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Hemophagocytic Lymphohistiocytosis
Hanny Al-Samkari1, Nancy Berliner2
1Massachusetts General Hospital, Boston, Massachusetts 02114;
Annual Review of Pathology
|September 22, 2017
Summary
Hemophagocytic lymphohistiocytosis (HLH) is a severe immune disorder causing organ damage. While stem cell transplants improve survival in children, adults face poor outcomes, necessitating new targeted therapies.
Area of Science:
- Immunology
- Hematology
- Genetics
Background:
- Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome.
- It involves overactive cytotoxic T lymphocytes, NK cells, and macrophages, leading to cytokine storms and multi-organ damage.
- HLH can be primary (genetic) or secondary (acquired due to infections, malignancy, or autoimmunity).
Purpose of the Study:
- To provide a comprehensive overview of Hemophagocytic lymphohistiocytosis (HLH).
- To discuss the clinical manifestations, diagnostic criteria, and current treatment strategies for HLH.
- To highlight the challenges in managing HLH, particularly in adults, and explore future therapeutic avenues.
Main Methods:
- Review of existing literature on Hemophagocytic lymphohistiocytosis.
- Analysis of clinical and laboratory features associated with HLH.
- Evaluation of treatment outcomes for HLH, including hematopoietic stem cell transplantation and emerging therapies.
Main Results:
- HLH presents with fever, splenomegaly, cytopenias, liver dysfunction, and neurological symptoms.
- Diagnostic hallmarks include hyperferritinemia, hypertriglyceridemia, and hemophagocytosis.
- Allogeneic hematopoietic stem cell transplantation offers survival benefits for children, but adult outcomes remain poor.
Conclusions:
- HLH is a critical condition requiring prompt diagnosis and management.
- Current treatments like immunosuppressants and etoposide have limitations, especially in adults.
- Novel targeted immunomodulatory agents show promise for improving HLH treatment outcomes.