Introduction: Hemophagocytic lymphohistiocytosis characterized by hemophagocytosis leading to uncontrolled inflammation; the most common etiology in secondary cases of hemophagocytic lymphohistiocytosis is viral infections, especially Epstein-Barr virus. Visceral leishmaniasis is a vectorborne protozoal disease caused by Leishmania donovani complex. It is common in tropical and subtropical regions, with 50,000-90,000 new cases annually.
Case Presentation: A 15-month-old Arab female was admitted to our hospital with 15 days of fever and decreased weight. On clinical examination, she had a markedly enlarged liver and spleen that were palpable 4 cm and 6 cm below the costal margin, respectively. The peripheral blood smear showed hypochromic microcytic anemia, poikilocytosis, reactive lymphocytosis, and mild thrombocytopenia. Bone marrow aspiration did not show malignancy or any other pathological findings. The patient was put on antibiotic therapy without improvement. Repeated bone marrow aspiration showed erythrophagocytosis; intracellular small round organisms looked like the amastigote form of Leishmania (Donovan bodies) with no evidence of malignancies. Her lab values showed ferritin greater than 500 ug/L, pancytopenia, and hypertriglyceridemia. The patient was diagnosed with hemophagocytic lymphohistiocytosis secondary to visceral leishmaniasis.
Conclusion: Hemophagocytic lymphohistiocytosis secondary to visceral leishmaniasis is an extensively rare phenomenon in the medical literature that causes challenges in diagnosis and management. Steroids should be used wisely to not cover the symptoms of infections or malignancy, and amphotericin B resistance should be kept in mind in unresponsive Leishmania cases.
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http://dx.doi.org/10.1186/s13256-024-04760-4 | DOI Listing |
Sci Rep
December 2024
International Collaboration On Repair Discoveries, School of Biomedical Engineering, University of British Columbia, Vancouver, BC, Canada.
Cytokine storm syndromes such as hemophagocytic lymphohistiocytosis (HLH), Adult-onset Still's disease (AOSD), and COVID-19 cytokine storm (CCS) are characterized by markedly elevated inflammatory cytokines. However clinical measurement of serum cytokines is not widely available. This study examined the clinical utility of C-reactive protein (CRP) and ferritin, two inexpensive and widely available inflammatory markers, for distinguishing HLH from AOSD and CCS.
View Article and Find Full Text PDFExpert Rev Clin Immunol
December 2024
Institute of Hematology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
Introduction: Besides cytokine release syndromes (CRS) and immune effector cell-associated neurotoxicity syndrome (ICANS), immune effector cell-associated HLH-like syndrome (IEC-HS) is increasingly recognized across CAR-T recipients. This emergent and fatal syndrome is difficult to separate from other disorders during the early phase, and urgently requires more integrated diagnostic and therapeutic frameworks.
Areas Covered: Existing literature has pointed out the potential role of unbridled proliferation of cytotoxic T lymphocytes, lymphopenia of natural killing cells, and hypercytokinemia in triggering the IEC-HS.
J Immunother
December 2024
Department of Hematology, Peking University First Hospital, Beijing, China.
Secondary hemophagocytic lymphohistiocytosis (HLH) syndrome, a fatal disorder characterized by NK/T-cell deficiency, cytokine storm, and organ damage, is rare in chronic lymphocytic leukemia (CLL). Ibrutinib, the first generation of irreversible Bruton's tyrosine kinase inhibitor, has been the first-line therapy for CLL. As an off-target effect, it can also block IL-2 inducible T-cell kinase (ITK), which is essential in maintaining normal NK and T-cell functions.
View Article and Find Full Text PDFJ Infect
December 2024
Guangzhou Medical Research Institute of Infectious Diseases, Infectious Disease Center, Guangzhou Eighth People's Hospital, Guangzhou Medical University, Guangzhou, Guangdong 510440, China. Electronic address:
Transpl Immunol
December 2024
National Clinical Research Center for Hematologic Diseases, Jiangsu Institute of Hematology, Department of Hematology, The First Affiliated Hospital of Soochow University, Suzhou 215000, China. Electronic address:
Hemophagocytic lymphohistiocytosis (HLH) is a severe disorder with poor clinical outcomes. Use of emapalumab, an IFN-γ inhibitor, enables primary HLH control in over 85 % of affected children. However, data on emapalumab use for Epstein-Barr virus-associated HLH (EBV-HLH) are limited.
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