Polymorphous lymphoproliferative disorder with Hodgkin-like features in common γ- chain–deficient severe combined immunodeficiency Mary A. Slatter, MD,

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Presentation transcript:

Polymorphous lymphoproliferative disorder with Hodgkin-like features in common γ- chain–deficient severe combined immunodeficiency Mary A. Slatter, MD, Brian Angus, FRCPath, Kevin Windebank, MD, Alison Taylor, MSc, Cathy Meaney, BSc, Tracey Lester, FRCPath, Gail Norbury, FRCPath, Sophie Hambleton, MD, Mario Abinun, MD, Terence J. Flood, MD, Andrew J. Cant, MD, Andrew R. Gennery, MD Journal of Allergy and Clinical Immunology Volume 127, Issue 2, Pages 533-535 (February 2011) DOI: 10.1016/j.jaci.2010.09.036 Copyright © 2010 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig 1 Morphologic and phenotypic characteristics of lymphoproliferative lesions from patients 1 and 2. A-D, Patient 1. A, Hematoxylin and eosin–stained preparation. Large cells with copious cytoplasm and large vesicular nuclei and prominent nucleoli, set in a background of reactive cells including histiocytes, fibroblasts, and lymphocytes. Some multinucleate forms are seen (arrow), which resemble HRS cells. B, Immunostaining for CD30. Note the intense membrane staining of the large neoplastic cells. C, Immunostaining for the B-lymphocyte–associated antigen CD79a. Note the intense cytoplasmic labeling of tumor cells. D, In situ hybridization for immunoglobulin light-chain mRNA. The 2 pictures represent the same area, and a blood vessel is represented on the left to highlight this. There are many more κ-positive cells than λ. Note that these are small lymphoid cells. The large cells shown in A to C did not express immunoglobulin light chains. E and F, Patient 2. E, Hematoxylin and eosin–stained preparation. Large cells with large hyperchromatic nuclei are set in a background of reactive cells including histiocytes, fibroblasts, and lymphocytes. Multinucleate forms were seen, and some cells of more typical HRS cell morphology were also present (inset). F, Hematoxylin and eosin–stained preparation. In this lower-power view, extensive tumor necrosis is demonstrated (yellow arrow). The bony trabeculum on the right is also necrotic. The blue arrow indicates a band of large neoplastic cells with hyperchromatic nuclei at the edge of the necrotic zone. (Original magnification × 400 except D and F, which were original magnification × 200.) Journal of Allergy and Clinical Immunology 2011 127, 533-535DOI: (10.1016/j.jaci.2010.09.036) Copyright © 2010 American Academy of Allergy, Asthma & Immunology Terms and Conditions