The rare Hematological disorder; A man with Hemophagocytic Lymphohistiocytosis (HLH)

• Main Authors: Majid Gholizadeh, Shirin Kianersi, Leila Noorazar, Vahid Kaveh, Elham Roshandel, Sina Salari
• Format: Article
• Language: English
• Published: Babol University of Medical Sciences 2021-07-01
• Series: Caspian Journal of Internal Medicine
• Subjects: hemophagocytic lymphohistiocytosis; splenomegaly; fever
• Online Access: http://caspjim.com/article-1-2374-en.html

Background: Hemophagocytic lymphohistiocytosis (HLH) is a rare disease with different causes. HLH has been categorized into two sub-groups; primary HLH which is associated with some gene mutations and secondary HLH that is developed by various causes, such as autoimmune disease, infections, and malignancies. However, the symptoms of both groups are identical and if left untreated, it will result in death. Case Presentation: In this study, we reported a 39 years old man had symptoms such as fever, weakness and chill for a month period of time. Firstly, due to pancytopenia in peripheral blood findings and clinical manifestations, he had been diagnosed with myelodysplastic syndrome (MDS) with an excess blast but the elevated liver enzymes and bilirubin were not consistent with this diagnosis. Hence, we recommended more investigation such as CT scan, bone marrow aspiration and bone marrow biopsy with immunohistochemistry tests. Finally, we found macrophages and histiocyte in bone marrow biopsy smear with Wright-Giemsa staining that engulfed the cells such as platelets and lymphocytes, so HLH syndrome was confirmed and treatment program with latest approved protocols started for the patient. Conclusion: HLH syndrome is a life-threatening disease that can be saved if timely diagnosed. Therefore, more consideration of all the laboratory findings and clinical signs of the patient can help to diagnose the disease more accurately. Also, we did a review of its pathophysiology, symptoms and therapeutic treatments.