Severe Axillary Lymphadenitis After BCG Vaccination: Alert for Primary Immunodeficiencies

Abstract

The bacilli Calmette-Guérin (BCG) vaccine is administered to all newborns in countries where tuberculosis is endemic. Immunocompromised hosts, namely patients with human immunodeficiency virus infection or primary immunodeficiencies, are especially prone to serious complications from this vaccine. We report three cases of BCG disease in children with primary immunodeficiencies: one with a partial recessive interferon-γ receptor 1 deficiency, who developed BCG dissemination; and two relatives with ZAP70 deficiency, a severe combined immunodeficiency, both of whom presented with regional and distant BCG disease. All had severe axillary lymphadenitis. These clinical cases underline the importance of considering the diagnosis of immunodeficiency in a child with severe axillary lymphadenitis after BCG vaccination and of disseminated BCG disease in an immunodeficient child in the appropriate clinical setting. Moreover, BCG vaccination should be delayed in every newborn with a family history of primary immunodeficiency until the condition has been ruled out.

BCG Adenitis: Action or Inaction?

“…Serious adverse events following BCG vaccination are likely to indicate immunodeficiency states. [8][9][10][11] Incidence of mild complications is said to be less than 1 per 1000 vaccinations whereas serious events like disseminated disease is less than 1 per million vaccinations. 12 Treatment options that have generally been taken in past for BCG adenitis include conservative follow up, anti-tubercular therapy, oral antibiotics mainly Erythromycin, needle aspiration, incision drainage and excision of lymphadenitis.…”

Live Attenuated Salmonella Vaccines Displaying Regulated Delayed Lysis and Delayed Antigen Synthesis To Confer Protection against Mycobacterium tuberculosis

“…Although there are antibiotics for effectively treating TB, strains of Mycobacterium tuberculosis resistant to multiple drugs are increasing annually, compromising our ability to treat TB (5). The only available vaccine, an attenuated strain of Mycobacterium bovis Bacille CalmetteGuérin (BCG), is effective in preventing serious complications of TB in infants and small children, but this vaccine does not confer long-lasting immunity to infection (6,29,69), its efficacy in preventing TB in adults is variable, and the vaccine can cause disseminated disease in immunocompromised individuals (64).…”

Diffuse Hyper Pigmented Subcutaneous Nodules as a Primary Manifestation of Disseminated Bacillus Calmette-Guerin Disease in Young Infants

“…Notably, presence of acid fast bacilli within the cytoplasm (intracellularly) is an uncommon yet interesting phenomena (16). Our patient fulfilled all diagnostic criteria of disseminated BCG infection, including presence Mycobacterium bovis BCG substrain on his skin as indicated by PCR, showing typical histopathological changes with granulomatous inflammation and positive tuberculosis complex-PCR in gastric washing, having systemic symptoms and more than two areas of involvement beyond the site of BCG vaccination (hepatosplenomegaly, pulmonary and skin involvement) (3,17). Patients with unrecognized primary immunodeficiency frequently experience severe complications of live bacterial or viral vaccines (for example BCG and rotavirus) in early infancy.…”

“…Patients with unrecognized primary immunodeficiency frequently experience severe complications of live bacterial or viral vaccines (for example BCG and rotavirus) in early infancy. Unusual vaccine adverse events could be the first signs of primary immune deficiencies (2,3,8,12,17); some authors have estimated the incidence of primary immune deficiencies from unusual systemic adverse events of live vaccines in early infancy (2). These complications may partly be the consequence of lack of standard newborn screening programs for primary immunodeficiency.…”