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Hemophagocytic syndrome in patients with long-term stable pulmonary sarcoidosis with progressive spleen and bone marrow lesion

An 83-year-old woman with asymptomatic pulmonary sarcoidosis presented to our hospital with fever and malaise for three months. Abdominal CT showed splenomegaly, and bone marrow examination revealed non-caseating granulomas. Pancytopenia was diagnosed due to bone marrow and splenic lesions of sarcoi...

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Bibliographic Details
Published in:Monaldi archives for chest disease 2023-01
Main Authors: Fujimoto, Hazuki, Kanzawa, Yohei, Senba, Hidemine, Washio, Tetsuo, Kato, Yukiko, Kawano, Kei, Mizuki, Shimpei, Ohnishi, Jun, Nakajima, Takahiro, Ishimaru, Naoto, Kinami, Saori
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Language:English
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Summary:An 83-year-old woman with asymptomatic pulmonary sarcoidosis presented to our hospital with fever and malaise for three months. Abdominal CT showed splenomegaly, and bone marrow examination revealed non-caseating granulomas. Pancytopenia was diagnosed due to bone marrow and splenic lesions of sarcoidosis. Steroid pulses were administered, but the patient died without response to treatment. Pathological autopsy results showed non-caseating granulomas and hemophagocytosis in the spleen and bone marrow. This suggested hemophagocytic syndrome, which was not suspected before death, in addition to sarcoidosis. In patients with splenomegaly and pancytopenia with history of pulmonary sarcoidosis, hemophagocytic syndrome should be considered in differential diagnosis.
ISSN:1122-0643
2532-5264
DOI:10.4081/monaldi.2023.2377