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Disseminated BCG infection following bone marrow transplantation for X-linked severe combined immunodeficiency
1Departments of Dermatology, Groote Schuur Hospital, Cape Town, South Africa.
Insights
Disseminated bacille Calmette-Guerin (BCG) infection in an infant with X-linked severe combined immunodeficiency (XSCID) required extensive treatment. The infant eventually recovered after bone marrow transplant and prolonged multi-drug therapy.
Area of Science:
- Immunology
- Infectious Diseases
- Pediatrics
Background:
- X-linked severe combined immunodeficiency (XSCID) is a primary immunodeficiency disorder.
- Bacille Calmette-Guerin (BCG) vaccination is used to prevent tuberculosis but can cause disseminated infection in immunocompromised individuals.
Observation:
- An 8-month-old boy with XSCID developed disseminated BCG infection after vaccination.
- The infection presented as skin abscesses and erythroderma, confirmed by skin biopsy showing granulomas and acid-fast bacilli (AFB).
Findings:
- The patient received a human leukocyte antigen (HLA)-identical bone marrow transplant (BMT).
- Treatment involved escalating antituberculous therapy (five drugs) and intravenous immunoglobulin infusions.
- Recurrent abscesses persisted for 7 months post-BMT despite aggressive treatment.
Implications:
- Disseminated BCG infection is a serious complication in XSCID patients.
- Bone marrow transplantation and prolonged multi-drug antituberculous therapy can lead to recovery.
- This case highlights the challenges in managing opportunistic infections in primary immunodeficiencies.
Abstract:
An 8-month-old boy with X-linked severe combined immunodeficiency (XSCID) developed disseminated bacille Calmette-Guerin (BCG) infection following BCG vaccination at birth. He initially presented with an abscess at the site of BCG vaccination and was begun on three-drug antituberculous treatment (rifampicin, isoniazid, and pyrazinimide). Dissemination was subclinical prior to a human leukocyte antigen (HLA)-identical bone marrow transplant (BMT) from his sister, following which he presented with an acute erythroderma. A skin biopsy specimen revealed granulomas with epithelial histiocytes and giant cells in the reticular dermis, and numerous acid-fast bacilli (AFB) were present on Ziehl-Nielsen stain. A diagnosis of disseminated BCG disease was made. Despite the addition of a fourth antituberculous agent, ethambutol, he did not recover and developed numerous skin abscesses over the following weeks. Examination of pus from these lesions demonstrated numerous AFB. Clarithromycin was added as a fifth antituberculous agent. Despite five-drug antituberculous therapy and monthly intravenous immunoglobulin infusions, recurrent abscesses containing AFB developed intermittently until 7 months posttransplant. At follow-up 1 year post-BMT he showed good general physical improvement. All abscesses had healed with scarring, and no further skin lesions had occurred.