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Hemophagocytic Lymphohistiocytosis: an Under-recognized and Life-threatening Condition.
J Nepal Health Res Counc
March 2023
Department of Internal Medicine, Rochester General Hospital, NY, USA. utsav.
Diagnosis of hemophagocytic lymphohistiocytosis is a challenge in Nepal because of limited resources and the high prevalence of tropical febrile illness mimicking hemophagocytic lymphohistiocytosis. We retrospectively reviewed medical records of 21 patients who were diagnosed with hemophagocytic lymphohistiocytosis from 2010 to 2015 at a single center in Nepal. Two patients had a mutation in their perforin gene and underwent successful haploidentical stem cell transplantation.
View Article and Find Full Text PDFHemophagocytic Lymphohistiocytosis (HLH) is a rare life-threatening condition characterized by widespread activation of the immune system leading to tissue damage all over the body. It is divided into primary HLH due to inborn error in lymphocytes, T cells, and macrophages and secondary HLH which is mostly due to infections, systemic connective tissue diseases, and lymphoid malignancies. Here, we report a 34-year-old man with a history of high-grade fever, chills, and rigor, eschar, splenomegaly with the laboratory findings of thrombocytopenia, hypochromic RBCs with anisocytosis and basophilic stippling, elevated transaminases, and a positive Weil Felix test along with positive PCR results for Orientia tsutsugamushi and the presence of IgG and IgM antibodies.
View Article and Find Full Text PDFHemophagocytic Lymphohistiocytosis Associated with Scrub Typhus: Systematic Review and Comparison between Pediatric and Adult Cases.
Trop Med Infect Dis
February 2018
Stroke Center, Jichi Medical University 3311-1, Yakushiji, Shimotsuke-city, Tochigi 329-0498, Japan;
Background: Scrub typhus is a mite-borne bacterial infection caused by . Hemophagocytic lymphohistiocytosis (HLH) is a potential severe complication. Most reported cases of HLH associated with scrub typhus were single cases or case series with a small sample sizes.
View Article and Find Full Text PDFEpstein-Barr Virus Associated Hemophagocytic Syndrome after Scrub Typhus Infection.
Infect Chemother
December 2016
Division of Infectious Diseases, Gyeongsang National University Hospital, Gyeongsang National University School of Medicine, Jinju, Korea.
There have been a small number of cases of scrub typhus-associated hemophagocytic syndrome (HPS), most of which were treated successfully using adequate antibiotics. Here, we report a case of Epstein-Barr virus (EBV)-associated HPS after scrub typhus infection that was not improved using antirickettsial treatment. A 73-year-old male who had been diagnosed with scrub typhus according to an eschar and a positive serology was transferred to our institution because of a persistent fever despite 7-day doxycycline therapy.
View Article and Find Full Text PDF[Clinical analysis of scrub typhus-associated hemophagocytic syndrome].
Zhonghua Er Ke Za Zhi
September 2014
Department of Emergency Medicine, Yu Ying Children's Hospital, Wenzhou Medical College, Wenzhou 325027, China.
Objective: To analyze the clinical manifestations and intervention against fulminant scrub typhus-associated hemophagocytic syndrome.
Method: The medical records for the onset time of hemophagocytic syndrome, the clinical course, the chest radiographic findings, laboratory data, antibiotic therapy, clinical outcome and its prognosis were retrospectively reviewed.
Result: (1) Four patients were diagnosed as scrub typhus based on clinical manifestations only, while 15 patients met the criteria of laboratory diagnosis.
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