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Washington University Experience | VASCULAR | Thrombotic Thrombocytopenic Purpura (TTP) | 2A0 Case 2 History
Case 2 History ---- The patient was a 53 year old female with a past medical history of diabetes, paranoid schizophrenia and a hysterectomy, who initially presented with several days of dark urine and stool, nausea and vomiting. During admission, blood smear examination revealed anisocytosis, poikilocytosis, microcytosis with severe schistocytosis, tear drop cells, and elevated platelets. Bone marrow biopsy showed erythroid hyperplasia, reversed myeloid to erythroid ratio of 1:2, normoblast erythropoiesis, mild increase in plasma cells and adequate serum iron stores. Therefore, thrombocytopenic purpura (TPP) was diagnosed at that time although this case is prior to the characterization of the role of ADAMTS13 in TTP. The patient was treated with glucocorticoid plasmapheresis and transfusion of packed red blood cells. However, the patient responded poorly to the therapy. The LDH remained elevated and platelet count was less than 10K. She developed two episodes of slurred speech with left hand numbness (head CT was negative). On 7/6, she was transferred to the general medicine service at Barnes Hospital for further treatment. During admission she received aggressive therapy for TTP including glucocorticoid, aspirin, plasmapheresis and Vincristine. On 7/8 she developed a generalized tonic-clonic seizure and was transferred to the medical intensive care unit for airway protection. Her head CT showed no bleeding and no focal lesions. Under continued multiple therapy, she began to improve clinically with no recurrent seizures. On 7/12 she was weaned from mechanical ventilation support and was transferred back to the general medicine service. However, on 7/16 her clinical condition began to deteriorate again in the setting of plasmapheresis. Since she did not respond to the therapy a splenectomy was performed on 7/19. She did well postoperatively with stable vital signs until 7/20 when she developed a second generalized tonic-clonic seizure. Her head CT at that time was still negative. Lab data showed severe thrombocytopenia with rising LDH and marked schistocytes in her blood smear. She was intubated and continued to receive plasmapheresis. However, during plasmapheresis she developed bradycardia and hypotension. CPR was carried out without success and she was pronounced dead on 7/20.
