TY - JOUR
T1 - An Unusual Case of EBV-Negative Primary CNS Lymphoma of Natural Killer/T-Cell Lineage
AU - Kajtazi, Naim I.
AU - Bafaquh, Mohammed
AU - Ghamdi, Juman Al
AU - AlEissa, Zahra
AU - Shmeikh, Arwa Al
AU - Alsaeed, Ali
AU - Sulaiman, Tarek
AU - Vizcaino, M. Adelita
AU - Al Hameed, Majed
AU - Raghunathan, Aditya
N1 - Funding Information:
We are deeply indebted to King Fahad Medical City’s nursing staff for their excellent care provided to the patients. Furthermore, we appreciate all the specialties involved in this patient’s multidisciplinary care, especially neurology, neurosurgery, ID, endocrinology, rheumatology, Neuro Critical Care Unit, physical and occupational therapists, and ancillary services of Medical Imaging and Laboratory Departments. The author(s) received no financial support for the research, authorship, and/or publication of this article.
Publisher Copyright:
© The Author(s) 2021.
PY - 2021/12
Y1 - 2021/12
N2 - Extranodal NK/T-cell lymphoma (ENKTL) is a well-defined cytotoxic lymphoma strongly associated with Epstein–Barr virus (EBV) infection, commonly affecting the nasopharynx and upper aerodigestive tract. Primary central nervous system (CNS) involvement is rare, and only 17 cases were previously reported in the literature. Here, we report the case of a 44-year-old male admitted with a 3-month history of personality changes and progressive right leg weakness. Brain magnetic resonance imaging studies (MRIs) revealed multiple rim-enhancing brain lesions bilaterally. An extensive clinical and laboratory workup was unrevealing, and 2 brain biopsies were initially considered inconclusive. Pertinently, no systemic lymphoproliferative disorder was identified. The patient initially experienced remarkable clinical improvement with dexamethasone, pulse methylprednisolone, and rituximab therapy. However, he eventually had rapid clinical deterioration, was found to have increased brain lesions, and died nearly 6 months after the initial presentation. During this time, the second brain biopsy was found to show involvement by T-cell lymphoma of NK-cell lineage, which was EBV negative. No post-mortem examination was done to identify any systemic lymphoma. This case serves to expand the spectrum of lymphomas involving the CNS.
AB - Extranodal NK/T-cell lymphoma (ENKTL) is a well-defined cytotoxic lymphoma strongly associated with Epstein–Barr virus (EBV) infection, commonly affecting the nasopharynx and upper aerodigestive tract. Primary central nervous system (CNS) involvement is rare, and only 17 cases were previously reported in the literature. Here, we report the case of a 44-year-old male admitted with a 3-month history of personality changes and progressive right leg weakness. Brain magnetic resonance imaging studies (MRIs) revealed multiple rim-enhancing brain lesions bilaterally. An extensive clinical and laboratory workup was unrevealing, and 2 brain biopsies were initially considered inconclusive. Pertinently, no systemic lymphoproliferative disorder was identified. The patient initially experienced remarkable clinical improvement with dexamethasone, pulse methylprednisolone, and rituximab therapy. However, he eventually had rapid clinical deterioration, was found to have increased brain lesions, and died nearly 6 months after the initial presentation. During this time, the second brain biopsy was found to show involvement by T-cell lymphoma of NK-cell lineage, which was EBV negative. No post-mortem examination was done to identify any systemic lymphoma. This case serves to expand the spectrum of lymphomas involving the CNS.
KW - Epstein–Barr virus (EBV) infection
KW - NK (natural killer) cells
KW - Primary CNS lymphoma
KW - extranodal NK/T-cell lymphoma (ENKTL)
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U2 - 10.1177/2632010X211065692
DO - 10.1177/2632010X211065692
M3 - Article
AN - SCOPUS:85121321347
SN - 1179-5557
VL - 14
JO - Clinical Pathology
JF - Clinical Pathology
ER -