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Biomedical subjects

I J Temperley

Publications and source records attributed to I J Temperley.

At least 37 records · Page 2Linked to original sources
PubMed1983

A pyruvate kinase variant with an increased affinity for phosphoenolpyruvate in an individual with congenital non-spherocytic haemolytic anaemia.

A red-cell pyruvate kinase (PK) variant from an individual with congenital non-spherocytic haemolytic anaemia was characterized according to the procedure recommended by the International Committee for Standardization in Haematology (ICSH 1979). Activity of the mutant enzyme in haemolysates was one fifth of that found in normal control subjects. The electrophoretic mobility and thermostability of the mutant enzyme were lower than those of the normal enzyme and its apparent affinity for phosphoenolpyruvate was higher.

Adenosine Triphosphate↗
PubMed1982

Lymphocyte sub-populations following splenic irradiation in patients with chronic lymphocytic leukaemia.

Four patients with chronic lymphocytic leukaemia were treated with splenic irradiation, 100 rads per week for 10 weeks. No patient had received prior therapy of any kind. All patients showed an excellent clinical response in terms of decrease in lymphocyte count in blood and bone marrow and decrease in lymph node and spleen size. B and T cell populations returned to normal in all patients as did the ratio of TG to TM cells. Two patients showed an increase in IgM levels, but the overall pattern of immunoglobulins showed no consistent change following treatment. No toxicity was incurred during treatment. We conclude that splenic irradiation is a safe and effective treatment of previously untreated patients with CLL and is associated with a return to normal of lymphocyte sub-populations. The role of the abnormal T cell population in the pathogenesis of the hypoimmunoglobulinaemia remains unclear.

B-Lymphocytes↗
PubMed1981

Ichthyosis and marrow involvement in malignant histiocytosis of the intestine.

It has recently been suggested that a number of small bowel lymphomas arise from histiocytic cells in the intestine. This has been referred to as malignant histiocytosis of the intestine (MHI) (Isaacson et al, 1979). Three such patients with small bowel malabsorption had the following features: subtotal villous atrophy of the jejunum, generalized ichthyosis, fever and lymphopenia are described. In all three, the bone marrow appearances were similar to those seen in histiocytic medullary reticulosis (HMR) (Scott & Robb-Smith, 1939). A tumour was present in the small bowel and/or mesentery of all patients and the histological lesion was similar to that described as MHI. Two patients had a response to combination chemotherapy. One patient had a complete remission of his disease but this relapsed after 1 year and proved refractory to chemotherapy. A second patient died following chemotherapy with gastro-intestinal perforation and septicaemia and the third patient died shortly after diagnosis and before chemotherapy could be commenced. It is suggested that the combination of signs, symptoms and pathological features described may reflect a specific clinical entity which has not previously been described.

Adult↗
PubMed1980

Congenital dyserythropoietic anaemia type II (HEMPAS): a family study.

A family having two affected siblings with congenital dyserythropoietic anaemia type II (HEMPAS) is described. The proband was diagnosed after referral for investigation of haemolytic anaemia. Clinical evaluation and in vivo red cell (RBC) survival and the sequestration studies in the proband indicated that the anaemia was due to a combination of ineffective erythropoiesis and premature destruction of RBCs in the spleen. Scanning electron microscopic examination of peripheral RBCs was undertaken and is reported. The polypeptide composition of RBC membranes was also examined using polyacrylamide gel electrophoresis after solubilisation in sodium dodecyl sulphate. These results are also reported.

Anemia, Dyserythropoietic, Congenital↗