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Biomedical subjects

M Seligmann

Publications and source records attributed to M Seligmann.

At least 19 recordsLinked to original sources

Hairy-cell leukaemia with polyarteritis nodosa.

In four patients a systemic vasculitis similar to polyarteritis nodosa developed within 2 years of the onset of hairy-cell leukaemia. Arteriographic studies in two patients revealed microaneurysms, and biopsy specimens in three patients revealed a vasculitis affecting medium-sized vessels. Blood neutrophilia and neutrophilic vascular infiltrate were absent. One patient had circulating immune complexes. Two patients responded to corticosteroids alone, one required cyclophosphamide as well as steroids, and one improved without chemotherapy. The association of vasculitis with hairy-cell leukaemia may provide insight into the pathogenesis of arteritis.

Adult

mu-chain disease. Report of two new cases.

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.

Diagnosis, Differential

Receptors for IgM on human lymphoblastoid B and T cell lines.

Receptors for the Fc of IgM were studied together with other lymphocyte markers on cells from nineteen human lymphoblastoid cell lines. Receptors for IgM were found on three of seven T lines and three of twelve B lines studied. Culture conditions needed for an optimal detection of the receptor were different for B and T lines. The presence of the receptor for IgM could not be correlated with that of any other membrane marker.

B-Lymphocytes

Hereditary C2 deficiency associated with common variable immunodeficiency.

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.

Adult

Acute lymphoblastic leukemia with pre-B-cell characteristics.

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.

Antigens, Neoplasm

Immunodiagnosis of alpha chain disease.

Since the early diagnosis of alpha chain disease (alphaCD)) is essential to successful treatment and to epidemiological studies, the available immunodiagnostic techniques were compared for their sensitivity, specificity and ease of performance on a panel of sixteen sera, comprising ten alphaCD sera and six control sera containing either IgA myeloma protein or high levels of polyclonal IgA. Immunoselection by immunoelectrophoresis into gel containing a specially developed anti-Fabalpha antiserum provided the most sensitive and specific detection system for alphaCD protein. The same technique using anti-light chain antiserum for immunoselection was also highly sensitive, but proved less specific, being prone to false positives with difficult IgA myeloma proteins. Somewhat less sensitive, but specific and simple to perform, was immunoelectrophoresis using an antiserum recognizing the conformational specificities of Fabalpha as well as those of the constant region of alpha chains. Immunoselection using the Ouchterlony or rocket techniques proved to be less sensitive and prone to false positives when some IgA myeloma sera were tested.

Antibody Specificity

Celluar immunoglobulins in human gamma- and alpha-heavy chain diseases.

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.

Adult

The immunological classification of acute lymphoblastic leukemias.

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.

Adolescent

Gamma heavy chain disease simulating alpha chain disease.

A young Turkish girl presented with all the clinicopathological features of a digestive form of alpha chain disease. A gamma heavy chain disease protein, however, was found in her serum and also in the cells invading the intestinal mucosa and mesenteric lymph nodes.

Child