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Medicine (MD/DO)
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USMLE Step 1
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Neurology, Ophthalmology & Otology
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Nervous System Cancer (Neuro-oncology)
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Primary Central Nervous System Lymphoma

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Primary Central Nervous System Lymphoma

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Primary Central Nervous System Lymphoma

(1) Wand CNS-Brain Lime-foam
DikeHAfifi
13 KEY FACTS
ASSOCIATIONS
EBV (Epstein Barr Virus)
Einstein-Bar Virus

Primary Central Nervous System Lymphoma is an extranodal, high-grade non-Hodgkin B cell neoplasm that originates in the brain, spinal cord, eyes, and cerebrospinal fluid without evidence of systemic spread. The most common association of primary CNS lymphoma is EBV which is always positive in patients with HIV-related PCNSL. EBV is thought to cause malignancy due to the activation of cell growth factors and chronic stimulation of the immune system.

HIV/AIDS
Band-AIDS

Primary CNS lymphoma is one of the late complications of HIV infection. Other defining malignancies which can be seen in AIDS are Kaposi sarcoma, non-Hodgkin lymphoma (NHL) of high-grade pathologic type and of B cell or unknown immunologic phenotype, and invasive cervical carcinoma.

Elderly
Old-man

Primary CNS lymphoma has been seen to have an increased incidence in elderly patients.

Immunocompromised
Moon-compromised

Immunocompromised conditions such as HIV/AIDS patients are thought to increase the risk of having primary CNS lymphoma.

SYMPTOMS
Confusion
Confucius

Confusion or changes in mental alertness can occur in primary CNS lymphoma patients.

Memory Loss
'Who am I' Thought Bubble

Memory loss is one of the findings seen in primary CNS lymphoma patients.

Seizures
Caesar

Seizures can happen in patient with primary CNS lymphoma.

Blurry Vision
Blurry Eyes

Lymphomas that present within the eye can result in symptoms such as blurry vision, photophobia, pain, and floaters.

DIAGNOSIS
MRI
M-R-eyes Machine

An MRI of the brain is recommended for these patients.

Single, Ring-Enhancing Lesion
Single Ring Leech

A single, ring-enhancing lesion characterizes a lesion seen in primary CNS lymphoma. The presence of EBV and HIV/AIDS will support this diagnosis. This needs to be differentiated by toxoplasmosis which poses a similar risk except with multiple rings enhancing lesions on the imaging study.

CSF Analysis
Brain-Spine-Fluid Sampler

CSF analysis should be obtained if leptomeningeal lymphoma is suspected, which includes flow cytometry, cytology, IgH (immunoglobulin heavy chain) and immunoglobulin light chain gene rearrangement, MYD88 mutational analysis and IL-10 if available.

TREATMENT
Methotrexate
Moth-T-Rex-ate

Methotrexate is proven to improve the survival of patients with primary CNS lymphoma.

Radiotherapy
Radio Therapeutic-massage

Whole-brain radiotherapy (WBRT) has a high response rate (>90%) in treating primary CNS lymphoma. But this treatment is related to a high risk of relapse.

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