Spontaneously Relapsing And Remitting Primary Cns Lymphoma In An Immunocompetent 45-year-old Man
partap, S., & Spence, A. M. (2006). spontaneously relapsing and remitting primary CNS lymphoma in an immunocompetent 45-year-old man. Journal of neuro-oncology, 80(3), 305–307. https://doi.org/10.1007/s11060-006-9192-3
View Original Source →Abstract
A 45-year-old immunocompetent man with primary central nervous system lymphoma (PCNSL) presented with multiple spontaneous relapses and remissions in the absence of steroid treatment. Because of the fluctuations with improvement in both the clinical course and MRI findings, alternative disorders were considered that led to delay of diagnosis and treatment prior to brain biopsy. This case and a handful of others with single remissions emphasize that PCNSL cannot be reliably ruled out by improving or disappearing symptoms, signs or traditional MR imaging abnormalities.
Case Details
Disease Location
Primary cns, multiple brain regions
Personal Characteristics
45 -year-old male no previous steroid treatments
Clinical Characteristics
Presented with a 2-day history of right sided weakness and slurred speech neuroimaging revealed left hemispheric lesions that enhanced with contrast and were associate with edema and mass effect, dw-imaging and adc studies were negative, but the lesions were thought to be infarcts and he was treated with aspirin hypercoagulability was negative other than heterozygosity of factor v leiden echocardiogram was negative for thrombus or cardiac dysfunction two weeks later he returned with worsening right hemiparesis, facial droop, and dysarthria brain CT raised the question of hemorrhage and MRI revealed large lesions in the left thalamus and cerebral peduncle with questionable hemorrhage gadolinium contrast revealed a homogenous, rond lesion in the left midbrain. Dwi, adc, and MRI angiography were negative. Repeat MRI a week later, he was stable. 4 months of being stable he then began to experience left hemiparesis, difficulty swallowing secretions, and hypophonia. MRI a months later revealed improvement of lesions in the peduncle and left basal ganglia, but the imaging revealed two new lesions in the right midbrain, thalamus, basal ganglia and medial temporal lobe that enhanced with gd dwi and adc were negative still csf studies revealed normal protein, glucose, and a white blood cell count of five. Negative csf studies included angiotensin-converting enzyme (ace), immunoglobulin g index, oligoclonal banding, cytocentrifuge for tumor cells and cultures for bacteria, fungi, and acid fast bacilli serum levels of lactate dehydrogenase, ace, serum lactate and pyruvate levels were normal. Studies for mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes at positions 2342, 3256, 3271 and 8356 were normal HIV was negative follow up MRI a months later showed improvement of right sided lesions but still malignant cells. Frontal lobe biopsy revealed large b-cell lymphoma in which flow cytometry showed abnormal large b-cell population having expression of CD45, 19, 20, 38, low CD10, and monoclonal lambda surface light chain restriction without CD5. The low CD10 suggested a follicle center origin. Findings were consistent with large b-cell lymphoma immunophenotyping revealed lymphocytes consisted of 44.5% b cells (CD19+), 54.2% t cells (CD3+), and 1.2% NK cells (CD3-, CD7+) testicular ultrasound, body CT and ophthalmologic exams were negative medicine treatment along with whole-brain radiotherapy was started. He declined cytarabine
Remission Characteristics
A week after finding for three large lesions, MRI appeared stable and remained stable for 4 months after finding the new lesions, an MRI a months later revealed remarkable improvement in the right sided lesions with continued malignant cells that were seen after treatment and radiotherapy, he has survived independently 18 months after diagnosis with stable MRI and neurobiological findings, cheif deficits are hypophonia and bradykinesia
Treatment & Mechanisms
Proposed Remission Mechanisms
Natural killer cells triggered by an immune response may have reduced the tumor burden
Clinical Treatment
Aspirin methotrexate, procarbazine and vincristine followed by whole brain radiotherapy
Non-Clinical Treatment
None reported