Ascp Hematology Microcytic Anemia 1

Front 1

What are the microcytic anemias?

Back 1

Iron deficiency

Thalassemia

Anemia of chronic disease

Sideroblastic anemia

Zinc deficiency

Lead toxicity

Front 2

What is the storage protein for iron within cells called?

Back 2

Ferritin. Ferritin is an ubiquitous intracellular protein that stores iron and releases it in a controlled fashion. The protein is produced by almost all living organisms. In humans, it acts as a buffer against iron deficiency and iron overload. Ferritin is found in most tissues as a cytosolic protein, but small amounts are secreted into the serum where it functions as an iron carrier.

Front 3

Plasma ferritin is used as an __ __ of the total amount of iron stored in the body.

Back 3

indirect marker

Front 4

What organ serves as the major repository of stored iron in the body?

Back 4

the liver

Front 5

How is the amount of ferritin in the body related to the amount of stored iron?

Back 5

Directly related

Front 6

What is the name of the protein that transports iron throughout the body?

Back 6

transferrin

Front 7

Where is transferrin made in the body?

Back 7

the liver

Front 8

What is the primary route of transferrin throughout the body?

Back 8

From storage sites to the bone marrow

Front 9

What is the measure of the blood's capacity to bind iron with transferrin?

Back 9

TIBC (total iron binding capacity)

Front 10

Within cells, iron is stored in a protein complex known as __ or __.

Back 10

ferritin or hemosiderin

Front 11

__ binds to free ferrous iron and stores it in the ferric state.

Back 11

Apoferritin

Front 12

As ferritin accumulates within cells of the reticuloendothelial system (RES), protein aggregates are formed as __.

Back 12

hemosiderin

Front 13

Which iron is more readily available: Iron stored in ferritin or hemosiderin?

Back 13

ferritin

Front 14

Iron in ferritin or hemosiderin can be extracted for release by cells of the .... ?

Back 14

reticuloendothelial system, which includes macrophages/monocytes.

Front 15

Under steady state conditions, the serum ferritin level correlates with .... ?

Back 15

total body iron stores

Front 16

Since serum ferritin levels correlate with total body iron stores, the more convenient test for checking total body iron stores is ?

Back 16

serum ferritin FR5Rl

Front 17

Ferritin concentrations increase drastically in the presence of an __ or __.

Back 17

infection or cancer

Front 18

__ is an up-regulator of the gene coding for ferritin, thus causing the concentration of ferritin to rise as it builds up in the body.

Back 18

Endotoxin

Front 19

Ferritin is usually found within cells, but is also present in smaller quantities in the ?

Back 19

plasma

Front 20

Ferritin levels usually have a direct correlation with the total amount of iron stored in the body. However, ferritin levels may be artificially high in cases of .... ?

Back 20

anemia of chronic disease

Front 21

In cases of anemia of chronic disease, ferritin is elevated in its capacity as an _____ and not as a marker for iron overload.

Back 21

acute phase protein

Front 22

The ranges for ferritin can vary between laboratories but are usually ... for males and for females?

Back 22

30–300 ng/mL (=μg/L) for males

18–115 ng/mL (=μg/L) for females.

Front 23

The most specific test of iron deficiency anemia (IDA) is ... ?

Back 23

serum ferritin; low is ferritin is positive. But remember that infection and chronic inflammation can raise the level, thus masking what would otherwise be a low level of ferritin.

Front 24

3 conditions that may result in a low ferritin include ... ?

Back 24

hypothyroidism, vitamin C deficiency or celiac disease

Front 25

If ferritin is high, there is iron in excess or else there is an _____ in which ferritin is mobilized without iron excess.

Back 25

acute inflammatory reaction

Front 26

If ferritin can be elevated by infections or inflammation, how can you test to make sure a high level isn't related to one of these issues?

Back 26

C-reactive protein levels will normal

Front 27

Hepcidin is a key regulator of the entry of __ into the circulation in mammals.

Back 27

iron

Front 28

Ferroportin is a transmembrane protein that transports iron from where to where?

Back 28

the inside of a cell to the outside of it

Front 29

Hepcidin inhibits iron transport by binding to the iron export channel __.

Back 29

ferroportin

Front 30

What is the relationship between the amount of hepcidin and the amount of intestinal iron absorption?

Back 30

inversely related

Front 31

What insoluble form of iron is formed in the body when there is excess iron?

Back 31

hemosiderin

Front 32

About how much iron is ingested and excreted on a daily basis?

Back 32

1-2 mg

Front 33

What is the normal iron content of the body?

Back 33

3-4 mg

Front 34

Where does most of the iron in the body exist?

Back 34

RBCs! (1.8-2g)

Front 35

What cells absorb iron in the gut?

Back 35

duodenal enterocytes; some iron enters the circulation and some is stored in the enterocytes.

Front 36

What MCV level indicates microcytic anemia?

Back 36

Less than 80 MCV.

Front 37

What are the 3 pathophysiologies of iron deficiency anemias?

Back 37

1. Reduction in iron availability

2. Reduction of heme synthesis

3. Reduced globin production

Front 38

What is the most common cause of anemia worldwide?

Back 38

iron deficiency anemia

Front 39

The 4 main causes of microcytic anemia?

Back 39

1. Iron Def. anemia

2. Thalassemia syndromes

3. Early Anemia of Chronic Disease (ACD)

4. Others, but very rarely; Sideroblastic anemia for example

Front 40

What are the 3 primary mechanisms for iron deficiency anemia?

Back 40

1. Blood loss (overt/occult)

2. Inadequate absorption (PPI, Celiac, H. pylori, etc)

3. Inadequate intake (poverty, fad diet, etc)

Front 41

In IDA, iron levels are increased or decreased?

Back 41

Decreased. But this is not diagnostic of IDA by itself.

Front 42

In IDA, transferrin levels are increased or decreased?

Back 42

Increased.

Front 43

In IDA, transferrin saturation is increased or decreased?

Back 43

Decreased. Not diagnostic of IDA by itself but a helpful discriminator

Front 44

In IDA, ferritin levels are increased or decreased?

Back 44

Decreased.

Front 45

What ferritin level is considered diagnostic of IDA?

Back 45

Less than 30ng/ml

Front 46

In IDA, soluble transferrin receptors are increased or decreased?

Back 46

Increased.

Front 47

In IDA, cytokine levels are increased or decreased?

Back 47

Normal, actually.

Front 48

Normal serum iron levels are ?

Back 48

40-160ug/dl

Front 49

Normal TIBC levels are ?

Back 49

200-400mg/dL

Front 50

Normal ferritin levels are ?

Back 50

30-250 ng/ml (less than 30 is diagnostic or IDA)

Front 51

Normal transferrin saturation levels are ?

Back 51

20%-50%

Front 52

Transferrin saturation is described as the .... ?

Back 52

ratio between free circulating iron and transferrin

Front 53

What kind of RDW level would you expect in IDA?

Back 53

Increased, but may normalize if the IDA is long standing. This does not rule out thalassemia.

Front 54

What is the gold standard of IDA diagnosis?

Back 54

bone marrow biopsy, but used as a last resort

Front 55

What disease is characterized by defects in the synthesis of one or more of the globin chain subunits of hemoglobin?

Back 55

Thalassemia

Front 56

Which thalassemia is caused by the deletion of an allele?

Back 56

alpha thalassemia

Front 57

Which thalassemia is caused by a mutation?

Back 57

beta thalassemia

Front 58

Hemoglobin A (the major adult hemoglobin) is made up of one pair of __ HGB and one pair of __ HGB.

Back 58

one pair of alpha and one pair of beta hemoglobins

Front 59

What kind of thalassemia is incompatible with extra-uterine life?

Back 59

alpha thalassemia major

Front 60

What kind of thalassemia is survivable with life-long transfusions?

Back 60

beta thalassemia major

Front 61

Beta thalassemia causes a lifelong excess of what?

Back 61

alpha globin chains

Front 62

When are beta thalassemias generally noticed?

Back 62

About 6 months of age when Hgb F changes to Hgb A

Front 63

Thalassemia patients often have an MCV below __, though all should have an MCV below 80.

Back 63

70 MCV

Front 64

Beta thalassemia trait is the same thing as ... ?

Back 64

beta thalassemia minor

Front 65

What are the lab results of thalassemia trait?

Back 65

Microcytosis out of proportion to degree of clinical anemia

Thal:MCV 60; Hct 35

IDA: MCV 60; Hct 25

Front 66

What type of RBC morphology might be seen in thalassemia?

Back 66

Microcytes, acanthocytes (spiculated cell membrane), and target cells

Front 67

The iron panel results for thalassemia are ... ?

Back 67

High iron

Low TIBC

High ferritin

RDW low; This is key in telling thalassemia from IDA. If IDA, the RDW will be high.

Front 68

Most definitive method for testing for thalassemia?

Back 68

electrophoresis

Front 69

What form of anemia if frequently found in patients with a long-standing infection, malignancy, or inflammation?

Back 69

ACD (Anemia of chronic disease)

Front 70

The iron panel results for a patient with ACD (anemia of chronic disease) would be ... ?

Back 70

Low iron

Low TIBC

High ferritin (If <30ug/L, suggestive of coexistant iron deficiency anemia)

MCV only slightly reduced, maybe even normal

Front 71

Patients with sideroblastic anemia will have what lab results?

Back 71

1. presence of ringed sideroblasts in bone marrow (>10%)

2. elevated serum iron levels

3. elevated transferrin saturation Essentials for dx of sideroblastic anemia

Front 72

Sideroblastic anemia can also be an important marker of what condition?

Back 72

acute leukemia

Front 73

Which is more common: inherited or acquired sideroblastic anemia?

Back 73

Acquired is far more common.

Front 74

What are the biggest causes of acquired sideroblastic anemia?

Back 74

drugs and toxins; alcohol abuse can cause this.

Front 75

Drugs that can cause sideroblastic anemia include ... ?

Back 75

ethanol (alcohol)

INH/Isoniazid

cyclosporine

chloramphenicol

busulfan

copper chelators

-penicillamine

-triethylene tetramine dihydrochloride

Front 76

3 toxins that can cause sideroblastic anemia?

Back 76

Lead

zinc

auto-Abs (not sure that this counts as a toxin, but...)

Front 77

__ __ can be seen on a blood smear of a patient with sideroblastic anemia.

Back 77

Basophilic stippling

Front 78

The iron panel results for sideroblastic anemia would be ... ?

Back 78

High iron

Normal TIBC

High Ferritin

Front 79

What type of environment is required to absorb iron?

Back 79

Acidic

Front 80

What decreases absorption of iron?

Back 80

Calcium, antacids, dairy. Acids, like OJ, help absorption.

Front 81

What is the daily requirement of iron for a male or non-menstruating female?

Back 81

1 mg per day (this is how much that is lost)

Front 82

What is the daily requirement of iron in menstruating female?

Back 82

3-4 mg per day

Front 83

What is the daily requirement of iron for a pregnant female?

Back 83

2-5 mg per day

Front 84

What are the etiologies of iron deficiency anemia ?

Back 84

Remember: NIMBLE

N: increased need --> pregnancy, lactation

I: decreased intake --> not getting through dietM: malabsorption --> crohn's, zinc deficiency, antacids

B: blood loss --> GI and menstruation

L: loss

E: excessive donation

Also: chronic illness, daily ASA, hemoglobinuria, iron sequestration

Front 85

What can happen to the tongue in iron deficiency anemia?

Back 85

Smoothing and sensitivity

Front 86

What can happen to fingernails in iron deficiency anemia?

Back 86

Spooning, also called koilonychia. This leads to brittle nails.

Front 87

A patient with IDA would have what retic index?

Back 87

ormal or decreased: less than 2.5

Front 88

What are the 4 MC iron studies to order?

Back 88

Ferritin: most accessible storage form of iron

TIBC: total iron binding capacity --> ability of iron to bind; indirect measure of transferrin

SI: serum iron --> amount of transferrin saturated w/iron

% saturation --> transferrin saturation

Front 89

What is the first lab value to decrease in iron studies?

Back 89

Ferritin, which would dip below 12

Front 90

What is the one thing that increases during IDA?

Back 90

TIBC increases. Everything else decreases.

Front 91

What would you see on a blood smear of a patient with IDA?

Back 91

Depending on the stage:

Microcytic, hypochromic

Anisocytosis and poikilocytosis

Target cells/pencil shaped cells

Front 92

What will be the LAST lab value to come back up to normal after treatment of iron deficiency anemia?

Back 92

Ferritin. If normal, iron stores are back to normal.

Front 93

What does zinc deficiency have to do with iron deficiency?

Back 93

You can't absorb iron without zinc. This leads to IDA.

Front 94

How long does HgbF persist in the body after birth?

Back 94

6 months

Front 95

What does HGB-S cause?

Back 95

sickle cell

Front 96

If alpha-thalassemia is caused by a gene deletion, beta-thalassemia is caused by a gene ____?

Back 96

mutation

Front 97

What key aspects on a CBC would make you suspect a Thalassemia?

Back 97

LOW MCV --> microcytic anemia

Low MCHNormal Ferritin --> rule out iron deficiency

Normal RBC

Front 98

What is the # 1 complication of chronic transfusions when it comes to severe thalassemias?

Back 98

Transfusional Hemosiderosis (hemochromatosis) AKA iron overload

Front 99

What do we call it if patient is missing all alpha- chains?

Back 99

hydrops fetalis

Front 100

TRUE or FALSE: the MCV of a patient with a thalassemia will always be lower than in an iron deficiency anemia patient

Back 100

TRUE; MCV tend to be really low in thalassemia patients.

Front 101

What would the reticulocyte count be of a patient with hemoglobin H disease?

Back 101

Increased: chronic hemolytic anemia, so ongoing need for production

Front 102

What would hemoglobin electrophoresis show for hemoglobin H disease?

Back 102

Increased hemoglobin H (10-40% of all hemoglobin)

Front 103

Besides intermittent transfusions, what else can you do for your patient with Hemoglobin H disease?

Back 103

Give them folic acid to help with hemoglobin production.

Front 104

Can beta chains be replaced in beta-thalassemia?

Back 104

Yes, by gamma and delta chains.

Front 105

Cooley's anemia is also known as ... ?

Back 105

Beta-thalassemia Major

Front 106

Why do you see bony changes, deformities, and fractures in patients with cooley's anemia and b-thalassemia intermedia?

Back 106

Hematopoiesis is occurring in other locations than normal bone marrow due to intense need

Front 107

What are the 3 classifications of ACD (anemia of chronic disease)?

Back 107

1. Anemia of Inflammation

2. Anemia of the Elderly

3. Anemia or Organ failure

Front 108

Name some diseases/disease states associated with anemia of inflammation?

Back 108

IBD (irritable bowel syndrome), RA (rheumatoid arthritics), malignancy, any chronic infection, trauma

Front 109

Does inflammation increases or decrease Hepcidin?

Back 109

increased

Front 110

Would Hepcidin levels be increased or decreased in ACD?

Back 110

increased

Front 111

What lab study would you draw to check Hepcidin levels?

Back 111

None. No such lab.

Front 112

Explain some of the pathophysiology behind anemia of chronic inflammation.

Back 112

Inflammation --> increased hepcidin --> decreased iron uptake in gut

-Decreased iron transfer into the bone marrow

-Decreased responsiveness to erythropoietin

-Increased hemolysis

Front 113

What is the serum iron level in a patient with ACD? Why?

Back 113

LOW serum ironHepcidin is often increased in ACD = decreased iron absorption in the gut

Front 114

What is the difference between TIBC levels in anemia of chronic disease and iron deficiency anemia?

Back 114

ACD --> low or normal TIBC

IDA --> High TIBC

Front 115

If you see basophilic stippling on a peripheral smear, what do you think of?

Back 115

lead poisoning

Front 116

What is the PRIMARY issue in sideroblastic anemia?

Back 116

Defect in biosynthesis of heme, specifically the porphyrin ring

Front 117

What is a specific CBC finding you could see in a patient with copper deficiency?

Back 117

neutropenia

Front 118

What do iron studies look like for a patient with sideroblastic anemia

Back 118

Everything is elevated EXCEPT TIBC with is decreased

Front 119

Is it possible to have sideroblastic anemia with normal iron?

Back 119

Yes, if the patient already has a iron deficiency present

Front 120

During an iron deficient erythropoiesis, what happens to the free erythrocyte protoporphyrin (FEP)?

Back 120

It increases.

Front 121

What is Total Iron Binding Capacity (TIBC)?

Back 121

Amount of transferrin that is in circulation.

Front 122

Which mechanism of acquired SA increase in urine d-ALA and copropophyrin, and increased FEP with ALA dehydrate and ferrochelatase most sensitive, found in children eating flaked paint and adults inhaling compounds for industrial processes causing shortened red cell survival and markedly abnormal heme synthesis?

Back 122

Lead

Front 123

Which mechanism of acquired SA involves 31% of hospitalized chronic alcoholics have SA especially with poor diet - may be deficient in B6, folic acid, and megaloblastosis and inhibits synthesis of B6 and exacerbates effect of folate deficiency?

Back 123

alcohol

Front 124

Which mechanism of acquired SA involves the possible findings of ringed sideroblast in various hematologic malignancies and evidence of abnormal stem cell clone that effect the RBC as well as other cell lines?

Back 124

Malignancy

Front 125

What is the accumulation of iron surpassing normal levels causing gross deposition in macrophages of spleen, liver and BM with generally no organ damage?

Back 125

Hemosiderosis

Front 126

What is a more severe form of iron overload that has progressed to involve a widespread deposition of iron in parenchymal tissue cells with organ injury which may be inherited or acquired?

Back 126

Hemochromatosis.

Front 127

What is the most common anemia of hospitalized patients thats associated with chronic infection, connective tissue disorders, renal disease, tissue injury, and malignant disease?

Back 127

Anemia of chronic inflammation

Front 128

What would a rouleaux morphology look like?

Back 128

stacking of cells, can be from mutliple myeloma as well as other conditions

Front 129

Where does iron absorption occur?

Back 129

proximal small intestine

Front 130

What are some reversible causes of sideroblastic anemia?

Back 130

alcoholism, drug induced (isoniazid and chloramphenicol), copper deficiency, lead poisoning

Front 131

What are pappenheimer bodies?

Back 131

hypochromic RBCs with coarse basophilic granules that stain positive for iron

Front 132

What is the pathogenesis of anemia of chronic disease?

Back 132

increased IL-6, increased hepcidin

Front 133

What is MCV? How is it calculated?

Back 133

avg red cell size; calculated Hct/RBC