A.Y., a 52-year-old kindergarten teacher, had myelofibrosis that had been in remission for 25 years. She had seen her hematologist regularly and had had routine blood testing since the age of 27. After several weeks of fatigue, idiopathic joint and muscle aching, weakness, and a frightening episode of syncope, she saw her hematologist for evaluation. Her hemoglobin was 9.0 g/dL and her hematocrit was 29%. Concerned that she was having an exacerbation, her doctor scheduled a bone marrow aspiration, and the results were positive for myelofibrosis.
A.Y. went through a 6-month therapy regimen of iron supplements in the form of ferrous sulfate tablets and received weekly vitamin B12 injections. Interferon was given every other week in addition to erythropoiesis therapy, which was unsuccessful. She was treated for presumed aplastic anemia. During treatment, she developed splenomegaly, which compromised her abdominal organs and pulmonary function. She continued to lose weight, and her hemoglobin dropped as low as 6.0 g/dL. Weekly transfusions of packed RBCs did not improve her hemoglobin and hematocrit.
After a regimen of high-dose chemotherapy to shrink the fibers in her bone marrow and a splenectomy, A.Y. received a stem cell transplant. The stems cells were obtained from blood donated by her brother, who was a perfect immunologic match. After a 6-month period of recovery in a protected environment, required because of her immunocompromised state, A.Y. returned home and has been free of disease symptoms for over 1 year.
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