Thrombotic Thrombocytopenic Purpura with Normal Initial Hemoglobin.

Thrombotic thrombocytopenic purpura (TTP) is a life threatening multisystem disorder considered a true medical emergency. Usually patients are said to present with low hemoglobin as well as low platelets. This is a case of patient with early TTP having normal hemoglobin at presentation. A 37 year old male with history of idiopathic thrombocytopenic purpura (ITP) about 20 years ago, Ulcerative Colitis s/p colon resection, Graves disease s/p ablation, Lupus anticoagulant, and TTP about 3 years prior to admission which was treated with plasma exchange, was in his usual state of health until the morning of admission when he developed red urine and petechiae on his shoulder after playing football with his son the day before. He also reported a small amount of blood when he blew his nose. He denied fevers, chills, headache, confusion, anuria or any recent viral illness. Physical exam on admission revealed stable vital signs without fever, mild scleral icterus, and diffuse petechiae on shoulders/chest and ankle. He was alert and oriented × 3. Lab data showed platelets 5 k/ul, hemoglobin 14.3 g/dl, white blood cells 7.7 k/ul, BUN 29 mg/dl, creatinine 1.0 mg/dl, total bilirubin 6.6 mg/dl, direct bilirubin 0.5 mg/dl, reticulocytes 1.3%, LDH 1245 u/l, and haptoglobin < 7 mg/dl. Direct Coombs was negative. Peripheral smear showed 5–6 schistocytes/HPF. DIC work up was negative. Urinalysis showed large occult blood, 83 RBCs and 300mg/dl of protein. We suspected an early presentation of recurrent TTP. He was treated with methylprednisolone 1mg/kg and FFP 2–3 units then therapeutic plasma exchange with initial good response. His hemoglobin dropped to 10.9 within the next 36 hrs. He required plasma exchange over 3 months as well as other treatments including steroids, vincristine, splenectomy, and rituximab. Eventually he remained in remission. This case is notable for the normal initial hemoglobin. Isolated thrombocytopenia is often said to be ITP, but we believe that in this case the patient presented so soon after the development of symptoms that the hemoglobin had not yet declined. As TTP can be fatal if not recognized and treated promptly, it may be prudent to evaluate all patients with acute profound thrombocytopenia for TTP.