Thymic alymphoplasia, monoclonal gammopathy, and Pneumocystis carinii pneumonia in an infant.

Becroft, D. M. O.; Douglas, R. Gordon

doi:10.1136/adc.43.230.444

Cited by 7 publications

(2 citation statements)

References 22 publications

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“…However, most of the case reports documenting monoclonal Ig in childhood also concern patients with immunodeficiency diseases: Wiskott-Aldrich syndrome (Radl et al, 1967;Blaese et al, 1971), ataxia telangiectasia (Cawley and Schenken, 1970), and severe combined immunodeficiency (Stoop, Ballieux, and Weyers, 1962;Schaller et al, 1966;Becroft and Douglas, 1968). In these cases and in those reported here, the patients had received neither y-globulin nor thymus or bone marrow grafts.…”

Section: Discussionmentioning

confidence: 69%

Serum monoclonal immunoglobulins in childhood

Danon¹,

Séligmann²

1973

Archives of Disease in Childhood

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Section: Discussionmentioning

confidence: 69%

Serum monoclonal immunoglobulins in childhood

Danon¹,

Séligmann²

1973

Archives of Disease in Childhood

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“…Associated immune defects have been offered as an explanation for extreme histiocytic reactions produced by identifiable infections such as rubella (Claman, Suvatte, Githens, and Hathaway, 1970) and atypical mycobacteria (Clinico-pathological Conference, 1970). There may be striking histiocytic proliferation in some children who have severe congenital defects in T-lymphocyte-mediated cellular immunity in association with thymic dysplasia (Becroft and Douglas, 1968). Widespread proliferation of pigmented lipid-containing histiocytes occurs in chronic granulomatous disease in which a metabolic defect in phagocytes leads to defective intracellular killing of certain bacteria (Carson, Chadwick, Brubaker, Cleland, and Landing, 1965).…”

Section: Discussionmentioning

confidence: 99%

Benign sinus histiocytosis with massive lymphadenopathy: transient immunological defects in a child with mediastinal involvement

Becroft¹,

Dix²,

Gillman³

et al. 1973

J Clin Pathol

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A 16-month-old boy presented with cervical lymphadenopathy and a mediastinal mass causing tracheal displacement. Treatment of what at first was suspected to be a malignant neoplasm was limited to low-dose irradiation of the mediastinum and biopsy excision of the cervical lymph nodes. There has been no recurrence of disease in the subsequent four years.The pathological features and clinical course correspond to a benign disease first described in 1969 in which there is massive proliferation of histiocytes in lymph node sinuses. No infectious cause was identified. Consistent but eventually reversible defects in lymphocyte response to phytohaemagglutinin and in augmentation of nitro-blue tetrazolium reduction by neutrophils during phagocytosis were demonstrated in the patient and in his monozygous twin. These defects in cellular immune function are believed to be important in the pathogenesis of the histiocytosis.

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Appearance of multiple benign paraproteins during early engraftment of soy lectin T cell-depleted haploidentical bone marrow cells in severe combined immunodeficiency

1986

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Recent advances in the prevention of graft-versus-host disease through postthymic T-cell depletion have allowed the use of haploidentical bone marrow cells for immunologic reconstitution of severe combined immunodeficiency disease. We report a male infant with severe combined immunodeficiency (with normal adenosine deaminase) who developed two IgG kappa and one IgA lambda paraproteins 7 weeks following the administration of 1.4 X 10(9) maternal bone marrow cells depleted of postthymic T cells by soy lectin agglutination and sheep erythrocyte rosetting. Serum IgG rose from 128 to 820 mg/dl, and IgA from 0 to 2400 mg/dl, peaking at 10 weeks postgrafting. By 14 weeks posttransplantation T-cell numbers and function had risen to normal (all dividing T cells had the donor karyotype) and paraprotein concentrations began to decline. These observations strongly suggest that the later-appearing T cells regulated the B-cell clones from which the paraproteins were derived. Failure of such function to appear could account for the increased incidence of B-cell lymphomas in severe combined immunodeficiency.

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Thymic alymphoplasia, monoclonal gammopathy, and Pneumocystis carinii pneumonia in an infant.

Cited by 7 publications

References 22 publications

Serum monoclonal immunoglobulins in childhood

Serum monoclonal immunoglobulins in childhood

Benign sinus histiocytosis with massive lymphadenopathy: transient immunological defects in a child with mediastinal involvement

Appearance of multiple benign paraproteins during early engraftment of soy lectin T cell-depleted haploidentical bone marrow cells in severe combined immunodeficiency

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