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Case Reports in Hematology
Volume 2017, Article ID 1262368, 8 pages
Case Report

Peripheral T-Cell Lymphoma of the Submandibular Salivary Gland as an Unusual Manifestation of Richter’s Syndrome: A Case Report and Literature Review

1Department of Intensive Methods of Therapy, V.A. Nasonova Research Institute of Rheumatology, Russian Academy of Medical Sciences, Kashirskoye shosse 34A, Moscow 115522, Russia
2Department of Pathology, N.N. Blokhin Russian Cancer Research Center, Russian Academy of Medical Sciences, Kashirskoye shosse 24, Moscow 115478, Russia

Correspondence should be addressed to Vadim R. Gorodetskiy; ur.liam@doolbyikstedorog

Received 31 July 2017; Accepted 22 October 2017; Published 3 December 2017

Academic Editor: Kazunori Nakase

Copyright © 2017 Vadim R. Gorodetskiy et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Richter’s syndrome is the development of high-grade non-Hodgkin lymphoma (NHL) or Hodgkin lymphoma in patients with chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL). In most patients with Richter’s syndrome, the high-grade NHL is diffuse large B-cell lymphoma. Only a small minority of CLL/SLL patients develop T-cell malignancies. Herein, we describe a 40-year-old male patient presenting with peripheral T-cell lymphoma not otherwise specified (PTCL-NOS) in the submandibular salivary gland, two years after the diagnosis of CLL/SLL. The PTCL-NOS consisted of small lymphocytes, which complicated diagnosis. Immunohistochemical, cytological, and molecular studies allowed the correct diagnosis of composite lymphoma (SLL/PTCL-NOS) of the submandibular salivary gland. The PTCL-NOS had a cytotoxic phenotype and aberrant expression of CD79a. There was no evidence to suggest that the PTCL-NOS of the submandibular salivary gland developed from an intimately associated submandibular lymph node or by PTCL-NOS dissemination. A review of the literature and presented case suppose that the PTCLs developed following CLL/SLL have the cytotoxic phenotype and can clinically mimic typical Richter’s syndrome.