2000 Associates' Presentations
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The Case of the Dysfunctional Bone Marrow
Amy Stella, MD, University of Wisconsin Medical School, Madison, WI
Case Presentation: Mr. B is a 37 y/o man who presented with a 4-month history of dyspnea on exertion, fatigue, cough and low-grade fevers. On exam he had bibasilar rales, a hyperdynamic precordium with a right ventricular heave, a wide fixed split S2 and a harsh 3/6 systolic murmur over the left upper sternal border. He had cervical and axillary adenopathy as well as splenomegaly. He was anemic with a hemoglobin of 7.8, thrombocytopenic and had a leukocytosis with 33% monocytes and 6% blasts. A bone marrow biopsy was consistent with MDS, specifically CMML with monosomy 7. He was also diagnosed with an ASD with bi-directional shunting and right ventricular hypertrophy with severe pulmonary hypertension. His MDS quickly transformed into acute myelomonocytic leukemia confirmed by bone marrow biopsy.
Discussion: MDS is a preleukemic state manifested by ineffective hematopoiesis. The cause is generally unknown. Prognosis is determined by cytogenetic abnormalities, percentage of blasts in the bone marrow, and number of cytopenic cell lines. The median survival of patients with the best prognosis is over 5 years, and those with the worst less than 6 months. Anywhere from 30-75% of patients with poor prognostic characteristics transform into AML. MDS is diagnosed by the clinical presentation and peripheral cytopenia with characteristic bone marrow findings. It is further classified as refractory anemia, refractory anemia with ringed sideroblasts, refractory anemia with excess blasts, chronic myelomonocytic leukemia and refractory anemia with excess blasts in transformation. Specifically, CMML is characterized by splenomegaly, peripheral monocytosis, <5% peripheral blasts and up to 20% blasts in the marrow. Treatment is with chemotherapy used to treat AML but there is a lower rate of complete remission, a shorter duration of remission and a higher rate of relapse. Attempt for cure is with an HLA-matched allogenic bone marrow transplant. Supportive care with transfusions of blood products and hematopoietic hormones such as erythropoietin and G-CSF may be used. This patient underwent surgical correction of his ASD followed by an HLA-matched allogenic BMT donated by his sister and is thus far doing well.
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