Disseminated Bacillus Calmette-Guérin (BCG) infections in infants with immunodeficiency

Abstract

Background: The Bacillus Calmette-Guérin (BCG) preparations are live-attenuated derivatives of Mycobacterium bovis. These products are used to vaccinate infants at birth, a practice that may result in a disseminated infection in those patients who have an unidentified immunodeficiency.

Case presentation: Patients who were immunized at birth with BCG and who developed a disseminated infection are reported here to emphasize the importance of taking an extensive medical history before ‎giving the BCG vaccine. Patient 1 has a sibling who had familial hemophagocytic lymphohistiocytosis. Patient 2 has a severe immunodeficiency with profound lymphopenia. Patient 3 has a sibling who had a disseminated BCG infection. Patient 4 has two siblings with an immunodeficiency disorder; one sibling passed away in infancy and one is receiving regular immunoglobulin infusions. Patient 5 has profound lymphopenia and his brother had cytomegalovirus (CMV) pneumonitis and passed away in infancy.

Conclusions: These unfortunate events could have been avoided by compiling the relevant clinical and laboratory information. These cases also underscore the importance of a strict adherence to the BCG vaccine policies. Local and international registries that estimate the birth prevalence of primary immune deficiencies are needed prior to implementing universal BCG vaccination administration.

Keywords: BCG; Danish-SSI 1331 strain; Immunodeficiency; Mycobacterium bovis; Newborns; Tuberculosis; Vaccines.

Fig. 1

Case 1. a skin lesions showing erythematous nodules. b, c H&E stain of a skin excisional lesion ‎showing infiltration by histiocytes in the dermis. d, e H&E stain of marrow biopsy, showing infiltration by histiocytes. f marrow specimen revealing infiltration by histiocytes ingesting acid-fast bacilli

Fig. 2

Case 2. a (age 9 months): chest x-ray at her initial presentation showing bilateral pneumonia. b left forearm image showing her well-defined erythematous to brownish scaly plaques of different sizes. c (age 24 months): multiple cystic TB lesions with ring enhancements are evident; one in the right parietal lobe (measuring 48 × 45 mm and associated with edema and compression of the ipsilateral lateral ventricle) and one in the left parietal lobe (measuring 33 × 23 mm). Other smaller lesions are seen scattered on both cerebral hemispheres. d, e (age 32 months): decrease in the size of these lesions as well as decrease in the thick enhancement of their peripheral walls following treatment

Fig. 3

Case 3. BCG vaccine-associated chronic (recurrent) cervical lymphadenitis since birth