Book contents
- Frontmatter
- Contents
- Preface
- Acknowledgements
- 1 Introduction
- 2 Normal structure, development and functions of the spleen
- 3 Post-traumatic and incidentally removed spleens
- 4 The spleen in hereditary blood cell abnormalities and auto-immune disorders
- 5 The spleen in immunodeficiency and systemic infections
- 6 Lymphomas involving the spleen
- 7 The spleen in myeloproliferative disorders
- 8 Pathology of the splenic stroma
- 9 Metastases and miscellaneous conditions
- 10 Summary: some key points in splenic differential diagnosis
- Index
5 - The spleen in immunodeficiency and systemic infections
Published online by Cambridge University Press: 14 August 2009
- Frontmatter
- Contents
- Preface
- Acknowledgements
- 1 Introduction
- 2 Normal structure, development and functions of the spleen
- 3 Post-traumatic and incidentally removed spleens
- 4 The spleen in hereditary blood cell abnormalities and auto-immune disorders
- 5 The spleen in immunodeficiency and systemic infections
- 6 Lymphomas involving the spleen
- 7 The spleen in myeloproliferative disorders
- 8 Pathology of the splenic stroma
- 9 Metastases and miscellaneous conditions
- 10 Summary: some key points in splenic differential diagnosis
- Index
Summary
Inherited immunodeficiency syndromes
General features
Splenectomy is rarely performed in patients with inherited immunodeficiency syndromes but, since deaths may occur in early life due to infection, spleens from such individuals may be seen at post-mortem examination. In those syndromes associated with less severe immunodeficiency, splenectomy may occasionally be performed incidentally during intra-abdominal surgery or the spleen may require removal following trauma, just as in any other individual. Therefore, it is useful to have an understanding of the pathological changes likely to be present due to underlying immunodeficiency. See Singer (1998) and Janeway et al. (1999) for overviews of clinico-pathological and immunological aspects of these conditions.
In general, red pulp cords and sinusoids are anatomically and functionally normal. In combined immune deficiency due to defective production of the T and B lymphoid cell lineages from early precursor cells, absence of the PALS and B cell follicles will be seen. Small B lymphocytes, plasma cells and CD8-positive T cells will also be absent from red pulp cords but this may only be detectable by immunohistochemistry. Immunodeficiency syndromes associated with this pattern of splenic lymphoid hypoplasia present as severe combined immunodeficiency (SCID) syndromes and have heterogeneous underlying genetic defects, such as cytokine receptor γ-chain deficiency in X-linked SCID.
Inherited immunodeficiency states predominantly affecting T cells
Selective depletion of T cells in the PALS and cords accompanies pure T cell deficiency such as that in DiGeorge's syndrome.
- Type
- Chapter
- Information
- Illustrated Pathology of the Spleen , pp. 63 - 76Publisher: Cambridge University PressPrint publication year: 2000