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Biomedical subjects

J M Lang

Publications and source records attributed to J M Lang.

At least 163 records · Page 9Linked to original sources
PubMed1976

Autophagia in myeloid precursors: an explanation for neutropenia in Chediak-Higashi syndrome?

Neutropenia is an almost constant feature of Chediak-Higashi syndrome (CHS). There is evidence for a central mechanism of neutropenia. Ultrastructural studies of the bone marrow from a child with CHS showed marked autophagic phenomena within myeloid precursor cells and mature neutrophils. Autophagic vacuoles were randomly distributed in the cytoplasm of the cells from the granulocytic series and some of them contained giant granules which thus appeared particularly resistant to the autophagic process. The vital cellular damage through endophagocytosis suggests the possibility of intramedullary destruction as an explanation for neutropenia.

Agranulocytosis↗
PubMed1975

[Acquired C1-esterase inhibitor deficiencies during lymphoid syndromes].

Very marked abnormalities of the complement system were discovered in two patients suffering from a lymphoid syndrome and an IgM 7S dysglobulinaemia. The abnormalities in the complement system were related to a deficiency in C1-estérase (C1 INH). Several findings suggest that such a deficiency is acquired, in particular the absence of any family history of angio-neurotic oedema and, above all, the detection of a marked fall in levels of the C1 fraction which does not exist in the congenital form of deficiency of the inhibitor. The IgM 7S immunoglobulins found in the serum of both patients are probably responsible for the abnormalities in the complement system observed. Such acquired deficiencies in C1 INH are extremely rare since only a few cases have been reported in the literaute, in particular two cases in patients with lymphosarcoma with a serum IgM 7S.

Aged↗
PubMed1975

Lymphosarcoma, cold urticaria, IgG1 monoclonal cryoglobulin and complement abnormalities.

A patient with lymphosarcoma and cold urticaria showed evidence of complement activation by the classical pathway with low levels of the early complement components, normal levels of late acting components, normal functioning of the alternate pathway and reduction of the C1-inhibitor level. The serum contained an IgG1 monoclonal cryoglobulin responsible for the complement activation. In vitro tests demonstrated a high capacity of the serum to activate C1.

Cold Temperature↗