Primary immunodeficiency are genetically determined diseases characterized by lack of the function/a component of the immune system. Malignancies are one of the causes of death in PID. It has been demonstrated by epidemiological studies that cancer risk is 1.6-2.3 times higher in primary immunodeficiency than in the general population. The most common types of malignancies in PID are hematological malignancies, especially lymphomas.
Aim: evaluation of cases of hemoblastosis in patients diagnosed with primary immunodeficiency in the Republic of Moldova
were evaluated 51 patients (36 patients – post-mortem) aged 0-18 years diagnosed with primary immunodeficiency at the Mother and Child Institute of the Republic of Moldova. For the diagnosis of hematological malignancies were used: bone marrow biopsy, biopsy and lymph node histology, immunohistochemistry.
Of the total number of cases, 3 (5.8%) children were identified with malignant neoplasms; with a boys : girls ratio=2:1. The mean age of diagnosis of malignancy was 9.5±8,6 years. In a child with Louis Bar syndrome and marked cervical lymphadenopathy, at the thoracic CT with multiple paraaortal and peribronchial ganglia, after thelymph node biopsy and histology, the diagnosis of non-Hodgkin's lymphoma, lymphoblastic form was etablished. For the other two children, non-Hodgkin's lymphoma, lymphoblastic form (in the patient with CVID) and acute lymphoblastic leukemia (in the Louis Bar patient) were identified in the morphopathological evaluation.
Patients with primary immunodeficiency have a high risk of developing cancer. Hematological malignancies is one of the most common types of malignancy in these patients.