Aashi
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A 55-year-old man with no significant history comes to the office complaining of fatigue and abdominal fullness for a month. He claims that over the past year, he has been admitted to the hospital five times for bacterial pneumonia. The physical examination is remarkable for a massively enlarged spleen and liver. There are no palpable lymph nodes. The remainder of the examination is unremarkable. Laboratory studies show: WBC 1,100/mm3, hemoglobin 8.5 mg/dL, hematocrit 25%, platelets 34,000/mm3 (neutrophils 40%, lymphocytes 58%, monocytes 0%, eosinophils 2%). A bone-marrow aspirate was attempted but was unsuccessful. What is the best treatment for this patient?
(A) Interferon
(B) Hydroxyurea
(C) Fludarabine
(D) Cladribine
(E) Cyclophosphamide, vincristine, and prednisone
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| prathapdoctor
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massive hepatosplenomegaly with absent of palplable lymphnodes,unsucceful bone marrow biopsy is suggestive of hairy cell leukemia which can cause panycytopenia also,so i will go with Cladribine.
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| robin082006
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How can we rule out bone marrow fibrosis in this case?
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| prathapdoctor
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the point that is not in favour of marrow fibrosis is relative lymphocytosis,do we see lymphoctyosis in myelofibrosis.i think we just see pancytopenia with myelopthsic anemia and tear drop cells.
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| fox
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I dont think the lymphocyte %ge is significantly high....Myelofibrosis seems likely. Anyway what is the Rx for myelofibrosis?
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| Aashi
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Dr pratap, u hit the bulls eye , it is indeed hairy cell leukemia..and the treatment is CLIDARABINE..
GL
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| nida
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Good Q
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| fox
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good going prathap doc
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| nadiabarati
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oh my God! Do we ned to know this much?!!! treatment for hairy cell leukemia?! Where did you study it from?!
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| dev
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myelofibrosis has no treatment as such. there are experimental protocols in which I am workin
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| webjeee
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hairy cell leukemia
How can you tell?
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| neuroblastoma
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hairy cell leukemia-- bone-marrow aspirate was attempted but was unsuccessful
DRY BONE MARROW TAP+ LYMPHOCYTOSIS ( with HAIRy projections
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| Justice
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"Dry" aspirate
Hairy cell leukemia
(D) Cladribine
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| sachida
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"dry" aspirate is not just hairy cell leukemia.
MDS, Myelofibrosis secodary to p.vera,leukemi can also cause dry taps.
In fact, on this question only thing against making diagnosis of mds is fact that there is significant organomegaly.
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| sachida
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MDS secondary to CLL can have splenomegaly too.
however, none of the answer choice are drug of choice in treating MDS. Commonly used drug at present are hypomethylating agents (VIdaza, Decogen) and Revlamid (when patient cytogenetics show 5q deletion)- I dont think board will test for 5q deletion but I just learned about it so wanted to share.
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| starcraftbw
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very good case.....
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