Hemophagocytic syndrome
Hemophagocytic syndrome | |
---|---|
Synonyms | N/A |
Pronounce | N/A |
Specialty | N/A |
Symptoms | Fever, enlarged liver and spleen, cytopenias |
Complications | Multi-organ failure |
Onset | |
Duration | |
Types | N/A |
Causes | Genetic mutations, infections, malignancies |
Risks | |
Diagnosis | Clinical criteria, laboratory tests |
Differential diagnosis | N/A |
Prevention | N/A |
Treatment | Immunosuppressive therapy, chemotherapy |
Medication | N/A |
Prognosis | Variable |
Frequency | Rare |
Deaths | N/A |
Hemophagocytic syndrome (HPS), also known as hemophagocytic lymphohistiocytosis (HLH), is a severe systemic inflammatory condition characterized by excessive immune activation and cytokine release. It is a life-threatening disorder that can lead to multi-organ failure if not promptly diagnosed and treated.
Classification[edit | edit source]
Hemophagocytic syndrome is classified into two main types:
- Primary (familial) HLH: This form is usually inherited and is associated with genetic mutations affecting the immune system. It often presents in infancy or early childhood.
- Secondary HLH: This form is triggered by infections, malignancies, autoimmune diseases, or other conditions that lead to excessive immune activation.
Pathophysiology[edit | edit source]
The pathophysiology of hemophagocytic syndrome involves the dysregulation of the immune system, particularly the natural killer (NK) cells and cytotoxic T lymphocytes. These cells fail to regulate the immune response properly, leading to uncontrolled activation of macrophages and lymphocytes. The activated macrophages engulf and destroy blood cells, a process known as hemophagocytosis, which contributes to the clinical manifestations of the syndrome.
Clinical Features[edit | edit source]
The clinical presentation of hemophagocytic syndrome is variable but often includes:
- Persistent high fever
- Hepatosplenomegaly (enlargement of the liver and spleen)
- Cytopenias (reduction in the number of blood cells)
- Hypertriglyceridemia
- Hyperferritinemia
- Coagulopathy
- Neurological symptoms
Diagnosis[edit | edit source]
The diagnosis of hemophagocytic syndrome is based on a combination of clinical criteria and laboratory findings. The HLH-2004 diagnostic criteria are commonly used and include:
- Fever
- Splenomegaly
- Cytopenias affecting at least two of three lineages in the peripheral blood
- Hypertriglyceridemia and/or hypofibrinogenemia
- Hemophagocytosis in bone marrow, spleen, or lymph nodes
- Low or absent NK cell activity
- Hyperferritinemia
- Elevated soluble CD25 (sIL-2R)
Treatment[edit | edit source]
The treatment of hemophagocytic syndrome involves addressing the underlying cause and suppressing the excessive immune activation. Common treatment strategies include:
- Immunosuppressive therapy: Corticosteroids, etoposide, and cyclosporine are often used to control the immune response.
- Chemotherapy: In cases associated with malignancies, chemotherapy may be necessary.
- Hematopoietic stem cell transplantation: This may be considered in familial cases or refractory disease.
Prognosis[edit | edit source]
The prognosis of hemophagocytic syndrome varies depending on the underlying cause, the timeliness of diagnosis, and the response to treatment. Early recognition and intervention are critical to improving outcomes.
Epidemiology[edit | edit source]
Hemophagocytic syndrome is a rare condition, with an estimated incidence of 1 in 50,000 to 1 in 100,000 children per year. It can occur at any age but is more common in infants and young children.
See also[edit | edit source]
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Contributors: Prab R. Tumpati, MD