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Perinatal/Neonatal Case Presentation

Hemophagocytic lymphohistiocytosis in a newborn infant born to a mother with Sjögren syndrome antibodies

Abstract

We encountered a neonatal patient with hemophagocytic lymphohistiocytosis (HLH) whose mother was positive for anti-Ro/SSA and anti-La/SSB antibodies. Complete atrioventricular block was found in a male patient at 29 weeks of gestation. The patient was born at 40 weeks of gestation. He showed severe circulatory disturbance at 22 h after the birth, and he also had elevated serum levels of aspartate aminotransferase (1027 IU l−1), alanine aminotransferase (121 IU l−1), lactic dehydrogenase (3490 IU l−1), ferritin (9769.7 ng ml−1) and soluble interleukin-2 (IL-2) receptor (3230 U ml−1). We could not find any known HLH genetic abnormality in the patient, but he fulfilled seven of the eight criteria for HLH. Serum levels of IL-6 and IL-8 had been already elevated in his cord blood, and serum levels of granulocyte-macrophage colony-stimulating factor and IL-8 were significantly increased on the second day of life. His symptoms regressed with the administration of hydrocortisone. We presumed that transplacental transfer of maternal antibodies could be related to the occurrence of HLH.

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Acknowledgements

The authors thank Professor E Ishii (Department of Pediatrics, Ehime University, Matsuyama, Japan), Dr I Ueda and Dr T Imamura (Department of Pediatrics, Kyoto Prefectural University of Medicine, Kyoto, Japan), and Dr H Horiuchi (Department of Cardiovascular Medicine, Kyoto University, Kyoto, Japan) for performing gene analysis for HLH. This work was supported by a grant from the Ministry of Health, Labor and Welfare of Japan (22390215).

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Correspondence to N Takahashi.

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Suzuki, Y., Takahashi, N., Yada, Y. et al. Hemophagocytic lymphohistiocytosis in a newborn infant born to a mother with Sjögren syndrome antibodies. J Perinatol 33, 569–571 (2013). https://doi.org/10.1038/jp.2012.147

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  • DOI: https://doi.org/10.1038/jp.2012.147

Keywords

  • autoimmunity
  • cytokines
  • maternal antibodies
  • neonatal lupus
  • hemophagocytic lymphohistiocytosis (HLH)

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