A heterogeneous group of LPDs arise because of various PIDs, acting through various mechanisms, of which unrestrained infection with EBV is the most common. Diffuse large B-cell lymphoma is the most common lymphomas in the setting of PID.
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abbreviation |
aetiology of LPD |
precursor lesions |
type of LPD |
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ataxia telangestasia |
AT |
abnormal ATM gene leads to defects of DNA repair |
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Wiskott-Aldrich syndrome |
WAS |
Defects of T-cell, B-cells, neutrophils and macrophages |
lymphoid hyperplasia, nodal plasmacytosis with monoclonal gammopathy |
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common variable immunodeficiency |
CVID |
chronic antigenic stimulation leads to lymphoid hyperplasia |
fatal infectious mononucleosis, DLBCL, rarely T-cell lymphoma |
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severe combined immunodeficiency |
SCID |
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X-linked immunoproliferative disorder (Duncan syndrome) |
XLP |
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fatal infectious mononucleosis, Burkitt lymphoma |
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Nijmegen breakage syndrome |
NBS |
defective DNA repair |
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hyper-IgM syndrome |
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mutation of CD40 affects T-cell/B-cell interactions and differentiation of class-switched plasma cells |
extensive proliferation of IgM-plasma cells (may be fatal), lack of germinal centres. |
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autoimmune lymphoproliferative syndrome |
ALPS |
FAS gene mutation blocks apoptosis with resulting accumulation of lymphoid cells |
lymphoid hyperplasia |
benign proliferations of CD4-/CD8- ab T-cells, rarely T-cell lymphoma, classical HD, NLPHD |
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Job's syndrome |
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Most LPDs secondary to PID are of B-cell lineage. However, EBV may cause down-regulation of B-cell antigens. CD19, CD20 and CD79a may therefore be aberrantly negative in PID-associated B-cell LPDs. EBV also causes CD30 expression in many LPDs. LMP-1 is commonly expressed.
Most LPDs associated with PIDs are aggressive.
World Health Organization Classification of Tumours, Tumours of the haematopoietic and lymphoid tissues, IARC Press 2001.
This page last revised 23.12.2002.
©SMUHT/PW Bishop