Lymphocytic erythrophagocytosis is caused by excessive secretion of cytokines by overproliferated lymphocytes leading to phagocytosis of erythrocytes by histiocytes. This serious disease is a rare and fatal complication in patients with severe combined immunodeficiency and usually follows a septic condition in these patients. The aim of the research is to highlight the severity of this complication and raise awareness.
This is a 6-year descriptive retrospective study. The inclusion criteria are age group less than 2 years and patients with severe combined immunodeficiency disease with a biological diagnosis of lymphophagy. The study includes the results of clinical examination, biological and septic analyses, and therapeutic parameters.
We studied 8 cases of severe combined immunodeficiency with lymphocytic phagocytosis out of more than 120 cases of severe combined immunodeficiency. The age of the patients ranged from 3 to 13 months, with an average of 6 months, and the sex of the patients was 5 males and 3 females. 4 out of 8 cases had splenomegaly, 4 had hepatomegaly, and 4 had nodules, and all cases had fever during hospitalization. Six cases had pulmonary sepsis, of which 3 had cytomegalovirus isolation, 4 had ear, nose, and throat sepsis, and 1 had urinary sepsis. The most important biological data confirm the collapse of antibody levels in most patients and the diagnostic criteria for lymphocyte phagocytosis were met. In terms of treatment, all patients received antibiotics, corticosteroids in 5 patients, antibodies in 7 patients, and immunosuppressants in 5 patients. All cases died despite receiving treatment.
Co-immunodeficiency disease is a serious disease, and its most serious complication is lymphocytic phagocytosis, which should be searched for in sepsis and during hospitalization, in addition to raising awareness about it because it is rare.
