Saturday, May 4, 2013

Case study

A 65 year old man came to our clinic with weakness
he was found with mild pancytopenia
the marrow was hypercellular and the cytogenic findings on the bone Marrow suggested del 5q
RAEB-1 was the comment of the pathologist based on blast count
and the rate of Erythrpoietin was below 500.

standard recommendation per the Atlas of Genetics

Treatment ofthis condition in the elderly patient is largely supportive, including blood transfusion in patients with symptomatic anemia. Anemic patients with low serum erythropoietin (EPO) levels may benefit of the administration of rHu-EPO. Low dose cytarabine can be used to reduce the burden of blasts. Myeloablative regimens including anthracyclines and cytarabine in conventional or high doses can be used in high-risk patients under 60 years. Allogeneic bone marrow transplantation may offer a chance of cure in young patients.
EvolutionThis is an oligoblastic leukemia, carrying a 20-40% probability of evolving into leukemia. In a study approximately 25% of the patient developed acute myeloid leukemia (AML) within 18 months.
The probability of RAEB to transform into AML is lower in the RAEB-1 group (approximately 50% of the patients develop acute leukemia within 6 years) than in the RAEB-2 group (approximately 50% at 18 months with overt leukemia).
PrognosisMedian survival of RAEB falls in the 1-2 year range. The best outcome is usually observed in RAEB-1.
Chromosomal abnormalities have independent prognostic significance and are to be included in risk assessment at diagnosis. Favourable cytogenetic features are normal karyotype, 5q- or 20q- isolated; unfavourable features are complex karyotype (i.e. 3 or more clonal anomalies) and abnormalities of chromosome 7q; other abnormalities identify patients in the intermediate cytogenetic-risk group.

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BASED ON ONLY ONE FINDING
5q deletion
most oncologists will propose REVLIMID to this patient
that is the power of Genetic findings!

and indeed if a complex Cytogenetic finding was present and no 5q minus, Azacytidine would have been the choice in the UNITED STATES....
==========so what is 5q minus?

AZACYTIDINE
generally given at 75mg/m2 SC 7 days per months
achieves 7% complete response and 16% partial remission with most response reached after 3 to 4 months of therapy.  It also decreases the AML transformation and improves survival (11 Vs 18 months)
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