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Successful pregnancy in a patient with familial sea-blue histiocyte syndrome

S A Fadilah1, N R Mazeni, S K Cheong

  • 1Department of Haematology and Transplantation, MAKNA-HUKM Cancer Institute, Hospital Universiti Kebangsaan Malaysia (HUKM), Jalan Yaacob Latif, Cheras, 56000 Kuala Lumpur.

A 27-year-old Indian woman at 23 weeks' gestation presented with decompensated liver cirrhosis, coagulopathy, restrictive lung disease with cor pulmonale and preeclampsia. She was diagnosed to have sea-blue histiocyte syndrome (SBHS) at the age of 13 years and was treated conservatively. There was worsening liver, respiratory and bone marrow function as the pregnancy progressed. She underwent a successful pregnancy despite her poor medical condition and advanced disease state. We described the first case of familial SBHS in a pregnant patient from Asia.