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_MS2 HemeOnc > 8/13- MDS/AML > Flashcards

8/13- MDS/AML Flashcards

(51 cards)

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1
Q

What is MDS?

A

Myelodysplastic Syndrome

  • Aka “refractory anemia”, “preleukemia”, or “smoldering leukemia”

Spectrum of clonal myeloid disorders characterized by:

  • Ineffective hematopoiesis
  • Cytopenias
  • Qualitative disorders of blood cells
  • Clonal chromosomal abnormalities
  • Variable tendency to evolve into acute leukemia
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2
Q

Can divide/organize MDS how? (dkfjadj)

A
  • “Clonal bicytopenia or tricytopenias”
  • “Oligoblastic leukemia”
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3
Q

Epidemiology of MDS?

A

Most common clonal hematologic disorder

  • 1-10/100,000 /year in Western countries
  • 3,000-12,000 cases/year in US

Disease of the elderly – incidence increases logarithmically after the age of 20

Men affected more than women (1.5x)

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4
Q

Pathogenesis of MDS?

A

Clonal expansion of a multipotential hematopoietic cell

Chromosomal deletions or additions involving most frequently:

  • Long arms (q) of chromsomes 5 and 7
  • Del of 5 and 7
  • Del of 11, 12, 13, 20
  • Trisomy 8

Major mechanism is ineffective hematopoiesis: defective maturation and death of marrow precursor cells.

  • Decreased production/sensitivity to hematopoietic growth factors
  • Increased production of hematopoietic inhibitors (TNFa, Ifng) inducing apoptosis
  • T-cell mediated immune dysregulation
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5
Q

Risk factors for MDS?

A

Radiation Drugs and toxins

  • benzene and other solvents
  • alkylating agents (-5, -7)
  • topoisomerase II inhibitors (11q23)
  • smoking
  • hair dyes
  • pesticides, herbicides, organic chemicals

Other hematologic disorders

  • aplastic anemia, paroxysmal nocturnal hemoglobinuria, congenital neutropenia

Genetic disorders

  • Down syndrome Fanconi’s anemia,
  • Neurofibromatosis (von Recklinghausen’s disease)
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6
Q

Clinical Manifestations of MDS?

A
  • Anemia: weakness, fatigue, palpitations, orthostasis
  • Thrombocytopenia: bleeding, bruising
  • Leukopenia: increased susceptibility to infection
  • Other: Sweet disease, polyarthritis, neuropathy, acquired alpha thalassemia, splenomegaly
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7
Q

DDx of MDS?

A
  • Drugs or toxic exposures
  • Infections
  • Rheumatic disorders
  • Deficiencies of vitamins B12, B6, or folic acid
  • Hypersplenism
  • Metastatic cancer
  • Acute leukemia
  • Aplastic anemia
  • Paroxysmal nocturnal hemoglobinuria
  • Myelofibrosis
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8
Q

What are features of the PBS in MDS?

A

Red cells:

  • Anisocytosis
  • Normocytic or mildly macrocytic indices
  • Low reticulocyte count
  • Howell-Jolly bodies, Cabot rings, basophilic stippling

White cells:

  • Small, poorly granulated neutrophils with hyper- or hypolobulated nuclei
  • Increased monocytes

Platelets:

  • Large, agranular
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9
Q

What is shown here?

A

Pseudo Pelger-Huet cell

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10
Q

What is shown here?

A

Stodtmeister cell

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11
Q

What is shown here?

A

A: Basophilic stippling

B: Howell-Jolly bodies

C: Cabot’s rings

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12
Q

Overview of bone marrow in MDS?

A
  • Erythroid Dysplasia
  • Myeloid Dysplasia
  • Megakaryocytic Dysplasia
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13
Q

What is shown here?

A

MDS

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14
Q

What is shown here? Condition/features? Prognosis?

A

Refractory Anemia

  • Dimorphic population
  • Less than 5% blasts in marrow
  • AML in 10% at 2 yrs
  • Median survival 3-6 yrs
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15
Q

What is shown here? Conditions/features? Prognosis?

A

Refractory Anemia with Ringed Sideroblsats

  • > 15% ringed sideroblasts
  • Less than 5% blasts in marrow
  • AML in 0% at 2 yrs
  • Median survival: 3-6 yrs
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16
Q

MDS is associated with what genetically?

A

Isolated del(5q)

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17
Q

Epidemiology of MDS?

A
  • Occurs primarily in middle-aged women
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18
Q

Characteristics of MDS?

A
  • Characterized by macrocytic anemia and normal or increased platelet count
  • Megakaryocytes with hypolobulated nuclei
  • Favorable clinical course with absence of leukemic transformation
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19
Q

International Prognostic Index (IPI) for MDS?

A
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20
Q

Standard treatment of MDS?

A

Supportive care:

  • Erythropoietin, G-CSF, GM-CSF, IL-11
  • Blood transfusion
  • Deferoxamine or Exjade (iron chelator)
  • Pyridoxine, folic acid
  • Danazol Immunomodulation
  • Antithymocyte globulin
  • Cyclosporine
  • Steroids

AML-style chemotherapy

Newer agents affecting DNA methylation

Bone marrow transplantation

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21
Q

What are 5-zacytidine (Vdiaza) and Decitabine (Dacogen)?

Response?

Side effects?

Study These Flashcards
A
  • Vidaza is prodrug for Decitabine, which reduces DNA methylation at cytosine residues and allows reactivation of silenced tumor-suppressor genes (demethylating agents)
  • Overall response rate 16-17% (CR 7%, PR 16%, improvement in 37%)
  • Significant side effects including cytopenias, GI disturbances, fever, fatigue and various aches and pains
22
Q

Organization/Division of the Leukemias?

23
Q

Acute leukemias are a group of disorders characterized how?

Study These Flashcards
A
  • Neoplastic transformation of hematopoietic stem cell (Clonality)
  • Malignant cells either do not differentiate or differentiate abnormally
  • Abnormal cells accumulate in blood, marrow and tissues
  • Normal hematopoiesis is inhibited
  • Fatal if untreated
24
Q

Relative frequency of leukemias?

Study These Flashcards
A

CLL: 32%

AML: 30%

ALL: 13%

Other: 13%

CML: 125

25
***_TEST TEST TEST_*** What is shown here?
**AML**! Acute Myelogenous Leukemia BE ABLE TO RECOGNIZE THIS - Can see Auer rods!
26
Epidemiology of AML? Percentage of acute leukemias in adults/children?
- 15-20% of acute leukemia in children - 80% of acute leukemia in adults - 12,000 new cases/yr in the US - 8,000 deaths/yr in US - Median age is 63 (Disease of the elderly!)
27
Risk factors for AML?
_Genetics:_ - Chromsomal abnormalities: Down's, Klinefelters - Chromsomal instability: Fanconi's anemia, Bloom's _Prior chemotherapy:_ - Alkylating agents: Cyclophosphamide, Mechlorethamine - Topoisomerase II inhibitors: Etoposide, Anthacyclines _Radiation exposure:_ Hiroshema survivors, Radiologists (before modern shielding) _Environmental exposure_: benzene _Prior hematologic abnormalities_: Myelodysplasia, MPNs
28
What is shown here?
29
WHO/FAB Classification of AML?
**AML with genetic abnormalities** - t(8;21) or inv (16) - 11q23 abnormalities - APL with t(15;17) AML with multilineage dysplasia - Evolution from MDS - No MDS, but dysplasia in 2 or more cell lines AML secondary to therapy - Radiation/alkylating agent - Topoisomerase II inhibitor AML NOS (FAB classification)
30
FAB Classification of AML?
31
M2 (AML with maturation) is associated with what translocation?
t(8:21)
32
M3 (Acute promyelocytic leukemia) is associated with what translocation?
t(15:17)
33
M4 (Acute myelomonocytic leukemia) is associated with what translocation?
inv 16
34
Clinical Presentation of AML?
**Cytopenias:** - Anemia (fatigue) - Leukopenia (infection/fever) - Thrombocytopenia (bleeding) **Hyperleukocytosis:** increased risk of CNS involvement - Leukostasis (blast count \> 50-100K, limited flexibility of blasts, lungs/brain most affected) **Extramedullary disease**: - Gums with M4/M5 - Chloromas with M2 - CNS Coagulopathy - DIC, especially with M3 (also M4 and M5)
35
Petechiae are usually indicative of what abnormality?
Low platelet count
36
What is shown here?
Hyperleukocytosis in pt with M4 - HUGE buffy coat!
37
Which form of AML is associated with gum infiltration?
M5
38
Which form of AML is associated with M3?
DIC Ex) - Platelets 5,000 - Fibrinogen 25
39
CNS hemorrhage may occur in pt with what form of AML?
**M3** (from DIC) - Most feared complication (bleed before you can treat the leukemia)
40
CNS leukemia may cause what nerve complications?
CN VII palsy (Bell's palsy)
41
Diagnosis of AML?
_Bone marrow exam_ - Wright-Giemsa stain - Sudan/special stains - Flow cytometry (aspirate) - Cytogenetics (aspirate) - Molecular rearrangements
42
Cytogenetics and prognosis in AML?
**Good prognosis:** - t(8:21) - inv(16) - t(15:17) **Intermediate:** - Normal **Poor:** - del 5/5q or 7/7q - Abnormalities of 11q23 - Multiple abnormalities
43
What is the most common subtype of AML? - Genetics? - Prognosis?
**M2 AML** (AML with maturation) - 50% have t(8:21) - Better prognosis than average
44
What demographic groups are most commonly affected by M3 AML? - Genetics? - Prognosis? - Treatment?
**M3 AML** (Acute Promyelocytic Leukemia/APL) - Seen in younger Hispanic pts - May see Auer rods or big chunky granules in PBS (emergency!) 80-100% have t(15:17) **Risk of DIC:** **bleeding, thrombosis** **Unique treatment:** all-trans-retinoic acid
45
What is this?
M2 AML, t(8:21)
46
What is this?
**M3 AML** - May see Auer rods or big chunky granules (emergency!)
47
What is shown here?
Abnormal cell with **t(15:17)** on **FISH** - Indicative of **M3 AML**
48
Treatment of AML? Complications? Maintenance therapy?
**Induction chemotherapy:** - Anthracycline plus - Cytarabine **Complications:** - Prolonged neutropenia - infection - Prolonged thrombocytopenia **Consolidation chemotherapy** **No maintenance except M3** **Bone marrow transplantation**
49
Treatment of APL (M3 AML)?
**ATRA (all transretinoic acid) + Chemotherapy** or **ATRA + Arsenic** (ATRA can put pts into remission, but doesn't keep them there; not enough)
50
Prognosis of AML for children? Adults?
_Children:_ - 90% complete remission - 40% long-term cure rate _Adults:_ - 65-70% complete remission - 30% long-term cure
51
Adverse prognostic factors in AML?
- Age \> 60 yo - Poor risk cytogenetics - Antecedent hematologic disorder - WBC \> 100K - Presence of multidrug resistance gene 1 (MDR) - Failure to achieve complete remission with first cycle

_MS2 HemeOnc (33 decks)

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