What is Evan's syndrome?

ANSWER: (B) ITP with hemolytic anemiaREF: Harrison's 18th ed ch: 115EVANS SYNDROME is the presence of simultaneous or sequential direct Coombs- positive autoimmune hemolytic anemia (AIHA) in conjunction with immune-mediated thrombocytopenia, with no known underlying etiology. The typical clinical course is chronic and relapsing, and therapy is generally progressive and poor.Signs of thrombocytopenia include purpura, petechiae, and ecchymoses. Signs of anemia include pallor, fatigue, and light-headedness. Jaundice may indicate hemolysis.Complications:Potential complications of Evans syndrome include the following:Hemorrhage with severe thrombocytopeniaSerious infection in patients with neutropeniaDiagnosis: Laboratory studies that may be considered include the following:Complete blood count (CBC) and Reticulocyte count Coombs test (direct antiglobulin test)Tests for antierythrocyte, antineutrophil, and antiplatelet antibodiesLupus antibody (lupuslike inhibitor) and antinuclear antibody (ANA) testsMeasurement of serum immunoglobulinsFlow cytometry of blood samplesBone marrow1 aspiration helps reveal aplastic anemia or an infiltrative disorder. It is usually indicated for excluding infiltrative processes in patients who present with pancytopenia.Gene mutation studiesManagementMedical therapy is the mainstay of management.In patients admitted for severe anemia or thrombocytopenia blood transfusions are indicated:Commonly used agents are as follows:First-line agent: PrednisoneIntravenous immune globulin (IVIg; for those with persistent immune cytopenia and those who require prolonged or high doses of steroids)Other pharmacologic therapiesAdditional therapies* Danazol* Cyclosporine* Azathioprine* Cyclophosphamide* Vincristine* Rituximab* Alemtuzumab* Splenectomy (has no dearly established role in treatment but may be considered in refractory cases)* Autologous and allogeneic stem cell transplantation