Thrombotic microangiopathic hemolytic anemia (TMHA) caused by antiphospholipid syndrome (APS) is generally associated with renal or neurological complications and plasma exchange is the first line of treatment. We present the case of a 72-year-old woman with APS who had chronic TMHA and pulmonary hypertension without other major complications. TMHA and pulmonary hypertension were refractory to plasma exchange but were treated successfully with anticoagulant monotherapy. Contrast-enhanced computed tomography and perfusion scintigraphy did not detect signs of pulmonary embolism. TMHA localized in pulmonary microvasculature which causes pulmonary hypertension is a very rare complication of APS.
2010 by The Japanese Society of Internal Medicine