Venous thromboembolism during hematological recovery from Evans syndrome: a case report

Evans syndrome is a rare autoimmune condition characterized by immune thrombocytopenia and autoimmune hemolytic anemia. Although bleeding is common during active thrombocytopenia, venous thromboembolism may occur during subsequent hematological recovery. A 31-year-old Indian woman presented with mucosal bleeding and petechiae and was found to have severe anemia (hemoglobin 5.5 g/dL) and profound thrombocytopenia (platelet count 2,000/µL). Evidence of hemolysis included a reticulocyte count of 8.6%, elevated lactate dehydrogenase (776 U/L), hyperbilirubinemia with a total bilirubin of 3.2 mg/dL and direct bilirubin of 0.4 mg/dL, and spherocytes and polychromasia on peripheral blood smear. The direct antiglobulin test was positive for IgG. Haptoglobin was not measured. These findings, together with the profound thrombocytopenia and exclusion of infectious and other identified secondary causes, supported a diagnosis of Evans syndrome. Antinuclear antibody was positive at a titer of 1:160, while antiphospholipid antibodies were negative. She was treated with corticosteroids and a single dose of romiplostim for persistent severe thrombocytopenia, with platelet recovery to approximately 100,000/µL at discharge. Two days after discharge, she re-presented with initially nonspecific left lower chest and left hypochondrial discomfort, followed by calf pain. Computed tomography pulmonary angiography demonstrated bilateral segmental pulmonary emboli with bilateral lower-lobe pulmonary infarcts, and lower-limb venous Doppler ultrasonography confirmed deep vein thrombosis of the left popliteal vein. Therapeutic anticoagulation was initiated. This case highlights venous thromboembolism occurring during early hematological recovery from severe Evans syndrome. The temporal association between platelet recovery and thrombosis underscores that severe thrombocytopenia does not eliminate thrombotic risk. It also illustrates how nonspecific symptoms may initially obscure venous thromboembolism and emphasizes the importance of clinical reassessment when symptoms evolve.