A 53‐year‐old woman presented with right arm numbness extending from the shoulder to the fingertips. She was taking a shower when she had difficulty raising her arm. The numbness and weakness spontaneously resolved within 10 minutes, and then suddenly reemerged in the left arm. Her husband called 911. The EMS team initiated a stroke activation. She noted bilateral blurry vision and floating bright spots in her visual felds, symptoms she typically had experienced during migraine attacks. She coincidentally reported an upper respiratory tract infection for 1 week. Her vital signs were normal, and she was alert and oriented. Her pupils were normal and there were no retinal abnormalities or papilledema. Her heart, lung, and abdominal examinations were normal. There was no extremity edema. Two 1‐cm purpuric lesions were noted on the fingers of her right hand. She had diffuse petechiae around her anterior neck, chest, and forearms, with a few resolving ecchymoses on her breast and abdomen. Her neurologic exam at the time of admission was normal; the numbness, weakness, and dysarthria had resolved. WBC was 7.4, hemoglobin 8.8, and platelet count 11. Coagulation studies were normal. Peripheral smear revealed schistocyles. Thrombotic thrombocytopenic purpura was the presumptive diagnosis, and an ADAMTS‐13 test was sent. During preparation for emergent plasmapheresis, her mental status rapidly declined, and the right arm weakness reemerged. Repeat CT scan of the head was negative. After the first plasmapheresis treatment, she became febrile. Her platelet count elevated to 19 and her mental status and jaundice improved. She was started on prednisone, and after the second plasmapheresis, her platelets increased to 19, her mental status returned to normal, and her hemoglobin stabilized at 9.1. An HIV test was negative.
It is important for the hospitalist to recognize that patients with TTP rarely have all 5 classic symptoms at the time of presentation. The general internist must have sufficient clinical suspicion to distinguish TTP from other common disorders such as stroke or complicated migraines. The only significant finding may be thrombocytopenia, which can occur in several other diseases. Early, only a few of the classic pentad may be present and should not delay treatment for suspected thrombotic thrombocytopenic purpura, as mortality can exceed 90% if left untreated.
The diagnosis of thrombotic thrombocytopenic purpura became likely when her strokelike neurologic complaints were paired with laboratory evidence of thrombocytopenia and hemolysis on peripheral smear. Treatment with plasmapheresis should be initialed as quickly as possible based on clinical suspicion and immediately available laboratory evidence. No clearly identifiable cause was found in our patient; so knowing her ADAMTS‐13 level may point to a hereditary predisposition that may have been setoff by a possible recent infection.
S. Schott, none.