Byung H. Park

B and T Lymphocytes in Primary Immunodeficiency Disease in Man

B- and T-cell populations in 32 patients with different forms of primary immunodeficiency disease were studied. The B-cells in peripheral blood were investigated with respect to surface immunoglobulins by means of immunofluorescence. The T-cell function was studied utilizing quantitation of proliferative response to phytochemagglutinin (PHA)(1) and delayed allergy to various antigens. In 10 patients lymph node lymphocytes were also evaluated 11 male children with infantile x-linked agammaglobulinemia were divided into two subgroups. One did not show immunoglobulin spots on peripheral blood lymphocytes at all, the other contained a very low percentage of IgM- and occasionally IgA bearing lymphocytes. Eight patients with common variable immunodeficiency had moderately decreased percentages of peripheral blood and lymph node lymphocytes with surface immunoglobulins, but these patients lacked immunoglobulin secreting cells. Four cases of isolated IgA deficiency had normal or high percentages, and two cases of ataxia-telangiectasia had high percentages of lymphocytes with IgA in so called receptor distribution in both peripheral blood and lymph nodes. In three patients with infantile combined immunodeficiency that had been corrected by marrow transplantation, the percentages of Ig-bearing lymphocytes increased to normal or high levels together with establishment of functional T-cell population and ultimate secretion of serum immunoglobulins. One case of Di George syndrome reconstituted by fetal thymus transplant showed gradual decrease of B lymphocytes in circulation parallel to restoration of T-cell population.

Metabolic activities in leukocytes of newborn infants

Leukocytes of the newborn infant manifest an activated metabolic state similar in some ways to that associated with phagocytosis. Oxygen consumption, hexose monophosphate pathway activity, and nitroblue tetrazolium (NBT) reduction by newborn leukocytes were significantly increased in the resting state. These cells of the newly born infant showed normal metabolic response to phagocytosis in vitro and normal staphylocidal capacity in the presence of pooled normal human serum, indicating a functional maturity of these leukocytes as phagocytes.

Nonparalytic poliovirus infections in patients with severe combined immunodeficiency disease

Four patients with severe combined immunodeficiency disease developed nonparalytic poliovirus infection after immunization with oral polio vaccine. Allogeneic bone marrow transplantation or administration of unirradiated peripheral lymphocytes induced evidence of graft-versus-host disease in all four of these patients and evidence of immunologic reconstitution in two. These patients stopped excreting poliovirus in their stools shortly after the first evidence of acceptance of the immunocompetent cells. The cells responsible for the immunologic reconstitution of these patients also appear to be responsible for eradicating the poliovirus infection.

Immunologic reconstitution of a patient with combined immunodeficiency disease

Transplantation of bone marrow cells from a matched sibling donor to a 4-month-old Negro boy with a severe combined immunodeficiency syndrome resulted in immunologic reconstitution. Severe GVH disease was avoided by grafting a small number of carefully aspirated marrow cells from an HL-A-identical female sibling. A mild GVH reaction appeared 12 days after transplantation but subsequently subsided. The patient was discharged 53 days after transplantation; at this time the quantitative serum immunoglobulins and the in vitro responses to PHA stimulation showed clear evidence of immunologic reconstitution. The patient remains well and has granulocytes with drumsticks in the peripheral blood, clearly indicating the presence of donor-derived cells.

Chronic Granulomatous Disease of Childhood : Surgical Considerations

Abstract Experience with 25 cases of chronic granulomatous disease of childhood seen at the University of Minnesota Hospitals is reviewed. A unique combination of granulomatous, suppurative disease is commonly encountered in lymph nodes, lung, liver, bones, skin, and other parenchymatous organs. Early definitive excisional drainage or extirpation of the involved organ has proved successful in the control of infection in these patients and the concept of en bloc resection of all infected tissue is presented. The unique combination of granuloma formation and suppuration that coexists in areas of infection in these children seems to justify this approach to therapy that is unresponsive to conventional antibiotics.

Metabolic studies of leukocytes of patients with osteogenesis imperfecta.

In contrast to the results of previously published reports, no significant abnormalities of quantitative tetrazolium dye reduction, oxygen consumption, or the hexose monophosphate shunt were found in the leukocytes of patients with osteogenesis imperfecta.

Variability in immunologic reconstitution following bone marrow transplantation

Abstract Following the first successful bone marrow transplant which corrected both a genetically based immunodeficiency disease and a second marrow transplant that corrected an immunologically based marrow aplasia, 25 additional successful marrow transplants have been reported. In this report three patients are described to illustrate both the variability in the clinical and laboratory manifestations of combined immunodeficiency disease and the variability in the degree and pattern of immunologic reconstitution following marrow transplantation. These three patients emphasize that many factors that may influence the successful outcome of marrow grafting are not well understood and each patient must be considered as a unique undertaking.

Immunodeficiency Diseases of Man**Original work has been aided by grants from The National Foundation–March of Dimes, U. S. Public Health Service (HE-06314, AI-08677, AI-00798, and NS-02042), and the American Heart Association.

Publisher Summary This chapter discusses immunodeficiency diseases of man. Hodgkins disease, a malignancy affecting reticulum cells, is a disease characterized by progressive loss of T-cell function, whereas B-cell function is well maintained in most instances. In contrast, myeloma, which on morphological grounds is clearly a disease that involves B2 or secretory B cells shows primarily deficits of the normal B-cell function. Chronic lymphatic leukemia has been shown to be a disease in which both T- and B-cell function may be disturbed early in the disease. However, in acute leukemias both T- and B-cell functions seem well maintained until extensive cytotoxic therapy produces profound deficiencies of either or both functional compartments. The chapter also lists several well-defined primary immunodeficiency diseases of man classified according to the cell population and functions which are deficient in each disease. The chapter highlights that perhaps the best single test for the adequacy of the T-cell population is a quantitative evaluation of the cells in peripheral blood or lymphoid tissues which will respond to stimulation with phytohemagglutinin.