phuluong2k
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A 25-year-old man of Eastern European Jewish descent is being evaluated for a right hip fracture and splenomegaly. He reports recurrent acute episodes of bone pains for the past 5 years. Complete blood count shows: Erythrocyte count..............2,000,000/mm3
Leukocyte count................3,300/mm3
Platelet count....................70,000/mm3
X-ray films demonstrate multiple osteolytic lesions in the vertebral column and femurs. A bone marrow aspirate reveals clusters of histiocytes showing fibrillary cytoplasm with a typical "crumpled tissue paper" appearance. The fibrillary material in the cytoplasm is PAS-positive. Which of the following is the most effective treatment available for this condition?
A. Administration of pyridoxine and folate
B. Administration of hematin
C. Allogeneic bone marrow transplantation
D. Cytotoxic drugs such as hydroxyurea
E. High-carbohydrate diet
F. Replacement therapy with alglucerase
G. Splenectomy
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| ARJ
Forum Guru
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Posts: 792
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I am sure it is Gaucher disease
So probably
Answer F. Replacement therapy with alglucerase
If its correct then
.....Recently I came to know the treatment.......Cannot remember the Source...Can Somebody help
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| coppisv
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if its gaucher and i m sure it is..they asked for effective treatment..wont it be bone transplant
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| tolito
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F.
bone marrow transplant has been replaced by enzyme replacement therapy cos of the morbidity and mortality from transplant ( lack of perfect donors)
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