We describe a 15 year-old girl with autoimmune hemolytic anemia and thrombocytopenic purpura (Evans syndrome) associated with subclinical Sjögren's syndrome.
On admission, she was anemic (Hb 7.1g/dl, reticulocytes 144‰) and had jaundice (T. bil. 3.2mg/dl, D. bil. 0.2mg/dl). Petechial hemorrhages were demonstrated on her lower extremities and chest wall. Platelet count was 2.0×104/μl, and serum LDH was increased (798U/l). Increased megakaryocytes and erythroid hyperplasia were identified in the bone marrow. Serum Fe was 77μg/dl, and haptoglobin was markedly decreased (5mg/dl). Both direct and indirect Coombs' tests were continuously negative. ANA was positive at 1:80 (speakled pattern), but anti-DNA antibody and anti-ENA antibody were negative. Anti SS-A antibody was possitive at 1:16, but anti SS-B antibody was negative. Ham test, sugar water test and Donath-Landsteiner antibodies were all negative. Osmotic fragility test, RBC enzymes and Hemoglobin analysis were normal. RBC life span (51Cr-method) was 7.3days (normal: 25_??_35days). Parotid sialography revealed diffuse sialectasis, and both lacrimal and salivary secretions determined by Schirmer test or Gum test were decreased. Keratoconjunctivitis sicca was demonstrated by the rose-bengal test.
She was diagnosed as Evans syndrome with Sjögren's syndrome, and treated with prednisolone, 40mg daily. Both hemolytic anemia and thrombocytopenia responded to the treatment.
This is the first case report of Coombs' test-negative Evans syndrome associated with Sjögren's syndrome.
