Lymphoproliferation following antigenic stimulation in severe combined immunodeficiency disease

Akhter, Javeed; Krantman, H J; Hopper, John E.; Rothberg, Richard M.

doi:10.1016/0090-1229(81)90147-1

Cited by 6 publications

(2 citation statements)

References 24 publications

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“…The lack of this regulation may lead to persistence of the antigen and to a polyclonal, uncontrolled response. The situation seems analogous with that reported in an infant girl with marked inpairment of both cellular and humoral immune functions [30]. She responded to diphtheria and tetanus toxoid immunizations with progressive lymphocytosis, generalized lymphadenopathy and hepatosplenomegaly , but did not produce antibodies to diphtheria and tetanus toxoid.…”

Section: Discussionsupporting

confidence: 78%

Immune capacity of the chicken bursectomized at 60 hours of incubation: transplantation of bone marrow cells of the bursectomized chickens into cyclophosphamide‐treated newly hatched recipients

Jalkanen

1983

Eur J Immunol

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Chickens bursectomized at 60 h of incubation are known to be able to produce immunoglobulins (Ig) but not specific antibodies. In the present work bone marrow (BM) cells of 2- and 10-week-old embryonically bursectomized (Bx) chickens were transferred to newly hatched cyclophosphamide-treated chickens for the purpose of studying whether the transplanted cells home into the bursa and gain a capacity to produce specific antibodies. BM cells of normal 2- and 10-week-old chickens were transferred as controls. Cells of 2-week-old control (Co) and of 2- and 10-week-old Bx chickens could to some extent reconstitute serum Ig of the recipients, but were totally incapable of homing into the bursa and of restoring the specific antibody production. Only BM cells of 10-week-old Co chickens could restore the production of specific antibodies without homing into the bursa, indicating their postbursal nature. These findings indicate (a) that 2- and 10-week Bx BM cells have irreversibly bypassed the bursal phase of education, (b) that also the normal BM at the age of 2 weeks contains cells that are capable of Ig but not of specific antibody synthesis and (c) that bursal microenvironment is not necessary for isotype switch, but is essential for creation and expansion of the antibody repertoire.

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Section: Discussionsupporting

confidence: 78%

Immune capacity of the chicken bursectomized at 60 hours of incubation: transplantation of bone marrow cells of the bursectomized chickens into cyclophosphamide‐treated newly hatched recipients

Jalkanen

1983

Eur J Immunol

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“…The epidermis contained foci of hyperkeratosis and exocytosis of the lymphocytes corresponding to a phenotype of suppressor T cells (27). Some patients demonstrated histologic data compatible with GVHD (27,31), but this finding was not constant (19,28). As in GVHD (32,33), there was a decrease in the number of Langerhans cells T6 (+) and an expression of HLA-DR antigens by keratinocytes (27).…”

Section: Discussionmentioning

confidence: 99%

Cutaneous Lesions in Severe Combined Immunodeficiency: Two Case Reports and a Review of the Literature

Llorente

Amoros

Frutos

et al. 1991

Pediatric Dermatology

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Two patients with severe combined immunodeficiency (SCID) in whom cutaneous lesions were the first clinical feature were studied. Neither the morphology nor the histology of the lesions was uniform, although we have noted some common findings that can, in subsequent cases, lead us to suspect SCID. The immunologic defects were not uniform, representing the two poles of the spectrum of SCID. We believe that early recognition of the skin lesions is very important, since the patient's life expectancy can be increased by a bone marrow transplantation (1).

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The Omenn's syndrome: Histological, immunohistochemical and ultrastructural evidence for a partial T cell deficiency evolving in an abnormal proliferation of T lymphocytes and S-100+/T-6+ Langerhans-like cells

Ruco

Stoppacciaro

Pezzella

et al. 1985

Vichows Archiv A Pathol Anat

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A 7 month old female infant was affected by a rapidly fatal familial disease highly reminiscent of Omenn's syndrome. She presented with widespread eczematous lesions, hepatosplenomegaly, superficial lymphadenopathy, peripheral blood lymphocytosis, eosinophilia and hyper-IgE. An axillary lymph node was involved by a marked proliferation of T-3 +/T-10-- lymphocytes admixed with S-100+/T-6+/Leu-3a+/Ia + reticular cells which lacked typical LC granules; cell suspension study revealed that 90%-96% of the lymph node cells were T-11+/T-3+ lymphocytes characterized by low expression of Leu-3a and T-8 antigens and by high expression of Ia antigens (52%). Peripheral blood T lymphocytes exhibited a similar distribution of surface phenotypes. The patient died of interstitial pneumonia and an autopsy was performed. The thymus was markedly atrophic and completely devoid of lymphocytes. The peri-arteriolar lymphoid sheets of the spleen were poorly developed and were mainly composed of T-8+ lymphocytes. The mediastinal nodes were rudimentary and were populated by T-3+/T-10+ lymphocytes with low expression of Leu-3a and T-8 antigens. Our results raise the possibility that Omenn's syndrome is a peculiar primary immunodeficiency in which, despite early thymic involution, some abnormal T lymphocytes still develop in the peripheral lymphoid organs. Antigenic triggering of these cells might result in prominent proliferations of T lymphocytes and Langerhans-like cells which lead to the clinical manifestation of the disease.

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Lymphoproliferation following antigenic stimulation in severe combined immunodeficiency disease

Cited by 6 publications

References 24 publications

Immune capacity of the chicken bursectomized at 60 hours of incubation: transplantation of bone marrow cells of the bursectomized chickens into cyclophosphamide‐treated newly hatched recipients

Immune capacity of the chicken bursectomized at 60 hours of incubation: transplantation of bone marrow cells of the bursectomized chickens into cyclophosphamide‐treated newly hatched recipients

Cutaneous Lesions in Severe Combined Immunodeficiency: Two Case Reports and a Review of the Literature

The Omenn's syndrome: Histological, immunohistochemical and ultrastructural evidence for a partial T cell deficiency evolving in an abnormal proliferation of T lymphocytes and S-100+/T-6+ Langerhans-like cells

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