Heme-Onc Part 1

Front 1

an RC should be the size of:

Back 1

a lymphocyte nucleus

Front 2

what two other cells should be present near RBC's?

Back 2

platelets and WBC's

Front 3

anisocytosis =

Back 3

disorder of RBC SIZE

Front 4

RC size is measured by:

Back 4

MCV,

RDW

Front 5

high RDW ~~

Back 5

lots of variation

check

Front 6

microcystosis is measured by:

Back 6

MCV

Front 7

microcystosis occurs in:

(4)

Back 7

1. iron deficiencies

2. thal's

3. lead poisoning

4. sideroblastic anemias

Front 8

macrocystosis occurs in:

(3)

Back 8

1. elevated reticulocyte counts

2. B12/folate deficiency

3. normal SE of AZT's/chemotherapy

Front 9

AZT =

Back 9

anti-retrovirals

Front 10

hypochromasia =

Back 10

RC's have too little Hb

- area of central pallor >1/3

(more pale = LESS Hb)

Front 11

hypochromasia is measured by:

Back 11

MCH

Front 12

**polychromasia features:**

(2)

Back 12

1. bluish tinge

2. RC's are LARGER

Front 13

polychromasia occurs in:

Back 13

hemolytic anemias

Front 14

poikilocytosis =

Back 14

variation in RC SHAPE

Front 15

target cells occur in:

(4)

Back 15

1. LIVER disease

2. thal's

3. HbC

4. splenectomy (minor)

Front 16

spherocytes occur in:

(2)

Back 16

1. hereditary spherocytosis

2. autoimmune hemolysis

Front 17

main spherocyte feature:

Back 17

1. LOSS of central pallor (more Hb/SA)

Front 18

spherocytes + bluish tinge =

Back 18

hemolytic anemia

Front 19

schistocytes are:

Back 19

RC fragments with sharp edges

Front 20

**schistocytes are a hallmark of:**

Back 20

MAHA,

of which TTP is one

Front 21

sickle cells - there might only be:

Back 21

one on the slide

Front 22

echinocytes features:

Back 22

small, regular projections - like a flower

Front 23

***when you see echinocytes, think:***

Back 23

RENAL disease

Front 24

echinocytes are aka:

Back 24

burr cells

Front 25

acanthocyte features:

Back 25

pointy, irregular projections

Front 26

when you see acanthocytes, think:

Back 26

LIVER disease

(burst quickly => horrible anemia)

Front 27

acanthocytes are aka:

Back 27

spur cells

Front 28

teardrop cells occur in __________________ processes

Back 28

myelophthistic

- diseases of *marrow infiltration*

Front 29

teardrop cells are seen in:

(3)

Back 29

1. myelofibrosis

2. tumor mets to marrow

3. granulomatous diseases

Front 30

Howell-Jolly bodies are:

Back 30

purple dots inside RC's

= nuclear fragments

Front 31

Howell-Jolly bodies are the result of:

Back 31

splenectomy or low-functioning spleen

- spleen isn't geting rid of nuclear fragments

Front 32

check

Back 32

check

Front 33

roeleaux =

Back 33

RC's in stack of coins

Front 34

rolo occurs as a result of:

Back 34

increased levels of Ig, as seen in:

multiple myeloma, inflammatory conditions,
Waldenstrom's macroglobin,
severe hypoalbuminemia

Front 35

agglutination =

Back 35

clumps of RC, due to coating with IgM

Front 36

basophilic =

Back 36

purple

- eosinophilic = red

Front 37

RC's in iron deficiency anemias:

(4)

Back 37

1. small

2. hypochromic

3. ~~ inc. # of platelets

4. s/ts target cells are present

Front 38

RC's in B-thal major:

(5)

Back 38

1. target cells

2. HJ bodies

3. nucleated RC's

4. basophilic stippling

5. teardrops

Front 39

***RC's in megaloblastic anemia:***

(2)

Back 39

1. macrocytic

2. hypersegmented PMN's

Front 40

megaloblastic anemia is the result of:

Back 40

B12/folate deficiency

Front 41

in Autoimmune hemolytic anemia, RC's are:

(2)

Back 41

1. polychromic

2. microspherocytes

Front 42

CBC includes:

Back 42

Hb, Hct, RBC count, MCV

- as well as MCH, MCHC, and RDW

Front 43

Hct =

Back 43

% of RBC's/blood

Front 44

equation for Hct =

Back 44

RBC x MCV

Front 45

anemia = less ______________ than normal for age and gender

Back 45

Hb / Hct

Front 46

anemia is a ________________, not a disease

Back 46

manifestation of disease

Front 47

acute hemorrhage => anemai, via hypovolemia, which includes:

(4)

Back 47

1. hypotension

2. orthostatic changes

3. syncope

4. shock

Front 48

shock =

Back 48

loss of blood to heart, brain, kidneys

Front 49

also associated with anemia: hypoxia, or:

Back 49

difficulty in O2 reaching the tissues

Front 50

analogy: RBC's are:

Back 50

trucks delivering O2 to tissue

Front 51

body responses to anemia:

(5)

Back 51

1. HR goes up => inc. SV

2. reflex vasoconstriction (to offset hypovolemia)

3. kidneys retain salt and water

4. inc. in erythrocyte 2,3-DPG (=> right shift => inc. O2 to tissues)

5. inc. erythropoietin synth.

Front 52

erythropoietin =

Back 52

hormone produced by renal mesangial cells in response to low tissue O2,

that goes to the marrow and tells it to inc. RC production

Front 53

b/c kidneys retain salt/water in response to anemia, beware of:

Back 53

fluid overload with transfusions

Front 54

anemias are classified by:

Back 54

1. erythropoietic response

2. MCV and Hb [ ]

Front 55

erythropoietic response =

Back 55

reticulocyte count

Front 56

erythropoietic response/reticulocyte count is a measure of:

Back 56

marrow function

Front 57

erythropoietic response is either:

Back 57

hypo- or hyperproliferative

Front 58

(hypoproliferation indicates:

Back 58

marrow isn't working right or lacks the nutrients to make new RC's)

Front 59

reticulocytes are:

Back 59

young RC's immediately released from marrow

- the end-result of erythropoiesis

Front 60

**reticulocytes are poluchromatophilic, meaning they are:**

Back 60

grayish-blue

Front 61

reticulocyte index =

Back 61

reticulocyte count x Hct/ideal Hct x 0.5

Front 62

absolute reticulocyte count =

Back 62

retic. % x RBC count

Front 63

a retic index of <2% or ARC of <75,000 indicates a problem with:

Back 63

RC production

- i.e. a hypoproliferative abnormality

Front 64

a retic index of >2% or ARC > 100,000 indicates good marrow function, and therefore suggests that the cause of anemia is:

Back 64

blood loss or RC destruction (hemolysis)

Front 65

MCV of <80 =

Back 65

microcytic anemia

~~ Hb synth problem

Front 66

MCV of >100 =

Back 66

macrocytic anemia




- megaloblastic

Front 67

macrocytic anemia is divided into:

Back 67

megaloblastic

or

non-megaloblastic

Front 68

megaloblastic macrocytic anemia is a result of:

Back 68

impairment of DNA synthesis

- cells can't divide properly

Front 69

treatment of anemia:

(2)

Back 69

1. UC

2. transfusions not always the right call

Front 70

**there is NO standardized:

Back 70

"must-transfuse" number

- there are ceilings though

Front 71

3 indications to transfuse:

Back 71

1. CV compromise (shock, angina, CHF)

2. hypoproliferative anemia with no/prolonged recovery

3. OR/surgery

Front 72

nutrients => bone marrow =>

Back 72

new RBC's => circulation

Front 73

megaloblastic anemias are characterized by defects in:

Back 73

DNA synthesis

Front 74

features of RC's in megaloblastic anemias:

(3)

Back 74

1. immature nucleus

2. inc. MCV

3. hypersegmented PMN's

Front 75

megaloblastic anemia is most often caused by:

Back 75

B12 / folate deficiencies

Front 76

non-heme presentations of megaloblastic anemias =

Back 76

1. beefy, red tongue

2. neuro/psych problems (ONLY in B12 deficiency)

Front 77

B12 features:

(2)

Back 77

1. aka cobalamine

2. comes only from animal prot's

Front 78

B12 absorption pathway: binds to:

Back 78

salivary R prot in acidic conditions

- parietal cells secrete IF

- pancreatic enzymes degrade R => B12 binds IF

Front 79

B12-IF is absorbed only in:

Back 79

***the distal ileum***

Front 80

B12 is transferred to tissues by:

Back 80

transcobalamine II

Front 81

causes of B12 deficiency:

(6)

Back 81

1. vegan diet

2. pernicious anemia (loss of parietal cells = no acid)

3. gastric bypass (can't make IF)

4. Crohn's disease (dec. absorption)

5. metformin (dec. absorption)

6. tape worms/organisms (compete for B12)

Front 82

diagnosis of B12 deficiency:

(3)

Back 82

1. low serum B12 (bad test - want to confirm with other two)

2. inc. homocysteine

3. inc. methylmalonic acid

Front 83

B12 deficiency takes ______ to develop

Back 83

years

- storage is great

Front 84

treatment of B12 deficiency generally requires:

Back 84

life-long IM injections of B12

Front 85

treating a B12-deficient pt with folate alone =>

Back 85

neuro/psych problems

- steals B12 away from myelin pathway

- neuro/psych problems might never go away, due to how long it takes for myelin to develop

Front 86

folic acid is absorbed through:

Back 86

every part of the SI

Front 87

normal amounts of folic acid:

Back 87

100 mcg for adults, 400 for preg, more for conditions with active cell turnover

Front 88

what do you do with excess folic acid?

Back 88

pee it out

Front 89

deficiencies in folic acid are seen only after:

Back 89

a couple of months - storage isn't great

Front 90

iron uptake depends mostly on:

Back 90

iron stores

- low stores = decreased absorption

Front 91

analogy: liver =

Back 91

iron savings,

RBC's = iron checking

Front 92

transferrin =

Back 92

iron transporter

Front 93

TIBC =

Back 93

total iron binding capacity

= 300 mcg Fe/dL

= amount that transferrin can carry

Front 94

what happens to TIBC when you're iron-deficient?

Back 94

it increases

Front 95

transferrin saturation =

Back 95

fraction of available iron-binding sites

- decreases when you're iron-deficient

Front 96

iron is predominantly stored in which protein?

Back 96

ferritin

Front 97

ferritin levels _____________________ when you're iron-deficient

Back 97

decrease a lot

Front 98

**iron leaves the body only when:**

Back 98

cells are lost

Front 99

the liver sends iron to the:

Back 99

marrow to make RBC's

Front 100

iron loss is most commonly seen in:

(3)

Back 100

1. blood loss

2. preg/lact

3. blood loss

Front 101

transfusions: one unit of blood transfusions =>

Back 101

inc. in Hb by 1 g/dL

(in adult of normal size)

Front 102

in the case of iron deficiency, iron stores will be used *before*

Back 102

Hb's iron

- iron stores, then transport iron, then Hb iron

Front 103

clinical presentation of iron deficiency:

(8)

Back 103

1. s/ts asymp, s/ts anemic

2. glossitis

3. sore on corners of mouth (angular chelitis)

4. koilonychia (spoon)

5. Pica

6. thrombocytosis

7. hair loss

8. RLS

Front 104

first sign of iron deficiency =

Back 104

inc. in RDW, followed by dec. in MCV

- Hb and Hct fall last

Front 105

**best measure of iron deficiency in otherwise healthy pt. =

Back 105

ferritin

(<15 = Iron-def)

Front 106

anemia can occur in chronic diseases b/c cytokines inc:

Back 106

hepcidin

Front 107

what does hepcidin do?

(3)

Back 107

1. dec. iron absorption from gut

2. dec. export from the liver

3. dec. transferrin and TIBC

Front 108

sum effect of hpcidin =

Back 108

**decreased serum iron**

Front 109

in AoCD's, ferritin can be:

Back 109

normal or elevated

Front 110

treatment of iron deficiencies:

(2)

Back 110

1. UC

2. oral iron supplements

Front 111

SE's of oral iron supplements =

(2)

Back 111

1. black stool

2. GI issues

Front 112

3 random things that block iron absorption:

Back 112

1. tea

2. egg yolks

3. whole grains

Front 113

RDW measures:

Back 113

variation in size of cells

Wide RDW ~~ wide cells and small cells

Front 114

Hb = a:

Back 114

tetramer

- 2 alpha chains, 2 B chains

- each chain's globin includes iron

Front 115

right shift =

Back 115

dec affinity for O2

=> inc. off=loading into tissue

Front 116

what causes a right-ward shift?

(3)

Back 116

1. inc. T

2. inc. 2,3-DPG

3. inc. [H+]

Front 117

a2y2 =

Back 117

HbF

Front 118

a2d2 =

Back 118

HbA2 (minor)

Front 119

a2B2 =

Back 119

HbA

Front 120

thalassemia =

Back 120

globin chain imbalance (i.e. NOT a2B2)

Front 121

clinical presentations of thalassemias are characterized as:

Back 121

minor (very mild symptom's, if any)

intermedia,

and major (pts are transfusion-dependent)

Front 122

B-thalassemias tend to appear in:

Back 122

Med Deli folk

Front 123

B-zero =

Back 123

*absent* B-globin chain synth

Front 124

***B+ thalassemia ~~***(

Back 124

*decreased* B-globin synthesis

Front 125

thalassemia major = deficiency in B-globin => relative excess of a-globin =>

Back 125

instability => ineffective erythropoiesis, destruction of brand-new RC's

=> MORE bad RC's made

=> splenomegaly

=> sequestration

=> worsening anemia

=> marrow expansion to meet demand for O2

=> skeletal deformaties (including frontal bossing and fractures)

Front 126

in thalassemia major, there is a danger of iron overload due to:

Back 126

transfusions or yper-absorption of iron from gut

Front 127

thal-major is also called:

(2)

Back 127

B-zero/B-zero

or

Cooley's

(severe anemia between 2 and 12 mths of life)

Front 128

B+/Bzero or B+/B+ = thal-intermedia: infants are:

Back 128

fine at birth, since HbF and HbA predominate

Front 129

treatment of B-thals:

(3)

Back 129

1. transfuse regularly

2. chelate to prevent iron overload

3. pot. stem cell transfer

Front 130

Hb is a:

Back 130

tetramer

- 2 alpha chains, 2 B chains

- each chain's globin includes iron

Front 131

right shift ~~

Back 131

dec. affinity for O2 = inc. off-loading into tissue

Front 132

alpha thal's ~~ which 2 people groups?

Back 132

1. SE Asians

2. Africans

Front 133

what is the typical genotype of Africans with alpha-thal?

Back 133

a - / a -

Front 134

what is the typical genotype of Asians with alpha-thal?

Back 134

a a / - -

Front 135

aa/a- genotype features:

(thal-minor)

Back 135

1. typically silent with minimal microcytosis

2. anemia is NOT present

3. electrophoresis is normal

Front 136

thal-intermedia features:

(aa/-- or a-/a-)

(3)

Back 136

1. mildly anemic (Hb = 10-11)

2. MCV ~ 70 (microcytic)

3. electrophor. is normal

Front 137

a-/-- genotype features

(thal-major)

(3)

Back 137

1. low MCV

2. high RDW

3. wide variation in presentation

Front 138

a-/-- thalassemia is also called:

Back 138

HbH disease

Front 139

what is HbH?

Back 139

B4 tetramers

Front 140

in HbH disease, HbH ppts out, forming:

Back 140

Heinz bodies => bite cells => hemolytic anemia

- splenomegaly also occurs

Front 141

what is the condition that --/-- genotype causes?

Back 141

hydrops fetalis

- NO alpha chains

- only y4 chains

=> intrauterine death, stillbirth

Front 142

what's another name for y4 Hb?

Back 142

Hb Barts

Front 143

hydrops fetalis CAN be:

Back 143

treated, in utero, by exchange transfusions

Front 144

HbS is the result of:

Back 144

6th AA in the BETA globin chain going from Glutamate to Valine (hydrophobic)

=> sickle cells

Front 145

sickle cells polymerize in microvasculature during:

Back 145

hypoxia, acidosis

- depolymerize when blood returns to lungs

=> cycles of polym/depolym

Front 146

cycles of sickle cell polym/depolym =>

Back 146

cell dehydration

=> threshold for sickling steadily decreases => irreversibly sickled => *obstruction*

Front 147

meanwhile, b/c sickle cells aren't as robust, they will:

Back 147

lyse

Front 148

ANOTHER problem with sickle cells:

Back 148

abnormally sticky to endo cells

Front 149

a de-oxygenated sickle cell is 50x less ______________ than deoxy HbA

Back 149

soluble

Front 150

3 genotypes of sickle cells:

Back 150

1. SS

2. SC

3. Sickle-/B-thal

Front 151

SC disease =

Back 151

SCA

Front 152

in SC disease, the Hb that's made is HbC, due to:

Back 152

change from glutamate to lysine

~~ W. Africa

Front 153

S-B-zero is ________________ from SS

Back 153

*indistinguishable*

S-B+ is milder

Front 154

hematologic manifestation of SCA =

Back 154

chronic hemolysis - anemia

Front 155

if a SCA pt's Hb falls below baseline, you need to start worrying about the following complications:

(3)

Back 155

1. parvovirus B19 inf (aplastic crisis)

2. splenic/hepatic sequestration

3. ACS

Front 156

2 other hematologic manifestations of SCA:

Back 156

1. thrombocytosis (=> inc. risk of venous clots)

2. pts. are functionally asplenic by adulthood

Front 157

SCA crises:

(4)

Back 157

1. hemolytic crisis (rare)

2. splenic sequestration crisis

3. aplastic crisis

4. vaso-occlusive crisis (most common)

Front 158

splenic sequestration crisis is common in children; what happens?

Back 158

profound anemia, hypovolemic shock (spleen takes nearly all circulation)

- need transfusion immediately

Front 159

aplastic crisis ~~

Back 159

parvovirus B19 inf => marrow suppression => dec. production of RC's => anemia

Front 160

vaso-occlusive crisis is due to ____________ and results in ____________________________

Back 160

ischemia;

pain in bones/joints

Front 161

vaso-occlusive crises of SCA are triggered by:

Back 161

exercise, cold, surgery, inf, dehydration, preg, stress, menstruation

Front 162

treating crises:

(3)

Back 162

1. UC

2. relieve pain

3. look out for aplastic crisis, ACS

Front 163

SCD => inc risk of:

Back 163

inf, especially to encapsulated bacteria

Front 164

SCD also ~~ inc. risk of inf with ______________, if being chelated

Back 164

Yersinia

(no seafood)

Front 165

respiratory complications of SCD:

(2)

Back 165

1. ACS

2. PHTN

Front 166

ACS = NEW sighting of:

(6)

Back 166

hypoxemia,

infiltrate on CXR,

fever,

chest pain,

dyspnea,

cough

Front 167

ACS is the most common:

Back 167

cause of death of SCA pts

Front 168

ACS is usually caused by:

Back 168

atypical org's like chlamydia and mycoplasma

- or by fat embolism

Front 169

treatment of ACS =

(3)

Back 169

1. **transfusions to lower HbS [ ]**

2. antib's

3. O2

Front 170

PHTN occurs in ______ of SCD adults

Back 170

1/3

=> substantial morbidity, death w/in 2 years

Front 171

treat for PHTN:

Back 171

**there is NO GOOD TREATMENT.**

Front 172

**median age of stroke in SCD =

Back 172

5 yo**

Front 173

is SCD stroke is acute, treat with:

Back 173

exchange transfusion

Front 174

is SCD stroke is chronic, treat with:

Back 174

regular transfusion

- can actually prevent it using doppler and finding obstruction in the CoW

Front 175

2 complications of blood transfusion:

Back 175

1. iron overload

2. allo-immunity

Front 176

iron is toxic; it causes:

Back 176

organ damage (heart, liver, endocrine failure)

Front 177

treat iron toxicity with:

Back 177

chelation

Front 178

allo-immunization =

Back 178

immune response against antigens from same species

Front 179

AfAm's have to avoid 3 particular blood groups:

Back 179

C, E, Kell

Front 180

other complications of SCA:

(4)

Back 180

1. renal

2. leg ulcers

3. avascular necrosis

4. priapism (inf that won't go away)

Front 181

AVN =

Back 181

death of tissue due to lack of blood supply

(usually in shoulders/hips)

Front 182

**best treatment for SCD =

Back 182

hydroxyurea

Front 183

**what does hydroxyurea do?**

Back 183

INC's HbF, thereby dec. [HbS]

Front 184

other effects of hydroxyurea:

(3)

Back 184

1. dec. crises

2. dec, episodes of ACS

3. PREVENTS PHTN

Front 185

SE of hydroxyurea:

Back 185

bone marrow suppression

Front 186

hydroxyiurea is contra-indicated in:

(3)

Back 186

1. preg

2. poor/erratic compliance

3. allergy

Front 187

ONLY cure for SCD =

Back 187

BMT

but 1/3 mortality rate upfront

Front 188

SC trait =

Back 188

one SC gene and one normal B globin gene

Front 189

features of SC trait:

(3)

Back 189

1. normal heme parameters

2. only a problem is you become severely hypoxic

3. may have renal issues

Front 190

renal issues in SC trait take the form of:

(2)

Back 190

1. isosthenuria

2. microscopic hematuria

Front 191

HbC disease features:

(4)

Back 191

1. microcytic

2. target cells

3. splenomegaly

4. mild hemolysis

Front 192

pregnant people with SCA are at a greater risk for:

(3)

Back 192

1. pain crises

2. PHTN

3. inf's

Front 193

surgery is riskier for people with SCD; reduce risk by:

Back 193

reducing [HbS] with *transfusion*