Fetoscopy and fetal blood sampling were performed in an attempt at prenatal monitoring of a pregnancy at risk for infantile hereditary agranulocytosis (Kostmann's disease). In smears of fetal blood three segmented neutrophils were found out of 200 nucleated cells (1 1/2 per cent). Their presence, although in a lower percentage than in six age-matched controls, was considered to indicate that the fetus was not affected. The newborn infant has developed normally and at the age of four months has a normal number of segmented neutrophils in his peripheral blood. Feasibility of prenatal diagnosis of infantile hereditary agranulocytosis is discussed.

Adult↗

PubMedDate not supplied

Conditions affecting the inhibitory action of RLP on radiation leukaemogenesis in mice.

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Animals↗

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Chediak-Higashi syndrome: ocular findings.

A case of Chediak-Higashi syndrome diagnosed by its characteristic ocular findings is described. The appearance of the optic disc and the contrast between total (or nearly total) lack of pigment in the pigment epithelium and the relatively unaffected choroidal pigmentation are believed to be observations of paramount importance in the differential diagnosis by ophthalmoscopic examination. Electrophysiological tests of our case showed abnormal responses and a further deterioration of the ERG and VEP on repeated examinations. Electron microscopic study of a conjunctival biopsy revealed the presence of pathognomonic giant intracytoplasmic lysosomal granules in stromal fibroblasts and, thus, can be used as an adjunct test in suspected cases of Chediak-Higashi syndrome.

Chediak-Higashi Syndrome↗

PubMedDate not supplied

New approaches for the prevention of rejection and graft-vs.-host disease in clinical bone marrow transplantation.

The two major barriers to successful allogeneic bone marrow transplantation (BMT) in animals and man are graft-vs.-host disease (GVHD) and the risk of graft rejection. GVHD is the result of alloreactivity of mature donor T-lymphocytes present in the graft-vs.-host tissues and can be completely prevented by pregraft depletion of T-lymphocytes. Graft rejection results from residual host immunocompetent lymphocytes that survive heavy chemoradiotherapy prior to allogeneic BMT. Host resistance to allograft cannot be eradicated even by conventional conditioning with high-dose cyclophosphamide (120 mg/kg) and lethal whole body irradiation (1,200 rad). In the present report we have utilized two new techniques to overcome GVHD and graft rejection following allogeneic BMT. GVHD can be prevented by a new monoclonal rat antihuman lymphocyte antibody, CAMPATH-1, which binds human complement, enabling donor serum to serve as the source of complement. Prevention of rejection of T-lymphocyte-depleted marrow allografts can be achieved by the application of total lymphoid irradiation (TLI) in addition to conventional chemoradiotherapy, prior to allogeneic BMT. TLI causes potent immunosuppression with minimal side effects. A combination of TLI for overcoming host resistance to allograft, and CAMPATH-1 for overcoming GVHD, leads to a relatively smooth posttransplant outcome with no evidence of GVHD and with no need for posttransplant immunosuppression.

Antilymphocyte Serum↗

PubMedDate not supplied

Survival of irradiated erythrocytes after transfusion to isologous mice.

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Animals↗

PubMedDate not supplied

XX/XY lymphoid chimerism in a boy with fatal lymphohistiocytic proliferation.

XX/XY chimerism, present in peripheral blood and bone marrow cells, but absent in other somatic tissues, was demonstrated in a 6-year-old patient. The initial clinical presentation with "butterfly" rash, pancytopenia with a hemolytic component, and hypergammaglobulinemia was suggestive of an immune disorder. Infiltration of skin, bone marrow, and lymph nodes with histiocytes, without disruption of the general configuration, was similar to the findings in familial lymphohistiocytosis. The results of specific cellular markers and of cytogenetic and immunologic studies are analyzed and the nature of the disease and origin of the chimeric state are discussed. The most likely explanations include a chronic graft-vs-host reaction induced by proliferation of foreign lymphoid cells derived from a blood transfusion that the child had received during infancy, or proliferation of neoplastic cells present in the transfused blood.

Child↗