Haematology of the Australian eastern quoll, Dasyurus viverrinus--II. Red cell enzymes and metabolic intermediates.

1. The activity of 21 red cell enzymes and three red cell metabolic intermediates were measured in adult Dasyurus viverrinus and compared with published data on other marsupials. 2. Phosphofructokinase (PFK), glyceraldehyde dehydrogenase (GAPD) and phosphoglycerate kinase (PGK) were elevated in comparison to other marsupials. 3. Enolase (ENO) and 2,3-diphosphoglycerate (2,3 DPG) were lower than in other marsupials.

Alcohol-associated haemolysis in Zieve's syndrome: a clinical and laboratory study of five cases.

In 1958 Zieve described a syndrome of jaundice, hyperlipidaemia, and transient haemolytic anaemia associated with alcohol abuse. The clinical and laboratory features of five cases are reviewed. All patients presented with acute abdominal pain and fever. Four had a history of a recent alcohol binge. Hyperlipidaemia was present in two patients; this subsided before the onset of haemolysis. The red cells showed features of an acquired pyruvate kinase deficiency: an increased autohaemolysis with only partial correction with glucose, low red cell ATP, and instability of pyruvate kinase when haemolysate was heated to 55 degrees C. These changes were not observed in a control group of chronic alcoholics without haemolysis.

Hemoglobin Hobart or alpha 20(Bl)His----Arg: a new alpha chain hemoglobin variant.

A new alpha chain hemoglobin variant, Hb Hobart, alpha 20(Bl)His----Arg, was detected in a 60-year-old female of British nationality. The proposita had a history of severe rheumatoid arthritis and had been treated for many years for a refractory microcytic anemia and/or iron deficiency. A hemoglobin electrophoresis screen indicated the presence of a hemoglobin variant, with electrophoretic characteristics similar to a Hb Lepore. However, the level of the variant (17.9%) and the presence of a minor variant Hb A2 band (0.4%) suggested that further investigation was indicated. The variant hemoglobin was purified by column chromatography and the alpha chain subjected to aminoethylation and tryptic digestion. Peptide mapping and amino acid analysis indicated that the histidine residue 20 had been substituted by an arginine residue. The substitution in Hb Hobart is at the first residue in the B Helix of the alpha chain of hemoglobin. As this is an externally placed amino acid in the hemoglobin molecule, a substitution at this position of the hemoglobin molecule would not be expected to cause any functional problems. A family study has shown that at least three other relatives are heterozygous for Hb Hobart. These family members have normal hematological findings.

Myeloproliferative and lymphoproliferative disorders in Tasmania, 1972-80: occupational and familial aspects.

All cases in a population-based series of myeloproliferative and lymphoproliferative (LP) disorders diagnosed in usual residents of Tasmania between 1972 and 1980 were asked for occupational histories and the occurrence of similar diseases in their close relatives. Occupational risks were observed for males who had farmed, mined, or worked in foundries and for women who had farmed or had been hairdressers. For individual diagnoses, the risk to farmers was seen to vary with age at diagnosis, sex, and region. With regard to familial risks, the LP and MP groups seemed to breed true, relatives being affected with a disorder from the other group not more often than would be expected by chance. The risks were of two kinds: Those to siblings and that between parent and child, particularly between mother and son. Occupational risks for the familial cases were limited to farming particularly in males diagnosed at 65 years of age or older. Females possessed elevated risks for the LP group only, and their highest relative risk was for those diagnosed before age 65. This evidence suggests that familial clustering of these disorders partly may be due to common environmental exposures in males from rural kindreds.

Myeloproliferative and lymphoproliferative disorders in Tasmania, 1972-80: patterns in space and time.

During the period 1972-80, 866 patients with myeloproliferative (MP) and lymphoproliferative (LP) disorders were diagnosed in Tasmania. The residential histories of these cases were compared with those of population-based controls. No space-time clustering was demonstrated by individuals of any single diagnosis or group of diagnoses, nor were any regional differences in incidence detected. However, urban-rural differences were observed. Rural exposures were important especially in early childhood and at specific intervals before onset for certain diagnoses. Farm residence in early life yielded heightened relative risks, especially for sufferers from chronic lymphocytic leukemia and acute nonlymphoblastic leukemia. Regional analysis demonstrated elevated risks of residence in orcharding and dairying regions and in an urban-industrial municipality. High risks were associated with latent periods before diagnosis. The findings suggest environmental agents, acting prenatally or in early life, as well as in adult life, as important risk factors in the later development of MP and LP disorders.

Normal infant after treatment of acute myeloid leukaemia in pregnancy with daunorubicin.

Daunorubicin, thioguanine and cytosine arabinoside were administered from the 17th to 34th weeks of pregnancy in a 23-year-old patient with acute myeloid leukaemia. The patient went into remission of her leukaemia, and a normal male infant was born after labour was induced in the 40th week. This experience supports the view that modern regimens of anti-leukaemic drugs may be administered during the second and third trimesters of pregnancy without harmful effects on the foetus.