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Disseminated bacillus Calmette-Guérin infections in patients with primary immunodeficiencies
C Abramowsky1, B Gonzalez, R U Sorensen
1Department of Pathology, Emory University School of Medicine, Atlanta, Georgia.
Insights
Bacillus Calmette-Guérin (BCG) vaccination can cause disseminated disease in immunocompromised individuals, particularly those with severe combined immunodeficiency. Histologic findings reveal histiocytic infiltrates with abundant acid-fast bacilli, mimicking neoplasms.
Area of Science:
- Pathology
- Immunology
- Infectious Diseases
Background:
- Bacillus Calmette-Guérin (BCG) vaccination is a common prophylaxis against tuberculosis.
- Immunodeficiency can predispose individuals to disseminated BCG infection.
- BCG vaccination practices vary globally, with widespread use in some regions.
Purpose of the Study:
- To describe the pathologic findings in patients with disseminated BCG infection following vaccination.
- To correlate histologic features with the degree of host immunodeficiency.
- To highlight the importance of considering BCG dissemination in immunocompromised patients from endemic regions.
Main Methods:
- Analysis of biopsy and autopsy materials from four patients with BCG infection.
- Identification of Mycobacterium BCG strains in a reference laboratory.
- Histopathologic examination of affected tissues, including skin, systemic organs, and bone marrow.
Main Results:
- Patients presented with skin nodules, systemic organ lesions, and bone marrow involvement.
- Three patients had regional BCG lymphadenitis; one had disseminated BCG without local reaction.
- Histology showed diffuse histiocytic infiltrates with poorly formed granulomas and abundant acid-fast bacilli (AFB).
- Massive histiocytosis sometimes resembled a spindle cell neoplasm.
- Findings varied based on host immunocompetence, with unimpeded AFB proliferation in severe immunodeficiency.
Conclusions:
- Disseminated BCG infection can occur in individuals with severe combined or T-cell immunodeficiency.
- Histologic patterns reflect the host's immune status, with a lack of granuloma formation in severe cases.
- BCG dissemination should be considered in immunocompromised patients vaccinated in countries where BCG is practiced.
Abstract:
The pathologic findings from biopsy or autopsy material in four patients, who were vaccinated with bacillus Calmette-Guérin (BCG) at birth in Chile, are presented. Two patients had severe combined immunodeficiency, and two had more restricted cellular (T-cell) immunodeficiency with no evidence of human immunodeficiency virus infection. The patients had distinct skin nodules and nodular lesions in systemic organs and bone marrow. Three patients had regional BCG lymphadenitis. One patient with severe combined immunodeficiency, however, had disseminated BCG without any local reaction. In all cases BCG strains of Mycobacterium were identified in a reference mycobacteriology laboratory. The histologic lesions in most patients usually consisted of diffuse histiocytic infiltrates with poorly formed granulomas and variable or no necrosis. Histiocytes were plump and engorged with numerous acid-fast bacilli (AFB). In some areas the massive histiocytosis resembled a spindle cell neoplasm. Other histologic findings supported the underlying immunodeficiency. The pattern of histiocytic response and degree of microbial killing depend on the host's immunocompetence. In the later stages of disease or in severe immunodeficiency, there is a lack of granuloma formation and unimpeded proliferation of AFB. These findings are reminiscent of nontuberculous mycobacterial infections in AIDS patients. Bacillus Calmette-Guèrin dissemination has to be considered in immunocompromised individuals when the patient comes from other countries in which such vaccinations are practiced.
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