primary effusion lymphoma
Introduction
Introduction to primary exudative lymphoma Primary exudative lymphoma (PEL) is a type of malignant lymphoma whose malignant cells are monoclonal B cells that express the CD38 and HHV-8 genes and are positive for EBV. basic knowledge The proportion of illness: 0.045% Susceptible people: no special people Mode of infection: non-infectious complication:
Cause
Cause of primary exudative lymphoma
(1) Causes of the disease
More common in HIV-infected patients, it can also occur in HIV-negative patients, and occasionally in patients after heart transplantation.
(two) pathogenesis
The tumorigenic effect of EBV in this disease has not been affirmed, unlike its role in PCNSL. The mechanism by which HHV-8 infection promotes tumor growth is not certain. A HHV-8-encoded latent-associated nuclear antigen may play an important role. It binds HHV-8 DNA to the host's chromosomes in mitotic cells, thereby separating HHV-8 episomes from daughter cells. Other studies have found that universally activated transcription factors (NF-B, possibly other factors) HHV-8-infected PEL cells are involved in survival, and competition to block apoptosis signals suggests that inhibition of NF-B leads to induction of apoptosis, and the viral IL-6 encoded by the HHV-8 genome promotes plasma cell and vascular proliferation. Cytokines, in vitro studies show that PEL cells secrete IL-6, and IL-6 antisense oligonucleotides almost completely inhibit the growth of these cell lines, but do not inhibit 2 B-cell lymphomas without HHV-8 infection. The growth of the cell line, the above in vitro results suggest that viral IL-6 is an autocrine growth factor that promotes PEL growth. In addition, human IL-10 expressed by PEL cells may be another important autocrine growth factor.
Prevention
Primary exudative lymphoma prevention
There is no effective preventive measure for this disease. Early detection and early diagnosis are the key to the prevention and treatment of this disease.
Complication
Primary exudative lymphoma complications Complication
No complications.
Symptom
Symptoms of primary exudative lymphoma Common symptoms Ascites pleural effusion pericardial effusion
The main manifestations are serous effusions such as ascites, pleural effusion and pericardial effusion. Clinically, patients often present with a large amount of effusion in the serous cavity and its related clinical manifestations. The serous exudate contains high degree of malignancy. Lymphocytes, but no detectable mass-like lesions.
Examine
Examination of primary exudative lymphoma
1. X-ray examination showed that the effusion of the serous cavity, chest X-ray and chest CT can be found in pleural effusion and (or) pericardial effusion and mild serosal thickening, no substantial lumps or enlarged mediastinal performance.
2. Mucosal cavity puncture and liquid examination can generally find malignant cells. Morphologically, the tumor cells are between diffuse large B-cell lymphoma (immunoblastic variant) and anaplastic large cell lymphoma. The type is positive for CD45 and CD30, but apparently lacks B cell and T cell-associated antigen expression, no c-MYC gene rearrangement, and HHV-8 is often detected in exudate.
According to the condition, clinical manifestations and symptoms, physical signs can be selected for electrocardiogram, B-ultrasound, biochemical examination.
Diagnosis
Diagnosis and diagnosis of primary exudative lymphoma
The clinical manifestations of serous effusion and the corresponding imaging findings are important clues for the diagnosis of this disease, and the diagnosis depends on the discovery of lymphoma cells in the exudate.
Mainly differentiated from DLBCL empyema, immunophenotyping of tumor cells can be differentiated from other morphologically similar lymphomas such as diffuse large B-cell lymphoma and anaplastic large cell lymphoma.
