Hemophagocytic syndrome

From WikiMD's Wellness Encyclopedia

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:

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:

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