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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References
Filipovich AH . Hemophagocytic lymphohistiocytosis (HLH) and related disorders. Hematology Am Soc Hematol Educ Program 2009; 127–131.
Suzuki N, Morimoto A, Ohga S, Kudo K, Ishida Y, Ishii E et al. Characteristics of hemophagocytic lymphohistiocytosis in neonates: a nationwide survey in Japan. J Pediatr 2009; 155: 235–238.
Janka GE, Schnider EM . Modern management of children with haemophagocytic lymphohistiocytosis. Br J Haematol 2004; 124 (1): 4–14.
Takahashi N, Uehara R, Kobayashi M, Yada Y, Koike Y, Kawamata R et al. Cytokine profiles of seventeen cytokines, growth factors and chemokines in cord blood and its relation to perinatal clinical findings. Cytokine 2010; 49: 331–337.
Stephan JL, Kone-Paut I, Galambrun C, Mouy R, Bader-Meunier B, Prieur A-M . Reactive haemophagocytic syndrome in children with inflammatory disorders. A retrospective study of 24 patients. Rheumatology 2001; 40: 1285–1292.
Larroche C, Mouthon L . Pathogenesis of hemophagocytic syndrome (HPS). Autoimmun Rev 2004; 3: 69–75.
Imashuku S, Ueda I, Teramura T, Mori K, Morimoto A, Sako M et al. Occurrence of haemophagocytic lymphohistiocytosis at less than 1 year of age: analysis of 96 patients. Eur J Pediatr 2005; 164: 315–319.
Kumakura S, Ishikura H, Kondo M, Murakawa Y, Masuda J, Kobayashi S . Autoimmune-associated hemophagocytic syndrome. Mod Rheumatol 2004; 14: 205–215.
Janka GE . Hemophagocytic syndromes. Blood Rev 2007; 21: 245–253.
Fukaya S, Yasuda S, Hashimoto T, Oku K, Kataoka H, Horita T et al. Clinical features of haemophagocytic syndrome in patients with systemic autoimmune diseases: analysis of 30 cases. Rheumatology 2008; 47: 1686–1691.
Wisuthsarewong W, Soongswang J, Chantorn R . Neonatal lupus erythematosus: clinical character, investigation, and outcome. Pediatr Dermatol 2011; 28: 115–121.
Stephan JL, Kone-Paut I, Galambrun C, Mouy R, Bader-Meunier B, Prieur AM . Reactive haemophagocytic syndrome in children with inflammatory disorders. A retrospective study of 24 patients. Rheumatology (Oxford) 2001; 40: 1285–1292.
Jordan MB, Hildemann D, Kappler J, Marrack P . An animal model of hemophagocytic lymphohistiocytosis (HLH): CD8+ T cells and interferon gamma are essential for the disorder. Blood 2004; 104: 735–743.
Maruyama A, Takahashi N, Gunji Y, Morimoto A, Yada Y, Koike Y et al. Congenital hemophagocytic lymphohistiocytosis in a preterm infant: ytokine profile and a review of the disease. J Pediatr Hematol Oncol 2011; 33 (5): e209–e212.
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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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
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