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Case Reports
. 2017 Sep;140(3):862-864.e4.
doi: 10.1016/j.jaci.2017.02.032. Epub 2017 Mar 30.

CTLA-4 haploinsufficiency in a patient with an autoimmune lymphoproliferative disorder

Zeynep Yesim Kucuk  1 , Louis-Marie Charbonnier  2 , Richard L McMasters  3 , Talal Chatila  2 , Jack J Bleesing  4
Affiliations
Case Reports

CTLA-4 haploinsufficiency in a patient with an autoimmune lymphoproliferative disorder

Zeynep Yesim Kucuk et al. J Allergy Clin Immunol. 2017 Sep.
No abstract available

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Conflict of interest statement

Disclosure of potential conflict of interest: The authors declare that they have no relevant conflicts of interest.

Figures

FIG 1
FIG 1. Histopathology of submandibular gland
A, Low power view (H&E, 11x) shows thick pink bands of collagen fibrosis, destruction of acini, and ectopic (inducible) MALT with secondary lymphoid follicles containing expanded germinal centers. A single secondary lymphoid follicle is highlighted by the box. B, Low power view (H&E, 11x) of a normal gland from a control patient shows an absence of fibrosis and preserved acini. Arrows point to a few subtle tiny lymphoid aggregates without germinal centers, which are not considered to be ectopic MALT. C, High power view (H&E, 44x) of the secondary lymphoid follicle, highlighted by the box. The center contains an expanded, pale-staining germinal center, which is surrounded by a dark blue outer mantle zone. D-K, Immunohistochemical stains (44x) confirm that the secondary lymphoid follicle has fully developed architecture. Abundant CD20+ B cells are present in both the germinal center and mantle zone, whereas relatively few CD3+ T cells are present in the germinal center. CD4+ T cells outnumber CD8+ T cells and there is no obvious increase in double negative T cells. The germinal center contains a mixture of BCL6+ follicular center cells, CD21+ follicular dendritic cells, and PD1+ follicular helper/regulatory T cells. BCL2 stains the B cells in the mantle zone and all of the T cells. It does not stain the BCL6+ follicular dendritic cells.
See this image and copyright information in PMC

References

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