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

Submitted: July 21, 2022
Accepted: December 27, 2022
Published: January 11, 2023
Abstract Views: 847
PDF: 181
Publisher's note
All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.

Authors

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.

Dimensions

Altmetric

PlumX Metrics

Downloads

Download data is not yet available.

Citations

Lower EE, Smith JT, Martelo OJ, Baughman RP. The anemia of sarcoidosis. Sarcoidosis 1988;5:51-5.
Kalajian AH, Van Meter JR, Callen JP. sarcoidal Anemia and Leukopenia Treated With Methotrexate and Mycophenolate Mofetil. Arch Dermatol 2009;145:905–9. DOI: https://doi.org/10.1001/archdermatol.2009.75
Yousif PA, Moshrefi HR, Mohamed MA, Meysami A. A rare and fatal case of hemophagocytic lymphohistiocytosis associated with sarcoidosis. Am J Case Rep. 2020; 21:e921306. DOI: https://doi.org/10.12659/AJCR.921306
Ynardağ H, Pamuk GE, Karayel T, Demirci S. Bone marrow involvement in sarcoidosis: an analysis of 50 bone marrow samples. Haematologia (Budap) 2002;32:419-25.
Longcope WT, Freiman DG. A study of sarcoidosis; based on a combined investigation of 160 cases including 30 autopsies from The Johns Hopkins Hospital and Massachusetts General Hospital. Medicine (Baltimore) 1952;31:1-132. DOI: https://doi.org/10.1097/00005792-195202000-00001
Baughman RP, Teirstein AS, Judson MA, et al. Clinical characteristics of patients in a case control study of sarcoidosis. Am J Respir Crit Care Med 2001;164:1885-9. DOI: https://doi.org/10.1164/ajrccm.164.10.2104046
Salazar A, Mañá J, Corbella X, et.al. Splenomegaly in sarcoidosis: a report of 16 cases. Sarcoidosis 1995;12:131-4.
Phillips J, Staszewski H, Garrison M. Successful treatment of secondary hemophagocytic lymphohistiocytosis in a patient with disseminated histoplasmosis. Hematology 2008;13:282-5. DOI: https://doi.org/10.1179/102453308X316013
Henter JI, Horne A, Aricó M, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007;48:124-31. DOI: https://doi.org/10.1002/pbc.21039
Janka GE, Lehmberg K. Hemophagocytic syndromes -- an update. Blood Rev 2014;28:135–42. DOI: https://doi.org/10.1016/j.blre.2014.03.002
Fardet L, Galicier L, Lambotte O, et al. Development and validation of the HScore, a score for the diagnosis of reactive hemophagocytic syndrome. Arthritis Rheumatol 2014;66:2613-20. DOI: https://doi.org/10.1002/art.38690
Kumakura S, Yohko M. Clinical characteristics and treatment outcomes of autoimmune-associated hemophagocytic syndrome in adults. Arthritis Rheumatol 2014;66:2297-307. DOI: https://doi.org/10.1002/art.38672
Balduini CL, Noris P, Loni C, Aiosa C: Hemophagocytic syndrome responding to high dose gammaglobulin as presenting feature of sarcoidosis. Am J Hematol 1997;54:88-9.

How to Cite

Fujimoto, Hazuki, Yohei Kanzawa, Hidemine Senba, Tetsuo Washio, Yukiko Kato, Kei Kawano, Shimpei Mizuki, Jun Ohnishi, Takahiro Nakajima, Naoto Ishimaru, and Saori Kinami. 2023. “Hemophagocytic Syndrome in a Patient With Long-Term Stable Pulmonary Sarcoidosis With Progressive Spleen and Bone Marrow Lesion”. Monaldi Archives for Chest Disease 93 (4). https://doi.org/10.4081/monaldi.2023.2377.