Myelodysplastic Neoplasms (MDN) Flashcards
What are myelodysplastic neoplasms & what are their general features?
- acquired clonal stem cell disorder
- peripheral blood cytpenias
- dyspoiesis in one or more lineage
- bone marrow hypercelularity (ineffective hematopoiesis)
- normal or increased marrow blasts (<20%)
- organomegaly is uncommon
What type of dyspoiesis is seen in the provided image?
hyposegmented & hypogranular neutrophil
dysgranulopoiesis
What type of dyspoiesis is shown in the provided image?
hypogranular band
dysgranulopoiesis
What type of dyspoiesis is seen in the provided image?
large, hypersegmented neutrophil
dysgranulopoiesis
What type of dyspoiesis is seen in the provided image?
dyserythropoiesis
multinucelated erythroid precursors with nuclear/cytoplasmic asynchrony & irregular nuclear contours
What type of dyspoiesis is indicated by the yellow arrows?
hypogranular platelets
(may be large, may be small, may be regular size)
What type of dyspoiesis is seen int he provided images?
dysmegakaryopoiesis
- Possible phenotypes
- increased/decreased numbers
- clusters
- odd numbered nuclei
- small size
- widely-spaced nuclei
What type of dyspoiesis is seen in the provided image?
Auer rods
(rod-shaped coalescent primary granules)
What are Auer Rods? They are seen in what conditions?
rod-shaped structures composed of fused lysosomes/primary neutrophilic granules
contain myeloperoxidase, lysosomal enzymes & crystalline inclusions
seen only in RAEB-2 (pre-leukemia) & some acute myeloid leukemias
What is karyorrhexis?
fragmentation of the nucleus of a dying cell
What is the general clinical picture seen with myelodysplastic neoplasms?
most commonly older adults (can be any age)
M > F
Symptoms are related to cytopenias
most patients are anemic & transfusion-dependent
What would you expect to seen in a peripheral blood smear from a patient with Refractory cytopenia with unilineage dysplasia (RCUD)?
cytopenia in one or two myeloid lineage
unilineage dysplasia
no or rare blasts
What would you expect to seen in the bone marrow sample from a patient with Refractory cytopenia with unilineage dysplasia (RCUD)?
unilineage dysplasia
<5% blasts
<15% ring sideroblasts
What would you expect to seen in a peripheral blood smear from a patient with Refractory anemia with Ringed Sideroblast (RARS)?
anemia
often dimorphic pattern (2 populations RBC)
no blasts
What would you expect to seen in a bone marrow sample from a patient with Refractory anemia with Ring Sideroblast (RARS)?
>15% of erythroid precursors are ring sideroblasts
unilineage dysplasia (erythroid only)
<5% blasts
increased iron in bone marrow
What is the genetic cause of MDS Associated with Isolated del(5q)?
isolated deletion of bands 31-33 of long arm of chromosome 5
What is the general clinical picture of an individual with MDS Associated with Isolated del(5q)?
elderly woman
relatively good prognosis for MDS
What would you expect to seen in a peripheral blood smear from a patient with MDS Associated with Isolated del(5q)?
macrocytic or normocytic anemia - sever & transfusion dependent
normal or increased platelet count (platelets may be large)
no/rare blasts <1%
No Auer rods
What would you expect to seen in a bone marrow sample from a patient with MDS Associated with Isolated del(5q)?
normal/increased megakaryocytes with hypolobulated nuclei
<5% blasts
no Auer rods
What would you expect to seen in a peripheral blood smear from a patient with Refractory Cytopenia with Multilineage Dysplasia (RCMD)?
cytopenia
multilineage dysplasia
no/rare blasts <1%
No Auer rods
no monocytosis
What would you expect to seen in a bone marrow sample from a patient with Refractory Anemia with Excess Blasts-1 (RAEB-1)?
unilineage/multilineage dysplasia
5-9% blasts
No Auer rods
What would you expect to seen in a bone marrow sample from a patient with Refractory Anemia with Excess Blasts-2 (RAEB-2)?
unilineage or multilineage dysplasia
10-19%
+/- Auer rods
myeloid maturation is arrested
Identify which of the provided bone marrow samples came from a patient with RAEB-1 & RAEB-2.
