8/28- HemeOnc Review

Question 1

What are the main 2 diseases that cause stem cell failure?

Answer 1

• Acquired
• Congenital (Fanconi’s)

Question 2

What causes stem cell failure (or aplasia)? What does BM look like?

Answer 2

Aplastic anemia - Empty marrow

Question 3

What is the condition where the stem cell differentiates but is ineffective? What does BM look like?

Answer 3

MDS - Cellular marrow but cytopenic pt - Dyspoietic cells

Question 4

What is the condition where the stem cells differentiate but proliferators are out of control? What does BM look like?

Answer 4

MPD (neoplasms) - Marrow is hypercellular - High Hct, WBC, or platelets - Abnl megakaryoctyes—fibrosis, big spleen

Question 5

What is the condition where the stem cells proliferate out of control but do not fully differentiate? What does BM look like?

Answer 5

Acute myelogenous leukemia (AML) - Cellular marrow with primitive cells (blasts) replace marrow and circulate

Question 6

What can cause pure red blood cell aplasia?

Answer 6

• Congenital (Diamond-Blackfan) - Parvovirus - Thymoma (autoimmune) - MDS (5q minus syndrome)

Question 7

T/F: Anemia of CRD involves platelet dysfunction? Why/why not?

Answer 7

True! Bleeding in uremic pts

Question 8

What may cause reticulocytosis (4)?

Answer 8

• Treatment/resolution of hypo-proliferative anemia (recovery after aplastic crisis, from relative hypoproliferative state like moving from Houston to Denver, or with treatment of iron, folate, or B12 deficiency) - Bleeding (in iron replete pt) - Myelophthisis (marrow w/ fibrosis or tumor) - Hemolytic anemia

Question 9

What are the main microcytic anemias (4)?

Answer 9

• Iron deficiency anemia - Sideroblastic anemia - Thalassemias - Anemia of chronic disease

Question 10

What are hepcidin levels/characteristics in the following states: - Anemia of chronic inflammation - Hemochromatosis - Renal failure

Answer 10

• Anemia of chronic inflammation: increased - Hemochromatosis: mutate or downregulated - Renal failure: not cleared; higher levels

Question 11

What are the key characteristics of Beta thal minor. Hb allotype specifics?

Answer 11

• Decreased beta chain production - Damage to RBCs from excess alpha - Increased A2 on electrophoresis!

Question 12

What are the key characteristics of Alpha thal minor. Hb allotype specifics?

Answer 12

• Decreased alpha chain production due to gene deletions - Common in Black Americans - Damage due to beta tetramer (hemoglobin H) - Normal hemoglobin electrophoresis

Question 13

What is a common cause of congenital sideroblastic anemia?

Answer 13

dALA synthase deficiency - Pyridoxine may be effective

Question 14

What is the inheritance of sideroblastic anemia?

Answer 14

X-linked (some AR)

Question 15

What is the most important cause of cobalamin (B12) deficiency from an impaired absorption standpoint?

Answer 15

• Gastric bypass - (Also pernicious anemia)

Question 16

What substances will be elevated in folate deficiency? B12 deficiency?

Answer 16

Folate - Increased homocysteine B12 - Increased homocysteine - Increased MMA

Question 17

What are some RBC characteristics you will see with liver disease (histo)?

Answer 17

• Target RBCs - Acanthocytes/spur cells

Question 18

What symptoms/signs are seen with macrocytosis?

Answer 18

• Megaloblastic anemia - Liver disease (target RBCs, acanthocytes/spur cells) - Extreme reticulocytosis - Hypothyroidism - Artifactual: cold agglutinin disease

Question 19

How do you treat hypoproliferative anemias?

Answer 19

• Iron - B12 - Folate - EPO

Question 20

What organs are affected in hemochromatosis? How?

Answer 20

• Liver: cirrhosis, hepatoma - Heart: congestive CM, conduction blocks/arrhythmias - Endocrine: diabetes, pituitary (hypogonadism, etc.) - Joints - Skin

Question 21

What gene commonly is affected in hemochromatosis?

Answer 21

• HFE gene near HLA-A on chrom 6 - Also TfR-2 (ferroportin) can cause the phenotype as well

Question 22

What could this be? (Pic 1)

Answer 22

• HUS - DIC

Question 23

When do we commonly see cold agglutination?

Answer 23

• Mycoplasmic infections - Indolent lymphomas

Question 24

What do EPO levels look like in secondary erythrocytosis vs. primary?

Answer 24

Secondary: EPO high Primary (polycythemia vera): EPO low

Question 25

What are common translocations in AML?

Answer 25

• t(8;21)- good prognosis - t(15;17)- AML type 3, acute promyelocytic leukemia (response to ATRA, presents with DIC) - inv(16)- AML type 4

Question 26

What translocation is commonly seen in Burkitt’s lymphoma? Gene expressed?

Answer 26

t(8;14) - Bcl-2

Question 27

What translocation is commonly seen in follicular lymphoma? Gene expressed?

Answer 27

t(14;18) - C-myc BCL6

Question 28

What are some tie ins/closely related lymphomas and leukemias?

Answer 28

(pic 2)

Question 29

What is Richter’s transformation?

Answer 29

CLL transforming into malignant lymphoma

Question 30

What do they following translocations correspond to? - t(14;18) - t(11;14) - t(8;14) - t(2;8) - t(8;22)

Answer 30

• t(14;18): Follicular lymphomas (NHL), BCl-2 - t(11;14): Mantle cell lymphomas, Cyclin D1 - t(8;14): Burkitt lymphomas, IgH/K/n and c-myc - t(2;8): Burkitt lymphomas, IgH/K/n and c-myc - t(8;22): Burkitt lymphomas, IgH/K/n and c-myc

Question 31

Describe staging process/criteria

Answer 31

Stage I: 1 LN region/structure Stage II: > 1 LN, same side of diaphragm Stage III: both sides of diaphragm Stage IV: extranodal sites beyond “E” designation (e.g. bone marrow, liver…) Also A: No B symptoms B: B symptoms E: single extranodal site S: spleen X: bulky

Question 32

Lytic bone lesions are seen in what condition?

Answer 32

Multiple myeloma

Question 33

What causes this? (pic 3)

Answer 33

Coumadin

Question 34

What is the initial anticoagulant of choice for new thrombosis?

Answer 34

DOACs (2) - NOT warfarin!!

Question 35

Where in pregnant women is thrombosis most commonly seen?

Answer 35

Left leg

Question 36

When is risk of thrombosis highest in pregnant women?

Answer 36

6 week period post partum

Question 37

What deficiency is common in newborns? Results in what?

Answer 37

Vitamin K deficiency - Causes hemorrhagic disease of the newborn

Question 38

What chemotoxic drugs cause neuropathy?

Answer 38

• Taxanes - Platinum (oxaliplatin) - Vinca alkaloids

Question 39

What chemotoxic drugs cause cardiac issues?

Answer 39

Anthracyclines (doxorubicin)

Question 40

What chemotoxic drugs cause hand-foot syndrome?

Answer 40

5FU/capecitabine

Question 41

What chemotoxic drugs cause nephrotoxicity?

Answer 41

Cisplatin

Question 42

What chemotoxic drugs cause diarrhea?

Answer 42

• 5FU - Irinotecan

Question 43

What are the side effects of Sunitinib (Sutent), VEGFR?

Answer 43

• Rash - Hand Foot - Diarrhea