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Gamma Heavy-chain Disease: Defining the Spectrum of Associated Lymphoproliferative Disorders Through Analysis of 13 Cases

Lookup NU author(s): Dr Christopher Bacon


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Gamma heavy-chain disease (gHCD) is defined as a lymphoplasmacytic neoplasm that produces an abnormally truncated immunoglobulin gamma heavy-chain protein that lacks associated light chains. There is scant information in the literature regarding the morphologic findings in this rare disorder, but cases have often been reported to resemble lymphoplasmacytic lymphoma (LPL). To clarify the spectrum of lymphoproliferative disorders that may be associated with gHCD, this study reports the clinical, morphologic, and phenotypic findings in 13 cases of gHCD involving lymph nodes (n = 7), spleen (n = 2), bone marrow (n = 8), or other extranodal tissue biopsies (n = 3). Clinically, patients showed a female predominance (85%) with frequent occurrence of autoimmune disease (69%). Histologically, 8 cases (61%) contained a morphologically similar neoplasm of small lymphocytes, plasmacytoid lymphocytes, and plasma cells that was difficult to classify with certainty, whereas the remaining 5 cases (39%) showed the typical features of one of several other well-defined entities in the 2008 WHO classification. This report demonstrates that gHCD is associated with a variety of underlying lymphoproliferative disorders but most often shows features that overlap with cases previously reported as "vaguely nodular, polymorphous" LPL. These findings also provide practical guidance for the routine evaluation of small B-cell neoplasms with plasmacytic differentiation that could represent a heavy-chain disease and give suggestions for an improved approach to the WHO classification of gHCD.

Publication metadata

Author(s): Bieliauskas S, Tubbs RR, Bacon CM, Eshoa C, Foucar K, Gibson SE, Kroft SH, Sohani AR, Swerdlow SH, Cook JR

Publication type: Article

Publication status: Published

Journal: American Journal of Surgical Pathology

Year: 2012

Volume: 36

Issue: 4

Pages: 534-543

Print publication date: 01/04/2012

ISSN (print): 0147-5185

ISSN (electronic): 1532-0979

Publisher: Lippincott Williams & Wilkins


DOI: 10.1097/PAS.0b013e318240590a


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