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Extranodal Marginal Zone Lymphoma–like Presentations of Angioimmunoblastic T-Cell Lymphoma

A T-Cell Lymphoma Masquerading as a B-Cell Lymphoproliferative Disorder

Kaffenberger, Benjamin MD*; Haverkos, Brad MD; Tyler, Kelly MD*; Wong, Henry K. MD, PhD*; Porcu, Pierluigi MD; Gru, Alejandro Ariel MD†,‡

The American Journal of Dermatopathology: August 2015 - Volume 37 - Issue 8 - p 604–613
doi: 10.1097/DAD.0000000000000266
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Abstract: Angioimmunoblastic T-cell lymphoma (AITL) is the second most common type of peripheral T-cell lymphoma worldwide, and in some countries, it is the most common form. Clinically, AITL usually presents with systemic symptoms, diffuse lymphadenopathy, hepatosplenomegaly, and common laboratory abnormalities such as hypergammaglobulinemia. Rashes are seen in 50%–80% of patients. AITL derives from follicular T-helper cells (TFH), that express germinal center markers and produce hyperactivation of B-cells seen in AITL. Although the histological features of AITL in the skin could be similar to pathological findings present in lymph node biopsies, herein, we present 2 cases of AITL with histological and immunophenotypic features that were somewhat suggestive of extranodal marginal zone lymphoma. Caution is urged to exclude the possibility of a systemic T-cell lymphoma such as AITL in cutaneous and lymph node B-cell proliferations.

*Division of Dermatology, Department of Internal Medicine, The Ohio State University Wexner Medical Center, Columbus, OH;

Division of Hematology, Department of Internal Medicine, The Ohio State University Wexner Medical Center, Columbus, OH; and

Division of Dermatopathology, Department of Pathology, The Ohio State University Wexner Medical Center, Columbus, OH.

Reprints: Alejandro Ariel Gru, MD, The Ohio State University Wexner Medical Center, Columbus, OH 43210 (e-mail: alejandro.gru@osumc.edu).

B. Haverkos is supported by the National Cancer Institute of the National Institutes of Health under Award Number T32CA165998 (PI Miguel Villalona and Steven Devine).

The authors declare no conflicts of interest.

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