Red Blood Cell Disorders/anemias

Front 1

where does Fe deficiency "act" in the paradigm of causing anemias

Back 1

can't even get Fe into Macrophages

Front 2

where does anemia of chronic disease 'act' in the paradigm of causing anemia

Back 2

limits transfer of iron from macrophages to rbcs (can't get into rbcs but accumulates in macrophages)

Front 3

where does sideroblastic anemia act in the paradigm of causing anemia

Back 3

can't incorporate iron into hemoglobin molecules so it accumulates in the mitochondria of rbcs in a perinuclear distribution (ringed sideroblasts)

Front 4

name four examples of intracorpuscular defects in causes of hemolytic anemia and are they hereditary vs. acquired

Back 4

1. Hereditary spherocytosis
2. G6PD
3. hemoglobinopathies (quantitative and qualitative)
4. Paroxysmal nocturnal hemoglobinuria (ACQUIRED)
(THE REST ARE HEREDITARY)

Front 5

name 3 categories for decreased rbc production causing anemia with examples for each

Back 5

1. stem cell failure (aplastic anemia, pure red cell aplasia)
2. nutritional (iron, folate, b12)
3. multifactorial (anemia of CI, sideroblastic, marrow infiltration, renal dz. HIV anemia, congenital dyserythropoietic anemias)

Front 6

what is the single most useful test in evaluating anemias

Back 6

reticulocyte count

Front 7

what stains are needed for reticulocyte counts

Back 7

- classically done on peripheral smear
- need supravital dye (methylene blue) to stain RNA

Front 8

some formulas: reticulocyte percentage

Back 8

# retics/1000 rbcs (misleading in anemia as rbcs go down and artificially elevate the number)

Front 9

some formulas: absolute reticulocytes

Back 9

% counted x (patient HCt/normal HCt)

Front 10

some formulas: reticulocyte production index (RPI)

Back 10

absolute reticulocytes/2
(% retics counted x pt Hct/nl Hct (45))/2

accounts for the fact that basophilic young retics take twice as long to develop into mature rbcs

Front 11

to conclude the anemia is primarily due to increased destruction, what would you expect the RPI to look like

Back 11

>2

Front 12

to conclude the anemia is due to decreased production, what would you expect the RPI to be

Back 12

<2

Front 13

what are modern techniques for measuring total/absolute retic count by automation (2)

Back 13

1. fluorochrome
2. flow

Front 14

what are three ways to gauge the size of an rbc

Back 14

- direct measurement (flow/forward scatter, electrical impedance)
- calculation (Hct/rcc)
- visualize on smear

Front 15

what is the MCV in microcytic anemia

Back 15

<80fl

Front 16

what is the MCV in macrocytic anemia

Back 16

>100fl

Front 17

what does a low ferritin means

Back 17

low iron stores in liver, spleen, marrow

Front 18

what are the results of the following studies in iron deficiency:
serum fe, TIBC, % sat, ferritin, S Tf R, HbA2

Back 18

serum fe: low
TIBC; increased
% sat: very reduced
ferritin: low
S Tf R: increased (upregulated receptors)
HbA2: low

Front 19

what are the results of the following studies in anemia of chronic disease:
serum fe, TIBC, % sat, ferritin, S Tf R, HbA2

Back 19

serum Fe: low (stuck in macrophages)
TIBC: down (not clear why)
% sat: nl or increased
ferritin: increased stores (just can't get out)
S Tf R: nl (no upregulation b/c no real decrease in Fe)
HbA2: nl

Front 20

when do you see in an increase in serum transferrin receptor

Back 20

upregulation of receptor (increase) with immature erythroid cells:
- iron deficiency
- hemolysis
- hyperactive erythropoiesis
- PV
* most useful in distinguishing between iron deficiency anemia and anemia of chronic inflammation

Front 21

where do you see a mild increase in HbA2

Back 21

b thal minor (makes sense: extra alpha chains around)

Front 22

when do you see a decrease in HbA2

Back 22

sideroblastic anemia (can't incorporate fe into globins so see slight decrease in HbA2)

Front 23

name 6 causes of microcytic anemia

Back 23

- iron deficiency anemia
- sideroblastic anemia
- anemia of chronic disease (usually normocytic but can be microcytic)
- thals (minor and major)
- HbE
- hyperthyroidism

Front 24

what does microcythemia?

Back 24

small rbcs

Front 25

where do you see microcythemia

Back 25

thal minor - increase RCC, so MCV/RCC is very low, usually less than 13

Front 26

what is arguably the best test for early iron deficiency

Back 26

reticulocyte hemoglobin concentration (by flow)

Front 27

where do you see an increase in free eryhtrocyte protoporphyrin and where don't you

Back 27

increased in casese where there is a reduced incorporation of Fe into heme, this is increased in most cases of anemia exc in thalassemias (?)

Front 28

name two categories of macrocytic anemias and what you might find in those categories

Back 28

1. megaloblastic anemia (oval macrocytes) - b12, folate, others (rare)
2. other causes (round macrocytes)
- liver disease, EtOHism, reticuloctosis, hypothyroidism, bone marrow disease (aplasia, MDS, infiltration)

Front 29

what is it

Back 29

spherocytes: loss of membrane but not volume, lose biconcave shape/central pallor
Increased MCHC

Front 30

causes of spherocytes

Back 30

hereditary spherocytosis
think extravascular hemolysis
warm AIHA
old blood
burns, venoms

Front 31

what is MCHC

Back 31

mean corpuscular Hb concentration - concentration of hb given volume of rbcs (hgb/hct)

Front 32

what is it

Back 32

elliptocytes

Front 33

what is it

Back 33

schistocytes fragments

includes helmet cells and horn cells (looks like keratocytes)

Front 34

what is it

Back 34

echinocytes (burr cells)
regular sharp projections

Front 35

what is it

Back 35

acanthocytes (spur cells)
blunt, few, irregular projections
LIVER disease
also: ab-lipoproteinemia, hypothyroidism, post-splenectomy, McLeod phenotype (no Kx), MDS

Front 36

what is it

Back 36

stomatocytes:
mouth/slit like central pallor
may be nonspecific - can be inherited (univalent N/K leak), Rh null phenotype
may be associated with pseudohyperkalemia

Front 37

what is it

Back 37

codocytes (target cells)
relative surplus of membrane, decreased osmotic fragility (cf spherocytes)
causes: liver disease (esp obstructive), hemoglobinopathies (hbC esp), asplenia, Fe deficiency, megaloblastic anemia

Front 38

what is it

Back 38

codocytes (target cells) and clams
think HgC

Front 39

what is it

Back 39

bite cells, can be subtle, may be accompanied by blister cells

Front 40

what is it

Back 40

dacrocytes (tear drops)
typically in myelofibrosis
may be nonspecific (including thals)

Front 41

what is it

Back 41

punctate basophilia (basophilic stippling)
small blue dots, regularly dispersed
reflect RNA (if fine, may be reticulocytes), or RNA complexes (course and more likely pathologic)
causes: lead poisoning, hemoglobinopathies (thals and unstable Hb), inherited pyrimidine 5 nucleotidase deficiency, MDS, infection

Front 42

what is it

Back 42

Howell Jolly body
nuclear remnant, dense, magenta and may feel like you have to go in and out of plane
typically seen in asplenia/post-splenectomy
also in megaloblastosis and MDS (perturbed DNA syn)

Front 43

two major causes of hyposplenism

Back 43

sickle cell disease
celiac disease

Front 44

what is it

Back 44

Pappenheimer body
multiple irregularly located (cf basophilic stippling) blue dots
reflect iron (asplenia or iron overload)

Front 45

what is it

Back 45

Heinz body
not seen on regular wright-giemsa stain, requires supravital dy (ex crystal violet) to see
causes: denatured Hb, typical of G6PFD or unstable hemoglobins)

Front 46

what is dimorphism on peripheral smears

Back 46

two discrete populations of RBCs, differing size (see MCV)
see in sideroblastic anemia (but also in treated microcytic anemia and macrocytic anemia as well as post transfusion)

Front 47

what is it

Back 47

rouleaux
loss of zeta potl on rbcs
due to increase in large, asymmetrical proteins in plasma (fibrinogen, Igs)
is the smear equivalent of an elevated ESR

Front 48

what is it

Back 48

autoagglutination of rbcs
cause: cold autoab, typically IgM
spurious RBC indices,"", need to keep specimen warm

Front 49

what is the solution for autoagglutination

Back 49

keep specimen warm

Front 50

what is it

Back 50

P. falciparum - only see ring forms and then banana-shaped gametocytes (no merozoites or schizonts); recognize in P falciparum the ring forms are smaller(<.33 of rbcs); often double dot chromatin (headphones), delicate (no amoeboid forms)

Front 51

what is it

Back 51

babesiosis
often 4 merozoites forming Maltese cross
- no schizonts gametocytes, pigment

Front 52

what spreads Babesiosis and what is this similar to

Back 52

Ixodes dammini (same tick as spreads Lyme disease)

Front 53

causes of elliptocytes

Back 53

loss of elasticity in hereditary elliptocytosis (not specific for HE though) - can look like cigar cell/pencil cells of iron deficiency anemia

Front 54

causes of schistocytes

Back 54

indicates traumatic intravascular hemolysis (microangiopathy)

Front 55

causes of echinocytes

Back 55

burr cells
associated with ARF, but may be mimicked by prolonged EDTA, acidic slides, prickel cells seen in pyruvate kinase deficiency (esp post splenectomy)

Front 56

causes of bite cells

Back 56

due to munching of Heinz body (not apparent on wright giemsa)
typical of oxidant induced damage, such as G6PD deficiency

Front 57

causes of dacrocytes

Back 57

myelofibrosis

Front 58

intracorpuscular defects resulting in hemolytic anemia: membrane/cytoskeleton defects

Back 58

HS
HE
Hereditary stomatocytosis
PNH

Front 59

intracorpuscular defects resulting in hemolytic anemia: enzymopathies

Back 59

G6PD
pyruvate kinase
pyrimidine 5' nucleotidase
glutathione synthetase

Front 60

intracorpuscular defects resulting in hemolytic anemia: hemoglobinopathies

Back 60

quantitative (thalassemia)
qualitative (abnormal variants affect solubility, stability, O2 affinity, etc)

Front 61

inheritance pattern of HS

Back 61

Autosomal dominant

Front 62

molecular problem(s) with HS

Back 62

heterogenous, usually ankyrin mutation with secondary spectrin deficiency (recall vertical defect)

Front 63

cellular explanation of HS

Back 63

loss of membrane, less deformable, splenic trapping

Front 64

sx of HS

Back 64

- moderate splenomegaly
- gallstones
- aplastic crises

Front 65

what might you use to make the dx of HS

Back 65

- family hx
- spherocytes
- increased MCHC,
- osmotic fragility
- autohemolysis

Front 66

what is the altered biochemistry in G6PD deficiency

Back 66

- enzyme in hexose monophosphate shunt, generates reducing power (NADPH, GSH) to protect against oxidative damage (H2O2)

Front 67

what is the wild type for G6PD deficiency

Back 67

B

Front 68

what are the four categories of G6PD

Back 68

1. chronic hemolysis
2. moderately severe hemolysis (Mediterranean, 70% Kurdish Jews)
3. mild hemolysis (10% african-americans, A-)
4. no functional affect (A+)

Front 69

why is the G6PD deficiency genetically persisted

Back 69

resistance to P. falciparum

Front 70

how is G6PD inherited

Back 70

X linked recessive, mutations lead to abnl protein folding

Front 71

what is the pathogenesis underlying G6PD

Back 71

oxidation of SH groups on globin, Hb denatures, ppts out as Heinz bodies, intravascular hemolysis (membrane damage) nd extravascular hemolysis (bite cells, blister cells, spherocytes)

Front 72

how is G6PD def diagnosed

Back 72

direct enzyme quantitation, or dye reduction tests (using brilliant cresyl blue or fluorescent spot test with UV light)

Front 73

what is a nuance to G6PD testing one should be aware of

Back 73

reticulocytes have "normal" amounts of protein activity so do assays after hemolytic episode or get rid of retics before testing

Front 74

which hemoglobinapathy affects Hb solubility

Back 74

HgS

Front 75

which hemoglobinapathy affects Hb stability

Back 75

Hb Zurich

Front 76

which hemoglobinapathy affects O2 affinity

Back 76

Hb Chesapeake

Front 77

which hemoglobinapathy affects ionic state of Fe in heme, the methemoglobinemias

Back 77

HbM Boston

Front 78

in standard gel electrophoresis for hemoglobinopathies, what is the first gel type you run

Back 78

cellulose acetate (alkaline pH 8.5)

Front 79

in standard gel electrophoresis for hemoglobinopathies, what could be a second gel you might run and why

Back 79

citrate agar (acid Ph) - used to help separate out the C and S hemoglobins

Front 80

in standard gel electrophoresis for hemoglobinopathies, what is the standard migration pattern for a cellulose acetate agar (alkaline

Back 80

A Fat Santa Claus
recall travels from neg to pos and "a" is faster than C

Front 81

in standard gel electrophoresis for hemoglobinopathies, some other variants seen (besides A Fat Santa Claus

Back 81

Barts, J, A2', CS

Front 82

in standard gel electrophoresis for hemoglobinopathies, if a citrate agar is run (acid ph), what is the comigration we care about

Back 82

ADAGE, so F runs fastest, then ADAGE, then O at origin and C and S run towards negative pole from center-based origin

Front 83

how are thals named

Back 83

named for the decreased/absent chain; even though left over chain is the one that wreaks the most havoc

Front 84

what does major and minor mean in the thals

Back 84

major - two genes defective
minor - one gene defective

Front 85

what would Hb electrophoresis look for in Bo thal major

Back 85

HbF 98
HbA2 2

Front 86

what would Hb electrophoresis look for in B+ thal major

Back 86

HbF 80
HbA 20
var A2

Front 87

what would Hb electrophoresis look for in Hb Lepore

Back 87

Hb F 80, AbN 20

Front 88

what is the genotype for B0 thal major

Back 88

Bo/Bo

Front 89

what is the genotype for B+ thal major

Back 89

B+/B+

Front 90

what is the genotype for Hb Lepore

Back 90

delta-beta fusion

Front 91

what would Hb electrophoresis look for in delta-betah thal

Back 91

Hb F 100

Front 92

what would Hb electrophoresis look for in alpha tha trait

Back 92

normal or maybe A2 reduction

Front 93

what would Hb electrophoresis look for in HbH

Back 93

Hb A 80
Hb F reduced
Hb A2 reduced
AbN 5-30

Front 94

what would Hb electrophoresis look for in alpha thal major

Back 94

abN 100 (hydrops fetalis - not compatible with life)

Front 95

what would Hb electrophoresis look for in Hb Constant Spring

Back 95

normal but 5-8 AbN

Front 96

which have the highest severity of sx on thals

Back 96

Bo thal major, B+ thal major, Hb Lepore (variable), alpha thal major (incompatible with life)

Front 97

generalizations re: alpha thal on genetics, ethnicity, solubility of excess chains, severity

Back 97

genetics: usually deletions
ethnicity: chinese/africans
solubility of excess chains: moderately reduced
severity: moderate

Front 98

generalizations re: beta thal on genetics, ethnicity, solubility of excess chains, severity

Back 98

genetics: point mutations/splicing
ethnicity: mediterraneans, south asians
solubility of excess chains: very reduced solubity
severity: worst

Front 99

causes of Sickle cell disease beyond homozygous HbSS

Back 99

SC, SD, Sbthal

Front 100

pathogenesis of sickle cell disease

Back 100

deoxygenation, polymerization, distorted rbc shape, secondary membrane changes (flip flop phospholipids, increase adhesion molecules, microvascular occlusion, ischemia)

Front 101

sx of sickle cell disease

Back 101

severe anemia (20-30) with
multiorgan ischemia
gallstones
increased infections (see separate flashcard)
crises

Front 102

increased susceptibility to infections is seen in sickle cell disease. which ones

Back 102

pneumococcus, hemophilus, salmonella

Front 103

what virus can provoke crises/aplasia in sickle cell disease patients

Back 103

parvovirus

Front 104

what is useful in the diagnosis of sickle cell disease

Back 104

clinical/family hx
peripheral blood smear
screening solubility (dithionite)
sickling (metabisulfite) tests
hb electrophoresis

Front 105

what is the % S in a simple heterozygote SA

Back 105

35-45%

Front 106

what is the % S in a dobule heterozygote

Back 106

25-30% (less S than baseline simple SA of 35-45%)

Front 107

what is the % S in a double heterozygote S-b+thal

Back 107

55-65% (more S than in baseline SA of 35-45%)

Front 108

what is the % S in a double heterozygote S-Bo thal

Back 108

>80% way more S than all others

Front 109

geographic distribution, sx and other details pertaining to: HbE

Back 109

- most common hemoglobinopathy worldwide: seen in SE asia (cambodia, thailand, Laos)
- microcytosis/mild anemia
- if double heterozygote with b thal leads to thal major

Front 110

geographic distribution, sx and other details pertaining to: Hb C

Back 110

west africans,
mild anemia in homozygotes
prominence of target cells ("C"odocytes), crystals, clams
if HbSC: see pita bread, gull wing cells

Front 111

geographic distribution, sx and other details pertaining to: HbM

Back 111

iron in ferric form (Fe3+) leads to methemoglobinemia and cyanosis: boston, Iwate, Saskatoon, Milwaukee

Front 112

unstable Hb can include what, is associated with what on peripheral smear, can be diagnosed with what

Back 112

- Torino, Hasharon, Zurich
- associated with formation of Heinz bodies
- diagnose with heat instability test, isopropanol or n-butanol test

Front 113

if hb reduces O2 affinity what happens and what are some ex of hb that do that

Back 113

- develop cyanosis
- examples: Kansas, Providence, Seattle, Beth Israel

Front 114

If Hb has an increased affinity for O2 what happens and what are some examples

Back 114

polycythemia
examples: J cape town, Chesapeake, Malmo, Bethesda

Front 115

is paroxysmal nocturnal hemoglobinuria intracorpuscular or extracorpuscular; inherited or acquired

Back 115

intracorpuscular and acquired (the only one)

Front 116

what are the underpinnings of paroxysmal nocturnal hemoglobinuria

Back 116

-Mutant PIG-A gene leading to absence of GPI anchor with loss of many proteins (including CD55, CD59, CD66, Cd14, CD16, CD48 and CD52)
- loss of CD55 and CD59 leads to increased complement mediated lysis

Front 117

what are patients with PNH at risk for

Back 117

aplastic anemia, AML, MDS
budd chiari syndrome

Front 118

which does PNH target rbcs, plts or wbcs

Back 118

all three

Front 119

what is the most common cause of morbidity/mortality in patients with PNH

Back 119

budd-chiari syndrome (recurrent venous thrombosis in hepatic veins - see abdominal pain, ascites, hepatomegaly)

Front 120

does hemolytic anemias usually lead to iron deficiency

Back 120

no, but interestingly PNH does b/c you pee out the iron

Front 121

what are the ways to diagnose PNH

Back 121

- Flow
- HAM test (looking for hemolysis)
- LAP score (low=GPI)??

Front 122

what are four mechanisms by which drugs could produce anemia through extracorpuscular means

Back 122

- Immune:
- hapten (drug binds to rbc membrane)
- ternary complex
- autoab
Non immuune:
direct rbc membrane damage

Front 123

warm antibodies produce AIHA through intravascular or extravascular mechanisms

Back 123

extravascular (spherocytosis)

Front 124

cold abs produce AIHA through extravascular or intravascular mechanisms

Back 124

both

Front 125

what is the underpinning of aplastic anemia

Back 125

due to stem cell failure loss or suppression

Front 126

numeric definitions of aplastic anemia:
- pmns
- plts
- retics
- bM

Back 126

- pmns: <500ul
- plts: <20x10^9
- retics: <1%
- bM: <25%

Front 127

name two congenital causes of aplastic anemia

Back 127

- fanconi
- dyskeratosis congenita

Front 128

what is the mode of transmission for fanconi anemia

Back 128

autosomal recessive

Front 129

at what age does the cytopenia seen fanconi's arise

Back 129

5-10 years of age

Front 130

what are some sx (6) seen in fanconi's anemia

Back 130

- pancytopenia
- cafe au lait spots
- short stature
- absent thumb
- MR
- renal abnormalities

Front 131

what are those with fanconi anemia at risk for

Back 131

- AML
-MDS
- some solid tumors (cervical, vulva, basal cell, GI, breast, liver)

Front 132

what are three ways in which fanconi can be identified

Back 132

-- flow
- breakage studies
- molecular

Front 133

what is a congenital cause of pure red cell aplasia

Back 133

diamond-blackfan

Front 134

what are some sx of diamond-blackfan

Back 134

pure red cell aplasia with
-craniofacial malformations,
-thumb or upper limb abnormalities,
cardiac defects,
urogenital malformations
-cleft palate. Low birth weight

Front 135

acquired causes of pure red cell aplasia

Back 135

autoimmune
thymoma
heme malignancies
infections (ex. parvovirus, HIV, hepatitis, EBV)
drugs (phenytoin, azathioprine, INH)
nutritional (B12, folate, riboflavin)

Front 136

most common cause of nutritional deficiency

Back 136

iron deficiency

Front 137

what is hemosiderin

Back 137

degraded protein shell from ferritin (the long term storage form of Fe), stains with Prussian blue, Perl

Front 138

what does hemosiderin stain with

Back 138

Prussian blue/Perl

Front 139

where is iron absorbed

Back 139

duodenum

Front 140

what is iron absorption regulated by (2)

Back 140

1. Divalent Metal Transporter 1
2. Product of HFE gene

Front 141

what is HFE gene and how does it relate to iron absorption

Back 141

gene product of HFE is a form of MHC lass I protein that is associated with the transferrin receptor and reduces the affinity of the transferrin receptor for Fe in the duodenum

Front 142

what are the two mutated forms of HFE and to what disease are they related

Back 142

Major C282Y and minor H63D, cause hemochromatosis

Front 143

what are some sx of iron deficiency anemia (5)

Back 143

- microcytic anemia
- pica
-koilonychia
- atrophic glossitis
- esophageal web

Front 144

what is Plummer-Vinson syndrome

Back 144

- microcytic anemia
- atrophic glossitis
-esophageal web

Front 145

how does iron deficiency affect peripheral smear outside of its effect on rbcs

Back 145

- thrombocytosis
- PMN hypersegmentation

Front 146

what are other causes of megaloblastic anemia are there besides B12 and folate deficiency

Back 146

- hereditary orotic aciduria
- Lesch-Nyhan syndrome
- thiamine responsive megaloblastic anemia

Front 147

what affect does megaloblastic anemia have on rbcs on peripheral smear

Back 147

- macroovalocytes
-tear drops
Howell-Jolly bodies
Cabot rings

Front 148

what affect does megaloblastic anemia have on other cell lines (besides rbcs) in peripheral smear

Back 148

- giant platelets
- hypersegmented and large PMNs

Front 149

what are megaloblasts

Back 149

earliest erythroid precursors that are enlarged with open chromatin and nucleoli

Front 150

bone marrow aspirate in iron deficiency anemia

Back 150

beware - don't diagnose as acute leukemia:
big megaloblasts developing into multinucleated, bizarre forms
granulocytes with giant bizarre forms
megas bizarrely multilobated

Front 151

what is a lab finding for megaloblastic anemia

Back 151

increased LDH (due to intramedullary deth)

Front 152

four ways to distinguish between folate and B12 deficiency

Back 152

B12 - neuro
B12 - infertility
time of onset: folate fast, B12 - years to develop)
etiology: B12 - absorptive problem; folate - decreased intake/increased requirements

Front 153

what is another name for B12

Back 153

cobalamin

Front 154

what two reactions are B12 involved in

Back 154

1. homocysteine to methionine (SAM)
2. methylmalonyl Coa to succinyl Coa

Front 155

where are the three sites of neurological complications in B12 deficiency

Back 155

1. posteriolateral columns of cor (subacute combined degeneration)
2. peripheral neuropathy
3. brain (megaloblastic madness)

Front 156

specific metabolites (related to B12 deficiency) that are thought to mediate neuro sx

Back 156

- SAM
- MMA
- propionic acid

Front 157

what is the more specific and what is more sensitive in evaluating for malabsorptive causes of B12 deficiency

Back 157

anti IF - specific (not sensitive)
anti-parietal cell ab sensitive (less spcific)

Front 158

where does folate participate biochemically

Back 158

one-carbon carrier in:
-purine synthesis
- conversion of homocysteine to methionine (this is B12 too)
- synthesis of dTMP for DNA synthesis
- in metabolism of FIGlu, product of histidine

Front 159

what two biochemical tests essentially excludes B12 deficiency

Back 159

MMA
Homocysteine

Front 160

what is the purpose of the Schilling test

Back 160

determine CAUSE of B12 deficiency but may need to partially treat B12 deficiency before doing Schilling test

Front 161

most common cause of anemia in hospitalized patients

Back 161

anemia of chronic inflammation

Front 162

underlying cellular/molecular process causing anemia of chronic inflammation

Back 162

-cytokines (impaired utilization of proper Fe stores - via hepcidin, IL6)
(also reduced EPO response, increased hemolysis, increased apoptosis in erythropoiesis)

Front 163

why does transferrin go down in anemia of CI

Back 163

it's a negative acute phase reactant

Front 164

what is sideroblastic anemia

Back 164

body has plenty of iron but can't incorporate it into hemoglobin

Front 165

can you find sideroblasts normally

Back 165

yes, type I sideroblasts (with 1-5 small granules) is considered "normal"

Front 166

what are causes of sideroblastic anemia

Back 166

- hereditary (xlinked - ex. mutation in erythroid specific ALA-synthase)

Front 167

look up pearson marrow pancreas syndrome/TRMA

Back 167

in context of sideroblastic anemia

Front 168

acquired causes of sideroblastic anemia

Back 168

- MDS
- drugs
-toxic (ex. lead, zinc, etoh)
- chronic diseases
- disturbance in B6 metabolism

Front 169

how does renal disease cause anemia

Back 169

- reduced EPO production
- hemolysis
- bleeding due to plt dysfunction
- iron deficiency from chronic hemodialysis
- anemia of CI

Front 170

how does HIV promote/cause anemia

Back 170

- inhibiting marrow function
- opportunitistic infections
- coincident neoplasia
- iatrogenic
- GI dz
- low EPO

Front 171

changes in BM of HIV patients

Back 171

-hypercellular (becomes hypocellular later in disease)
- dysplastic naed micromegakeratocytes (megs are CD4)
- plasma,lympho, histiocytosis
- granulomas
- serous or mucinous fat atrophy
- reticulin and collagen fibrosis

Front 172

what are congenital dyserythropoietic anemias

Back 172

collection of autosomal recessive d/os with ineffective erythropoiesis, anemia, cholelithiasis and iron overload

Front 173

where is the Ham test positive

Back 173

PNH
type II congenital dyserythropoietic anemia (HEMPAS)

distinction: in type II HEMPAS, lysis doesn't occur with acidified serum