Hematology Week 3: Myelodysplastic Syndrome Flashcards
Myelodysplasia Definition
abnormal appearance of myeloid lineage
Myelodysplasia Syndrome is a
- hematological malignancy
- clonal stem cell disorder
- characterized by dysplasia ineffective hematopoiesis
- increased risk of AML (“preleukemia”)
Myelodysplasia Syndrome is characterized by?
- Cytopenias
- ineffective hematopoiesis
- dysplasia
*
Myelodysplasia Syndrome is a clonal stem cell disorder meaning that?
BM is replaced by clonal cells
Progeny of an abnormal clonal multipotent stem cell
- cytogenetic abnormalities
- gene mutations
- epigenetic changes
retains ability to differentiate
Myelodysplasia Syndrome Pathogenesis
- a normal stem cell gains some type of insult which can cause increased apoptosis cells don’t survive as well as usual
- cells accrue more genetic alterations that can lead to increased cell survival and reduced apoptosis
- leads to AML
Myelodysplasia Syndrome Clinical Presentations
3 listed
- Cytopenias
- Dysplasia
- Ineffective Hematopoiesis
Dx of Myelodysplasia Syndrome
4 listed
- Cytopenias
- Evidence of dysplasia
- Careful assessment of blasts <20%
- Exclude causes of secondary dysplasia (vitamin deficiency, HIV+, etc..)
Patients with Myelodysplasia Syndrome have increased risk for?
3 listed
10-40% will progress to AML
Epidemiology of MDS
more common in med except for del(5q) subtype in females
Erythroid Dysplasia in Myelodysplasia Syndrome
2 listed
- Erythroid multinucleation
- Ring sideroblasts
Neutrophil Dysplasia in Myelodysplasia Syndrome
2 listed
- Hypolobation (2 nuclei pseudo-paltrow hewwit cells)
- Hypogranulation
Platelet Dysplasia in Myelodysplasia Syndrome
4 listed
- Giant platelets
- hypogranular platelets
- Hypolobated megakaryocytes
- Multinucleated megakaryocyte
Identify Dysplasia
Erythroid Multinucleation
Identify Dysplasia
Ring sideroblasts
Identify Dysplasia
Neutrophil hypolobation
Pseudo-Pelger-Huet cells
Identify Dysplasia
Neutrophil hypogranulation
Identify Dysplasia
abnormal granules and blasts
Identify Dysplasia
Giant Platelets
Identify Dysplasia
Hypogranular platelets
Identify Dysplasia
Hypolobated Megakaryocytes
Identify Dysplasia
Multinucleated Megakaryocytes
AKA Pon ball megakaryocyte
List of dysplasias in Myelodysplasia Syndrome
8 listed
- Erythroid multinucleation
- Erythroid Ring sideroblasts
- Neutrophil Hypolobation
- Neutrophil Hypogranulation
- Giant Platelets
- Hypogranular platelets
- Hyperlobated Megakaryocytes
- Multinucleate Megakaryocytes
Non-neoplastic Differential Dx of Myelodysplasia Syndrome
7 listed
Vitamin/Nutrient Deficiency
6 listed
denture cream has ______ and can cause ________.
- zinc
- copper deficiency
Infection as a diff dx of myelodysplasia syndrome
Tests for Parvo Virus
2 listed
Parvo virus titers/PCR
Tests for HIV
2 listed
HIV serology/NAT
Tests for Hepatitis
2 listed
- HSV
- CMV
*
Tests for EBV
2 listed
- EBV serology
- NAT (Nucleic acid Testing)
Toxins as a diff dx of myelodysplasia Syndrome
- liver function tests
- coagulation studies
- Lead levels (lead exposure)
The leading cause of macrocytosis in adults
Alcohol use
Neoplastic Differential Dxs of Myelodysplasia Syndrome
7 listed
Myeloproliferative neoplasms
Acute Myeloid Leukemia
Revised International Prognostic Scoring System (IPSS-R)
- Assigns a risk category that correlates with survival and risk of transformation to AML
- most important component by far is the cytogenetic abnormalities
cytogenetic abnormalities that are looked for
5 most common
These are the most common
- del(20q)
- Del(7q)
- Trisomy 8
- i(17q)
- del(5q) - women
Y chromosome as men age
men can lose their Y chromosome as they age doesn’t mean they have a neoplasm
IPSS-R prognostic score values
Treatment of Myelodysplasia Syndrome Low Risk
- Growth Factors
- Supportive Care
Treatment of Myelodysplasia Syndrome High Risk
2 listed
- Induction chemotherapy
- HSCT
- if these treatments can be tolerated as most patients are >70
Supportive care/Growth Factors for Treatment of Myelodysplasia Syndrome
7 listed
- if someone is not very symptomatic and their risk score is low can try to decrease the symptoms
- If having anemia with fatigue may consider transfusing them
- if transfusion dependent but don’t meet criteria for aggressive therapy or cannot tolerate it then consider EPO
- if they are thrombocytopenic and are bleeding or if platelets are very low will give platelet transfusions
- If they have neutropenia then infection control (antibiotics) and GCSF for neutrophil count
Immune Treatments for Treatment of Myelodysplasia Syndrome
Thalidomide/Lenalidomide
Thalidomide/Lenalidomide
Decrease transfusion need
most effective in patients with del(5q)
Pharmacology of thalidomide
Angiogenesis inhibitor and immunomodulator
Chemotherapy in MDS
patients with high-risk disease
Conventional chemotherapy
- Cytarabine (ara-C)
hypomethylating agents
- Azacitidine
- Decitabine
- Decrease cell proliferation
- improve blood counts
Only curative treatment option for MDS
HSCT usually for healthy younger patients and is the only curative option
