A case report of lymphohistiocytic hemophagocytosis syndrome with Syntaxin11(STX11) positive gene and co-infection with Leishmania

Mohsen Jafari; Maryam Ghodsi; Maryam Noory; Mahdieh Mousavi Torshizi; Mohammad Kaji Yazdi

doi:10.18502/crcp.v8i6.16233

Mohsen Jafari Department of Pediatric Infectious Diseases, Bahrami Children Hospital, Tehran University of Medical Sciences, Tehran, Iran
Maryam Ghodsi Department of Pediatric, School of Medical Bahrami Hospital, Tehran University of Medical Sciences, Tehran, Iran
Maryam Noory School of Medicine, Tehran University of Medical Sciences, Tehran, Iran
Mahdieh Mousavi Torshizi Bahrami Children’s Hospital Tehran University of Medical Sciences, Tehran, Iran
Mohammad Kaji Yazdi Pediatric Hematology and Oncology Department, Bahrami Children’s Hospital, Tehran University of Medical Sciences, Tehran, Iran

DOI: https://doi.org/10.18502/crcp.v8i6.16233

Keywords: Hemophagocytic Lymphohistiocytosis; Leishman Body; STX11

Abstract

Background and Purpose: Hemophagocytic lymphohistiocytic syndrome is a rare, significant, and life-threatening disease. We are reporting this case with the hope that it may contribute to saving some patients if treated appropriately.

case: A 4-year-old boy presented with fever and pancytopenia. He was treated based on the symptoms that matched the criteria of (HLH). Furthermore, the bone marrow biopsy report revealed the presence of Leishman’s bodies.

Conclusion: Hemophagocytic lymphohistiocytic syndrome is a rare, important and life-threatening disease that we are reporting this case due to the hope of saving some patients if treated. A 4-year-old boy with fever and pancytopenia, who was treated according to the symptoms matching the criteria of HLH, as well as the bone marrow biopsy report showing Leishman’s bodies. HLH is a life-threatening disease based on cumulative increase and unrestricted excessive activity of T lymphocytes, cytokines and macrophages, which occurs in two primary and secondary forms, which requires the rejection of all risk factors, symptoms similar to the disease, including infections, lymphoproliferative and autoimmune diseases and other causes, and after ruling out other causes, the patient should be treated quickly with the treatment protocol of this disease, which is reported.due to the rarity of this disease and the urgent need for timely diagnosis and treatment; we are trying to report this patient