British Society for Haematology,slide session,annual scientific meeting,Glasgow, 2008

A morphology session is held each year at the Annual Scientific Meeting of the British Society of Haematology. Prior to the meeting this year, eight morphology cases were made available to BSH members as glass slides and also digitally as ‘virtual slides’. A panel of invited commentators who had no prior knowledge of the diagnosis discussed the eight cases. An initial limited history and blood count are given with representative images from the case material; this is followed by the discussants’ comments and suggested diagnosis. The actual clinical diagnosis is then given with other relevant information.     

CONTRASTING EFFECTS OF SUBCUTANEOUS PULSATILE GnRH THERAPY IN CONGENITAL ADRENAL HYPOPLASIA AND KALLMANN''S SYNDROME

A patient with congenital adrenal hypoplasia (AH) and hypogonadotrophic hypogonadism was treated with pulsatile subcutaneous GnRH therapy for 16 weeks in an attempt to induce puberty. No rise in serum LH or FSH concentrations occurred despite increasing doses of GnRH (2.8 micrograms/pulse-22.4 micrograms/pulse). In contrast a similar programme of therapy successfully initiated the biochemical changes of puberty in a patient with Kallmann's syndrome. Both patients before therapy had low basal serum LH and FSH concentrations with blunted LH and FSH responses to GnRH stimulation. After 1 week, serum LH and FSH rose into the normal adult range in the patient with Kallmann's syndrome. This study fails to confirm a previous report which suggested that intermittent low dose GnRH therapy may be of value in inducing puberty in AH. The reasons for the difference of pituitary responsiveness to GnRH in AH and Kallmann's syndrome are unclear at present.     

Congenital amegakaryocytic thrombocytopenia with congenital deformities and a leukemoid blood picture in the newborn

Two infants with absent radii are described. Both had hemorrhagic manifestations which occurred within 24 hours of birth, and from which they eventuallydied.

The most important hematologic feature was absence of megakaryocytes inthe bone marrow.

Five cases showing a similar picture have been recorded, four as amegakaryocytic thrombocytopenia, and one as congenital leukemia.

It is suggested that these cases are related on the one hand to Fanconi’s anemia,and on the other to congenital leukemia.

Submitted on July 3, 1956 Accepted on August 16, 1956     

Assessment of cardiac function by M-mode echocardiography in selenium-deficient phenylketonuric children

Selenium (Se) deficiency is associated with cardiac and skeletal muscle dysfunction. Twenty well children aged 2-16 years (10 male) attending the Phenylketonuria (PKU) Clinic at the Royal Children's Hospital, Brisbane, had low Se levels (mean 0.29 +/- 0.02 s.e.m. mumol/L; normal range 0.56-1.16 mumol/L). Their myocardial function was assessed at rest and after exercise provocation by M-mode echocardiography in order to exclude occult left ventricular dysfunction. At rest, fractional shortening (FS) was normal (mean 38.1 +/- 1.1 s.e.m. %, n = 20). After exercise, FS increased significantly (P less than 0.001) from 37.6 +/- 1.4% to 44.3 +/- 1.2%, n = 12). This was associated with a significant rise (P less than 0.001) in heart rate (HR) from 77.3 +/- 3.1 beats/min to 125.8 +/- 5.2 beats/min (n = 12). The normal resting FS and normal increase in FS and HR with exercise is evidence against significant cardiac impairment in this group of Se-deficient children.     

Prophylactic single-dose metronidazole before abdominal hysterectomy

Summary. The vagina is a common source of non-sporing anaerobic bacteria which may be the cause of postoperative infection after hysterectomy. A single vaginal pessary containing 1 g of metronidazole inserted the night before abdominal hysterectomy reduced the mean duration of postoperative pyrexia from 15·4 to 8·3 h ( P <0·05).