DOI: 10.1097/INF.0b013e318245810e

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PMID: 22330170

Issn Print: 0891-3668

Publication Date: 2012/03/01


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BCG Lymphadenitis in Neonates With Familial Hemophagocytic Lymphohistiocytosis

Yasser Wali; Ismail Beshlawi

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Excerpt

Familial hemophagocytic lymphohistiocytosis (FHLH) is an autosomal recessive immune disorder characterized by fever, hepatosplenomegaly, pancytopenia, hypertriglyceridemia, hypofibrinogenemia, markedly elevated inflammatory cytokines, and impaired cytotoxic activity of lymphocytes. This disease is invariably fatal in infancy if the treatment is not initiated immediately with specific chemotherapy.1 In Oman, we have previously reported a number of families (10) with known FHLH mutation, mainly in the perforin gene, and our patients commonly present in the first 2 months of life.2,3 Due to the high rate of consanguinity and interfamilial marriage in Oman, almost all the new cases of hemophagocytic lymphohistiocytosis can be traced to these families. BCG is a live attenuated bacterial vaccine that is included in the expanded program on immunization in Oman, which is administered on the first day of life.4
We recently treated 2 cases (male, 1 week old and female, 9 weeks old) coming from known families, fulfilling all the diagnostic criteria of FHLH, and both of them have no perforin by FACS analysis and perforin gene mutations (674G→C(Arg225Pro)). After initiating chemotherapy as per HLH 2004 protocol,5 they developed severe BCG adenitis confirmed by excisional biopsy. They needed triple antituberculous therapy for 3 months and continued to receive isoniazid and rifampin for 1 year. This has delayed the definitive treatment of these patients with bone marrow transplant until the adenitis resolves.
As these patients came from known high risk families of FHLH, this complication could have been prevented by proper counseling, highlighting the need to screen the newborns of such families for FHLH known mutation before administering BCG vaccine. We recommend delaying the BCG vaccine for these patients until recovery of their immune system.
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