Biomedical subjects
Publications and source records attributed to J F Greally.
A case of sarcoidosis in an eleven year-old boy which developed four months following B.C.G. vaccination is presented. This was associated with lymphopaenia of undetermined origin which persisted after the pulmonary lesions subsided.
A kindred is described in which neutropenia and low serum immunoglobulin A levels has occurred. The affected members have variable clinical courses which do not appear to relate to the degree of neutropenia. The bone marrows show a maturation delay in granulocyte production, with increased numbers of lymphocytes. In vitro cultures of the bone marrows failed to produce granulocyte colonies. Studies of peripheral blood and bone marrow lymphocyte subpopulations give inconsistent results, although a general increase in T suppressor lymphocytes compared with T helper lymphocytes emerges. A therapeutic trial of corticosteroid and then of lithium carbonate was without effect on one of the patients. The pathogenetic basis for this probably X-linked disorder remains unclear.
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Peripheral blood mononuclear cells (PBMC) from normal donors, pre-cultured at 37 degrees C for 24 hr before the addition of mitogen, demonstrated an enhanced proliferative response. This may be due to the loss of a subpopulation of suppressor cells during the incubation period. Still further enhancement was observed when pre-culturing was prolonged for 48 hr, while cells pre-incubated at 4 degrees C showed no increased responsiveness. Concanavalin A (Con A) pre-activated PBMC supressed the mitogen response of responder cells. More marked suppression was observed when the concentration of Con A used to induce the suppressor cells was increased. It was not possible to activate suppressor function in cells which had been kept in vitro for longer than 48 hr. These findings support the concept of the existence and function of suppressor cells, and that the suppressive influence is short-lived in vitro culture.
A case of chronic lymphatic leukaemia with cold agglutinins is presented in which peripheral blood lymphocytes of varying maturity appeared to possess T- and B-cell markers. A cold agglutinin IgM in type is identified in the serum and at the cell surface. This seems to be responsible for the spontaneous rosetting of sheep red blood cells (SRBC); A single clonal origin of these cells is suggested with maturation arrest. It is also suggested that with configurational change in lymphocyte surface cryoglobulins may sometimes produce spontaneous rosetting with SRBC.
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An improved screening test for both adenosine deaminase and nucleoside phosphorylase is described.
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