and 78.8% for influenza, pneumococcus, and COVID-19, respectively. Only one patient (7.1%) who received the influenza vaccine developed an upper respiratory tract infection. However, viral panel analysis could not be performed for this patient as they did not present to the hospital. The COVID-19 vaccination rate was notably higher than that of other vaccines, likely due to increased awareness during the pandemic, aided by public advisories and media influence. We observed higher vaccination rates for the COVID-19 vaccine compared to other vaccines (influenza and pneumococcal vaccines). Although we observed the potential impact of social and governmental influence in increasing vaccination rates, it is crucial to acknowledge that vaccination decisions in IEI patients must be individualized.

Management and outcome of COVID-19 in CTLA-4 insufficiency

AbstractDespite the high incidence of COVID-19 worldwide, clinical experience with severe acute respiratory syndrome coronavirus 2 (SARS-COV-2) in inborn errors of immunity remains limited. Recent studies have shown that patients with defects in type 1 interferon (IFN)-related pathways or those with autoantibodies against type 1 IFNs develop severe COVID-19. We reported the clinical course of 22 patients with CTLA-4 insufficiency and COVID-19 and retrospectively examined autoantibodies against type 1 IFNs at baseline. Data were obtained from the patient interviews and chart reviews. Screening for anti-IFN autoantibodies was performed using a multiplex particle-based assay. Student t test, Mann Whitney, analysis of variance, or χ2 tests were used where appropriate. Twenty-two patients aged from 8 months to 54 years, with genetically confirmed CLTA-4 insufficiency, developed COVID-19 from 2020 to 2022. The most common symptoms were fever, cough, and nasal congestion, and the median duration of illness was 7.5 days. Twenty patients (91%) developed mild COVID-19 and were treated as outpatients. Two patients were hospitalized because of COVID-19 pneumonia but did not require mechanical ventilation. Ten (45%) patients were vaccinated at the time of their first COVID-19 infection. Eleven patients received outpatient treatment with monoclonal antibodies against the SARS-CoV-2 spike protein. During the study period, 17 patients were vaccinated against SARS-CoV-2, with no severe vaccine-related adverse effects. Although median anti-S titers following vaccination or infection were lower in patients receiving immunoglobulin replacement therapy (IGRT) (349 IU/dL) than in those not receiving IGRT (2594 IU/dL; P = .15); 3 of 9 patients on IGRT developed titers >2000 IU/dL. All patients tested negative for autoantibodies against IFN-α, IFN-β, and IFN-ω at baseline. Most patients with CTLA-4 insufficiency and COVID-19 had nonsevere disease, lacked autoantibodies against type 1 IFNs, and tolerated messenger RNA vaccines with few adverse effects. Whether our findings can be extrapolated to patients receiving CTLA-4-targeting checkpoint inhibitors requires further studies.

X-linked agammaglobulinemia: 35 years of experience in a tertiary pediatric hospital in Latin America

Background: X-linked agammaglobulinemia (XLA) is a primary immunodeficiency characterized by arrested B cell development, leading to reduced numbers of B lymphocytes and serum immunoglobulin (Ig) levels, due to mutations in the BTK gene located on the X chromosome (Xq21.3- Xq22). The aim of this study was to describe the clinical and immunologic characteristics of patients with a diagnosis of XLA, seen in a tertiary pediatric hospital in Mexico in the department of immunodeficiency in the last 35 years.Methods: A cross-sectional, retrospective and observational study of patients diagnosed with XLA with follow-up in a reference hospital in Mexico City, in a period of January-1987 to July-2022 was performed, data related to their disease status were taken and statistical analysis were analyzed using SPSS.V.16.0.Results: Information was collected from 44 patients with the diagnosis of XLA. The age of onset was 24 months. The age at diagnosis was 60 months. A delay in diagnosis of 37 months was identified, 54 % of patients required I.V. antibiotics before diagnosis, and 45% had a family member with immunodeficiency. The mean Ig levels were IgG: 359 mg/dl, IgA: 24 mg/dl, and IgM: 25 mg/dl. The 77% had a history of bacterial infections before diagnosis, and 6% had allergic symptoms. The main infection was pneumonia in 75%, acute otitis media in 50%, sinusitis in 59%, meningitis in 13%, cutaneous abscesses in 15%, 36% percent developed complications such as bronchiectasis, and 31% sepsis. Once the diagnosis was established, all patients received Immunoglobulin replacement therapy (IRT). 47% of patients were administered antibiotic prophylaxis.Conclusions: The diagnosis of XLA should be suspected in a patient with recurrent clinical manifestations of encapsulated bacterial infections. The diagnosis is confirmed with serum immunoglobulin levels, two standard deviations below for age, when the flow cytometry is available it can help with the diagnosis. The first contact physician can make the diagnosis.

POS1178 PRESCRIBING RITUXIMAB IN PATIENTS WITH AUTO-IMMUNE DISEASES AND ACQUIRED HYPOGAMMAGLOBULINEMIA: DESCRIPTION OF THE RISK OF SEVERE INFECTION IN 121 PATIENTS BEFORE THE SARS-Cov2 ERA

BackgroundRituximab (RTX) induces rapid, usually complete and prolonged depletion of circulating B cells, and also hypogammaglobulinemia in some patients. There are limited data regarding the risk of severe infection events (SIE) when initiating or continuing rituximab in patients with acquired hypogammaglobulinemia, especially in patients suffering from autoimmune diseases (ADs) other than rheumatoid arthritis (RA) (1).ObjectivesTo describe the risk of severe infectious events (SIE) following initiation (rituximab-naïve patients [RNP]) or continuation of RTX therapy (rituximab-continuing patients [RCP]) in patients suffering from severe ADs other than RA and acquired hypogammaglobulinemia.MethodsWe conducted a single-center retrospective cohort study at the University Hospital of Toulouse (France) between 2010 and 2018. Patients were included if they had received at least one dose of RTX in the year following the evidence of hypogammaglobulinemia (defined as gammaglobulins [GG]≤ 6g/L on serum protein electrophoresis) in the setting of ADs other than RA. The primary outcome was the occurrence of a SIE within 2 years after the date of first RTX infusion (T0) prescribed after the evidence of hypogammaglobulinemia. SIE were infections either fatal or requiring hospitalization.ResultsWe included 121 patients (37 RNP and 84 RCP): 48 had ANCA-associated vasculitis (AAV), 48 multiple sclerosis (MS, n=41) or neuromyelitis optica (NMO, n=7), and 21 another severe AD. RTX was prescribed as induction therapy in 39 patients and as maintenance therapy in 82; 112/121 patients were followed for 2 years. Mean GG level were 5.5 g/L (IQ25-75 4.6-5.7) at T0, 5.5 g/L (IQ25-75 5-6.4) at one year, 5.7g/L (IQ25-75 4.8-6.1) at two years and 8 patients had a decrease of their GG level below 4g/L. Ten patients received immunoglobulin replacement therapy (IGRT) mostly after infection (n=6).Twenty-six patients (23.2%) had at least one SIE during follow-up: 12.8 % in the MS/NMO group with a 2-year incidence at 6.9 (3.1-15.3) per 100 person-years, 29.5 % in the AAV group with a 2-year incidence at 18.3 (9.3-20.1) per 100 person-years, 33.3 % in the ‘other ADs’ group with a 2-year incidence at 22.2 (10.6-46.5) per 100 person-years. Infection was opportunistic in 8 patients (33.3%) and 4 died from SIE.Risk factors of SIE at T0 were male gender (61.5% vs. 39.5% p<0.05), lung disease (65.4% vs. 37.2% p=0.01), renal failure (59.1% vs. 28.6% p=0.01), a higher Charlson comorbidity index (p=0.001), a previous treatment by cyclophosphamide (53.8% vs. 30.2%; p=0.03), ≥ 5 mg/d prednisone (69.2% vs. 33.7%, p=0.003), lack of pneumococcal vaccination (61.5% vs 31.4%, p=0.01). GG level was 5.3 g/l [4.1-5.6] in the ‘SIE’ group vs 5.6 g/l [4.8-5.8] in the ‘no SIE’ group (p=0.04). Incidence of SIE was 46% and 20.2% among patients with GG< 4 g/L or GG≥ 4 g/L, respectively (p=0.07). No multivariable analysis provided reliable results.ConclusionOur study provides useful information for clinicians considering initiating or continuing rituximab therapy in patients with acquired hypogammaglobulinemia before Sars-Cov2 era. Prospective studies are necessary to improve the knowledge on outcome of patients treated by rituximab despite low immunoglobulins levels. Prophylactic IGRT may be appropriate in higher risk patients, especially if the GG level is below 4 G/L.

Serum Allergen-Specific IgE among Pediatric Patients with Primary Immunodeficiency.

Background: Allergy is a clinical condition that reflects a deviated function of the immune system. The purpose of this study was to evaluate serum allergen-specific IgE (sIgE) along with clinical manifestations of allergy in patients with diagnosed primary immunodeficiency (PID). Methods: 72 patients, aged 1–17 years, diagnosed with PID and hospitalized between July 2020 and February 2021 were included in the study. Blood samples were obtained by venipuncture. sIgE (30 allergens), blood eosinophil count, as well as total IgE and IgG were measured and assessed in relation to a detailed medical examination. Results: Serum sIgE was detected in the blood of 50% of the patients in the study group, which significantly correlated (p < 0.0001) with clinical symptoms of allergy. During the period of the study, 61.1% of the patients showed symptoms of allergy, with 77.27% of them having tested positive for sIgE. The total IgE level was elevated in 18.06% of the patients and correlated with clinical symptoms of allergy (p = 0.004). An elevated total IgE level was not observed in children receiving immunoglobulin replacement therapy. Conclusion: The study showed that serum sIgE and total IgE together might be a plausible diagnostic tool for PID patients. However, for patients receiving immunoglobulin replacement therapy, the assessment of total IgE is not useful.

Seasonal Influenza Vaccine: Uptake, Attitude, and Knowledge Among Patients Receiving Immunoglobulin Replacement Therapy

Influenza is a potential cause of severe disease in the immunocompromised. Patients with hypogammaglobulinemia, in spite of adequate replacement therapy, are at risk of significant morbidity and adverse outcomes. A seasonal vaccine is the primary prophylactic countermeasure to limit disease. The aim of this study was to evaluate the attitude, knowledge, and influenza vaccine uptake among Irish patients receiving immunoglobulin replacement therapy (IgRT), as well as uptake in co-habitants. Fifty-seven percent of patients receiving IgRT at a regional immunology referral center completed a questionnaire evaluation. Seventy-six percent of IgRT patients received the influenza vaccine for the 2019 season. Ninety-eight percent recognized that influenza could be prevented with vaccination, and 81% deemed it a safe treatment. Ninety-three percent correctly identified that having a chronic medical condition, independent of age, was an indication for vaccination. Despite excellent compliance and knowledge, many were not aware that vaccination was recommended for co-habitants, and only 24% had full vaccine coverage at home. Those who received advice regarding vaccination of household members had higher rates of uptake at home. This study demonstrates awareness and adherence to seasonal influenza vaccine recommendations among patients receiving IgRT. Over three quarters felt adequately informed, the majority stating physicians as their information source. We identified an easily modifiable knowledge gap regarding vaccination of household members. This data reveals a need to emphasize the importance of vaccination for close contacts of at-risk patients, to maintain optimal immunity and health outcome.

Non-neutralizing antibodies and limitations of serologic testing for severe acute respiratory syndrome coronavirus 2 in patients receiving immunoglobulin replacement products

With the growing surges in coronavirus disease 2019 (COVID-19) cases caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) across the globe, there has been a growing concern regarding its implications among patients with primary immune deficiency disorders (PIDs). Common variable immunodeficiency is a common PID characterized by a failure in B cell differentiation with defective immunoglobulin production, making the patients uniquely susceptible to recurrent infections.1 Although current evidence suggests a higher risk for more severe disease in patients with both primary and secondary immune deficiency states, the exact burden of COVID-19 in these disease states is unknown.

Clinical and immunological features of 44 common variable immunodeficiency patients: the experience of a single center in Turkey

Introduction and objectivesCommon variable immunodeficiency (CVID) is one of the most prevalent forms of primary immunodeficiency characterized by hypogammaglobinemia. Its heterogeneous clinical features include recurrent respiratory tract infections and other complications such as gastrointestinal, autoimmunity, and lymphoproliferative disorders. The aim of this article is to evaluate the general characteristics of CVID patients. Materials and methodsClinical and immunological features of 44 CVID patients were evaluated retrospectively with long-term follow-up. Patients who participated in the study were diagnosed according to the criteria of the European Society for Immunodeficiency Diseases (ESID). ResultsThe median age at onset of symptoms was 2.75 years (range 6 months to 17 years), and the median age at diagnosis was 7.75 years (range 4–20 years). The average delay in diagnosis was 4.6 years (range 1–14 years). Positive family history was 18.2%. Before treatment, patients’ median total serum IgG was 271.5mg/dL, median IgA was 7.5mg/dL, and median IgM was 21mg/dL. Infections were the most common clinical manifestation, and 63.6% of patients presented with sinopulmonary infection as the first manifestation. Bronchiectasis developed in 23 CVID subjects, while bronchiectasis was detected prior to CVID diagnosis in eight patients. All patients received immunoglobulin replacement therapy, and one patient died because of granulomatous lymphocytic interstitial lung disease (GLILD). ConclusionsCVID is a heterogeneous group of immunologic disorders with unknown etiology. There are significant differences in the clinical presentation and prevalence of CVID-related complications among countries. Local guidelines for diagnosis and clinical follow-up are needed.

Utilization of intravenous or subcutaneous immunoglobulins in secondary immune deficiency (ULTIMATE): A retrospective multicenter study

IntroductionWe conducted a retrospective multicenter cohort study of patients receiving Immunoglobulin replacement therapy (IgRT) for secondary immune deficiency (SID) during 2012. MethodsData were retrospectively collected from the first dose of Ig administered in 2012 to 1 year afterward in terms of the indication for IgRT, as well as efficacy and safety. ResultsIn total, 16 hospitals participated in the study, and 368 patients were included. Indications for IgRT were non-Hodgkin lymphoma (82 [22.3%] patients), multiple myeloma (76 [20.7%]), chronic lymphocytic leukemia (64 [17.4%]) and other (79 [21.5%]). Only 89 (24.2%) patients received IgRT according to 2011 European Medical Agency (EMA) recommendations; 196 (53.3%) received prophylactic antibiotics and 262 (76.2%) had an IgG level < 4 g/L before IgRT initiation. ConclusionIn this study, whatever the criteria, only 24.2% of patients with SID who received IgRT met EMA recommendations, which suggests a misuse of IgRT in SID.

Hematopoietic Stem Cell Transplantation for Progressive Combined Immunodeficiency and Lymphoproliferation in Patients With Activated Phosphatidylinositol–3–OH Kinase δ Syndrome Type 1

To examine clinical features, laboratory findings, treatment, and outcomes of patients with activated phosphatidylinositol-OH-3 kinase δ syndrome type 1 (APDS1) and evaluate hematopoietic stem cell transplant (HSCT) as a potential treatment option.This study included 23 patients with confirmed PIK3CD mutations. Nine of these patients underwent HSCT.This was a retrospective study to characterize clinical characteristics, laboratory findings, treatment, and outcomes of patients with APDS1. Patients were identified by patient registry, physician referral, or having been previously reported in literature. Data were obtained by review of medical records and physician questionnaires.Common clinical features of patients with APDS1 included recurrent infections, lymphoproliferation, and enteropathy, with a median age at symptoms onset of 11 years old. The majority (65%) of patients received immunoglobulin replacement, and some additionally had prophylactic antimicrobial agents. Eleven transplantations were performed for recurrent infections or treatment refractory disease among 9 total patients. Fludarabine-based reduced-intensity conditioning regimens were used in all transplants (90–180 mg/m2), and 55% used human leukocyte antigen–mismatched donors. All but 1 patient ultimately achieved engraftment, but 36.4% had initial graft failure and required retransplantation, boosts of peripheral blood stem cells, or growth hormone administration for delayed T lymphocyte recovery. More than 90% of transplanted patients had an adverse complication, most commonly mild graft-versus-host disease. Despite this, most symptoms were improved post-HSCT, and no patients required ongoing immunoglobulin replacement by day 100 post-HSCT. The 30-year overall survival was 86.1%, and event-free survival was 39.6%.HSCT should be considered in patients with APDS1 and recurrent infections and/or treatment refractory lymphoproliferation. Clinical symptoms improved posttransplantation, although adverse complications and graft failure were frequent.Patients with APDS1 are at increased risk for recurrent infections, bronchiectasis, lymphoproliferation, chronic viral infections, and lymphoma. This article highlights the role that HSCT may play in the future management of patients with APDS1. Although not without its own risks, early HSCT can improve clinical symptoms and reduce the lifetime risk for potentially fatal complications in this disease. Investigators in this study closely examine HSCT regimens for APDS1 patients among various international medical centers, which serves as the first critical step toward improving event-free and overall survival for these patients.

B-cell differentiation and IL-21 response in IL2RG/JAK3 SCID patients after hematopoietic stem cell transplantation

AbstractAllogeneic hematopoietic stem cell transplant (HSCT) typically results in donor T-cell engraftment and function in patients with severe combined immunodeficiency (SCID), but humoral immunity, particularly when using donors other than matched siblings, is variable. B-cell function after HSCT for SCID depends on the genetic cause, the use of pre-HSCT conditioning, and whether donor B-cell chimerism is achieved. Patients with defects in IL2RG or JAK3 undergoing HSCT without conditioning often have poor B-cell function post-HSCT, perhaps as a result of impairment of IL-21 signaling in host-derived B cells. To investigate the effect of pre-HSCT conditioning on B-cell function, and the relationship of in vitro B-cell function to clinical humoral immune status, we analyzed 48 patients with IL2RG/JAK3 SCID who were older than 2 years after HSCT with donors other than matched siblings. T follicular helper cells (TFH) developed in these patients with kinetics similar to healthy young children; thus, poor B-cell function could not be attributed to a failure of TFH development. In vitro differentiation of B cells into plasmablasts and immunoglobulin secretion in response to IL-21 strongly correlated with the use of conditioning, donor B-cell engraftment, freedom from immunoglobulin replacement, and response to tetanus vaccine. Patients receiving immunoglobulin replacement who had normal serum immunoglobulin M showed poor response to IL-21 in vitro, similar to those with low serum IgM. In vitro response of B cells to IL-21 may predict clinically relevant humoral immune function in patients with IL2RG/JAK3 SCID after HSCT.

Clinical spectrum and features of activated phosphoinositide 3-kinase δ syndrome: A large patient cohort study

BackgroundActivated phosphoinositide 3-kinase δ syndrome (APDS) is a recently described combined immunodeficiency resulting from gain-of-function mutations in PIK3CD, the gene encoding the catalytic subunit of phosphoinositide 3-kinase δ (PI3Kδ).ObjectiveWe sought to review the clinical, immunologic, histopathologic, and radiologic features of APDS in a large genetically defined international cohort.MethodsWe applied a clinical questionnaire and performed review of medical notes, radiology, histopathology, and laboratory investigations of 53 patients with APDS.ResultsRecurrent sinopulmonary infections (98%) and nonneoplastic lymphoproliferation (75%) were common, often from childhood. Other significant complications included herpesvirus infections (49%), autoinflammatory disease (34%), and lymphoma (13%). Unexpectedly, neurodevelopmental delay occurred in 19% of the cohort, suggesting a role for PI3Kδ in the central nervous system; consistent with this, PI3Kδ is broadly expressed in the developing murine central nervous system. Thoracic imaging revealed high rates of mosaic attenuation (90%) and bronchiectasis (60%). Increased IgM levels (78%), IgG deficiency (43%), and CD4 lymphopenia (84%) were significant immunologic features. No immunologic marker reliably predicted clinical severity, which ranged from asymptomatic to death in early childhood. The majority of patients received immunoglobulin replacement and antibiotic prophylaxis, and 5 patients underwent hematopoietic stem cell transplantation. Five patients died from complications of APDS.ConclusionAPDS is a combined immunodeficiency with multiple clinical manifestations, many with incomplete penetrance and others with variable expressivity. The severity of complications in some patients supports consideration of hematopoietic stem cell transplantation for severe childhood disease. Clinical trials of selective PI3Kδ inhibitors offer new prospects for APDS treatment.

High Readmission Costs in Patients with Respiratory Syncytial Virus Following Haematopoietic Stem Cell Transplantation

BackgroundRespiratory syncytial virus (RSV) is a common cause of respiratory viral infections and is associated with increased morbidity and mortality in patients undergoing haematopoietic stem cell transplantation (HSCT). Little is known about the economic burden associated with RSV infection. We sought to analyse the readmission rates and costs associated with RSV infection in a single UK centre.Patients and methodWe undertook a retrospective case analysis of 48 consecutive patients diagnosed with RSV infection post HSCT between December 2010 and April 2014. The frequency of infection was 35/227 (17%) in allogeneic HSCT and 13/180 (7%) in autologous HSCT recipients.Patient characteristics: male 28, median age 55 (range 23 to 71), diagnosis: acute leukaemia 15, multiple myeloma 16, myelodysplasia 6, aplastic anaemia 5, chronic leukaemia 3, other 3. In patients who had received an allogeneic transplant 22 had reduced intensity conditioning (RIC) regimen and 13 myeloablative conditioning. One patient received a cord blood transplant (CBT), 14 had a sibling allograft, whereas 20 had a volunteer unrelated donor (VUD) HSCT. In 20 patients, Alemtuzumab was part of the conditioning regimen. Twenty-seven patients were on immunosuppressive drugs (ciclosporin, mycophenolate mofetil, tacrolimus or corticosteroids) at time of diagnosis and 15 had active graft versus host disease (GVHD). At diagnosis, 37 patients were lymphopenic with lymphocytes below 1.5x10e9/L.Diagnosis was established by polymerase chain reaction (PCR) assay for respiratory viruses and was performed as part of routine screening and in patients with respiratory symptoms. At diagnosis, 26 patients presented with lower respiratory tract infection (LRTI) as defined by new infiltrate on chest X-ray (CXR), signs on auscultation or hypoxia, whereas 22 experienced upper RTI. A CXR was performed in 39 patients and revealed infective or inflammatory changes in 12 patients, all with LRTI signs.The primary indication for treatment was LRTI signs. Patients who were felt to be at high risk of progression to LRTI also received therapy. In total, 42 patients received Ribavirin: orally 16; aerosolised 17; aerosolised and orally combined 5; aerosolised and IV combined 4. Six patients received no treatment. The median duration of treatment was 7 days (range 5 to 47 days). Additional therapy was given: 13 patients received immunoglobulin replacement, 33 had concomitant antibiotic treatment and 11 also received antifungal treatment.ResultsRibavirin was well tolerated with mild side effects associated with aerosolised administration: claustrophobia 2, headaches 1, nausea 1. Twenty patients were admitted for treatment of RSV infection, 6 of which required intensive care unit (ICU) admission including 4 who required invasive ventilation. Fifteen patients were treated as out-patients. Median length of ward stay was 10 days (range 4 to 55 days). ICU admission was associated with a median stay of 5 days (range 4 to 60 days). An additional 13 patients were diagnosed as in-patients and RSV was not felt to have extended their hospital stay. After a median follow-up period of 6.5 months (range 0-39): 33 patients are alive, 15 patients died. The causes of death were sepsis 3, relapsed disease 3, GVHD 2, gastrointestinal bleed 1, stroke 1. In 5 patients the cause of death was attributed to RSV infection, 4 having had previous ITU stay. No RSV-related deaths were recorded in the group treated with oral Ribavirin.Admission costs were calculated based on the 2014 hospital tariff. Median cost was £3,700 for ward stay (range £1,480 to 20,350) and ICU admission with a median cost of £7,340 (range £5,872 to 88,080). The median cost of aerosolised ribavirin (6 g/day 2014 pharmacy prices) was £800 (range £570 to 1,596). The median cost of oral ribavirin (1000 mg/day based on 70kg) was £98 (range £28 to 658) and for the IV formulation median cost was £7,920 (range 3,394 to 12,445).ConclusionsRSV in post HSCT patients remains a challenge due to the high frequency of infection and high rates and costs of readmission. Oral ribavirin may be an option for lower risk patients and strict isolation of patients in waiting areas to prevent transmission could be a cost effective measure. Prompt initiation of treatment in high risk patients is essential due to increased morbidity and mortality associated with RSV- associated LRTI. DisclosuresNo relevant conflicts of interest to declare.

Pathogen-specific IgG antibody levels in immunodeficient patients receiving immunoglobulin replacement do not provide additional benefit to therapeutic management over total serum IgG

Pathogen-specific IgG antibody levels in immunodeficient patients receiving immunoglobulin replacement do not provide additional benefit to therapeutic management over total serum IgG

Survey of infection in patients receiving antibody replacement treatment for immune deficiency

Background:0 Primary antibody deficiency disorders are a heterogeneous group of disorders, which are treated by regular infusions of immunoglobulin. Despite replacement treatment, patients remain susceptible to infection. Effective management of...