Caspian Journal of Internal Medicine، جلد ۱۲، شماره Case Supplement، صفحات ۴۳۹-۴۴۳

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عنوان انگلیسی The rare Hematological disorder; A man with Hemophagocytic Lymphohistiocytosis (HLH)
چکیده انگلیسی مقاله 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.
کلیدواژه‌های انگلیسی مقاله Hemophagocytic Lymphohistiocytosis, Splenomegaly, Fever

نویسندگان مقاله | Majid Gholizadeh
Hematopoietic Stem Cell Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran


| Shirin Kianersi
Hematopoietic Stem Cell Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran


| Leila Noorazar
Hematopoietic Stem Cell Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran


| Vahid Kaveh
Firouzgar Hospital, Iran University of Medical Sciences, Tehran, Iran


| Elham Roshandel
Hematopoietic Stem Cell Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran


| Sina Salari
Hematopoietic Stem Cell Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran



نشانی اینترنتی http://caspjim.com/browse.php?a_code=A-10-1854-1&slc_lang=en&sid=1
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کد مقاله (doi)
زبان مقاله منتشر شده en
موضوعات مقاله منتشر شده Hematology
نوع مقاله منتشر شده case report
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