Warm Autoimmune Hemolytic Anemia (WAHA) is the most common form of autoimmune hemolysis and there is a growing body of evidence of an association between SARS-CoV-2 infection, WAHA and a hyperinflammatory state, including hemophagocytic lymphohistiocytosis/macrophage activation syndrome. However, there is no literature to date of WAHA or hyperinflammatory state following administration of anti-SARS-CoV-2 monoclonal antibody treatment. This report documents a case of a patient with history of WAHA who developed brisk hemolysis and a hyperinflammatory state consistent with hemophagocytic lymphohistiocytosis/macrophage activation syndrome after COVID-19 infection and treatment with an anti-SARS-CoV-2 monoclonal antibody. He was successfully treated with multimodal treatment involving steroids, intravenous immunoglobulins, rituximab, anakinra, and vincristine with resolution of the hemolysis.
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http://dx.doi.org/10.1002/ccr3.8426 | DOI Listing |
J Allergy Clin Immunol
December 2024
Division of Immunology, Boston Children's Hospital, Harvard Medical School, Boston, Mass. Electronic address:
Proc (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 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.
View Article and Find Full Text PDFInt J Hematol
December 2024
Children's Cancer Center, National Center for Child Health and Development, Tokyo, Japan.
Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) has a wide range of clinical presentations and is sometimes life-threatening. It is often treated with systemic corticosteroids and etoposide, but no optimal treatment has been identified. Dexamethasone palmitate (DP) contains a combination of dexamethasone and a lipid emulsion and is selectively taken up by activated macrophages.
View Article and Find Full Text PDFJ Allergy Clin Immunol
November 2024
Division of Immunology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA. Electronic address:
Background: Overt immune activation by viral infections can lead to cytokine storm syndromes, such as hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS).
Objective: We aim to compare the immune response to different viral pathogens to understand the connection between infections and cytokine storm syndromes.
Methods: We recruited children who presented to the emergency room with fever for ≥ 3 days.
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