Han MR, Hwang JH, Cha S, Jeon SY, Jang KY, Kim N, Lee CH. Hemophagocytic lymphohistiocytosis triggered by relapsing polychondritis: A case report. World J Orthop 2024; 15(8): 813-819 [DOI: 10.5312/wjo.v15.i8.813]
Corresponding Author of This Article
Chang-Hoon Lee, MD, PhD, Assistant Professor, Doctor, Department of Internal Medicine, Jeonbuk National University Medical School, 20 Geonji-ro, Jeonju 54907, South Korea. rednose2000@hanmail.net
Research Domain of This Article
Hematology
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
World J Orthop. Aug 18, 2024; 15(8): 813-819 Published online Aug 18, 2024. doi: 10.5312/wjo.v15.i8.813
Hemophagocytic lymphohistiocytosis triggered by relapsing polychondritis: A case report
Mi-Ran Han, Jeong-Hwan Hwang, Seungah Cha, So-Yeon Jeon, Kyu Yun Jang, Namsu Kim, Chang-Hoon Lee
Mi-Ran Han, Jeong-Hwan Hwang, Seungah Cha, So-Yeon Jeon, Chang-Hoon Lee, Department of Internal Medicine, Jeonbuk National University Medical School, Jeonju 54907, South Korea
Kyu Yun Jang, Department of Pathology, Jeonbuk National University Medical School, Jeonju 54907, South Korea
Namsu Kim, Department of Laboratory Medicine, Jeonbuk National University Medical School, Jeonju 54907, South Korea
Author contributions: Han MR wrote the manuscript; Cha S, Jang KY, and Kim NS collected and validated the data; Hwang JH and Jeon SY revised the manuscript; Lee CH was in charge of patient treatment and designed the manuscript; All authors have read and approved the final manuscript.
Informed consent statement: Written informed consent was obtained from the patient for publication of this case report and accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflict of interest.
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).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Chang-Hoon Lee, MD, PhD, Assistant Professor, Doctor, Department of Internal Medicine, Jeonbuk National University Medical School, 20 Geonji-ro, Jeonju 54907, South Korea. rednose2000@hanmail.net
Received: May 15, 2024 Revised: July 17, 2024 Accepted: July 26, 2024 Published online: August 18, 2024 Processing time: 90 Days and 6.5 Hours
Abstract
BACKGROUND
Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening disorder caused by abnormal histiocytes and T cell activation. In adults, it is predominantly associated with infections, cancers, and autoimmune diseases. Relapsing polychondritis (RP), another rare disease, is diagnosed based on symptoms without specific tests, featuring cartilage inflammation characterized by swelling, redness, and pain, rarely inducing HLH.
CASE SUMMARY
A 74-year-old woman visited the emergency room with a fever of 38.6 °C. Blood tests, cultures, and imaging were performed to evaluate fever. Results showed increased fluorescent antinuclear antibody levels and mild cytopenia, with no other specific findings. Imaging revealed lymph node enlargement was observed; however, biopsy results were inconclusive. Upon re-evaluation of the physical exam, inflammatory signs suggestive of RP were observed in the ears and nose, prompting a tissue biopsy for confirmation. Simultaneously, persistent fever accompanied by cytopenia prompted a bone marrow examination, revealing hemophagocytic cells. After finding no significant results in blood culture, viral markers, and tissue examination of enlarged lymph nodes, HLH was diagnosed by RP. Treatment involved methylprednisolone followed by azathioprine. After two months, bone marrow examination confirmed resolution of hemophagocytosis, with normalization of hyperferritinemia and pancytopenia.
CONCLUSION
Thorough physical examination enabled diagnosis and treatment of HLH triggered by RP in patients presenting with fever of unknown origin.
Core Tip: Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening condition typically triggered in adults by conditions such as cancer, infections, and autoimmune disorders, resulting in immune system over-activation. Relapsing polychondritis (RP), an uncommon disease, is diagnosed through physical examination. In contrast to primary HLH, which necessitates stem cell transplantation is the only definitive cure, acquired HLH can be managed with therapies such as chemotherapy and immunosuppressive therapy, tailored to the underlying cause. The patient received treatment for HLH using RP therapies and was successfully cured with methylprednisolone (1 mg/kg) and azathioprine.
