Disseminated BCG Infection Following Bone Marrow Transplantation for X‐Linked Severe Combined Immunodeficiency

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.

Successful treatment of BCG‐related immune reconstitution inflammatory syndrome following ex vivo T‐cell–depleted haploidentical hematopoietic stem cell transplantation: A case report

“…Three of them had no prior evidence of infection, yet developed either local or disseminated infection following HSCT as in our case . Sixteen of them had infection prior to HSCT, and dissemination process had occurred before transplantation as a result of inadequate immunity …”

“…Abu Arja et al reviewed 19 reported cases of BCG infection that had been reported in post‐transplant patients with PID . Three of them had no prior evidence of infection, yet developed either local or disseminated infection following HSCT as in our case .…”

Update on cutaneous complications of Bacille Calmette-Guérin immunization

“…The first case report of acute erythroderma and recurrent BCG abscesses was presented after bone marrow transplant [32].…”

Successful management of cutaneous BCG dissemination in a child affected by SCID and receiving allogeneic hematopoietic stem cell transplant

“…3,4 Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is the only treatment for SCID, but a high transplant-related mortality rate is reported in the case of disseminated BCG infection. 5 We report a case of successful management of a disseminated BCG infection in a child with a diagnosis of X-linked SCID undergoing allo-HSCT.…”

“…In patients with T-cell defects systemic dissemination of the organism can occur, but true infection with signs and symptoms of mycobacterial disease is very rare, with an incidence rate ranging from 1 to 4.3 cases per 1 million infants vaccinated; in these cases the mortality rate is very high (60%). [4][5][6][7][8] Our patient affected with T-B+ SCID presented a localized infection at the site of inoculum and satellite lymph node, a consequence of BCG infection that became disseminated during pre-engraftment bone marrow aplasia induced by allo-HSCT conditioning regimen, despite 4 months of previous, presumptively effective anti-BCG therapy with isoniazid, rifampin and ethambutol. [9][10][11] This dissemination was finally controlled by this combination of antibiotics, with temporary addition of amikacin (3 months) and levofloxacin (6 months) 11,12 in the early postengraftment period, and immune reconstitution (despite immunosuppression for management of GvHD).…”