Myelodysplastic Syndromes-Usera Flashcards
What is this:
a group of clonal hemtopoietic stem cell diseases characterized by one or more peripheral blood cytopenia(s), with dysplasia in one or more myeloid lines.
Myelodysplastic syndromes
With myelodysplastic syndromes there is an increased, but not inevitable, risk for developing (blank)
acute leukemia
Who typically gets myelodysplastic syndromes?
older adults
around 70
male predominance
What does MDS (myelodysplastic syndromes) look like?
blood- cytopenia
marrow- cellular
maturation- variable
What does MPD (myeloproliferative disorder) look like?
blood-cythemia
marro-cellular
maturation- yes
What does Acute leukemia look like?
blood-variable
marrow-cellular
maturation-no
How do you classify myeodysplastic syndrome?
cytopenia
degree of dysplasia in peripheral blood and bone marrow
blast count
What are the IPSS thresholds for cytopenias?
hemoglobin less than 10g/Dl
ANC less than 1.8 X 10^9 /L
platelets less than 100 X 10^9/L
What are four types of myelodysplastic syndromes?
Refractory Anemia (RA)
Refractory Anemia with Ringed Sideroblasts (RARS)
Refractory Anemia with Excess Blasts (RAEB-1)
Refractory Anemia with Excess Blasts (RAEB-2)
In the myelodysplastic syndrom Refractory Anemia, what do you see in the peripheral blood?
anemia
no blasts
In the myelodysplastic syndrome Refractory Anemia, what do you see in the bone marrow?
Dysplastic erythroids
less than 5% blasts
less then 15% ringed sideroblasts
In the myelodysplastic syndrome Refractory anemia with ringed sideroblasts (RARS), what do you see in the peripheral blood?
anemia
dimorphic red cell pop
pappenhemier bodies
no blasts
In the myelodysplastic syndrome Refractory anemia with ringed sideroblasts (RARS), what do you see in the bone marrow?
dysplastic erythroids
less than 5% blasts
GREATER than 15% ringed sideroblasts
In the myelodysplastic syndrome Refractory anemia with excess blasts (RAEB), what do you see in the peripheral blood?
Bi-or pancytopenia
In RAEB-1=lless than 5% blasts, NO AUER RODS
In RAEB-2=5-19% blasts or Auer rods
In the myelodysplastic syndrome Refractory anemia with excess blasts (RAEB), what do you see in the bone marrow?
5-9% blasts, no auer rods RAEB-1
10-19% blasts or auer rods RAEB-2
Dysplasia
In the myelodysplastic syndrome MDS with del (5q)- 5q syndrome, what do you see in the peripheral blood?
anemia, normal to increased platelets
less than 5% blasts
no auer rods
In the myelodysplastic syndrome MDS with del (5q)- 5q syndrome, what do you see in the bone marrow?
Hypoblated megakaryocytes
less than 5% blasts
No auer rods
Isolated 5q-karotype
WHy do you get myelodysplastic syndromes?
abnormal stem cell clone proliferates
Clonal cytogenic abnormalities are present in (Blank) percent of MDS cases
50
What are the initiating stimuli of myelodysplastic syndrome?
unknwon but involve genetic damage
Primary or de novo MDS occur without known history of chemotherapy or radiation. What are some possible etiologies?
benzene exposure
cigarettes
family Hx of heme neoplasms
SOme heritble conditions that are associated with increased risk for MDS are (Blank X 3)
Fanconi’s anemia
Schwachman-Diamond syndrome
Diamond-Blackfan syndrome
What the clinical findings of myelodysplastic syndromes?
fatigue and weakness palor ecchymoses hemorrhage infection
What are the lab findings of the erythrocytes in myelodysplastic syndromes?
Anemia (HgB less than 10) macroscytosis (MCV greater than 110) oval macrocytes dimorphic anemia in RARS Reticulocytopenia Anisocytosis, poikilocytosis Basophilic stippling Howell-jolly bodies nucleated RBC membrane abnormalities
(blank) is a medical term meaning that a patient’s red blood cells are of unequal size. This is commonly found in anemia and other blood conditions.
Anisocytosis
(blank) refers to the presence of poikilocytes in the blood. Poikilocytes are abnormally shaped red blood cells as seen on a blood film in humans and many wild and domestic species of animals, though they are common in some clinically normal small ruminants, particularly goats.
Poikilocytosis
(blank) aka Punctate basophilia refers to an observation found when observing a blood smear in which erythrocytes display small dots at the periphery. These dots are the visualization of ribosomes and can often be found in the peripheral blood smear, even in some normal individuals.
Basophilic stippling
(blank) are histopathological findings of basophilic nuclear remnants (clusters of DNA) in circulating erythrocytes. During maturation in the bone marrow late erythroblasts normally expel their nuclei, but in some cases a small portion of DNA remains
Howell–Jolly bodies
What are the lab findings of the granulocytes in myelodysplastic syndromes?
Leukopenia Neutropenia left-shift sometimes present agranular/hypogranular neutrophils abnormal granules pseudo-pelgeroid nuclei (nonsegmented dense looking nuclei) hypersegmentation ring nuclei
What are the lab findings of the platelets in myelodysplastic syndromes?
Thrombocytopenia Thrombocytosis Giant platelets Hypogranular platelets Abnormal granulation Megakaryoblasts
What are the lab findings of the bone marrow in myelodysplastic syndromes?
variable cellular amounts
<20% myeloblasts
Dysplastic features in all cell lines common
Abnormal localization of immature precursors
Ringed sideroblasts
What are these lab findings of MDS with bone marrow dyserythropoiesis?
Megaloblastoid erythropoiesis Giant erythroid precursors Multinucleation Nuclear fragmentation Abnormal nuclear shape Nuclear budding Karyorrhexis Irregular nuclear or cytoplasmic staining Vacuolization Basophilic stippling Ringed sideroblasts
(blank) is the destructive fragmentation of the nucleus of a dying cell whereby its chromatin is distributed irregularly throughout the cytoplasm
Karyorrhexis
What are these lab findings of MDS with bone marrow dysgranulopoiesis?
Abnormal granulation Hypogranularity Agranularity Abnormal size granules Absent secondary granules Hyposegmentation Hypersegmentation
What are these lab findings of MDS with bone marrow dysmegakaryopoiesis?
Increased megakaryocytes Normal megakaryocytes Decreased megakaryocytes Micromegakaryocytes Hyperchromatic nuclear staining Nuclear hyposegmentation Decreased granularity Giant platelets
What will you find in the peripheral blood of myelodysplastic Refractory anemia?
Anemia
Macrocytosis (MCV <1% of nucleated cells
What will you find in the bone marrow of Refractory Anemia?
Hypercellular Erythroid hyperplasia Dyserythropoiesis Megaloblastoid changes Ringed sideroblasts <5% of cellularity
What will you find in the peripheral blood of myelodysplastic RA with ringed sideroblasts?
Anemia
Macrocytosis (MCV <1% of nucleated cells
What will you find in the bone marrow of myelodysplastic RA with ringed sideroblasts?
Hypercellular Erythroid hyperplasia Dyserythropoiesis Megaloblastoid changes Ringed sideroblasts >15% Blasts <5% of cellularity
IF you see sideroblastic anemia dont just to AML because only (blank) percent involve AML
1-2%
What will you find in the peripheral blood of myelodysplastic RAEB-1?
Cytopenia in two cell lines Normo- or macrocytic anemia Oval macrocytes Reticulocytopenia Dysgranulopoiesis Monocytes <5% of nucleated cells
What will you find in the bone marrow of myleodysplastic RAEB-1?
Hypercellular Dyserythropoiesis Dysgranulopoiesis Dysmegakaryopoiesis Ringed sideroblasts present Blasts 5-9% of cellularity Prognosis correlates with blast percentage
What will you find in the peripheral blood of myelodysplastic RAEB-2?
Cytopenia in two cell lines Normo- or macrocytic anemia Oval macrocytes Reticulocytopenia Dysgranulopoiesis Monocytes 5% of nucleated cells
What will you find in the bone marrow of myleodysplastic RAEB-2?
Hypercellular Dyserythropoiesis Dysgranulopoiesis Dysmegakaryopoiesis Auer rods may be present Blasts 10-19% of cellularity Higher risk of leukemia
What are myelodysplastic syndrome varients?
dont fit into subtype, trilineage dysplasia with less than 5% blasts in marrow, survival is poor with worsening dysplasia
What is the hierarchical approach to classification of myelodysplastic syndrome varients?
AML
RAEB-2
RAEB-1
RA
What is therapy related MDS?
- occurs following chemotherapy or radiation therapy for another disease
- related to duration, amount, an courses of therapy
- difficult to classify as specific MDS
- unfavorable prognosis with high risk leukemia
What are the cytogenetics of myelodysplastic syndromes?
Frequently involve chromosomes 5 and 7
Isolated 5q- syndrome is a specific Dx associated with good prognosis
Complex karyotypes (>3 abnormalities) are associatd with worse prognosis
Detection of chromosomal abnormalities in myelodysplastic syndromes improves with increasing (blank)
blast count
What is the prognosis of myelodysplastic syndromes?
Median survival worsens with increasing blast percentage and trilineage dysplasia
Median survival (all types) is < 2 years
Leukemic transformation 10-40%
Scoring systems aid in prognosis
What are the therapies for myelodysplatic syndromes?
Supportive care RBC transfusion for anemia Platelets for bleeding prophylaxis Antibiotics for infections, neutropenia Growth factors (GM-CSF, EPO) Chemotherapy for poorer prognosis Bone marrow transplantation
Chemotherapy with the hypomethylating agents (blank) and (blank) has been shown to decrease blood transfuion requirements and to retard the progression of MDS to AML
5-azacytidine and decitabine
