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Biomedical subjects

B Safai

Publications and source records attributed to B Safai.

At least 19 recordsLinked to original sources

Selective IgA deficiency and circulating immune complexes containing bovine proteins in a child with chronic graft versus host disease.

We have previously shown that a selective absence of serum and secretory immunoglobulin A (IgA) may lead to the development of circulating immune complexes which appear to contain bovine milk antigens. We report here that high levels of circulating immune complexes were found in the serum of a child who was treated for severe combined immunodeficiency by bone marrow transplantation but in whom the IgA-producing cells subsequently failed. As increasing amounts of complexes appeared over a two year period, the child had a parallel progression of an apparent chronic graft versus host disease including a Sjögrens syndrome and scleroderma. Very large amounts of complexes were eventually formed but the level fell 77 per cent after milk was excluded from the diet. Chemical studies on the complexes showed that the majority of complexes did contain bovine milk proteins, and fluorescence antibody staining of skin biopsy samples showed the presence of dense deposits of bovine casein in the dermis. The relationship between bovine protein-antigen antibody complexes and the chronic graft reaction remains uncertain.

Antibodies

Immunity in wart resolution.

The involvement of the humoral and cell-mediated immune systems in the regression of wart infection has been investigated extensively in recent years. This review examines the supporting evidence for the roles of humoral and cellular immunity in wart regression and its possible implications. From the available data it does not appear that a conclusion can be drawn that only humoral or cell-mediated immunity is involved, or that both are essential, in the regression of wart infection. Studies by several investigators, however, suggest that, since agents known to stimulate the cell-mediated immune system have been reported to be followed by the successful resolution of warts, the cell-mediated immune system appears to play a critical role in wart resolution. It may be that the direction which should be taken in eradication of warts resistant to conventional modalities of treatment is one which relies upon the stimulation of the patient's immune system in a very specific manner. Probably the most efficient way to accomplish this, based on available data, would be by autogenous vaccination. Following the patient's humoral and cell-mediated immune response prior to, during, and following treatment with autogenous vaccination may help to elucidate the precise mechanism by which the successful resolution of warts occurs.

Antibody Formation

Once weekly total-skin electron-beam therapy for mycosis fungoides: 7 years' experience.

A total of 115 patients with mycosis fungoides were given total-skin electron-beam therapy (TSEB), utilizing 3.5-mev electrons at doses of 400 rads to the entire skin surface once a week for 6--8 consecutive weeks. Prompt relief of symptoms and regression of lesions were observed in all patients. Of the 81 patients at risk for 12--91 months (median, 24 months) following TSEB, initial unmaintained remission lasted 6--69 months (median, 19 months). No untoward immediate or late effects have been noted in the bone marrow or normal skin which was irradiated. The duration of remission following TSEB correlated well with lymphocyte responsiveness to various mitogens and antigens, but not with the initial response. Thymic hormone factor levels (Facteur Thymic Serique) were elevated in the majority of these patients with mycosis fungoides.

Adult

Pyoderma gangrenosum and myeloproliferative disorders. Report of a case and review of the literature.

The exact mechanism involved in the pathogenesis of pyoderma gangrenosum (PG) still remains unclear, yet there is an increasing number of reports associating PG with immunologic abnormalities. A correlation between PG and myeloproliferative disorders has also been described. We describe a patient with chronic myelocytic leukemia in whom PG developed during the course of illness. We present an immunologic analysis of this case, speculation on the pathogenesis of PG, and a review of the literature. We report the futility of current therapeutic modalities in the treatment of PG.

Adult

A novel lymphocyte differentiating factor in serum of patients with mycosis fungoides and Sezary syndrome.

Sera from 13 patients with mycosis fungoides and 2 with Sezary syndrome were tested for activity that induces lymphocyte differentiation. Induction of Thy-1.2 antigen and surface immunoglobulin were used, respectively, to measure T- and B-cell differentiation. The indicator cells were null lymphocytes from the spleens of congenitally athymic nude mice. Normal serum induced some T-cell but no B-cell differentiation. The T-cell-inducing activity was ascribed to thymic hormone and declined with advancing age. A totally different pattern emerged with patient serum. T-cell-inducing activity was significantly more active than in normal serum (p less than 0.001). This activity did not decline with advancing age and was not inhibited by a concentration of ubiquitin, which blocks nonspecific beta-adrenergic induction. B-cell-inducing activity was also present. This novel serum factor (or factors) is a potent inducer of T- and B-lymphocyte differentiation and is associated with neoplastic lymphoproliferation of the T-cell series.

Adult

Antibody patterns to herpesviruses in Kaposi's sarcoma. II. Serological association of American Kaposi's sarcoma with cytomegalovirus.

The prominent finding of this extended serologic analysis on American and African Kaposi's sarcoma (KS) patients and appropriately matched control groups is the detection of a specific serologic association of cytomegalovirus (CMV) with American KS patients. All American KS sera contained CMV antibodies and their geometric mean titers (GMT) were significantly higher than those in sera of melanoma patients (GMT ratio k = 5.3 to 7.7 by complement fixation [CF], k = 8.9 by indirect hemagglutination [IHA]) or in sera of age- and sex-matched healthy controls (k = 12.6 to 16.0 by CF, k = 12.6 by IHA). The result is strongly reminiscent of the data obtained previously for European KS. Although the GMT to CMV of African KS patients were similar to the GMT of the American KS groups, their significance cannot be demonstrated due to the high background of CMV infections in the control groups. Complex mechanisms are hypothesized, by analogy with the Epstein-Barr virus (EBV) involvement in Burkitt's lymphoma (BL), for a CMV involvement in the development of KS.

Aged

Rosette-formation with mouse erythrocytes: VI. T, B, and third population lymphoid cells in mycosis fungoides and effect of leukopheresis.

Peripheral blood from 16 patients with mycosis fungoides and two patients with Sézary syndrome was examined for T, B, and third-population (K) cells, using a battery of surface markers. T lymphocytes as determined by spontaneous rosette formation with sheep erythrocytes and third-population cells as determined by the Ripley rosette test were present in normal proportions. Surprisingly, B lymphocytes, as determined by surface immunoglobulin and receptors for mouse erythrocytes, were either lacking or were present in low proportions in some patients. Normal proportions were present in others. Repeat studies of two of three patients lacking B lymphocytes, following treatment, revealed normal or low proportions of B cells. Two patients with mycosis fungoides had increased proportions of "null" cells. Study of lymphoid cell subpopulations before and after leukopheresis in a single patient demonstrated a decrease in T cell proportions associated with a concomitant increase in the proportions of "null" cells following this therapy.

Alopecia

Cutaneous manifestation of internal malignancies (I). Acanthosis nigricans.

Skin changes may be the first clue to a neoplastic process at a stage when it still is treatable (Such as the development of Acanthosis Nigrican (AN) in an otherwise healthy adult). Ninety percent of the neoplasm responsible for the development of AN originate in the abdomen. The tumor, even in a subclinical state, seems to possess unidentified properties that activate the dermatosis.

Acanthosis Nigricans

Electron-beam therapy for mycosis fungoides.

The lesions of mycosis fungoides are radiosensitive and because extensive areas of the body are usually involved, the treatment of choice is with the electron beam. Since 1971, a fractionation scheme of weekly doses for irradiation of the entire skin surface has been practiced with a 6 MeV linear accelerator that generates 3.5 MeV electrons. One hundred and five patients were treated in this way by delivering 400 rads once weekly for six consecutive weeks. All patitents enjoyed complete disappearance of the skin lesions at once. Of the 62 patients whom we were able to follow-up after electron-beam therapy, for periods ranging from one to nearly seven years (median, two years), the duration of remission was one-half to nearly five and a half years (median, one and one-half years). The treatment was well tolerated and no unusual skin reactions or hematopoietic depression occurred.

Adult

Association of blue rubber bleb nevus syndrome with chronic lymphocytic leukemia and hypernephroma.

A male patient with clinical and pathologic features of blue rubber bleb nevus syndrome is described. Multiple hemangiomata were disseminated throughout the skin and gastrointestinal tract, and typical findings of B cell chronic lymphocytic leukemia and renal cell carcinoma were evident. This case demonstrates a unique instance of associated neoplasia in blue rubber bleb nevus syndrome.

Adenocarcinoma

Human immunodeficiency disease: impairment of cellular interactions leading to abnormal mediator production in mixed lymphocyte culture reaction.

Leukocyte migration inhibitory factor (LMIF) production in mixed lymphocyte culture (MLC) reactions is the result of cellular interactions based on two separate phenomena: the capacity of lymphocytes to stimulate in MLC, and the capacity of lymphocytes to respond in MLC. Puromycin-treated lymphocytes are capable of stimulating allogeneic cells for LMIF production, but are unable to respond with synthesis of LMIF (one-way MLC-LMIF test). We have studied the stimulating and responding capacity of lymphocytes from patients with different immunodeficiency syndromes in a one-way MLC-LMIF assay. Lymphocytes from patients known to have qualitative and quantitative defects of T cell or B cell functions (Hodgkin's disease, mycosis fungoides, thymoma, chronic lymphatic leukemia) were found to respond poorly as measured by mediator production although their stimulating fuction was frequently retained. Patients with advanced solid tumors often had both MLC-stimulating and responding functions depressed. There was no apparent correlation between mitogen response and MLC-induced LMIF responses or between MLC proliferative response (as measured by thymidine incorporation) and mediator production. Studying of stimulatory and responding capacity of lymphocytes in the MLC-LMIF assay provides a new tool for assessing immunocompetence and allows for in vitro evaluation of cellular interactions that may play an important role in vivo.

Candidiasis, Cutaneous