U.K. consensus statement on the use of extracorporeal photopheresis for treatment of cutaneous T-cell lymphoma and chronic graft-versus-host disease.

Extracorporeal photopheresis (ECP) has been used for over 30 years in the treatment of erythrodermic cutaneous T-cell lymphoma (CTCL) and over 20 years for chronic graft-versus-host disease (cGVHD). The lack of prospective randomized trials has led to different centres having different patient selection criteria, treatment schedules, monitoring protocols and patient assessment criteria. ECP for CTCL and cGVHD is available only at six specialized centres across the U.K. In the recent Improving Outcomes Guidance the National Institute for Health and Clinical Excellence endorsed the use of ECP for CTCL and because of the complexity of treatment supported its use in specialized centres and also suggested the need for expansion of this service. In 2005 consultants and senior nurses from all U.K. sites and from Scandinavia formed a Photopheresis Expert Group. This group's first aim was to produce a consensus statement on the treatment of CTCL and cGVHD with ECP using evidence-based medicine and best medical practice, in order to standardize ECP eligibility, assessment and treatment strategies across the U.K.

Outcome of consecutive pregnancies in a patient with Bombay (Oh) blood group.

A young lady with a rare Bombay (Oh) blood group had two successive uneventful pregnancies. Her serum contained a potent high-titre anti-H and serological as well as chemiluminescence tests, suggesting that the antibody was haemolytic. Her husband was of the normal H status. Theoretically, both babies should have been positive for the H antigen and should have suffered from haemolytic disease of the newborn. This apparent conundrum could be owing to the weak expression of the H antigens on the infant red cells.

Refractory hyperhaemolysis in a patient with beta-thalassaemia major.

We report the case of a 1-year-old girl with newly diagnosed beta-thalassaemia major. Following an initial blood transfusion with phenotypically matched blood, she developed a haemolytic anaemia which progressed with subsequent transfusions. The Direct Antiglobulin test (DAT) was strongly positive with C3d and weakly with IgG. The only free antibodies detected were a weak anti-H and a weak cold auto-antibody, which did not exhibit a wide thermal range. The indirect Donath-Landsteiner and Ham's tests were negative. There was no sustained clinical response to steroids, immunoglobulin infusions or splenectomy. An HLA identical sibling donor was available for allogeneic bone marrow transplantation (BMT) and the haemolysis resolved during the immunosuppressive transplant conditioning. Such hyperhaemolysis without significant red cell alloantibodies has previously been reported in patients with sickle cell anaemia, but only rarely in patients with beta-thalassaemia major.

Identification of an HLA-A11 serological variant and its characterization by sequencing based typing.

We have identified an HLA-A11 variant allele, A*1105, segregating in a Caucasoid family. The variant antigen expressed by this allele failed to cross-react with most Caucasoid anti-HLA-A11 antisera tested. Sequencing based typing has been used to characterize this new allele and this showed that it has a novel mutation at a polymorphic position (502) in exon 3. In comparison with A*1101, the mutation (A-->G) results in an amino acid change from positively-charged lysine to negative glutamate and this may explain the altered HLA-A11 serological profile exhibited by this antigen. The new allele was found in a patient with acute lymphoid leukaemia (ALL), her father and two siblings.

Five-day storage of platelet concentrates. II. In-vivo studies.

Following in-vitro tests it was concluded that platelet concentrates stored for 5 days at 22 degrees C in polyolefin containers, coded PL732, should be as effective in clinical practice as similar concentrates stored in the standard PVC containers, coded PL146. These predictions have been confirmed by the following in-vivo tests; autologous survival studies in volunteers, determination of recovery, platelet increment calculations 1 and 24 h after transfusion and clinical appraisal after transfusion of haemorrhagic thrombocytopenic patients. Bacteriological cultures of the platelet concentrates were sterile after storage for more than 5 days. It can be concluded that the 5-day storage of platelet concentrates in these containers is a practical proposition.

Five-day storage of platelet concentrates. I. In-vitro studies.

Platelet concentrates have been prepared in standard PVC (PL-146) packs and polyolefin (PL-732) packs. Comparison of pH and hypotonic stress of the platelet concentrates has shown that significantly improved values are obtained after storage of the concentrates at 22 C in PL-732 packs for 5 days than after storage for 3 days in PL-146 packs. There was no significant difference under conditions of the test between vertical and horizontal agitation of the concentrates. Since both pH and hypotonic stress recovery have shown a relationship to post-transfusion recovery and viability, evidence is presented to support the 5-day storage period of concentrates prepared and stored in PL-732 packs.