8/28- HemeOnc Review Flashcards Preview

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Flashcards in 8/28- HemeOnc Review Deck (43)
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1

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

- Acquired

- Congenital (Fanconi's)

2

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

Aplastic anemia - Empty marrow

3

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

MDS - Cellular marrow but cytopenic pt - Dyspoietic cells

4

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

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

5

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

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

6

What can cause pure red blood cell aplasia?

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

7

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

True! Bleeding in uremic pts

8

What may cause reticulocytosis (4)?

- 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

9

What are the main microcytic anemias (4)?

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

10

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

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

11

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

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

12

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

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

13

What is a common cause of congenital sideroblastic anemia?

dALA synthase deficiency - Pyridoxine may be effective

14

What is the inheritance of sideroblastic anemia?

X-linked (some AR)

15

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

- Gastric bypass - (Also pernicious anemia)

16

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

Folate - Increased homocysteine B12 - Increased homocysteine - Increased MMA

17

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

- Target RBCs - Acanthocytes/spur cells

18

What symptoms/signs are seen with macrocytosis?

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

19

How do you treat hypoproliferative anemias?

- Iron - B12 - Folate - EPO

20

What organs are affected in hemochromatosis? How?

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

21

What gene commonly is affected in hemochromatosis?

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

22

What could this be? (Pic 1)

- HUS - DIC

23

When do we commonly see cold agglutination?

- Mycoplasmic infections - Indolent lymphomas

24

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

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

25

What are common translocations in AML?

- 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

26

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

t(8;14) - Bcl-2

27

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

t(14;18) - C-myc BCL6

28

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

(pic 2)

29

What is Richter's transformation?

CLL transforming into malignant lymphoma

30

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

- 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