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9 Cards in this Set
- Front
- Back
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Evans syndrome is the combination of autoimmune hemolytic anemia and kostmann syndrome. T/F
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False- Combo of AIHA and ITP, in the absence of known underlying etiology.
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What is the pathophysiology of Evans Syndrome?
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Pathophysoiogy is unknown, a disorder of both humoral and immune regulation. Hypogammaglobulinemia and other autoimmune problems have been reported.
Autoantibodies may be produced against antigens on the surface of erythrocytes, platelets or neutrophils. Immune destruction on red cells, platelets or neutrophils generally occures in the peripheral circulation, though targeted cells may also be removed in the bone marrow. |
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What is the clinical presentation of a patient with Evans Syndrome?
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Present with AIHA or ITP separately or concomitantly.
AIHA- pallor, fatigue, jaundice and if severe cardiopulmonary failure. ITP- petechiae, bruising and mucutaneous bleeding, hemoglobinuria |
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How is a diagnosis of Evans Syndrome made?
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Diagnosis of exlusion. CBC, retic, bili, direct coombs (may be positive for IgG or complement, antibodies, QIGs, BMA, T cell subsets by flow.
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What is Autoimmune lymphoproliferative syndrome (ALPS)?
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50% of patients with Evans Syndrome have ALPS.
It is a rare disorder that presents in early childhood and is characterized by abnormal lymphocyte survival (defective apoptosis). Majority have genetic mutations in the FAS gene. |
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What are treatments included for first line therapy in the treatment of ALPS?
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- Corticosteroids
- IVIG (2gm/kg) in divided doses |
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What is the second line therapy in the treatment of ALPS?
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- Immunosuppressive agents (CSA, MMF, danazol)
- Rituximab - Vincristine - Splenectomy |
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What is the third line therapy in the treatment of ALPS?
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- Cyclophosphamide
- Alemtuzumab - Vincristine-loaded platelets Reserved for patients with severe relapsing disease dispite second-line therapy HSCT may be the only therapy to offer long-term remission |
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What is the prognosis for Evan's Syndrome?
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Usually chronic, with frequent exacerbations and remissions; occasionally fatal. Episodes of ITP appear more frequently and are more difficult to control than AIHA.
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