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Biomedical subjects

M Seligmann

Publications and source records attributed to M Seligmann.

At least 73 records · Page 4Linked to original sources
PubMed1983

Waldenström's macroglobulinemia and peripheral neuropathy: a clinical and immunologic study of 25 patients.

We investigated, by indirect immunofluorescence, the binding of monoclonal IgM to human peripheral nerve in 25 patients with Waldenström's macroglobulinemia and peripheral neuropathy. In 10 cases (40%), an antibody activity against the myelin sheaths was demonstrated. The reactivity was mediated by the F(ab')2 fragments of the IgM. Prior delipidation of nervous tissue was needed to allow full expression of the target antigen(s). In nine cases, the IgM reacted with both peripheral and central myelin of primates, but not mouse or rabbit nervous tissue. In one case, the IgM reacted only with human peripheral nerve. The patients, whose IgM had an antibody activity to myelin antigen(s), had some distinct hematologic and neurologic features. The peripheral neuropathy always antedated the hematologic symptoms by several years. The serum level of the monoclonal IgM was low in all cases, and overt lymphoid malignancy was frequently absent. In other patients with neuropathy, the monoclonal IgM, which lacked antimyelin antibody activity, displayed either cross-idiotypic antigenic determinants or anti-intermediate filament antibody activity. These results taken together emphasize the heterogeneity of antibody activity of the monoclonal IgM from patients with Waldenström's macroglobulinemia and peripheral neuropathy.

Antibodies, Monoclonal↗
PubMed1983

Association of the human type C retrovirus with a subset of adult T-cell cancers.

To determine whether the human T-cell lymphoma-leukemia virus (HTLV) is associated with particular cancers, patient sera were surveyed for HTLV-specific antibodies. An association was seen with aggressive cancers of mature T-cells, specifically Japanese adult T-cell leukemia (ATL) and T-cell lymphosarcoma cell leukemia (TLCL), a similar cancer of Caribbean blacks. Ninety to 100% of these patients possessed HTLV-specific antibody. Forty-seven and 20% of relatives of ATL and TLCL patients, respectively, and 12 and 4% of healthy donors from ATL and TLCL endemic areas were also antibody positive. Visceral organ involvement, hypercalcemia, and skin manifestation, features of ATL and TLCL, were often seen in other antibody-positive patients. Childhood cancers, most cutaneous T-cell and all non-T-cell leukemias and lymphomas, myeloid leukemias, Hodgkin's disease, and solid tumors were not associated with HTLV. Healthy United States donors and European patients with non-malignant diseases were antibody negative. HTLV is thus associated with a subtype of adult T-cell leukemia-lymphoma, clustered in viral endemic areas, with apparent racial and geographic predilection.

Adult↗
PubMed1983

[Treatment of chronic autoimmune thrombopenic purpura with intravenous immunoglobulins. Study of 18 cases].

The authors reported the effect of high dose intravenous immunoglobulin therapy in 18 patients with chronic autoimmune thrombocytopenic purpura. A good but transitory response was observed in 60% of the cases. The intensity of the response was variable, the duration was between 11 and 26 days. The mechanism of the effect of this therapy is poorly understood. Since this therapy is expensive and the response inconsistent and transitory, the indications are limited.

Adolescent↗
PubMed1982

[Light chain or monoclonal immunoglobulin deposition disease: physiopathogenic concepts].

Although recently identified, this disease is by no means exceptional. It is characterized by the deposition in various organs of an amorphous substance which differs from the amyloid substance and contains monoclonal immunoglobulin determinants: either a light kappa or lambda chain, or a light and a heavy chain. The severity of the disease is due to various organs being involved, notably the kidneys. There is in every case a monoclonal plasmocytic or lymphoplasmocytic proliferation which may appear as benign. In almost one-third of the cases no monoclonal immunoglobulin can be detected in the serum. In a study of immunoglobulin biosynthesis, 6 out of 8 patients showed striking structural abnormalities. The relationship between these very unusual lg's and tissue deposition is discussed in detail.

Chemical Phenomena↗
PubMed1982

[Mechanisms and prognosis of neutropenia in Felty's syndrome. 27 cases (author's transl)].

Twenty patients with Felty's syndrome were investigated. Isotopic studies of polymorphonuclear neutrophils, bone marrow biopsies and autoradiographies, and cultures of granulous stem cells showed that neutropenia resulted from three mechanisms acting simultaneously: hypermargination of the neutrophils predominantly in the spleen, decreased production of granulocytes in the bone marrow, and peripheral hyperdestruction of the neutrophils. Anti-granulocyte antibodies were detected in 3/12 patients. Other factors present in the serum of 2/4 patients seem capable of inhibiting the growth of granulocytic stem cells. Secondary bacterial infection (77%) may explain the severity of the prognosis: 13 out of 27 patients died 4 years on average after neutropenia was diagnosed.

Agranulocytosis↗
PubMed1982

Mitogen-induced maturation of chronic lymphocytic leukemia B lymphocytes.

Peripheral blood lymphocytes from eight patients with untreated B-derived chronic lymphocytic leukemia (CLL) (only one had a serum monoclonal immunoglobulin) were stimulated by Nocardia, phytohemagglutinin, and pokeweed mitogen. This stimulation resulted in the occurrence in all but one case of large cytoplasmic immunoglobulin-containing cells with a predominantly lymphoblastic (six cases) or plasmacytic (one case) appearance. These blast cells contained the same immunoglobulin chains as those of the surface immunoglobulins on fresh lymphocytes except for delta chains that were not found by cytoplasmic immunofluorescence. In three cases, intracytoplasmic immunoglobulin inclusions that were present in both small lymphocytes and stimulated blast cells provided further evidence of the differentiation of the leukemic clone. In another case, both small leukemic B lymphocytes and large B blast cells expressed in vitro a receptor for sheep erythrocytes. A switch from IgM to IgG synthesis was observed in this patients, whose cells showed the strongest response to the activators. The most effective activator was Nocardia, then phytohemagglutinin, whereas an effect of pokeweed mitogen was observed only when the fresh lymphocytes contained a fair percentage of T cells. Leukemic cells, in most patients, were able to differentiate without appreciable B-cell proliferation.

B-Lymphocytes↗
PubMed1981

Malignant lymphoma supervening in chronic lymphocytic leukemia and related disorders. Richter's syndrome: a study of 25 cases.

Richter's syndrome (RS) has been defined as "histiocytic" lymphoma (HL) or Hodgkin's disease (HD) supervening in the course of chronic lymphocytic leukemia (CLL) and related disorders. The clinical, histologic, and immunologic findings in 25 cases (11 women, 14 men) of RS are presented. The initial diagnosis was CLL in 19 cases, diffuse well-differentiated lymphocytic lymphoma in 2 cases, and Waldenstrom's macroglobulinemia in 4 cases. The interval between the initial diagnosis and that of RS ranged from 0 (two cases) to 120 months (median 49 months). At the time of diagnosis of RS, the initial lymphoproliferative disorder was in apparent complete remission in only two cases. The lymphoma was disseminated in at least 18 cases. The overall median survival was four months, but complete remission was achieved in six cases and has been maintained for 15 to 77 months. In four of these six cases, the RS was localized. The histologic diagnosis of HD was made in only two cases. In the other 23 cases, the diagnosis was HL, but in five of these cases, the proliferation was heterogeneous and was considered as an early aspect of HL. Immunologic studies of lymph node cell suspensions were performed in seven cases. In all cases, the B-lymphocytic origin of the lymphoma cells could be ascertained. Detailed studies in four cases showed that lymphoma cells carried SIg of the same isotype and light chain type as that of SIg detected on CLL cells or of monoclonal serum Ig. In these cases, the lymphoma was actually related to the initial B-cell chronic lymphoid disease.

Adult↗
PubMed1981

Diversity of immunoglobulin expression in leukaemic cells resembling B-lymphocyte precursors.

Approximately 20% of patients with acute lymphocytic leukaemia (ALL) have leukaemic blasts with features of pre-B cells which are the recently characterized precursors of B lymphocytes in normal development (for a review, see ref. 2). Pre-B cells isolated from normal bone marrow or fetal liver, and malignant cells from patients with pre-B cell leukaemia, are rapidly dividing lymphoid cells that contain cytoplasmic immunoglobulin mu heavy chains, but have no detectable surface immunoglobulin. The resemblance of immunoglobulin-containing ALL cells to normal precursors of B lymphocytes and their availability in relatively pure preparations allowed us to explore them as models of early stages in the differentiation of the B-lymphocyte line. We report here observations on the occurrence of intermediate pre-B/B-cell phenotypes, immunoglobulin isotype switching and the asynchrony of immunoglobulin heavy and light chain expression in 30 cases of ALL and 3 cases of chronic myelogenous leukaemia in lymphoblastic crisis (CML-BC).

B-Lymphocytes↗
PubMed1981

Peripheral neuropathy and plasma cell neoplasias: a report of 10 cases.

Ten patients with peripheral polyneuropathy associated with plasma cell neoplasias are reported. Progressive sensorimotor polyneuropathy was the presenting complaint in all patients. CSF protein concentration was elevated in most patients. The electrophysiological and pathological changes were consistent with a primary segmentary demyelinating disease. All patients were male and younger than the average patient with myeloma. None presented with high tumour mass or overt multiple myeloma. Six were affected with single or multiple plasmacytomas with osteolytic lesions. Unusual haematological features such as polycythaemia, thrombocytosis and lymphadenopathy were often combined with the polyneuropathy. Skin hyperpigmentation, gynaecomastia and diabetes mellitus were noted in some patients. Complete recovery of the polyneuropathy was observed in some patients after either cyclophosphamide and corticosteroid therapy or radiotherapy of localized plasmacytoma, suggesting a direct relationship between the plasmacytic proliferation and the neuropathy. The nature of the postulated factor produced by the plasma cells and responsible for nerve injury is discussed and the importance of a careful search for plasma cell proliferation in men with obscure polyneuropathies is outlined.

Adult↗
PubMed1981

Immunological phenotypes of human leukemias of the B-cell lineage.

The recent results are summarized of some immunological studies of various human leukemias of the B-cell lineage. The study of pre-B leukemias has outlined the diversity of immunoglobulin expression in leukemic cells resembling B-lymphocyte precursors and has provided insights into the biology of the progenitors of hematopoietic cells. Acute leukemia with Burkitt cells represents a very homogeneous group close to classical Burkitt's lymphoma. Mitogens are able to induce in vitro differentiation of leukemic cells from chronic lymphocytic-B leukemia and from hairy cell leukemia. Hairy cells become able to express new phenotypic characteristics after stimulation by phytohemagglutinin.

Antibodies, Neoplasm↗
PubMed1980

Synthesis of abnormal immunoglobulins in lymphoplasmacytic disorders with visceral light chain deposition.

Three patients presented with renal or more diffuse tissue deposits of a nonamyloid material reactive with anti-kappa antibody by immunofluorescence. All patients had progressive renal failure with the nephrotic syndrome and extensive tubular basement membrane deposits. Glomerular lesions were conspicuous but heterogeneous. One patient also had hepatic deposits with peliosis at histopathologic examination. An underlying lymphoplasmacytic disorder was found in all patients: multiple myeloma in one, pleomorphic lymphoplasmacytic malignancy analogous to Waldenström's macroglobulinemia in one and bone marrow monoclonal plasmacytosis without overt myeloma in one. Biosynthesis experiments in two cases showed production of abnormal kappa chains which were not detected in appreciable amounts in serum and urine. These light chains had an aberrant size (abnormally short or large), their apparent molecular weight was larger in secretion than in cytoplasmic extracts (suggesting their glycosylation) and they were secreted as polymers. These results suggest a causal relationship between production of abnormal light chains and tissue deposition.

Adult↗
PubMed1980

Synthesis of abnormal heavy and light chains in multiple myeloma with visceral deposition of monoclonal immunoglobulin.

In a patient treated for IgA kappa myeloma, bone marrow relapse and a sharp drop in the serum IgA level paralleled tissue deposition of non-amyloid material reactive with anti-kappa anti-alpha sera in immunofluorescence studies of kidney and liver biopsies. Clinical manifestations were progressive renal failure with nephrotic syndrome, with both tubular and glomerular lesions (including nodular glomerulosclerosis), hepatomegaly, cardiac and neurological symptoms. Biosynthesis experiments showed the production of alpha chains diminished in length by about one domain which were rapidly degraded predominantly after secretion and of two species of light chains; normal-sized light chains which assembled with alpha chains and abnormally short ones which were secreted as free light chains. The apparent molecular weight of the light chains was larger in secretions than in cytoplasmic extracts, suggesting their glycosylation. These results suggest a causal relationship between tissue deposition and production of abnormal immunoglobulins by a variant clone, the emergence of which was possibly induced by Melphalan therapy.

Bone Marrow↗