Almasaabi M, Masood S, Elsayed G. Concurrent sarcoidosis and hemophagocytic lymphohistiocytosis: A case report. World J Clin Cases 2026; 14(25): 124208 [DOI: 10.12998/wjcc.124208]
Corresponding Author of This Article
Maryam Almasaabi, MBBS, Research Fellow, Department of Gastroenterology, Mediclinic Middle East Hospital, Abu Dhabi - Al Shamkha - Street 27 - Villa 9, Abu Dhabi W67, United Arab Emirates. maryamalmasaabi@outlook.com
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case-report
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Almasaabi M, Masood S, Elsayed G. Concurrent sarcoidosis and hemophagocytic lymphohistiocytosis: A case report. World J Clin Cases 2026; 14(25): 124208 [DOI: 10.12998/wjcc.124208]
World J Clin Cases. Sep 6, 2026; 14(25): 124208 Published online Sep 6, 2026. doi: 10.12998/wjcc.124208
Concurrent sarcoidosis and hemophagocytic lymphohistiocytosis: A case report
Maryam Almasaabi, Sarah Masood, Ghassan Elsayed
Maryam Almasaabi, Sarah Masood, Ghassan Elsayed, Department of Gastroenterology, Mediclinic Middle East Hospital, Abu Dhabi W67, United Arab Emirates
Co-first authors: Maryam Almasaabi and Sarah Masood.
Author contributions: Almasaabi M, Masood S, and Elsayed G contributed to study conceptualization, data collection, manuscript writing and revision, and data interpretation; Elsayed G contributed to radiological analysis and endoscopic procedures, project supervision, and critical manuscript review, and provided final approval of the manuscript; Almasaabi M and Masood S contributed equally to this work and share co-first authorship, as both were equally involved in the study design, data acquisition, data analysis, and primary drafting of the manuscript; and all authors have read and approved the final version of the manuscript.
AI contribution statement: Portions of this manuscript were edited using AI tools for language refinement. The authors were responsible and agree to be accountable for all scientific content.
Informed consent statement: Informed written consent was obtained from the patient for the publication of this report and any accompanying images.
Conflict-of-interest statement: All authors declare that they have no conflict of interest to disclose.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Corresponding author: Maryam Almasaabi, MBBS, Research Fellow, Department of Gastroenterology, Mediclinic Middle East Hospital, Abu Dhabi - Al Shamkha - Street 27 - Villa 9, Abu Dhabi W67, United Arab Emirates. maryamalmasaabi@outlook.com
Received: June 9, 2026 Revised: August 5, 2026 Accepted: August 28, 2026 Published online: September 6, 2026 Processing time: 86 Days and 13.5 Hours
Abstract
BACKGROUND
Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening hyperinflammatory syndrome driven by excessive cytokine release and uncontrolled immune activation. While HLH is commonly associated with infections, malignancies, and autoimmune diseases, its occurrence secondary to sarcoidosis is exceptionally rare. Sarcoidosis is a multisystem granulomatous disorder characterized by noncaseating granulomas, most frequently affecting the lungs and lymphatic system. Coexistence of these two conditions presents significant diagnostic and therapeutic challenges, particularly in adult populations in whom the established diagnostic criteria may be less sensitive.
CASE SUMMARY
We report the case of a 57-year-old man with a 2-year history of unexplained weight loss, recurrent nocturnal fever, generalized lymphadenopathy, and hepatosplenomegaly. His medical history included hypertension, type 2 diabetes mellitus, hyperlipidemia, and gout. Initial laboratory and imaging workup were inconclusive. Progressive clinical deterioration prompted further hematological evaluation, revealing pancytopenia with abnormal peripheral smear findings, although bone marrow biopsy was unremarkable. Serum angiotensin-converting enzyme levels were mildly elevated. Subsequent endoscopic ultrasound-guided fine needle aspiration of a subdiaphragmatic lymph node demonstrated florid noncaseating granulomatous inflammation consistent with sarcoidosis. Based on multidisciplinary team discussion and application of the HScore (174), a diagnosis of HLH secondary to systemic sarcoidosis was established. The patient was initiated on high-dose corticosteroid therapy, with a plan for gradual tapering and consideration of additional immunosuppressive therapy. Early recognition and prompt treatment were critical to prevent further clinical deterioration.
CONCLUSION
This case highlights a rare but important association between sarcoidosis and HLH, underscoring the need for maintaining a high clinical suspicion in patients presenting with systemic inflammatory features and granulomatous disease. Early multidisciplinary evaluation and timely initiation of immunosuppressive therapy are essential to improve outcomes in this potentially fatal condition.
Core Tip: This case emphasizes the importance of considering sarcoidosis in adults presenting with unexplained hemophagocytic lymphohistiocytosis (HLH) after exclusion of infectious and malignant causes. Early use of the HScore facilitated prompt diagnosis and treatment initiation. Notably, the patient showed improvement with corticosteroid therapy alone, supporting a stepwise, individualized treatment approach in clinically stable sarcoidosis-associated HLH.
