The obstetric and gynaecological management of women with inherited bleeding disorders--review with guidelines produced by a taskforce of UK Haemophilia Centre Doctors' Organization.

The gynaecological and obstetric management of women with inherited coagulation disorders requires close collaboration between obstetrician/gynaecologists and haematologists. Ideally these women should be managed in a joint disciplinary clinic where expertise and facilities are available to provide comprehensive assessment of the bleeding disorder and a combined plan of management. The haematologist should arrange and interpret laboratory tests and make provision for appropriate replacement therapy. These guidelines have been provided for healthcare professionals for information and guidance and it is also intended that they are readily available for women with bleeding disorders.

POEMS syndrome and Waldenström's macroglobulinaemia.

A 58 year old man presented with a three year history of impotence, night sweats and ankle swelling. On examination, the patient fulfilled the diagnostic criteria for POEMS syndrome, but was unusual in that he also had underlying Waldenström's macroglobulinaemia with IgM kappa paraproteinaemia. The patient was treated with intermittent chlorambucil and made a good recovery. POEMS syndrome has been described in association with osteosclerotic myeloma and Castleman's disease. The paraprotein involved is usually IgG or IgA with lambda light chains. This case indicates that the presence of lambda light chains is not essential for the pathogenesis of POEMS syndrome. It also emphasises the diversity of plasma cell dyscrasias that can manifest as POEMS syndrome.

A retrospective audit of the combined pituitary function test, using the insulin stress test, TRH and GnRH in a district laboratory.

OBJECTIVE: To assess the value of the combined insulin stress test (IST), thyrotrophin-releasing hormone (TRH) and gonadotrophin hormone-releasing hormone (GnRH) tests. DESIGN: A retrospective audit of 232 such tests performed between 1980 and 1989 inclusive. PATIENTS: One hundred and ninety-seven patients with known or suspected pituitary disease. MEASUREMENTS: IST, TRH and GnRH responses were retrieved from laboratory records. Case notes were surveyed for clinical data and additional results. RESULTS: A basal serum cortisol level of less than 100 nmol/l (or less than 200 nmol/l in patients who had recently received glucocorticoid replacement therapy) accurately predicted a subnormal response to hypoglycaemia. All patients with a basal cortisol level of greater than 400 nmol/l, except those who had recently received steroids, showed a normal cortisol response. In retrospect, by consideration of such basal values, 55% of ISTs could have been avoided if the only aim was to assess cortisol reserve. A deficient growth hormone (GH) response to hypoglycaemia was, however, common in patients with a normal cortisol response. Two-thirds of patients with GH deficiency would have been missed if an IST had been avoided on the basis either of basal cortisol levels alone, or of cortisol responses to an alternative test which did not test GH reserve. There was poor agreement between the pituitary response to TRH and GnRH and basal levels of thyroxine and gonadotrophins respectively, suggesting that these releasing hormone tests are misleading. CONCLUSIONS: The IST provides information regarding pituitary function not provided by other tests of the hypothalamic-pituitary-adrenal axis, so that the choice between the IST and alternative tests must depend on a critical assessment of what information is required. Routine TRH and GnRH testing appears to yield little information of practical clinical value.