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2025 ; 16
(6
): 195-200
Nephropedia Template TP
gab.com Text
Twit Text FOAVip
Twit Text #
English Wikipedia
Successful Treatment of Primary Central Nervous System T-Cell Lymphoma With
Induction Chemotherapy Followed by Consolidation With High-Dose Chemotherapy and
Autologous Stem Cell Rescue
#MMPMID40642741
Holley N
; Waris S
; Elzahrany H
; Howell D
; Randall C
; Saifuddin A
; Safi SUD
J Med Cases
2025[Jun]; 16
(6
): 195-200
PMID40642741
show ga
Primary central nervous system T-cell lymphoma (PCNSTL) is an exceptionally rare
subtype of non-Hodgkin lymphoma, comprising only 2% of primary CNS lymphoma
cases. Due to its rarity, PCNSTL is often misdiagnosed, lacks standardized
treatment guidelines, and carries a poor prognosis. We present a unique case of a
59-year-old-woman with a history of hypertension and hyperlipidemia who initially
presented with sudden-onset aphasia and right-sided weakness. Suspected initially
of having a cerebrovascular accident (CVA), she received tenecteplase and
underwent investigation of CVA, which was largely unremarkable. Six months later,
she returned with progressive nausea, vomiting, confusion, and word-finding
difficulties. A magnetic resonance imaging (MRI) of the brain showed lesions in
the left cerebellum and frontal lobe with vasogenic edema. A suboccipital
craniotomy and biopsy confirmed anaplastic lymphoma kinase (ALK)-negative
anaplastic large cell lymphoma. Positron emission tomography-computed tomography
(PET-CT) showed no systemic disease and cerebrospinal fluid (CSF) analysis showed
lymphomatous involvement. The patient was initiated on six cycles of
methotrexate, cytarabine, and thiotepa (MATRix regimen without rituximab),
followed by high-dose chemotherapy (HDC) with carmustine and thiotepa and
autologous stem cell transplantation (ASCT). She tolerated treatment and
transplant without complications and remains in complete remission 18 months
post-transplant. To our knowledge, this is the first reported case of PCNSTL
treated successfully with MATRix followed by HDC-ASCT. This case highlights the
importance of considering rare CNS lymphomas in patients with atypical neurologic
presentations and suggests the use of HDC-ASCT as a promising approach in a
disease with no established standard of care.