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A 59-year-old otherwise healthy male sought medical evaluation because of three
months of fatigue. Physical
examination was unremarkable. A
CBC showed a WBC of 5,600 with normal differential, a platelet count was
150,000 and a hemoglobin 8.9. The erythrocyte MCV was 106.
Iron, B12 and folate studies were normal.
An erythropoietin level was reported at 500 units (markedly
elevated).
The bone marrow
was hypercellular for age, M:E ratio 3:1, with mild reticulin fibrosis.
The erythroid series was left shifted with dyserythropoiesis.
The myeloid series was slightly left shifted with pseudo Pelger-Huet
abnormalities present. Megakaryocytes
appeared normal in morphology and number.
Myeloblast count was estimated at 7%. Iron stores were increased; there was no
increase in ring sideroblasts. Flow
cytometry showed a CD34+ blast population of 9% and maturing myeloid cells with
aberrant expression of CD33, HLA-DR and CD56. Cytogenetic analysis showed the
following karyotype: 47, XY, +8,
20q-, -Y [11]; 46, XY [9].
The
patient has two healthy siblings, 56 and 62 years of age.
HLA typing is being arranged.
What
would you recommend?
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