Clinical Hematology Review

Front 1

Causes of macrocytic megaloblastic anemia.

Back 1

- Defective nuclear maturation due to impaired DNA synthesis.

- From Folic acid or Vit B12 deficiency

Front 2

Causes of macrocytic non-megaloblstic anemias.

Back 2

- Chronic liver Dz

- Alcoholism

- Acute blood loss

- Arsenic/ chlordane intoxification

Front 3

Megaloblastic findings in BM

Back 3

Large RBC precursors

Large Granulocyte precursors

N:C asynchrony

Front 4

Peripheral smear findings in megaloblastic anemia.

Back 4

MCV 100-160 fL

MCH is increased

RBC count is low

Low retic count, rare polychromasia

Hyper segmented neutrophils

Front 5

True or False: Hyper segmented neutrophils are found in macrocytic non-megaloblastic anemia.

Back 5

False

Front 6

True or False: Macroovalocytes are seen in megaloblastic anemia and normal macrocytes are seen in non-megaloblastic?

Back 6

True

Front 7

True or False: Retics are increased in megaloblastic and normal/decr. in macrocytic non-megaloblastic anemia.

Back 7

False

Front 8

Chemistry findings in macrocytic anemia

Back 8

Inr. LDH

Inc. uncong. bilirubin

Normal Ferritin, TIBC, and Iron

Front 9

RBC morphology in macrocytic anemia

Back 9

Schistocytes, tear cells, spherocytes, target cells

Front 10

Cause of PA

Back 10

- Parietal cell atrophy and decreased intrinsic factor (IF)

- Autoimmune destruction of parietal cells

- Malabsorbtion syndromes

Front 11

What is needed for a Dx of Megaloblastic anemia?

Back 11

- Peripheral smear findings

- Folic acid/ B12 levels

Front 12

Principle of the Schilling Test?

Back 12

- Labeled B12 is given PO

- 24 hr urine levels are determined

- if B12 is normal, its a problem with IF

- if B12 is decreased, its a problem with malabsorption.

Front 13

4 Hereditary Membrane Defect Anemias

Back 13

Spherosytosis

Elliptocytosis

Stomatosytosis

Xerocytosis

Front 14

Hereditary shperocytosis

Back 14

- Spectrin and ankyrin deficiency

- Unable to deform when passing through capillaries

- leaves cells round and osmotically fragile

Front 15

Clinical subtypes of Hereditary Elliptocytosis

Back 15

- Common hereditary elliptocytosis

- SE Asian ovalocytosis

- Spherocytic hereditary elliptocytosis

- Hereditary pyropoikilocytosis

Front 16

Hereditary pyropoikilocytosis

Back 16

- Recessive disorder

- alpha/beta spectrin subject to degredation

- African Americans

- *Red cell budding*

- MCV 50-75 fL

- Lower temp. RBC destruction

Front 17

3 stages of IDA

Back 17

1. Depletion of iron stores

2. Iron-deficient erythropoiesis

3. Iron-deficiency anemia

Front 18

Sideroblastic anemia

Back 18

- Pappenheimer bodies, basophilic stippling

- Accum. of iron in mitochondria

- 5-aminolevulinic synthetase deficiency

- Primary: RARS

- Secondary: alcohol, lead, antibiotics

Front 19

Anemia of Chronic Disease/ Anemia of Inflammation (AOI)

Back 19

- Stored Iron is not released from macrophages

- Stored Iron not transported to BM

- EPO production is decreased

- Pronormoblasts are not responsive to EPO

Front 20

Alpha Thalassemia

Back 20

- 2 alpha chains from each parent (4 total_

- No Alpha chains: Barts Hydrops fetalis

- 1 Alpha chain: Hgb H Dz.

- Hgb H formed, not Hgb A

- golf-ball inclusions

- 2 Alpha chains: Alpha Thal Trait

- mild anemia

- 3 Alpha chains: Silent Carrier

Front 21

Beta Thalassemia

Back 21

- 1 Beta chain inherited from each parent

- either 1 or 0 beta chains inherited

- little to no Hgb A production

- compensate by producing Hgb F 60-95%

- point mutations disrupt gene expression for B-chains

Front 22

Iron overload

Back 22

accumulation of excess iron in reticuloendothelial cells

Front 23

Hemachromatosis

Back 23

tissue damage resulting from excess iron

Front 24

Sickle Cell Dz (SS)

Back 24

- Valine substituted for glutamic acid on 6th pos of both B-chains

- 60% Hgb S, 40% Hgb F

-

Front 25

Sickle Cell Trait (AS)

Back 25

- One B-chain is normal

- 60% Hgb A, 40% Hgb S

- Testing: Metabisulfite

- dithionite testing

- Isoelectric focusing

Front 26

Hgb C Dz. (CC)

Back 26

- sub lysine for glutamic acid on 6th pos of both B- chains

- Target cells

- Hgb C crystals (bars of gold)

- >90% Hgb C, <7% Hgb F

Front 27

Hgb C Trait (AC)

Back 27

- Hgb C gene inherited from 1 parent

- Target cells

- 60-70% Hgb A, 30-40% Hgb C

Front 28

Normochromic Anemia: biochemical deficiencies

Back 28

- G6PD deficiency

- Pyruvate Kinase deficiency, RBCs cannot generate ATP from ADP

- Methemoglobin reductase deficiency, cyanosis

Front 29

What is the most common deficiency anemia?

Back 29

G6PD deficiency

Front 30

Aplastic anemia

Back 30

Pancytopenia

Dry Tap

Front 31

Fanconi's Anemia

Back 31

Genetic

AML

Front 32

PNH

Back 32

Pancytopenia

CD 55, 59 markers

Front 33

CAS

Back 33

Cold auto-Ab causes hemolysis

Front 34

PCH

Back 34

Anti-P

Front 35

Gaucher's Dz

Back 35

Glucocerebrosidase deficiency

Crumpled tissie paper appearence.

Front 36

Neimann-Pick Dz

Back 36

- Sphingomyelinase deficiency

- Foam cells

Front 37

Tay-Sachs Dz

Back 37

- Hexosaminidase deficiency

- Vacuolated lymphs

Front 38

Myeloperoxidase

Back 38

- Used to stain peroxidase granules

- Will not stain lymphocytes

- Differentiates ALL from AML

Front 39

Sudan Black B

Back 39

- Stains primary and secondary granules of myeloid cells

- differentiates AML from ALL

Front 40

Napthol AS-D Chloroacetate

Back 40

Stains myeloblasts, segs, basophils, and mast cells

Front 41

Alpha-Napthyl Butyrate/Acetate Esterase

Back 41

Monoblasts/Monos stains a strong positive

Front 42

Terminal Deoxynucleotidyl Transferase

Back 42

Stains immature lymphoid cells

Front 43

Acid Phosphotase

Back 43

Stain T-lymphs

Front 44

Tartrate resistant AP

Back 44

Stains B lymphs in hairy cell leukemia

Front 45

Perdiodic Acid-Schiff (PAS)

Back 45

Stains erythroblasts in erythroleukemia

Front 46

CMDs

Back 46

- PV

- ET

- CML

- MMM