Myelodysplasia

Question 1

Definition

Answer 1

Increased number of blasts in the bone marrow, immature blood cells (normal 1-2%, AML > 20%)
Malignant proliferation of multipotent stemcells - retain the capacity to differentiate, but not in an effective manner

Question 2

Criteria WHO ??

Answer 2

Refractory anemia: cytopenia in one or two cell lines
RA with ringed sideroblast
Del (5q) - anemia, blasts < 5%, normal/elevated platelet count

Question 3

Myelodysplastic syndromes cause

Answer 3

Aquired clonal blood disorders

• ineffective hematopoiesis with apoptosis of myeloid precursors
• ineffective hematopoiesis: pancytopenia

Primary: most cases unknown
Secondary: Chemo or radiation therapy

Question 4

Symptoms

Answer 4

Asymtomatic - anemia, thrombocytopenia, neutropenia

Infections, pallor, fatigue, bleeding, palpitation. glossitis, koilonychia,

Question 5

Dx

Answer 5

Bone marrow biopsy, typically shows dysplastic marrow cells with blasts or ringe sideroblasts

Sideroblasts (sidero- + -blast) are nucleated erythroblasts (precursors to mature red blood cells) with granules of iron accumulated in the mitochondria surrounding the nucleus - cannot incorporate the iron into the heme

Cytogenic studies

CBC
Peripheral smear
BM aspiration

Question 6

Tx

Answer 6

Mainly supportive 
RBC and platelet transfusion: anemia and bleeding
EPO: RBC
G-CSF: neutropenia 
5q- = Lenalidomide