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Z - M2 Heme/Onc > Heme/Onc - Week 2 Review - Part 1 > Flashcards

Heme/Onc - Week 2 Review - Part 1 Flashcards

1
Q

Myeloproliferative Disorders - Overall Keys (5)

A

1) Myeloid Equivelent of Chronic Leukemia
2) Late Adulthood
3) High WBC with Hypercellular Marrow
4) Increased Risk for hyperucricemia + gout (high RBC turnover)
5) Progression to either marrow fibrosis or acute leukemia

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2
Q

Major Classes of Myeloproliferative Disorders (4)

A

1) CML
2) Polycythemia Vera (PV)
3) Essential Thrombocythemia (ET)
4) Myelofibrosis

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3
Q

Chronic Myeloid Leukemia (CML) - Genetics + Key Facts (3)

A

Genetics - 9,22 Philadelphia BCR-Abl Translocation

Key Facts

1) Spleenomegaly - Common + indicates risk for AML/ALL Progession
2) Key cells are BASOPHILS + Granulocytes

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4
Q

CML vs. Leukemoid Reaction - 3 Major Keys

A

Leukimoid Reaction = Acute Infection

CML - Negative Leukocyte Alkaline Phosphate (LAP) Stain —— Positive in Leukimid
CML - Increased Basophils (vs. Neutrophils)
CML - Lots of multilobar granulocytes

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5
Q

Polycythemia Vera - Definition + Cause + Treatment

A

Description - Neoplastic Disease of Myeloid RBCs (to many RBCs)

Genetics JAX2/STAT5 Mutation

Treatment - Phlebotomy to reduce viscosity

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6
Q

Polycythemia Vera - S/Sx

A

1) Blurry Vision
2) Venous Thrombosis
3) Flushed Fash
4) Itching After Bathing

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7
Q

Reactive Polycythemia vs. Polycythemia Vera - 2 Keys

A

Reactive Polycythemia - Response to Hypoxia (E.g. High Altitude)

Reactive Polycythemia = HIgh EPO and Low SaO2

Polycythemia Vera = Low/Normal EPO + Normal SaO2

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8
Q

Essentila Thrombocythemia (ET) - Definition + Genetics + Key Distinguishing Features (3)

A

Neoplastic Proliferation of platelets

Genetics - JAK2/STAT5 Mutation

Keys

1) Symptoms due to bleeding and clotting
2) Diagnosis of exclusion for platelets > 450,000
3) Giant + Dysfunctional Clonal Platelets (vs. reactive)

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9
Q

Diseases with Target Cells (4)

A

1`) Hemoglobin C Disease

2) Asplenia
3) Liver Disease
4) Thalasemia

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10
Q

Myelofibrosis - Definition + Genetics + S/Sx (3)

A

Neoplastic proliferation of Megakaryocytes

Genetics - JAK2 Kinase - Excess PDGF from the Megakaryocytes triggers marrow fibrosis

S/Sx

1) Splenomegally (extramedulary hematopoesis)
2) Increased Bleeding/Thombosis
3) Increased Infection (Spleen can’t keep up)

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11
Q

Myelofibrosis - Histology (3)

A

Histology - Due to Splenic RBC Production

1) Tear Drop Dacrocytes (from pinches/sheers on early realease)
2) Nucleated RBCs
3) Immature Cells

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12
Q

Normal Bone Marrow - Key Feature

A

1) Normal Cellularity of Vertebrae never drops below 50% - Femur etc. drop off

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13
Q

Aplastic Anemia - Definition + Key Features (3)

A

Marked reduction of all three marrow cell lines - error at the common myeloid progenitor level

1) Pancytopenia in the peripherial blood
2) Cellularity < 10%
3) Typically lymphoid + plasma elemnts with few progenitors and no MKs

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14
Q

Aplastic Anemia - Key Clinical Features (4)

A

1) Low Hb (Anemia - Fatigue)
2) Thrombocytopenia (Easy Bruising/Racoon Eyes + Bleeding)
3) Leukopenia (Infection)
4) Red/Dark Urine (PNH)

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15
Q

Aplastic Anemia - Causes (5) + Treatment (4)

A

Causes

1) Drugs
2) Radiaiton
3) Viral Infection
4) Chemicals
5) Autoimmune

Treatment

1) Treat Underlying Condition
2) Marrow Transfusion
3) Marrow Stimulation (GM-CSF)
4) Immunosuppresion in T-Cell Idopathic Cases

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16
Q

Aplastic Anemia Causes - Acquired (2) + Chemical (3)

A

Acquired

1) Loss HSC in the Niche (e.g. loss of CXCR4 or 12)
2) Autoimmune destruction of marrow stem cells

Chemical

1) Benzene
2) Pesticides
3) Heavy Metals

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17
Q

Drug Causes of Aplastic Anemia (4) + Viral Causes (5)

A

Drugs

1) Chloramphenicol,
2) Carbamazepine
3) Phenytoin
4) Quinine

Viral
1-2) - Hep B/C
3) HIV
4) Parvovirus B19
5) HHV (IN Immunosuppressed Patients)
18
Q

Paroxysmal Nocturnal Hemoglobinuria (PNH) - Definition + Pathophysiology + Major Complications (2)

A

Definition - Normocytic Anemia with Intravascular Hemolysis

Pathophysiology - Aquired GPI Defeciency - Can’t hold DAF on the outside of RBCs - No protection against compliment - at night respiratory acidosis triggers compliment which lysis the RBCs - Intravascular hemolysis leads to high Hb in the urine

Major Complications - Iron Deficiency + AML Progression

19
Q

Paroxysmal Nocturnal Hemoglobinuria (PNH) - Markers (2) + Genetics (1)

A

Markers - CD55(-) (DAF) and CD59(-) - Missing without GPI

Genetics - PIG-A Gene

20
Q

Parvovirus B19 - Infection + Importance for Heme Onc + Associated Disease (5)

A

Infects progenitor red cells and halts erythropoiesis

Major problem for people with pre-existing marrow stress

Associations

1) Fifths Disease (Health Kids)
2) Arthritis
3) Red Cell Aplasia (Immuno-compromised)
4) Hydrops Fetalis

21
Q

Inherited Bone Failure Syndromes (5)

CLASS

A

1) Dyskeratosis Congenita (DKC)
2) Fanconi Anemia
3) Shwachman-Diamond Syndrome
4) Pure Red Cell Aplasia (PRCA)
5) Diamond-Blackfan Anemia

22
Q

Dyskeratosis Congenita (DKC) - Genetics + S/Sx (2)

A

Inherited Bone Failure Snydrome

X-Linked Recessive Lack of Teomere Protection

S/Sx Cutaneous (Hyperpigmented Macules + Palms/Soles

23
Q

Fanconi Anemia - Genetics + Pathophysiology + Clinical Features (3)

A

Autosomal Ressive Knock out of Fanconi Complrex

No Fanconi = no protection of DNA Repair/Cell Cycle Arrest - Leasds to unchecked repairs

Clinical Features

1) Anemia
2) Cancer
3) Renal Tubule Acidosis

24
Q

Shwachman-Diamond Syndorme - Genetics + S/Sx (3)

A

Mutations in SBDS of Chromosome 7

Exocrine Pancreatic Insufficiency + Skeletal Issues + Marrow Failure

25
Q

Pure Red Cell Aplasia (PRCA) - Pathophysiology + Causes (5)

A

Aplasia of the erythroid cell line only

Aquired Causes

1) Thmoma
2) CLL
3) Parovirus B19
4) Myesthenia Gravis

Congenital = 5) Diamond Blackfan

WILL BE ON EXAM DO NOT GIVE THIS A 5

26
Q

Diamond Blackfan - Genetics + S/Sx (5)

A

1) Ribosomal Protein 19 (Autosomal Dominant)

S/Sx

1) PURE RED CELL APLASIA
2) Tri-pharyngeal Thumb
3) Snub Nose
4) Short Statue
5) Cleft Lip

27
Q

First Major Cancer Causation Link

A

Chimney Sweeps - Scrotum Cancer

28
Q

5 Major Causation Established Occupational Exposures

A

1-2) - Asbestos - Lung Cancer + Mesothelioma

3) Benzene - Leukemia
4) Rubber Worker - Lung Cancer
5) Vinyl Chloride (PVC) - Angiosarcoma of Liver

29
Q

Epidemiological Triangle - 3 Points

A

1) Agent
2) Host
3) Environment

30
Q

Bradford Hill Guidelines - Points 1-4

A

1) Temporality - Exposure Comes First - Only Mandatory One
2) Strength (Magnitude of Association) - Relative Risk/Odds Ratio
3) Dose Response - Linear - Possible for threshold
4) Reversibility - Following Cessation of Exposure - Not common in cancer - once the oncogenesis starts it doesn’t stop

31
Q

Bradford Hill Guidelines - Points 5-8

A

5) Consistent - Multiple trials in multiple locations
6) Biological Plausibility - Proposed Mechanism
7) Specificity of Association - Exposure associated with specific disease
8) Analogy - Analogous to a known causative agent

32
Q

Bradford Hill Guidelines - For Radon Points 1-4

A

1) Temporality - Established
2) Strength - Small Risk but large and wide exposure
3) Dose Response - Radon is linear without threshold
4) Reversibility - Not for radon

33
Q

Bradford Hill Guidelines - For Radon Points 5-8

A

5) Consistency - CHina + EU + US
6) Biological Mechanism - Alpha radiation damaging DNA via double strand breaks - shown in mice and cells
7) Specificity - Radon inhaled - expect lung cancer - Shown Link
8) Analogy - Residential Radon - Lung Exposure = Same as Occupational

34
Q

U.S EPA Rankings of Carcinogen Classification (5)

A

A) Sure Carcinogen
D) Inadequate Evidence
E) Not likely to be a carcinogen

35
Q

International Agency for Research on Cancer Classification (4)

A

1) Known Carcinogen

2) Not a Carcinogen

36
Q

Ionizing Radiation - Causation + Key Points (4)

A

Sufficient to induce cancer but not necessary

Key Points

1) 1 Sv of Radiation = 5% Risk of Cancer
2) Occupational Exposure = Radium Dial Painters + Uranium Miners
3) Evidence Strong for Ionizing but not for Non-Ionizing
4) Background Radiation = 3.1 mSv annually - CT = 10 mSv

37
Q

Asbestos - Causation

A

Sufficient but necessary for lung cancer - Sufficient + necessary for mesothelioma

38
Q

Shift Work - Causation + Key Points (2)

A

Mixed Evidence - Probably Human Carcinogen (2A)

1) Discount most negative studies (poor designs)
2) Worst Risk - Breast Cancer (1.5-2X)

39
Q

Viruses - Causation

A

Necessary and Sufficient - Hep. B + HPV

40
Q

Diesel Exhaust - Causation + Key Point

A

May but sufficient but not necessary (lung cancer)

Key Points
1) IARC - Carcinogenic but hard to split from cigarettes

Z - M2 Heme/Onc (10 decks)

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