Thymic pathology in primary and secondary immunodeficiencies - PubMed (original) (raw)
Review
Thymic pathology in primary and secondary immunodeficiencies
C Nezelof. Histopathology. 1992 Dec.
Abstract
For the sake of clarity and in agreement with the World Health Organization immunodeficiency classification, it is important to distinguish the congenital, inherited malformative lesions called generically 'thymic dysplasia' from the secondary, acquired changes, designated under the broad term of 'severe thymic atrophy'. Thymic dysplasia represents the archetype of thymic changes in cellular immunodeficiency, since there is no example of a thymic dysplasia associated with a normal T-cell function. Thymic dysplasia is observed in several inherited diseases, the most frequent of them being severe combined immunodeficiency. More than the depletion of lymphoid cells, the lack of differentiation of the thymic epithelium, responsible for the absence of Hassal's corpuscles, is the main and constant feature of this condition. Thymic dysplasia underscores the crucial role of the thymic epithelium in the normal differentiation of the T-cell population. Severe thymic atrophy is secondary to various causes, including prolonged protein malnutrition and immunosuppressive or cytotoxic drugs, graft versus host reaction and, chiefly today, chronic viral infection, especially with HIV-1. The morphological changes are similar and are characterized by a partial lymphoid depletion, involving mainly the CD1+ population, necrosis and calcification of epithelial cells, the frequent presence of plasma cells and, more significantly, fibrohyaline changes of the basement membrane of the vessels and thymic epithelium. The severity of the atrophic changes and the immunodeficiency-related manifestations depend on the duration of the aetiological factors and, more significantly, with their early occurrence, within the first months of life. The mechanisms underlying thymic atrophy are poorly understood. A primary impairment of lymphoid cells seems at present to be the most likely hypothesis.
Similar articles
- The thymus gland in secondary immunodeficiency.
Linder J. Linder J. Arch Pathol Lab Med. 1987 Dec;111(12):1118-22. Arch Pathol Lab Med. 1987. PMID: 3314787 Review. - Thymic lymphocytes and thymic epithelial cells in 4 cases of congenital immunodeficiency.
Stallmach T. Stallmach T. In Vivo. 1991 May-Jun;5(3):249-53. In Vivo. 1991. PMID: 1716494 - Severe combined immunodeficiency disease: a pathological analysis of 26 cases.
Gosseye S, Diebold N, Griscelli C, Nezelof C. Gosseye S, et al. Clin Immunol Immunopathol. 1983 Oct;29(1):58-77. doi: 10.1016/0090-1229(83)90007-7. Clin Immunol Immunopathol. 1983. PMID: 6192960 - Severe combined immunodeficiency with thymic mast cell hyperplasia.
Wise WS, Still WJ, Joshi VV. Wise WS, et al. Arch Pathol Lab Med. 1976 May;100(5):283-6. Arch Pathol Lab Med. 1976. PMID: 946756 - [Inborn immunodeficiencies].
Müller-Hermelink HK, Müller J. Müller-Hermelink HK, et al. Verh Dtsch Ges Pathol. 1991;75:32-50. Verh Dtsch Ges Pathol. 1991. PMID: 1724840 Review. German.
Cited by
- Death from human cytomegalovirus infection in a girl with congenital thymic dysplasia.
Liu Y, Zhu Y, Liu W, Wan C, Guo Q. Liu Y, et al. Virol J. 2022 Nov 8;19(1):179. doi: 10.1186/s12985-022-01915-x. Virol J. 2022. PMID: 36348432 Free PMC article. - Hormonal control of T-cell development in health and disease.
Savino W, Mendes-da-Cruz DA, Lepletier A, Dardenne M. Savino W, et al. Nat Rev Endocrinol. 2016 Feb;12(2):77-89. doi: 10.1038/nrendo.2015.168. Epub 2015 Oct 6. Nat Rev Endocrinol. 2016. PMID: 26437623 Review. - Thymopoiesis in mice depends on a Foxn1-positive thymic epithelial cell lineage.
Corbeaux T, Hess I, Swann JB, Kanzler B, Haas-Assenbaum A, Boehm T. Corbeaux T, et al. Proc Natl Acad Sci U S A. 2010 Sep 21;107(38):16613-8. doi: 10.1073/pnas.1004623107. Epub 2010 Sep 7. Proc Natl Acad Sci U S A. 2010. PMID: 20823228 Free PMC article.
Publication types
MeSH terms
LinkOut - more resources
Full Text Sources