BOF: Malignant (Myelodysplastic Syndrome)
- Aug 9, 2016
- 1 min read
A 67 year old woman is seen in the haematology follow up clinic with the following investigation results. She is well, with no previous medical history of note and asymptomatic. Hb:10 g/dL, MCV 102fl, WCC 2.4 x10⁹/L, N 0.9 x10⁹/L, MC 1.0, Plts 97 x10⁹/L, Ferritin 170, B12 & Folate normal, Erythropoietin 310u/L Film: Some neutrophils appear hypogranular, Bone marrow aspirate: Dyserythropoiesis with 23% ringed sideroblasts. Myeloblasts <5%, Cytogenetics: Normal karyotype by G banding. She has required 3 units of blood transfused over the past 10 weeks. Which ONE of the following is the best subsequent treatment plan?
a) Transfusion support alone
b) Iron chelation is combination with transfusion
c) Recombinant humon erythropoietin (Epo)
d) Recombinant human erythropoietin (Epo) with Granulocyte Colony Stimulating Factor (GCSF)
e) Lenalidomide
Answer:
d) Recombinant human erythropoietin (Epo) with Granulocyte Colony Stimulating Factor (GCSF)
Explanation:
This is a low risk myelodysplastic syndrome. In patients with low risk, low endogeneous Epo (<500iu/l) with minimal transfusion requirements, good responses can be seen with recombinant Epo, and GCSF may improve responses from 20-30% to 40-60%. The relative impact of RARS over RA on outcome and recombinant growth factor responsivness remains contentious. In 5q- associated myelodysplastic syndrome, lenalidomide shows excellent responses and transfusion independence in around half of patients but carries a risk of early worsening neutropenia and a small increased risk of venous thrombosis.
References:
Bejar R, Steensma DP. Recent developments in myelodysplastic syndromes. Blood 2014;124(18):2793-2803.
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