Primary central nervous system lymphoma

Currently, the exact etiology of this disease does not exist. There are two theories: some studies show that the spread of a malignant clone can occur among normal B-lymphocytes located in the central nervous system ; the theory is supported by evidence of lymphoma in the white matter of the brain. The second theory is based on the fact that a clone of malignant lymphocytes with the help of special adhesion molecules can travel and penetrate the central nervous system. ^[one]

PCLCS is considered a rather rare disease, however, the frequency of cases over the past two decades has increased by 7-10 times, both in people with and without immunodeficiency . Of all primary brain tumors, primary CNS lymphoma accounts for about 4-7%, 2-3% of all non-Hodgkin lymphomas and 4-6% of all extranodal lymphomas. The average age of patients is 60-65 years. ^[one]

Primarily, central nervous system lymphomas are represented by single or multiple intracerebral nodes , which are localized in the frontal lobe (in about 50% of cases), in the corpus callosum and in the deep brain structures (in about 40% of cases). In 35% of cases, multifocal growth of volumetric education is noted. The leptomeningeal variant (16%) and spinal cord lesions (<1%) are much less common. In most cases, it is a diffuse B-cell large-cell lymphoma (DBCL) - more than 90% of cases. ^[one]

The disease is manifested mainly by neurological symptoms, depending on the location of the lesion. Often there are changes in the psyche, increased intracranial pressure and convulsive seizures.

1. Medical history
2. Assessment of focal neurological symptoms during physical examination
3. General laboratory tests ( OAC , blood biochemistry, coagulation , bone marrow examination, OAM )
4. IHC confirmation of lymphoproliferative disease.
5. Unilateral trepanobiopsy of the bone marrow.
6. MRI and CT
7. PET study (In order to clarify the primary foci)
8. In men, an ultrasound examination of the testicles is mandatory. ^[one]

The main therapy consists in irradiating the entire brain in a total dose of 40-50 Gy. However, after the procedure, 80% of people have relapses of the disease. The prognosis for relapses is absolutely unfavorable. The average survival after relapse is 12-18 months.

Methods for the prevention of central nervous system lymphoma currently do not exist, since the etiological factor leading to the development of the disease is unknown. ^[one]

1. ↑ ^{1 2 3 4 5} Abdurakhmanov Jamal Tinovich. Primary lymphoma of the central nervous system in adults // Klin. Rec. - 2017 .-- 2017.