Omenn's syndrome—Pathologic arguments in favor of a graft versus host pathogenesis: A report of nine cases

Jouan, H; Deist, F Le; Nézelof, C

doi:10.1016/s0046-8177(87)80376-3

Cited by 45 publications

(27 citation statements)

References 15 publications

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“…At present, most authors consider the etiopathology of Omcnn and Omenn-like syndromes to be an autologous graft-versus-host reaction in an immunocompromised host [4], Immunocompetent cells from the mother could give rise to this syndrome, as has been described [5]. However, in most cases and also in our case, no chimerism could be demonstrated.…”

Section: Discussionsupporting

confidence: 51%

A Case of Omenn-Like Immunodeficiency Syndrome

Morren

Lierde²,

Lacquet³

et al. 1992

Dermatology

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Section: Discussionsupporting

confidence: 51%

A Case of Omenn-Like Immunodeficiency Syndrome

Morren

Lierde²,

Lacquet³

et al. 1992

Dermatology

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“…Death usually occurs from overwhelming infection in the 1st year of life [5], although successful BMT has been recorded with a HLA identical donor [5]. The pathogenesis of Omenn syndrome remains unclear, but maternal engraftment in a primary cellular immunodeficiency has been suggested [4,5]. In our view this is unlikely, as maternal engraftment could not be demonstrated by HLA tissue typing in our patient.…”

Section: Discussionmentioning

confidence: 67%

Successful bone marrow transplantation and treatment of BCG infection in two patients with severe combined immunodeficiency

et al. 1991

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We report successful bone marrow transplantation (BMT) in two patients with severe combined immunodeficiency (SCID), who had developed BCG infection following neonatal vaccination. Patient 1 had Omenn Syndrome, associated with hypertrophic non-obstructive cardiomyopathy. Patient 2 had SCID due to adenosine deaminase deficiency. This communication demonstrates that with appropriate anti-mycobacterial cover, immunological reconstitution together with full recovery from BCG infection can be achieved by BMT. As demonstrated by persistent negative Mantoux tests, specific cell-mediated immunity to BCG was not acquired following BMT. We suggest that these children may continue to be at risk from mycobacterial infection.

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“…In classic OS, T lymphocytes originate from the host, predominantly infiltrate skin and gut and induce tissue damage through a poorly defined autoimmune-like mechanism [9]. These lymphocytes are oligoclonal, as indicated by the restricted heterogeneity of their T cell receptor repertoire and probably arise from leakiness in the context of a genetically determined SCID [6-81.…”

Section: Discussionmentioning

confidence: 99%

CD30 cell expression and abnormal soluble CD30 serum accumulation in Omenn's syndrome: Evidence for a T helper 2‐mediated condition

et al. 1996

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Omenn's syndrome (OS) is a severe immunodeficiency, characterized by clinical and laboratory features reminiscent of a T helper type-2 (Th2) response. CD30, a member of the tumor necrosis factor receptor superfamily, has been found to be preferentially expressed by human T cell clones exhibiting a Th2-line profile and function. We investigated whether there are derangement in CD30 expression in tissues, and/or abnormalities in soluble CD30 (sCD30) levels in the serum, or both, of three children with OS and one child with maternal engraftment and Omenn's-like syndrome (OLS). Large proportions of tissue-infiltrating T lymphocytes from all four patients expressed CD30, whereas in control tissues, including peripheral blood, CD30+ T lymphocytes were extremely few or absent. In addition, levels of sCD30 were abnormally increased in all patients' sera. T cell clones were generated from sorted CD30+ and CD30-peripheral blood T cells of the patient with OLS who showed unusually high numbers of circulating CD30+ T lymphocytes. Most CD4+ T cell clones derived from CD30+ cells showed a Th2-like cytokine profile, whereas the majority of clones generated from CD30-T cells were Th1. These findings support the hypothesis that Th2 cells are involved in the pathogenesis of OS. Moreover, they provide evidence that detection of CD30+ T cells in tissues, increased levels of sCD30 in biological fluids, or both, reflect the presence of immune responses characterized by prevalent activation of T cells producing Th2 cytokines.

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Omenn's syndrome—Pathologic arguments in favor of a graft versus host pathogenesis: A report of nine cases

Cited by 45 publications

References 15 publications

A Case of Omenn-Like Immunodeficiency Syndrome

A Case of Omenn-Like Immunodeficiency Syndrome

Successful bone marrow transplantation and treatment of BCG infection in two patients with severe combined immunodeficiency

CD30 cell expression and abnormal soluble CD30 serum accumulation in Omenn's syndrome: Evidence for a T helper 2‐mediated condition

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