Virus associated hemophagocytic syndrome
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Virus-Associated Hemophagocytic Syndrome | |
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Specialty | Hematology, Infectious disease |
Symptoms | Fever, fatigue, splenomegaly, cytopenias, hepatomegaly, rash |
Complications | Multiorgan failure, Infections, Liver failure |
Causes | Viral infections (Epstein-Barr virus, Cytomegalovirus, HIV, others) |
Risk factors | Immunocompromised state, genetic predisposition |
Diagnostic method | Blood tests, bone marrow biopsy |
Differential diagnosis | Sepsis, Lymphoma, Autoimmune diseases, Macrophage Activation Syndrome |
Treatment | Immunosuppression, Antiviral drugs, Corticosteroids, Intravenous immunoglobulin |
Prognosis | Variable |
Frequency | Rare |
Virus-Associated Hemophagocytic Syndrome (VAHS) is a rare, life-threatening condition characterized by an overwhelming activation of the immune system, leading to severe inflammation and damage to various organs. It is a form of Hemophagocytic Lymphohistiocytosis (HLH) triggered by viral infections. The syndrome is marked by fever, splenomegaly, cytopenias, hepatomegaly, and rash. The pathophysiology involves excessive activation of macrophages and T cells, leading to the phagocytosis of blood cells in the bone marrow and other tissues.
Causes[edit | edit source]
VAHS is most commonly triggered by viral infections such as the Epstein-Barr virus (EBV), Cytomegalovirus (CMV), Human Immunodeficiency Virus (HIV), and others. Individuals with a weakened immune system or a genetic predisposition are at a higher risk of developing the syndrome.
Symptoms[edit | edit source]
Common symptoms include persistent fever, fatigue, splenomegaly (enlarged spleen), cytopenias (low blood cell counts), hepatomegaly (enlarged liver), and skin rash. The condition can rapidly progress to multiorgan failure if not treated promptly.
Diagnosis[edit | edit source]
Diagnosis of VAHS involves a combination of clinical findings and laboratory tests, including blood tests showing cytopenias and elevated levels of triglycerides and ferritin. A bone marrow biopsy is often performed to confirm the presence of hemophagocytosis.
Treatment[edit | edit source]
Treatment aims at controlling the underlying viral infection and suppressing the excessive immune response. This may include the use of antiviral drugs, corticosteroids, immunosuppressive drugs, and intravenous immunoglobulin. Early diagnosis and treatment are critical for improving outcomes.
Prognosis[edit | edit source]
The prognosis of VAHS varies depending on the underlying viral trigger, the promptness of diagnosis, and the effectiveness of treatment. Without treatment, the syndrome can be fatal due to multiorgan failure.
See also[edit | edit source]
- Hemophagocytic Lymphohistiocytosis
- Macrophage Activation Syndrome
- Infectious mononucleosis
- Immunodeficiency
Virus associated hemophagocytic syndrome Resources | ||
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