Processing and CD3+/CD19+ Depletion of Cadaveric Vertebral Bone Marrow for Primary Immunodeficiency Patients Undergoing Sequential Bilateral Orthotopic Lung Transplant (BOLT) and Bone Marrow Transplant (BMT)

ConclusionFor details of pre/post processing, see Table. Since early 2017 we introduced chest/abdominal cavity wash with 1:1 saline-betadine mix and scrubbing T11-L4VB logs with chlora-prep that has eliminated bacterial contamination for the last 3 products. The first 2 were infused without adverse events following IRB/FDA approved antibiotic prophylaxis.Initially, processing of the VBs was arduous, using a scalpel and wire brush to clean the logs, then dissecting into small pieces with a mallet and osteotome. Since early 2017 a Stryker RemB saw has been used. A physical task that had taken 2 technologists 3 hours to complete has become almost effortless and one tech is freed up. The target pre-cryo CD34+ dose and viabilities have met target at all times, in fact CD34+ recovery has increased>70%, see Table. The entire process (dissection, crushing, filtering, CD3/CD19 depletion and cryopreservation) can be completed in 12 hours with 3 technologists.In summary, we have developed a complex sequence of procedures that even exceeded our target of 2E+06 CD34/kg pre-cryo dose (mean: 4.49E+06) leading to prompt and multilineage engraftment in 3 of the first 4 analyzable patients.
Source: Cytotherapy - Category: Cytology Source Type: research

Related Links:

Condition:   Primary Immune Deficiency Disorder Intervention:   Biological: Octanorm Sponsor:   Octapharma Completed
Source: - Category: Research Source Type: clinical trials
ConclusionExome sequencing as a first-tier test for PIDs granted a diagnosis for 28% of patients. Importantly, molecularly defined diagnoses indicated altered therapeutic options in 34% of cases. In addition, exome sequencing harbors advantages over gene panels as a truly generic test for all genetic diseases, including in silico extension of existing gene lists and re-analysis of existing data.
Source: Genome Medicine - Category: Genetics & Stem Cells Source Type: research
Abstract Background/Aims: Primary immunodef iciency (PID) is a serious comorbid condition in adult asthmatics that have frequent exacerbations, which requires monthly replacement of intravenous immunoglobulin (IVIG). However, the prevalence and clinical significance of PID in adult asthmatics in Korea have not yet been reported. The aim of this study is to assess the prevalence of PID and its association with asthma exacerbation in Korean adult asthmatics. Methods: A total of 2,866 adult asthmatics were enrolled in this study. The PID group was defined as subjects who had lower levels of immunoglobulin G (IgG...
Source: The Korean Journal of Internal Medicine - Category: Internal Medicine Authors: Tags: Korean J Intern Med Source Type: research
DiGeorge syndrome (DGS) is a primary immunodeficiency characterized by various degrees of T-cell deficiency. In partial DGS (pDGS), other risk factors could predispose to recurrent infections, autoimmunity, and allergy. The aim of this study was to assess the effect of different factors in the development of infections, autoimmunity, and/or allergy in patients with pDGS. We studied 467 pDGS patients in follow-up at Great Ormond Street Hospital. Using a multivariate approach, we observed that palatal anomalies represent a risk factor for the development of recurrent otitis media with effusion. Gastroesophageal reflux/dyspha...
Source: Blood - Category: Hematology Authors: Tags: Pediatric Hematology, Immunobiology and Immunotherapy Source Type: research
Guidelines for screening for primary immunodeficiencies (PID) are well-defined and several consensus diagnostic strategies have been proposed. These consensus proposals have only partially been implemented due to lack of standardization in laboratory procedures, particularly in flowcytometry. The main objectives of the EuroFlow Consortium were to innovate and thoroughly standardize the flowcytometric techniques and strategies for reliable and reproducible diagnosis and classification of PID of the lymphoid system. The proposed EuroFlow antibody panels comprise one orientation tube and seven classification tubes and corres...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
Primary immunodeficiency diseases (PID) are clinically and immunologically heterogeneous group of disorders of immune system. Diagnosis of these disorders is often challenging and requires identification of underlying genetic defect complemented by comprehensive evaluation of immune system. Flow cytometry, with its advances in last few decades has emerged as an indispensable tool for enumeration as well as characterization of immune cells. Flowcytometric evaluation of immune system not only provides clues to underlying genetic defect in certain PIDs and helps in functional validation of novel genetic defects, but is also u...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
ConclusionsRespiratory complications are common in patients with HIgM syndrome. A proper awareness of respiratory manifestations in patients with HIgM may result in improved management, reduced morbidity and mortality, and an improvement in the quality of life of the patients.
Source: Journal of Clinical Immunology - Category: Allergy & Immunology Source Type: research
We report the first two cas es of this phenomenon to affect children.
Source: Journal of Clinical Immunology - Category: Allergy & Immunology Source Type: research
ConclusionsThe field of PIDs in India remains largely unexplored and we are faced with various challenges in the diagnosis of PIDs due to lack of awareness as well as absence of equipped immunological laboratory support. The authors propose a methodical step-wise laboratory diagnostic approach that can facilitate early diagnosis and timely intervention of these mis/underdiagnosed disorders.
Source: Indian Journal of Pediatrics - Category: Pediatrics Source Type: research
ngen JJM, Orfao A Abstract Fluorochrome selection is a key step in designing multi-color antibody panels. The list of available fluorochromes is continuously growing, fitting current needs in clinical flow cytometry to simultaneously use more markers to better define multiple leukocyte subpopulations in a single tube. Several criteria guide fluorochrome selection: i) the fluorescence profiles (excitation and emission), ii) relative brightness, iii) fluorescence overlap, iv) fluorochrome stability, and v) reproducible conjugation to antibodies. Here we used 75 samples (45 bone marrow and 30 blood) to illustrate Eur...
Source: Journal of Immunological Methods - Category: Allergy & Immunology Authors: Tags: J Immunol Methods Source Type: research
More News: Bone Marrow Transplant | Children | Cytology | Lung Transplant | Primary Immunodeficiency Disease | Stem Cell Therapy | Stem Cells | Transplant Surgery | Transplants