[Morphology of Chediak-Higashi syndrome]. / Morfologiia sindroma Chediaka-Khigasi.

The authors observed two boys born to cousin sib parents. The boys presented with a variant of primary immunodeficiency--Chediak-Higashi syndrome. The diagnosis was based mainly on the presence of giant azurophil granulations in proband phagocytes. The disease studied morphologically ran a specific course with development of reactive generalized histiocytosis and erythrophagia. The latter are thought to arise in response to defective phagocytosis. The patients suffered recurrent infections from which they died.

[Structure of the thymus, immune status and the pathological process]. / Struktura timusa, immunnyi status i patologicheskii protsess.

Morphological changes in the immune system organs are described which allow the pathologist to diagnose primary syndromes of immunodeficiency. The criterion for the evaluation of the infant immune status is a thymic structure at various stages of ontogenesis as compared to the structure of a normal thymus after an antigenic stimulation.

[Various hereditary immune deficiencies linked to the X-chromosome]. / Nekotorye nasledstvennye immunodefitsity, stseplennye s X-khromosomoi.

The organs of immune system in hereditary immunodeficiencies linked to X-chromosome, particularly in Nezelof's syndrome and chronic granulomatosis (4 cases) were studied histologically. The alterations of thymus found in Nezelof's syndrome suggest the disturbance of its morphogenesis at the embryonal stage, those found in chronic granulomatosis suggest the postnatal disturbance of thymus morphogenesis. The children die of polytopic infections foci. Generalized BCG-itis is observed in children with chronic granulomatosis.

[Collector system of the thymus]. / Kollektornaia sistema timusa.

A method of silver impregnation of the microcirculatory bed was used to disclose in thick sections (30-80 micrometers) of the guinea-pig thymus cortical zone a vascular network consisting of arteriolo-capillaro-venular loops resembling those seen in the derma papillary layer. The arteriolar and venular loops belong to the cortical zone, whereas the capillary loops lie in the corticomedullary zone. Since the cortical layer demonstrates a local distinctly marked histohematic barrier, poorly accessible for lymphocytes with the capillary loop wall being readily permeable, the lymphoid cells, before egress from the organ, travel across the entire cortical layer where they undergo influences of numerous differentiation factors over a certain period of time. It is assumed that the microcirculatory network disclosed is the basis for the thymus collector system responsible for egress of mature T cells from the organ.