A case of a 61-year male presented with chief complaints of fever for three months. Diagnosis of hemophagocytic lymphohistiocytosis was made after liver biopsy when Positron-Emission Tomography revealed multiple fluoro deoxy glucose avid lesions in the liver. The patient of this disease typically presents with fever, splenomegaly, hyperferritinemia, hypertriglyceridemia, raised liver enzymes, and marrow features suggestive of hemophagocytosis. The treatment is usually systemic corticosteroids, chemotherapy with etoposide, cyclosporine, or hematopoietic stem cell transplantation, depending on the cause. A diagnosis like hemophagocytic lymphohistiocytosis can easily be missed in developing countries with low resources like Nepal. Hence, this disease should be suspected in patients presenting with pyrexia of unknown origin by treating physicians.
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http://dx.doi.org/10.31729/jnma.8626 | DOI Listing |
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.
View Article and Find Full Text PDFProc (Bayl Univ Med Cent)
July 2024
Department of Pathology, University of New Mexico School of Medicine, Albuquerque, New Mexico, USA.
This case report presents the management of a 25-year-old woman with refractory hemophagocytic lymphohistiocytosis (HLH) secondary to adult-onset Still's disease. Despite initial treatment with corticosteroids and biologic therapies, including anakinra and tocilizumab, her condition continued to deteriorate, necessitating intensive care unit admission. Following multidisciplinary consultation, ruxolitinib therapy, a Janus kinase (JAK) inhibitor targeting hyperinflammation, was initiated.
View Article and Find Full Text PDFJ Med Case Rep
December 2024
Department of Pathology, Faculty of Medicine, University of Colombo, Colombo 08, Sri Lanka.
Introduction: Primary bone marrow diffuse large B-cell lymphoma is a rare clinical entity, and the "bone marrow-liver-spleen" type of diffuse large B-cell lymphoma is rarer, with only a few published cases in literature. Though bone marrow-liver-spleen-type diffuse large B-cell lymphoma has unique presentations such as fever, cytopenias, and hemophagocytic lymphohistiocytosis, no cases with cold autoimmune hemolytic anemia have been reported.
Case Presentation: A 39-year-old Sri Lankan woman, previously healthy, presented with shortness of breath, productive cough, and fever for 4 days.
Front Oncol
December 2024
Department of Geriatrics, Hematology & Oncology Ward, Guangzhou First People's Hospital, Guangdong Medical University, Guangzhou, Guangdong, China.
Hodgkin lymphoma variant of Richter syndrome (HL-type RS) is a very rare disease, in which chronic lymphocytic leukemia (CLL) or small lymphocytic lymphoma (SLL) is transformed into novel Hodgkin lymphoma. The most important prognostic factor of HL-type RS is the clonal relationship between HL-type RS and the preexisting CLL/SLL. Detailed confirmation of clonally unrelated HL-type RS cases have not been reported.
View Article and Find Full Text PDFFront Immunol
December 2024
Clinical Laboratory, Children's Hospital Affiliated to Shandong University, Jinan, Shandong, China.
Background: Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) is a severe hyperinflammatory disorder induced by overactivation of macrophages and T cells. This study aims to identify the risk factors for the progression from infectious mononucleosis (EBV-IM) to EBV-HLH, by analyzing the laboratory parameters of patients with EBV-IM and EBV-HLH and constructing a clinical prediction model. The outcome of this study carries important clinical value for early diagnosis and treatment of EBV-HLH.
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