Reactive Hemophagocytic Lymphohistiocytosis Secondary to Ovarian Adenocarcinoma: A Rare Case Report

Abstract

Xiao-Yan Li,1,* Shu-Min Zhu,2,* Xin-Yuan Li,2,* Rui-Sheng Dong,3 Ai-Ai Zhang,1 Shu-Jing Li,4 Yu-Lan Geng2 1Department of Laboratory Medicine, Shanxi Province Fenyang Hospital, Fenyang, 032200, People’s Republic of China; 2Department of Laboratory Medicine, The First Hospital of Hebei Medical University, Shijiazhuang, 050031, People’s Republic of China; 3Department of Imaging Medicine, Shanxi Province Fenyang Hospital, Fenyang, 032200, People’s Republic of China; 4Department of Radiology, The First Hospital of Hebei Medical University, Shijiazhuang, 050031, People’s Republic of China*These authors contributed equally to this workCorrespondence: Xiao-Yan Li, Department of Laboratory Medicine, Shanxi Province Fenyang Hospital, No. 186 of Shengli Road, Fenyang, Shanxi, 032200, People’s Republic of China, Tel [phone], Fax [phone], Email [email protected] Yu-Lan Geng, Department of Laboratory Medicine, The First Hospital of Hebei Medical University, No. 89 of Donggang Road, Shijiazhuang, Hebei, 050031, People’s Republic of China, Tel [phone], Fax [phone], Email [email protected]: Hemophagocytic lymphohistiocytosis (HLH), a syndrome of immune hyperactivation and abnormal regulation that causes life-threatening inflammation, is mainly characterized by fever, hepatosplenomegaly, cytopenia, and other symptoms. Reactive HLH (rHLH) is typically secondary to immune deregulation caused by underlying rheumatologic, infectious, or malignant conditions. Malignancy-associated HLH (M-HLH) continues to be a critical health problem worldwide. Most malignancies associated with HLH are hematologic tumors, and M-HLH in non-hematologic tumors very rarely occurs.Case Report: A 34-year-old Chinese woman had a history of persistent fever, acute dizziness, and bicytopenia. She was found to have developed bilateral ovarian cancer. Additional tests showed splenomegaly, hemophagocytes in the bone marrow, low natural killer activity, and hyperferritinemia, which met the diagnostic criteria put forth in the Histiocyte Society HLH-2004. The patient was treated with correcting anemia, increased platelets, and glucocorticoid therapy but showed no response. She progressively deteriorated and died 55 days later.Conclusion: Hemophagocytic lymphohistiocytosis related to a solid tumor is extremely rare. To the best of the authors’ knowledge, the present case was the first to report rHLH secondary to ovarian adenocarcinoma. It is very significant for a better understanding of the disease mechanisms of HLH and should attract the attention of hematologists and other clinicians as the condition progresses and the cost of treating it increases.Keywords: ovarian adenocarcinoma, reactive hemophagocytic lymphohistiocytosis, hyperferritinemia, cytopenia, high reticulocyte, natural killer activity

Keywords

• ovarian adenocarcinoma
• reactive hemophagocytic lymphohistiocytosis
• hyperferritinemia
• cytopenia
• high reticulocyte
• natural killer activity