Features of the hidden immunodeficiency in a newborn baby whose mother suffered from leukemia

Abstract

Background & Objective: To study the features of the pathomorphological changes in the intestines, spleen and thymus gland in a baby with the hidden congenital immunodeficiency provoked by the BCG vaccination. Method: We present a case of the death of the newborn baby (14 days of life), whose mother suffered from acute leukemia in youth. The baby was born without pathologies. Therefore, she was vaccinated against hepatitis B (Engerix-B) and tuberculosis (BCG) according to the immunizations schedule. In a week the state of health of the baby became worse, the disease progressed despite the pharmacological treatment and led to multiple organ failures that caused the death. During the postmortem examination the changes in the intestines, spleen and thymus gland were observed and were studied in detail by using the histologicalimmunohistological (CD3/CD79α/CK-pan) methods.Results: The histological study of the intestines revealed the diffuse foci of necrosis, strong leukocyte infiltration and vascular congestion with multiple hemorrhages. Perivascular T-cells hypoplasia was observed in the spleen. Thymus lobules were reduced, cortex thickness (lymphocyte loss and reticular stroma collapse) and proliferation of the epithelial reticular cells were observed. Numerous Hassall's corpuscles, different in their size, were observed. The results of the immunohistochemical study of the spleen showed the absence of the perivascular T-cells and the follicular hyperplasia of B-lymphocytes at the same time. Small number of CD3/CD79a-positive cells was observed in the thymus gland, CK-pan - significantly expressed in epithelial-reticular cells. The abovementioned changes indicate the development of the congenital T-cells immunodeficiency that led to the ulcerous-necrotic enterocolitis. Conclusion: This clinical case demonstrates the importance of early diagnosis of immune status in the newborns, whose parents have leukemia in anamnesis. BCG-vaccination against the background of T-cell immunodeficiency may lead to the dangerous complications that threaten baby’s life.