Affiliation:
1. Janice Skinner is Adult Nurse Practitioner, School of Medicine, Department of Medicine Division of Hematology, Johns Hopkins Bayview Medical Center, Sydney Kimmel Cancer Center, 300 Mason Lord Dr, Baltimore MD 21224 (). Benedicta Yankey is Nurse Clinician, Sydney Kimmel Cancer Center, Johns Hopkins University, Baltimore, Maryland. Brenda K. Shelton is Clinical Nurse Specialist, Sydney Kimmel Cancer Center, Johns Hopkins University, Baltimore, Maryland.
Abstract
Hemophagocytic lymphohistiocytosis is a life-threatening condition associated with hyperinflammation and multiple organ dysfunction. It has many causes, symptoms, and outcomes. Early recognition is critical for treatment. Fever, cytopenias, coagulopathy, and hepatosplenomegaly are hallmark findings. Identifying the trigger event is crucial but challenging because of the varied presentations and infrequent provider experience. Diagnostic features include anemia, thrombocytopenia, neutropenia, elevated ferritin, hypertriglyceridemia, hypofibrinogenemia, hemophagocytosis (in bone marrow, spleen, or lymph nodes), low or absent natural killer cells, and elevated soluble interleukin 2 receptor assay. Primary treatment goals are eliminating the underlying trigger and suppressing hyperinflammation with steroids, immunoglobulins, or immunomodulators. Specific treatment includes corticosteroids, etoposide, and antithymocyte globulin followed by hematopoietic stem cell transplantation in patients with refractory or relapsing disease. Prompt immunochemical therapy is essential but often complicated by a high risk of treatment-related morbidity and disease recurrence. Despite these challenges, improvements in diagnostic technology and treatment have enhanced survival.
Subject
Critical Care,Emergency Medicine,General Medicine
Cited by
28 articles.
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