Block 3 Path Exam -- Intro To Hematopoiesis & Iron Metabolism Lectures (# 1-2)

Front 1

Bones in which active hematopoiesis occurs

Back 1

VERTEBRA, STERNUM, PELVIS
Ribs, skull, scapula
Proximal long bones

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EPO
(Erythropoietin)

Back 2

Source -- kidney
INCREASES RBC production
Used in anemia of RF, chronic disease, malignancy

Front 3

G-CSF and GM-CSF

Back 3

Sources -- monocytes, fibroblasts, endothelial cells
Note: Production is increased by inflammation (IL-1 [from monocytes])
INCREASE granulocyte release from BM
INCREASE BM production of all cell lineages
Used in neutropenias, BM transplantation

Front 4

IL-3

Back 4

Source -- activated T lymphocytes
Causes proliferation of ALL hematopoietic precursors
SYNERGISTIC with G-CSF, GM-CSF, SCF

Front 5

SCF
(Stem Cell Factor)

Back 5

Sources -- fibroblasts, BM stomral cells
Enhances stem cell survival
Mitogenic
SYNERGISTIC with IL-6, IL-3, G(M)-CSF
Used in BM failure

Front 6

IL-5

Back 6

Source -- marrow stromal cells
Eosinophil growth factor

Front 7

IL-1

Back 7

Source -- stimulated monocytes
Stimulates production of IL-2 and other cytokines (G-CSF)

Front 8

Tumor Necrosis Factor

Back 8

Stimulates production of GM-CSF, M-CSF, and IL-1

Front 9

Retinoic acid

Back 9

Promotes granulocyte differentiation

Front 10

Cytokines induced by inflammation and infection

Back 10

GM-CSF, IL-3, IL-6

Front 11

Growth factors produced by Acute Myelogenous Leukemias

Back 11

GM-CSF, G-CSF, M-CSF
Produced in an AUTOCRINE fashion

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Erythroblast
(Pronormoblast)

Back 12

Earliest identifiable erythroid cell
Fine chromatin, nucleoli
Basophilic cytoplasm
Found in the BM

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Orthocromic Normoblast

Back 13

Pink cytoplasm
Nucleus is extruded following this stage
Found in the BM

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Reticulocyte

Back 14

Larger than mature RBCs
Slightly bluish-grey color (persistent RNA)
Polychromasia -- the reaction to stain (methylene blue)

Front 15

Erythropoietin

Back 15

Regulator of erythrocyte production
Production of this is mediated by TISSUE O2 LEVELS

Front 16

2,3 DPG

Back 16

Modulates the oxygen affinity of Hgb
Inc. 2,3 DPG results in Inc. O2 release

Front 17

Anisocytosis

Back 17

Variation in RBC size (RDW)

Front 18

Poikilocytosis

Back 18

Variation in RBC shape

Front 19

Schistocytes

Back 19

Fragmented RBCs
Split or broken by physical means
3 main causes: DIC, TTP, HUS

Front 20

Target Cells

Back 20

Concentrated Hgb in the center
Seen in thalassemia, liver disease, and post-splenectomy

Front 21

Spherocytes

Back 21

Spherical RBCs, WITHOUT central pallor

Front 22

Ovalocyte

Back 22

Egg-shaped RBCs

Front 23

Elliptocytes

Back 23

Oblong RBCs, with nearly parallel sides

Front 24

Acanthocyte

Back 24

SPIKY RBC WITHOUT central pallor
Commonly seen in liver disease

Front 25

Burr Cell

Back 25

RBC with knobby surface bumps
Usually, these are artifact
Can be seen in Anemia of Renal Failure

Front 26

Acute Intermittent Porphyria

Back 26

Defect in PORPHOBILINOGEN DEAMINASE
Signs -- ab pain, NEUROLOGIC defects

Front 27

Variegate Porphyria

Back 27

Defect in protoporphyrinogen oxidase
Same symptoms as AIP, plus light sensitivity

Front 28

Porphyria Cutanea Tarda

Back 28

PARTIAL defect in URO-DECARBOXYLASE
Signs -- dermal photosensitivity
Can be triggered by estrogen and alcohol

Front 29

Protoporphyria

Back 29

Due to decreased Heme Synthetase (ferrochelatase)
Dermal photosensitivity is increased

Front 30

Thalassemia

Back 30

Hgb synthesis disorder
Decr. globin chain synthesis
Often entails chronic hemolytic anemia

Front 31

Spectrins

Back 31

Proteins that link to form "scaffolding" for RBCs

Front 32

Ankyrins

Back 32

Proteins that bind RBC membrane to "scaffolding"

Front 33

Normal HCT values

Back 33

Women -- .35 - .47
Men -- .40 - .52

Front 34

Normal Hgb values

Back 34

Women -- 120 - 160
Men -- 130 - 180

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Normal MCV values

Back 35

80 - 100 fl for both men and women

Front 36

3 major patterns of anemia

Back 36

Hemolytic (Inc. R-cytes, Decr. M:E)
Hypoproliferative (Decr. R-cytes, Incr. M:E)
Ineff. Erythropoiesis (Decr. R-cytes, Decr. M:E)

Front 37

Hemosiderin

Back 37

Storage form of iron
Commonly seen in MACROPHAGES

Front 38

Heme Oxygenase

Back 38

Separates iron from heme @ enterocyte membrane

Front 39

Ferroportin
Hephaestin

Back 39

Transport iron across BASOLATERAL membrane
Hand iron off to transferrin

Front 40

Divalent Metal Transporter 1

Back 40

Protein that transports ferric iron across apical membrane

Front 41

Hepcidin

Back 41

Primary regulator for the absorption and release of iron
Manufactured in the liver
High plasma iron --> Incr. hepcidin --> Decr. release of iron
Low plasma iron --> Decr. hepcidin --> Incr. release of iron

Front 42

Ceruloplasmin

Back 42

Important in mobilizing iron from macrophages
Iron oxidized and incorporated into ferric transferrin

Front 43

Sequence of iron depletion in anemia

Back 43

BM --> RBCs --> other tissues

Front 44

Type of anemia that Iron Def. Anemia is

Back 44

Microcytic
Hypochromic

Front 45

Serum Iron (SI)

Back 45

All iron bound to transferrin

Front 46

Total Iron Binding Capacity

Back 46

Approximates amount of transferrin
% Saturation = SI divided by TIBC

Front 47

Soluble transferrin receptor

Back 47

Reflects the total body mass of cellular transferrin receptor

Front 48

Type of Anemia that anemia of inflammation is

Back 48

Normocytic
Normochromic

Front 49

Type of anemia that Anemia of RF is

Back 49

Normocytic
Normochromic

Front 50

Type of anemia that Sideroblastic Anemia ia

Back 50

Microcytic
Hypochromic
Note: has "ringed sideroblasts"