SummaryThe outcome of 81 adult aplastic anaemia patients who had successful cytogenetics at diagnosis and received immunosuppressive therapy was evaluated. Ten patients had an abnormal karyotype, six of which had a trisomy. Four of five evaluable patients with a trisomy responded. One patient with monosomy 7 achieved a complete response and later developed haemolytic paroxysmal nocturnal haemoglobinuria but no recurrence of monosomy 7. None of the patients with a non-numerical karyotypic abnormality responded. No significant differences in survival or later clonal disorders were observed between patients with a normal karyotype and those with an abnormal karyotype.
List of recommendations• All laboratories performing H&I testing for allogeneic HPC transplantation should follow EFI standards and be accreditated by EFI and UKAS/CPA. (Grade 1A)• HLA typing definitions as described by Nunes et al. 2011 and here should be used (Grade 1A)• HLA typing results should use official WHO HLA Nomenclature (Grade 1A)• The clinical urgency should be made available to the individual performing the related and unrelated donor search (Grade 1B)• HLA high resolution typing should be performed on potential matching; mismatching and haploidentical related donors when familial haplotypes cannot be fully assigned (Grade 1A)• Patients and selected related donors should be typed for HLA-A, -B, -C, -DRB1 and -DQB1 (+/-DPB1) (Grade 1A).• All patients and donors must have their HLA type confirmed on a second sample pre-transplant (Grade 1A).• The patient should be high resolution typed prior to submitting the HLA type for an unrelated donor search (Grade 1A)• A 10/10 high resolution HLA-A, -B, -C, -DRB1 and -DQB1 matched unrelated PBSC or bone marrow donor should be used where possible (Grade 1A).• Where a 10/10 matched PBSC or bone marrow donor is not available a single mismatch at HLA-A, -B, -C, -DRB1 or -DQB1 is acceptable (Grade 1A).• Alternative progenitor cell donors (cord blood or haplo-identical) should be considered early in the donor search when a patient is unlikely to have an HLA matched unrelated donor (Grade 1A).• HLA-DRB3, -DRB4, -DRB5 typing should be performed and, when a choice of otherwise equally matched and appropriate (e.g. CMV status) donors is available, mismatches for these should be minimized (Grade 2A). • For unrelated donor selection, HLA-DPB1 typing should be performed and when a choice of otherwise equally matched and appropriate (e.g. CMV status) donors is available, non-permissive mismatches should be minimised (Grade 2C).• For mismatched related and unrelated donor selection, HVG mismatches are favoured over bi-directional and GVH mismatches (Grade 2C).• UCB units should be HLA typed to high resolution HLA-A, -B, -C, -DRB1, -DQB1 (Grade 1B ).• Selection of UCB units should follow national consensus guidelines published by Hough et al. (Grade 1A).• HLA alloantibody testing of the recipient should be performed at the time of donor search and should be repeated at the time of donor work-up request if an HLA mismatched donor is selected (Grade 1A).• The clinical team must be made aware of any HLA alloantibody incompatibility for a selected donor (Grade 1A).• When a choice of equally well matched donors is available, avoid selection of donors against which the patient has HLA alloantibodies (Grade 1A).• HLA alloantibody testing should be performed in cases of failed engraftment if the donor is HLA mismatched (Grade 1B).• The guideline published by Emery et al., 2013 recommending CMV matching between patient and donor should be followed (Grade 1A).• Major ABO incompatibilities should be avoided when there is a choice of HLA and CMV matched donors (Grade 1A)• Male donors ...
The first episode of TRALI seems to be due to the action of HLA-A2 and granulocyte-specific IgM antibodies. The second episode may have been due to the action of lipid neutrophil-priming agents in the donors' units in association with the patient's underlying pulmonary condition (i.e., recovering from lung injury). TRALI can recur if a patient requires further transfusion support shortly after an initial episode of TRALI.
Transfusion related acute lung injury (TRALI) is one of the complications of blood transfusion and can result in major morbidity or mortality. The diagnosis depends upon the application of strict clinical criteria defining acute lung injury (ALI) and a temporal relationship to blood transfusion. We present the clinical and immunogenetic findings of 96 suspected TRALI cases investigated between 1996 and 2004. During this time period the national haemovigilance scheme (UK) defined TRALI as a reaction occurring either during or within 24 h of blood transfusion. Using clinical, laboratory and post mortem evidence, 64/96 cases could be defined as TRALI in our series. Sensitive techniques were employed to screen for HLA class I, class II and granulocyte specific antibodies in donor serum. Donor derived antibodies were detected in 58/64 (90%) of cases. Recipient derived DNA or cells were not always available but incompatibility was confirmed by the presence of the cognate antigen on recipient leucocytes or by crossmatching in 47/64 (73%) of cases. Cases referred prior to 2001 were not tested for HLA class II antibodies. By applying strict clinical criteria and using sensitive techniques a white blood cell antibody mediated immunological pathophysiology can be implicated in the majority TRALI cases.
Transfusion-associated graft-vs.-host disease (TA-GvHD) can occur following transfusion of blood products containing immunocompetent lymphocytes, usually from HLA homozygous donors, into immunocompromised patients sharing one HLA haplotype with the donor. The diagnosis of TA-GvHD may be delayed due to the initial nonspecific clinical features involved. Investigations to detect the presence of donor-derived cells in the blood and/or affected tissues of the recipient are essential to confirm the diagnosis. We report the investigation of suspected TA-GvHD using short tandem repeat (STR) analysis, to detect the presence of donor cells (chimerism), in an immunocompetent patient admitted for coronary artery bypass surgery. Peripheral blood and skin biopsies (from affected and nonaffected sites) from the patient and peripheral blood samples from the implicated donors were taken for HLA typing and STR analysis. STR analysis revealed the presence of donor material in the patient's peripheral blood sample and in DNA extracted from the affected skin biopsy but not the unaffected biopsy, suggesting lymphocytes from this donor were responsible for the development of TA-GvHD. Furthermore, HLA typing results supported the diagnosis of TA-GvHD. These data demonstrate the use of STR and HLA analysis as effective tools in the diagnosis of TA-GvHD.
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