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Emma Heme/Onc > Hematopoiesis, Aplastic Anemia, HypOproliferative Disorders, WBC > Flashcards

Hematopoiesis, Aplastic Anemia, HypOproliferative Disorders, WBC Flashcards

1
Q

T/F

hematopoietic progenitor cells lack the ability to self-renew

A

T

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2
Q

myeloid growth facs

A

Erythropoietin (Epo)
Thrombopoietin (Tpo)
Granulocyte colony-stimulating factor (G-CSF)
Granulocyte-macrophage colony-stimulating factor (GM-CSF)
Macrophage colony-stimulating factor (M-CSF)
Interleukin-3 (IL-3)
Interleukin-6 (IL-6)
Stem cell factor/mast cell growth factor (SCF)

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3
Q

Epo MOA

A

Acts as a true hormone (produced at one site, circa in the blood and acts on a target organ)

Prod in kidney in response to local hypoxia –> acts on erythroid progenitors in the bone marrow to boost rbc prod

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4
Q

Tpo MOA

A

Prod in liver at constant rate

Binds to receptor on megakaryocyte and precursors in bone marrow –>Mk maturation/platelet prod

ALSO binds to same receptor on circulating platelets (if xs platelets –> free Tpo drops, platelet prod slows. If low platelets –> free Tpo rises, platelet prod incr.)

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5
Q

Pool of unbound Tpo is inversely related to

A

Pool of unbound Tpo is inversely related to NUMBER OF PLATELETS

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6
Q

myeloid growth facs act thru cell surface receptors to trigger _____ and _____

A

myeloid growth facs act thru cell surface receptors to trigger DIMERIZATION and ACTIVATION OF CELL KINASES

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7
Q

From the _____ stage on, cells are post-mitotic

A

From the METAMYELOCYTE stage on, cells are post-mitotic

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8
Q

mature blood cells circulate for how long

A

100-120 d

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9
Q

normal % of reticulocytes in blood

A

1%

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10
Q

clinical use of Epo

A

anemia of chronic kidney failure

chemo-induced anemia

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11
Q

clinical use of Tpo

A

chemo-induced thrombocytopenia

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12
Q

clinical use of G-CSF

A

chemo-induced neutropenia

aplastic anemia

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13
Q

clinical use of GM-CSF

A

chemo-induced neutropenia

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14
Q

site of active bone marrow hematopoiesis in adults

A

spine, pelvis, ribs, sternum, and skull

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15
Q

site of active bone marrow hematopoiesis in child

A

entire skeleton

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16
Q

usual site of BM biopsy

A

posterior superior iliac crest

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17
Q

bone marrow failure

A

cytopenia due to dec production

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18
Q

Aplastic anemia

A

bone marrow failure due to deficiency of hematopoietic stem cells –> in aplasia (lack of hematopoiesis) and pancytopenia (low rbc/wbc/platelet counts in the peripheral blood)

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19
Q

normal pyelogram contains ___ immature blast cells

A

normal pyelogram contains LESS THAN 5% immature blast cells

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20
Q

normal pyelogram

myeloid precursor:erythroid precursor

A

normally 2-4x as many myeloid precursors than erythroid precursors

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21
Q

fat makes up __% of the marrow normally

A

fat makes up 50-60% of the marrow normally

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22
Q

congenital aplastic anemia

A

fanconi anemia
Schwachmann-Diamond Syndrome \
Dyskeratosis congenita
Amegakaryocytic thrombocytopenia

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23
Q

Fanconi Anemia

A

congenital aplastic anemia

AR, Ashkenazi Jews

5-9 y/o (congenital malformations, abnormal thumbs, cytopenia)

damage due to defect in DNA repair pathway, 12 Dif gene mutations IDed

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24
Q

Schwachman-Diamond

A

congenital aplastic anemia

Rare, AR, homozygous mut SBDS (rRNA metabolism, mitotic spindle)

exocrine pancreatic deficiency, skeletal abnormalities, bone marrow failure

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25
Q

dyskeratosis congenita

A

congenital aplastic anemia

rare, XL or AD or AR

ectodermal dysplasia, BM failure, cancer predisposition

mut cause dysfunctional telomerase (pts have abnormally short telomeres, stem cell transplant is only cure)

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26
Q

Congenital amegakaryocytic thrombocytopenia

A

congenital aplastic anemia

rare, AD

infancy w/ isolated thrombocytopenia, absent megakaryocytes in BM

mut in MPL (gene for Tpo receptor)

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27
Q

acquired aplastic anemia

causes

A

Radiation, toxins/chemicals (benzene, organic solvents, cancer chemotherapy)

Drugs: methotrexate, chloramphenicol, gold, NSAID

Autoimmune disorders: lupus, mixed cyroglobulinemia, idiopathic

Lymphoid malignancy (thymoma/pure red cell aplasia)

Pregnancy

Virus (HIV, HBV, HCV, parvovirus B19)

Paroxysmal Nocturnal Hemoglobinuria

Unknown

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28
Q

Paroxysmal Nocturnal Hemoglobinuria (PNH)

A

acquired syndrome of hemolysis and BM failure

somatic mutation in PIG-A in HSC –> dec GPI-anchor needed for surface proteins on rbc

some of these surface proteins (CD55, CD59) limit complement action, so PNH cells susceptible to complement lysis –> hemolysis

high freq thrombosis

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29
Q

2 principle causes of severe aplastic anemia

A
  1. immune mediated stem cell suppression

2. stem cell damage/loss

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30
Q

2 main tx approaches for severe aplastic anemia

A
  1. immune suppression

2. stem cell replacement by transplantation

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31
Q

hypOproliferative anemias

A

Fe deficiency
B12 and folate deficiency
Anemia of chronic disease

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32
Q

anemia

physiologic definition

A

Erythropoiesis in bone marrow does not match demand for oxygen-carrying capacity (rbc) in peripheral blood circulation

inadequate delivery of O2 to tissues

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33
Q

anemia

operational definition

A

dec in Hb protein in red cells by >2 SD below normal mean

dec in Hct % (mL of RBC’s per 100 mL of blood)

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34
Q

Mean corpuscular volume (MCV)

A

size of rbc

calc via Hgb/RBC

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35
Q

Mean corpuscular hemoglobin (MCH)

A

Hb in single rbc

pg/cell

(Hb/rbc)

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36
Q

MCHC

A

Hb in given vol of rbc

g/dl

(Hgb/Hct)

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37
Q

RDW

A

rbc size distribution

%

if uniform size, low RDW

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38
Q

anemia signs/sx

A 
fatigue
pallor
SOB
tachycardia, palpitations
orthostatic HTN
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39
Q

reticulocyte

A

youngest normal RBC entering circulation from BM

contains RNA remnants, ribosomal machinery

visualized w/ supravital stains

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40
Q

reticulocyte count

A

(% of rbc that have RNA remnants) * total rbc #

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41
Q

reticulocyte index

A

adjusts reticulocyte count in anemic state

reticulocyte count (%) * pt’s Hct / 40

(should be >2 in anemic pt if BM is inc reticulocytes appropriately)

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42
Q

hypOproliferative anemia

criteria

A

anemia with RI <2 and/or absolute reticulocyte count less than nil

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43
Q

hypERproliferative anemia

A

RI elevated and/or absolute reticulocyte inc in pt w/ anemia

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44
Q

hypOproliferative anemias

A 
iron deficiency
B12 deificency
folate deficiency
Epo deficiency
hypOthyroidism
lead/other toxins (EtOH)
Infection (Parvo B19)
BM failure (Fanconi's anemia)
Myelodysplastic syndromes
Anemia of chronic disease
45
Q

low MCV (microcytic hypo proliferative anemias)

A

iron deficiency*
Pb tox
ACD

46
Q

normal MCV (microcytic hypo proliferative anemias)

A

ACD *
kidney disease
red cell aplasia

47
Q

high MCV (microcytic hypo proliferative anemias)

A

B12 deficiency*
Folate deficiency*
Myelodysplastic syndrome
Alcoholism

48
Q

Fe deficiency causes

A 
prematurity, rapid growth (infants)
diet (toddlers)
adolescents (menstruation, diet)
pregnancy (depletion of stores)
adults (overt or occult blood loss, Malabsorption, genetic)
49
Q

Fe deficiency signs/sx

A 
fatigue, pallor, dyspnea
hair loss
PICA
restless legs
spoon shaped nails (koilonychia)
angular cheilitis
swollen/sore tongue
50
Q

hephaestin

A

ferrioxidase that reoxidases iron for release from enterocyte to plasma transferrin

51
Q

ferroportin

A

key transport protein in exchange of Fe from enterocyte to transferrin

52
Q

transferrin

A

secreted by hepatocytes

major transport protein in blood

delivers Fe to cells possessing appropriate cell-surface transferrin receptors

53
Q

intestinal Fe absorption varies inversely w/ expression of ____ in ____

A

intestinal Fe absorption varies inversely w/ expression of HEPCIDIN in LIVER

54
Q

primary Fe regulatory hormone

A

hepcidin

prod in liver

synthesized in response to inflammation/inc Fe stores

55
Q

hepcidin prod is inhibited by

A

hepcidin prod is inhibited by INC Epo (DEMAND FOR Fe)

56
Q

______ downregulates Ferroportin

A

HEPCIDIN downregulates Ferroportin

–> dec Fe absorption by enterocyte, dec Ferroportin-mediated Fe export from enterocyte to blood, dec Fe release from macrophage of RES

57
Q

Ferritin

A

usable form of Fe

H2O soluble protein-iron complex w/ outer shell (apoferritin)

serum ferritin reflects Fe stores

58
Q

serum _____ reflects Fe stores

A

serum FERRITIN reflects Fe stores

59
Q

Fe deficiency CBC

A 
anemia
microcytosis (low MCV)
Hypochromia (low MCH)
dec rbc 
low reticulocyte count
60
Q

rbc should be roughly the size of ____ nucleus

A

lymphocyte nucleus

61
Q

Fe deficiency
serum Fe studies

serum Fe:
TIBC:
Ferritin:
Transferrin Sat:

A

serum Fe: low
TIBC: high
Ferritin: low
Transferrin Sat: low

62
Q

tx of Fe deficiency

A

oral Fe (6 mg/kg daily, acidic cond)

IV Fe (2 mg/kg IV over 1hr, Fe sucrose)

transfusion (0.5mg Fe in each ml whole blood, only severe sx, AE: infection, rxns, Ab form)

63
Q

Pb toxicity

A

Pb inhibits final steps in heme synthesis

Inhibits RNA breakdown

RNA aggregates in RBC (basophilic stippling)

anemia w/ low MCV

multiple non-hematologic comorbidities

64
Q

Pb tox signs/sx

A

exposure to lead paint, exhaust fumes, contaminated H2O

sx: abd pain, anemia, neuro, fatigue, malaise, irritability
dx: serum Pb

65
Q

anemia of chronic illness

A

normocytic anemia (MCV usually not less than 70)

assoc w/ inflammatory disorders

dysregulation of Fe transport, utilization and storage via induction of hepcidin expression by inflammatory cytokines

Fe released from enterocytes/macrophages is inhibited (down regulated ferroportin)

dec plasma Fe, normal BM Fe stores

66
Q

folic acid

A

water-soluble vitamin

green leafy veggies, fortified grains, some fruits

easy absorption in duodenum/upper jejunum (polyglutamate conjugation and methylation)

67
Q

folate deficiency causes

A

diet (old age, poverty, goats milk)

malabsorption (Chron’s, celiac sprue, GI surgery)

inc demands (pregnancy, hemolysis, rapid growth)

drugs (folate antagonists like methotrexate)

liver disease, EtOH

68
Q

B12

A

animal (liver, red meat, some dairy)

complex absorption mech (R-binder proteins, intrinsic factor (parietal cells), receptors on terminal ileum, transcobalamin I/II)

daily req 1-2mg

bod stores 2-3mg

69
Q

causes of B12 deficiency

A

malabsorption (gastrectomy, lack of intrinsic factor, pernicious anemia)

GI (ideal resection, stricture, celiac, tapeworm)

Transcobalamin II deficiency

NO toxicity

Meds (PPIs, metformin)

70
Q

pernicious anemia

A

Ab-mediated destruction of parietal cells

reduced IF prod

dx via finding anti-IF Abs

macro-ovalocytic rbc
giant hyperhsegmented neutrophils

71
Q

B12 neuropathy

A

progressive, affects peripheral sensory nerves and postero-lateral SC columns

tingling, balance/walking issues

impaired methylmalonyl CoA mutase

symmetrical, lower>upper limbs

impaired methylmalonyl CoA mutase

accumulation of S-adenosyl homocysteine and odd-chain FA

72
Q

folate and B12 predispose to ____ defects

A

neural tube defects (anencephaly, spina bifida)

impaired methylation of myelin and lipids

73
Q

B12 deficiency labs

serum B12:

RBC folate:

Serum folate:

A

serum B12: low

RBC folate: normal/low

Serum folate: normal/high

74
Q

folate deficiency labs

serum B12:

RBC folate:

Serum folate:

A

serum B12: normal

RBC folate: low

Serum folate: low

75
Q

in addition to checking serum B12 levels, evaluation of B12 deficiency can also include

A

checking for inc homocysteine and methylmalonic acid in blood (more sensitive than B12 in borderline cases)

endoscopy to evaluate for atrophic gastritis

blood for parietal Abs (in PA)

76
Q

tx for B12/folate deficiency

A

administer vitamin
(may need to give both vitamins to correct hematology and neuropathy)

if absorption a problem –> replace parenterally (IV or IM)

77
Q

indicators of response to B12/folate deficiency tx

A

inc appetite in 24-48 hr

inc reticulocyte count (3d)
Hb inc (2-3g/dl q2wk)
normal platelets and WBC (7-10d)

peripheral neuropathy may improve

spinal cord damage=IRREVERSIBLE

78
Q

Fe overload

A

inc Fe absorption (hereditary hemochromatosis, chronic liver disease, ineffective red cell production)

inc Fe intake (African siderosis)

transfusional (Fe chelation needs in chronic transfusion states)

79
Q

Hereditary hemochromatosis

A
  • mutation in HFE chromosome 6
  • AR
  • xs absorb of Fe from GI tract
  • inc transferrin sat and ferritin
  • iron deposition in liver/heart/organs
  • skin pigmentation/arthropathy
  • inc transferrin saturation/high ferritin
  • HFE mutation testing
  • tx: phlebotomy
80
Q

wbc

A

leukocytes

2 groups:
phagocytes (neutrophils, eos, basophil)
immunocytes (lymphocytes and plasma cells)

81
Q

neutrophils

A

usu >50% circulating WBCs in adult

exit vasculature –> tissues to kill invaders

1st to arrive in inflammatory response

82
Q

bands (WBC)

A

one step prior to mature eos

higher # w/ acute bacterial infection

83
Q

eosinophils

A

sim to neutrophils - phagocytosis but less responsive

worms/wheezes/weird diseases…
ellergies, parasites, TB, sarcoidosis, Addison’s disease, Hodgkin’s disease

84
Q

basophils

A

uncommon in peripheral blood

histamine release –> inflammation

histolog: blue, coarse granules, can barely see nucleus underneath

if >5-10%, consider CML

85
Q

monocytes

A

function:

  • phagocytosis
  • present antigen to T-cell lymphocytes
  • secrete cytokines

lifespan:

  • marrow–>brief
  • circ–> 20-40 hr
  • tissue–> months/yrs (differ to macrophage)
86
Q

inc monocyte count

A 
chronic infection (TB)
chronic inflammation (sarcoid)
chronic neutropenia
recovery from neutropenia
preleukemia
myeloproliferative syndrome
87
Q

dec monocyte count

A

bone marrow failure
corticosteroids
myelosuppressive drugs

88
Q

dohle bodies

A

cytoplasmic inclusions, ribosome rich ER seen in sepsis, G-CSF use

89
Q

Plger-Huet anomaly

A

majority of granulocytes have bilobed nucleus, rather than typical 3-5 lobes
benign hereditary disorder
myelodysplastic syndrome

90
Q

granulopoiesis growth facs

A

IL-1, IL-3, IL-5 (eos), IL-6, IL-11

GM-CSF, G-CSF, M-CSF

released from stromal cells and T lymphocytes (stim by endotoxin, IL1, TNF)

91
Q

chemoattractants

A
  • complement cleavage fragments
  • products released by bacteria/host cells
  • neutrophils –> C5a
  • GCPR
92
Q

leukocyte adhesion molecules

A

glycoprotein molecules (B2 integral and selectin ligand –> bind B2 ligand and selectin on endothelial cells)

93
Q

opsonization

A

antigens are bound by Ab or complement (C3b) –> enhanced phagocytosis

94
Q

neutralization (oxygen-independent)

A
  • phagosome-lysosome fusion and acidification

- hydrolytic and proteolytic enzymes

95
Q

neutralization (oxygen-dependent)

A

respiratory burst

NADPH oxidase reduces O2 to H2O2 (hydrogen peroxide), which then works w/ myeloperoxidase to kill bacteria.

96
Q

left shift in wbc

A

more immature granulocytic forms seen (band cells)

97
Q

inherited causes of leukocytosis

A

Down syndrome

WBC dysfunctional disorders

98
Q

acquired causes of leukocytosis

A 
inflammation
stress
infection
meds
malignancy
asplenia
99
Q

neutropenia causes (decreased production, congenital)

A

kostmann’s syndorme
benign (ethnic/familial)
cyclic
bone marrow failure (rare)

100
Q

neutropenia causes (decreased production, acquired)

A

drug
infectious
malignanct
toxicity

101
Q

neutropenia causes (increased destruction)

A

infection
immune (neonatal alloimmune, AI, SLE)
splenomegaly
drug

102
Q

inherited phagocytic functional disorders

A
  • myeloperoxidase deficiency
  • leukocyte adhesion defect
  • chediak-hegashi syndrome
  • specific granule deficiency
  • hyperimmunoglobin E, recurrent infection syndrome (Job’s)
  • chronic granulomatous disease (CGD)
103
Q

chronic granuomatous disease (CGD)

A

XLR (gene codes for NADPH oxidase enzyme)

  • DEFECT IN RESPIRATORY BURST ENZYME COMPLEX
  • severely diminished H2O2 prod (can’t destroy organisms)
  • problem w/ catalase + microbes (breakdown H2O2)
  • severe skin/sinopulmonary infec/granulomas/abcesses/sepsis
  • lymphadenopathy, hepatosplenomegaly
  • nitroblue-tetrazolium (NBT) NEGATIVE
104
Q

Which disease has trouble killing catalase + organisms?

Name some catalase + organisms

A

chronic granuomatous disease (CGD)

Staphylococci, E coli, Klebsiella species, Pseudomonas, fungi aspergillus, nocardia

105
Q

myeloperoxidase deficiency

A
  • usually partial defect

- respiratory burst affected but can still produce H2O2

106
Q

leukocyte adhesion defect

A
  • extremely rare AR disorder
  • beta integrin and selection ligand can’t adhere to endothelial surfaces
  • unable to phagocytose bacteria coated w/ C3
  • DELAYED UMBILICAL CORD LOSS, poor wound healing, bacterial infections
  • leukocytosis
107
Q

Chediak-Higashi Syndrome

A

AR

  • failure of phagolysosome formation, neutropenia
  • partial albinism, giant lysosomes, neuropathy, lymphoma
  • recurrent skin/systemic infections, Staph aureus
108
Q

Job’s Syndrome

A

hyperimmunoglobin E (recurrent infection syndrome)

  • rare AD, STAT3 mut
  • inc IgE, eczema, recurrent skin/sinopulmonary infections
  • defect in chemotaxis

Emma Heme/Onc (16 decks)

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