Biomedical subjects
Publications and source records attributed to J C Brouet.
Two cases of infantile acute lymphoblastic leukaemia, Burkitt type, are reported. The cytogenetic study revealed a translocation t(8;14) similar to that of Burkitt's lymphoma. This chromosomal rearrangement is considered as a further argument which favours uniqueness of the two diseases.
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We report two cases of mu-heavy-chain disease. Both patients were affected with a lymphoproliferative disease that shared several suggestive features with the previously reported cases of mu-chain disease: the presence of vacuolated plasma cells in bone marrow, a small amount of alpha 2 moving abnormal mu-chain protein, and urinary kappa Bence Jones protein in one case.
A t(8;22)(q24;q11) translocation was found in blood, bone marrow, and ascites cells from a European Burkitt's lymphoma. Cell surface markers were identified as monoclonal IgG. The relationship between these two unusual findings is questionable in this cytologically typical Burkitt's lymphoma.
Cytogenetic findings on five patients with ALL Burkitt's type are reported. A t(8;14) (q23;q32) translocation identical to that found in Burkitt's lymphoma cells was found in each case. The relationship between ALL, L3 type and Burkitt's tumours is discussed.
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The monoclonal immunoglobulin (Ig)M from 5 to 16 patients with Waldenström's macroglobulinemia and a polyneuropathy shared cross-idiotypic antigenic determinants as demonstrated by hemagglutination and hemagglutination inhibition experiments as well as by precipitin reactions. This reactivity was located to the Fab (and not Fc) fragment of the protein. The IgM from 73 patients with macroglobulinemia but without neuropathy all gave negative reactions. In contrast, the monoclonal IgG from a patient with polyneuropathy also possessed similar idiotypic determinants. Since cross-idiotypic determinants are usually related to the combining site of a monoclonal Ig, this finding suggests that the monoclonal Ig of these patients may mediate the nerve injury via their antibody activity, which could be directed either to a nerve antigen or to some component involved in the pathogenesis of the neuropathy.
Homozygous C2 deficiency in a 19-year-old boy was associated with variable immunodeficiency manifested by marked hypoimmunoglobulinemia and impaired antibody responses, normal circulating B lymphocytes, and subnormal T-cell functions. Neither antilymphocytic autoantibodies nor chromosomal abnormalities were found. Serum immunoglobulin levels were within normal limits in his parents and brother who were heterozygous for C2 deficiency. The patient's lymphocytes were homozygous at the HLA-D locus but expressed an antigen different from DW2.
Blast cells from 6 of 50 patients with acute lymphoblastic leukemia (ALL) displayed intracytoplasmic mu chains in the absence of detectable light chains and surface immunoglobulins. These cells also expressed lalike and common ALL antigens. Terminal deoxynucleotidyltransferase was detectable in 2 of 5 cases tested. These blast cells are probably related to early B-cell precursors (pre-B cells). In 4 of 6 cases the disease had a tumoral presentation; the prognostic significance of this new subgroup, which accounts for 20% of patients with non-T non-B ALL, remains to be established.
Proliferating cells from twenty-four patients with alpha- or gamma-heavy chain disease (HCD) were studied by direct immunofluorescence and in several cases by biosynthesis experiments with 14C-amino acid incorporation. In twenty-two patients, the cells contained the HCD proteins only and no light chain synthesis could be detected. Conversely, apparently non-secreted monotypic light chains were found in one case of gamma-HCD and one case of alpha-HCD. The proportion of proliferating cells containing cytoplasmic heavy chains, their appearance and the presence or not of surface heavy chains showed great variation from patient to patient. In some cases, the proliferation predominantly affected either plasma cells or lymphocytes whereas in others the disease seemed to correspond to a proliferation of HCD protein-bearing lymphocytes with persistent maturation into plasma cells. Large cell lymphomas supervening on alpha-HCD belonged to the same proliferating clone as the clone secreting the HCD protein, as shown by surface markers and biosynthesis experiments which demonstrated synthesis but no secretion of HCD proteins. In one patient with gamma-HCD, the cells carried surface gamma and delta chains.
The authors describe a case which demonstrates that nephrotic syndromes occuring during the course of a myeloma are not always of the amyloid type. They emphasize the possibility of a dysglobulinemia being involved in a glomerular nephropathy syndrome, especially when there are histological lesions of a glomerulosclerosis. The published literature is reviewed and the possible pathogenesis of these nephropathies briefly discussed: either protein deposits, the nature of which has not been fully determined, or mesangial modifications induced by the filtered light chains.
The chromosomes of an Epstein-Barr virus-negative European Burkitt's lymphoma cell line were studied. All the cells carried the t(8;14) translocation. One clone had 51 chromosomes and was (+1,+7,+16,+15,+21), whereas another clone also had 51 chromosomes but was (+1q+,+7,+16,+15,+21). A third clone had 46 chromosomes (4q+/-).
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Several rabbit antisera to T cells obtained from various sources (thymus, peripheral blood, brain, T-derived leukemias) were studied with the aim to obtain reagents specific for a subset of T cells. Sera were first absorbed on human tissues and B cells; thereafter these T cell-specific sera were additionally absorbed with T cells of different origin and especially with leukemic T cells, which are likely to represent a clonal expnasion of a subset of T cells with potentially unique antigenic markers. Three antigenically distinct subpopulations of T cells were delineated. The relationship of these subsets with previously defined human T cell subpopulations (T subsets with a receptor for the Fc or IgG or IgM or with a receptor for a lectin from wheat germ agglutinin) was investigated.
The main methods used in order to assess the immunological markers on the blast cells from patients with acute lymphoblastic leukemia (ALL) are critically evaluated. The classification of ALL according to the phenotype of the leukemic cells and their presumed origin is reviewed. The correlations with clinical features and prognosis, as well as their possible therapeutic implications, are discussed.
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