Deep vein thrombosis followed by internal jugular vein thrombosis as a complication of in vitro fertilization in a woman heterozygous for the prothrombin 3' UTR and factor V Leiden mutations.

Thrombosis of the internal jugular vein is a rare event but one that can have serious consequences. Most cases reported in the literature have occurred in patients with indwelling central venous catheters, in association with head and neck sepsis, or in hypercoagulable states. However, a small number of cases have been associated with in vitro fertilization and more often with the ovarian hyperstimulation syndrome (OHSS). We report the case of a 30-year-old woman heterozygous for both the prothrombin 3' UTR mutation and for the factor V Leiden mutation who presented with a proximal deep vein thrombosis following in vitro fertilization. She subsequently developed an internal jugular vein thrombosis extending into the subclavian and axillary vein despite therapeutic anticoagulation with a low molecular weight heparin. Thromboembolic events can occur in the absence of other clinical features of OHSS, especially in patients with underlying prothrombotic abnormalities. Neck pain and swelling in a pregnant woman, especially one that has undergone in vitro fertilization, should be taken seriously and investigated with duplex scanning and/or MRI. Women with a personal or family history of thrombosis undergoing in vitro fertilization should be made fully aware of the potential thrombotic risks and should be considered for a thrombophilia screen.

Hemolytic transfusion reactions due to anti-e+f detectable only by nonstandard serologic techniques.

A patient was transfused with a total of 14 units of red blood cells (RBCs) over 33 days (January 14 to February 15) at two hospitals. Febrile transfusion reactions were noted on three occasions, and hemoglobinuria was seen twice. Alloantibodies were not detected in a sample dated February 14, following a transfusion reaction, and this sample was referred to the North London Blond Transfusion Centre. Further samples were also obtained from before and after all transfusions at both hospitals. The patient's RBCs typed as A, D+, probable Rh phenotype (cDE/cDE). The direct antiglobulin test was negative, and serum samples following the second transfusion were red/brown in color. Serologic investigations were inconclusive on all samples taken until February 13 (after the fourth transfusion). At this time, a weak anti-e reacting by manual polybrene technique and an anti-e+f reacting by two-stage papain technique were detected. The serum also contained potent HLA antibodies. The patient subsequently received leukocyte-depleted group A, cDE/cDE RBCs with out any untoward effect. This case demonstrates the importance of a complete transfusion history and emphasizes that alloantibodies detectable only by nonstandard techniques can be clinically significant.

Systematic pre-deposit autologous blood provision for elective surgery: an important contribution to hospital blood supply.

From the 8th of September 1987 to the 31st of July 1988 all patients scheduled for major elective orthopaedic surgery were systematically offered the facility of pre-deposit autologous blood storage. Of 251 patients, 204 (81%) were eligible to pre-donate, and of these only 9 declined to do so. 72% of the participants required only autologous blood at operation, 23% required some additional homologous blood. During the study period, autologous blood accounted for 10% of all blood transfused in the district, making a significant contribution to the local blood supply.

Red cell 3-phosphoglycerate level as a diagnostic aid in pyruvate kinase deficiency.

A case of pyruvate kinase (PK) deficiency is described in which the diagnosis was aided by measurement of the 3-phosphoglycerate (3PG) concentration. Review of the literature on the levels of red cell metabolites in 52 families with PK deficiency confirmed that a rise in 3PG is a valuable indicator of a functional deficiency of PK. Estimation of 3PG is relatively easy (and accurate). Furthermore, reticulocytosis, which sometimes makes the diagnosis of PK deficiency more difficult, has minimal effect on the level of 3PG in comparison with all other glycolytic intermediates or PK activity.

Thalassaemia intermedia: interaction of the triple alpha-globin gene arrangement and heterozygous beta-thalassaemia.

Five patients with heterozygous beta-thalassaemia with an unusually severe clinical picture, low haemoglobin levels occasionally requiring blood transfusion, splenomegaly and unusually prominent basophilic stippling were found to have co-inherited a triple alpha-globin gene arrangement on one chromosome (alpha alpha alpha/alpha alpha). It seems probable that the expression of a single extra alpha-globin gene is sufficient in some patients with heterozygous beta-thalassaemia to give rise to a clinically significant degree of dyserythropoietic anaemia.

Distribution of immunoglobulin G subclasses in anti-A and anti-B sera.

Sera from 117 immunologically normal subjects, who had been selected for the presence of high titre ABO system antibody on routine screening, were further evaluated for the presence of IgG and its subclasses IgG1, IgG2, IgG3, and IgG4 using an indirect antihuman globulin technique. Subjects of all ABO groups had the capacity to produce IgG antibodies within each subclass, but those of group O produced the broadest spectrum of IgG subclasses and greatest strength of reactions.

Hodgkin-cell leukaemia of B-cell origin.

A case of lymphocyte-depleted nodular sclerosing Hodgkin's disease with a terminal leukaemic phase is described. Circulating Hodgkin cells were shown to be of B-cell origin by immunological phenotyping and the demonstration of clonal immunoglobulin gene rearrangement.

Cellular basis of immune response to antigens of ABO blood-group system. Capacity to provide help during response to T-cell-dependent ABO-system antigens is restricted to individuals of blood group O.

It is postulated that the near-identity of the A and B antigens in man makes it impossible for individuals of groups A, A2, or B to provide T-cell help during a response to T-dependent non-self antigens by B2 lymphocytes. Thus, people of these groups are unable to produce immune IgG antibody. The presence of naturally occurring IgM antibody to non-self antigen from the age of 6 months is due to a separate system of T-cell-independent B1 cells which have arisen during evolution to protect the host against polysaccharide-encapsulated bacterial pathogens.