Heme- Exam #2

Front 1

What is Anisocytosis?

Back 1

It's a variation in cell SIZE

Hint 1

Front 2

What part of a CBC is increased with anisocytosis?

Back 2

Red cell distribution width (RDW)

Front 3

What classifies an RBC as microcytic?

Back 3

When it's < 80 fL

Front 4

In what diseases do you see microcytosis?

Back 4

-Iron deficiency anemia

-thalassemia

-Chronic blood loss i.e. bleeding ulcers or GI bleeds

Front 5

What does a blood smear look like with microcytic cells?

Back 5

It's thinner blood so its spread out more on the smear

Front 6

What is macrocytosis?

Back 6

When the MCV is >100 fL

Front 7

In what disorders would you see you macrocytic cells?

Back 7

- vitamin b12 deficiency enter

- folate deficiency

- liver disease

- hypothyroidism

- myelodysplastic disorders

Front 8

What does polychromatophilic mean & what causes it?

Back 8

It means variation in color - other - red or blue

- due to artifactual changes from staining and pH

Front 9

What is dimorphism?

Back 9

- 2 populations of cells

- indicative of blood transfusions

Front 10

What is polychromasia?

Back 10

When ribosomal RNA gives RBCs a blue tinge

i.e. reticulocytes stained with super vital stain new methylene blue to confirm (but not always)

Hint 10

Front 11

What are reticulocytes stained with and what do they look like?

Back 11

- not artefactual because they are not refractive

- have to have at least two inclusions or it could be Heinz bodies

- stained with new methylene blue

Front 12

What are schistocytes?

Back 12

Fragmented red blood cells

Hint 12

Front 13

What are helmet cells

Back 13

Another name for schistocytes

Hint 13

Front 14

What is poikilocytosis?

Back 14

Change in red cell SHAPE

Hint 14

Front 15

In what dIseases do you see target cells?

Back 15

- B12 / folate deficiency

- thalassemia

- hemoglobin C disease

- liver disease

Hint 15

Front 16

What are red cells called that have 2-10 spiny or thorny projections?

Back 16

Acanthocytes

Hint 16

Front 17

In what dIseases do you see acanthocytes?

Back 17

- lipid metabolism disorders

- liver disease

- spleen removal

- alpha beta lipoproteinemia

Front 18

What are the RBCs called that have >30 short pointed evenly spaced projections?

- also called echinocytes

Back 18

Burr/crenated cells

Hint 18

Front 19

What causes burr cells?

Back 19

- when there's a chemical change in the blood

- renal disease from toxins building up

- can be from drying artifacts and water in stain

Front 20

What causes schistocytes to form?

Back 20

- mechanical hemolysis i.e. heart valve replacement

- DIC = dissociated intravascular coagulation

- 3rd & 4th degree burns

Front 21

In what conditions do you see spherocytes?

Back 21

- hereditary spherocytosis

- autoimmune hemolytic anemia

- burn patients

Hint 21

Front 22

What are Dacrocytes?

Back 22

Teardrop cells

Hint 22

Front 23

In what conditions do you see teardrop cells?

Back 23

- megaloblastic anemia (B12/folate)

- myelofibrosis: RBCs are being infiltrated in the bone marrow so they are being 'squeezed'

- bone marrow metastasis

Front 24

In what conditions do you see elliptocytes and/or ovalocytes?

Back 24

- hereditary elliptocytosis and Ovalocytosis

- megaloblastic anemia

- microcytic anemia ( iron deficiency & thallasemia)

Front 25

What is another name for a sickle cell?

Back 25

Oat cells... Because they have 2 pointed ends

Hint 25

Front 26

What causes cells to be sickled?

Back 26

- Issue with hemoglobin S or C

- decreased oxygen tension in the blood (running marathons or high altitudes)

Front 27

What body part removes sickled cells?

Back 27

The spleen

Front 28

What are the cells that have an elongated central pallor that looks like a mouth?

Back 28

Stomatocytes

Hint 28

Front 29

What causes stomatocytes to form?

Back 29

- membrane abnormalities

- liver disease

- hereditary stomatocytosis

Front 30

What are Howell Jolly bodies?

Back 30

Inclusions made of very dense chromosomal DNA remnants

Hint 30

Front 31

How can you tell HJ bodies apart from platelets?

Back 31

Platelets will push away from the hgb causing a halo effect and will be more in focus than HJ bodies

Front 32

What is 1 condition that causes HJ bodies?

Back 32

There will be an increase in these inclusions with a spleenectomy

Front 33

Inclusions that look like a stack of cannonballs with smaller dots aggregated together are called...

Back 33

Pappenheimer bodies

Hint 33

Front 34

What conditions do you see pappenheimer bodies form in?

Back 34

- chemical intoxication

- heavy metal exposure

- sideroblastic anemia

- spleen dysfunction

Front 35

What are pappenheimer bodies made of?

Back 35

Non-heme iron deposited in RBC mitochondria

Front 36

What stain do you use to find/see pappenheimer bodies?

Back 36

Prussian blue

Front 37

What causes basophilic stippling?

Back 37

- lead poisoning

- thalassemia

Front 38

What are fine to coarse granules of polyribosome aggregates evenly distributed in cell with mitotic spindles?

Back 38

Basophilic stippling

Hint 38

Front 39

What are inclusions that look like lined up dots/stippling and are mitotic spindle remnants?

Back 39

Cabot rings

Hint 39

Front 40

What conditions cause Heinz bodies to form?

Back 40

- thalassemia

- Hemoglobin S & H

- G6PD shunt defect

- Exposure to oxidizing drugs (aspirin, vit. C, anti-malarial meds)

- fava bean reaction

Front 41

What are Heinz bodies made of?

Back 41

Denatured precipitated hgb

Hint 41

Front 42

What stain do you use to display Heinz bodies?

Back 42

Supravital stain

Front 43

How are hgb crystals formed?

Back 43

Formed in-vitro (in test tube) due to drying process

Front 44

What is made from sodium citrate additive & are dense & rigid?

Back 44

Hemoglobin C crystals

Hint 44

Front 45

What's another name for hgb C crystals?

Back 45

Washington monument crystals

Hint 45

Front 46

What inclusions look like a gloved hand & are rigid?

Back 46

Hemoglobin SC crystals

Hint 46

Front 47

What does it mean when bacteria/fungi (yeast)/parasites are found in the blood?

Back 47

It's a sign a patient Is septic and you report it immediately

Front 48

What organism causes accole form rings on RBC edges?

Back 48

Plasmodium falciparum

Hint 48

Front 49

What organism causes Schuffner's dots to form?

Back 49

Plasmodium vivax

Hint 49

Front 50

What causes water artifacts to form?

Back 50

-humidity

- not drying a slide completely before staining

- they are very refractive and shiny

Front 51

This cytoplasmic change causes larger/darker staining granules & is the body's response to bacterial infection

Back 51

Toxic granulation

Hint 51

Front 52

This cytoplasmic change is found in the cytoplasm as residual RNA

Back 52

Dohle Bodies

Hint 52

Front 53

This morphology is caused from coag & bleeding disorders b/c they don't like sticking together

- exhibit hypogranular platelets (lg) and neutrophils (polymorphonuclear)

Back 53

Cytoplasmic agranulartiy

Front 54

Back 54

Intracellular bacteria

Front 55

This is a dying/dead cell & it's starting to break down & dissolve

Back 55

Pyknotic cells

Hint 55

Front 56

- These have 6+ lobes

- Associated with Megaloblastic Anemia

- Even 1 can be significant

Back 56

Hypersegmented neutrophils

Hint 56

Front 57

Back 57

Atypical Lymphs- just means it's moving away from normal morphology

Front 58

These are seen in VIRAL RXNS i.e. Infectious Mono

Back 58

Reactive Lymphocytes

Hint 58

Front 59

These are fragile, look like lymphs that are broken apart

- Common in Chronic Lymphocytic Leukemia (CLL)

Back 59

Smudge Cells

Hint 59

Front 60

Back 60

Abnormal platelets

Front 61

These are agranular, the size of RBCs or larger, found in leukemias

- If more than 1 is found in 5 oil immersions fields- REPORT IT

Back 61

Giant, agranular platelets

Hint 61

Front 62

Back 62

Budding platelets from a megakaryoblast/cyte

Front 63

Back 63

platelet clumping: seeing this means clots are starting to form

- Do we need to redraw the patient?

Front 64

Back 64

Platelet satellitosis: Plts adhere to neutrophil surfaces.

- To prevent this, draw blood in Na+ citrate tube.

Front 65

This is a group of disorders that have a hgb synthesis defect due to iron deficiency or abnormal iron utilization

Back 65

Microcytic Hypochromic anemias

Front 66

This group of disorders include:

- Fe+ deficiency anemia

- Anemia of Chronic Disease

- Sideroblastic anemia

Back 66

Microcytic hypochromic anemia

Front 67

Anemia of Chronic disease includes what?

Back 67

1) Chronic Infection

2) Autoimmune diseases

3) Chronic Inflammation

4) Malignancies

Hint 67

There are 4

Front 68

This is a type of microcytic hypochromic anemia that has a globin chain synthesis disorder

Back 68

Thalassemia(s)

Front 69

- Cellular growth

- Oxygen transport

- RBC proliferation

** what are these 3 things indicative of?

Back 69

Normal Iron Requirements

Front 70

What is the Avg. adult iron content in the body?

Back 70

3500-4000 mg

Front 71

Where are the primary areas in the body that iron is stored in?

Back 71

- 2/3 in hemoglobin

- 1/3 in BM, liver & spleen

Front 72

How much iron is stored as ferritin or hemosiderin?

Back 72

~90%

Hint 72

It's a percentage

Front 73

What are the daily requirements of iron?

Back 73

- Infants: 1.0 mg

- Children: 0.5 mg

- Menstruating women: 2.0 mg

- Pregnant/lactating women: 3.0 mg

- Adult men/women: 1.0 mg

Front 74

How much iron is absorbed from your diet?

Back 74

5-10%

Front 75

What are some foods that ⬆ Fe+ absorption?

Back 75

- Acidic foods

- Vitamin C

Front 76

What kind of foods are iron rich?

Back 76

- Meat

- Legumes

- Green veggies i.e. spinach

- Cereal (fortified)

- Prunes

- Whole grain bread/ oatmeal

- Oysters/shrimp

Front 77

What are some things that affect iron absorption?

Back 77

- Amount & type of iron in diet

- Current iron stores

- Erythropoietic needs (if you're anemic, the body needs more)

Front 78

Where is Transferrin + Fe +3 located?

Back 78

Plasma

Front 79

This is an iron binding transport protein found in plasma & extracellular fluid

Back 79

Transferrin

Front 80

This is a receptor mediated ferric transferrin

Back 80

Transferrin receptor

Front 81

Used for iron storage

Back 81

Ferritin

Front 82

This binds w/ transferrin receptors... reducing the affinity for transferrin

Back 82

hemochromatosis gene protein (HFE)

Front 83

This is an iron transport protein from GI lumen into the duodenal mucosal cell

Erythroblast --> cytoplasm

Back 83

Divalent metal transporter 1 (DMT 1)

Front 84

This is involved in iron metabolism.

- ⬆ in iron overload

- ⬇ in iron deficiency

Back 84

Hepcidin

Front 85

This is a measure of transferrin bound iron

Males: 65-170 ug/dL

Females: 50-170 ug/dL

*Morning specimens preferred due to diurnal variation

Back 85

Serum Iron

Front 86

This is the total amount of iron that can be bound by transferrin in plasma or serum

- Range: 250-450 ug/dL

- 1/3 is normally saturated

- It's an indirect measurement of transferrin concentration

Back 86

Total Iron Binding Capacity (TIBC)

Front 87

This is measured as the max. amount of iron that's bound to plasma or serum

- <16% indicates iron deficiency

Males: 20-50%

Females: 15-50%

Back 87

Transferrin Saturation

Front 88

What is the formula for calculating Transferrin Saturation?

Back 88

% Saturation=

(serum iron/TIBC) x 100%

Front 89

This is directly proportional to the amount of stored iron & is a better measurement than TIBC or serum iron

Back 89

Ferritin

Front 90

What is Ferritin's normal reference range?

Back 90

12-30 ng/mL

Front 91

This is inversely proportional to the amount of body iron

- ⬆ in iron deficiency ONLY when iron stores are depleted

- No increase w/ Anemia of Chronic Disease

Back 91

Transferrin Receptor

Front 92

This is a heme precursor that incorporates iron into the hgb molecule

Back 92

Free Erythrocyte Protoporphyrin (FEP)

Front 93

This component inversely correlates w/ ferritin levels

Back 93

Zinc Protoporphyrins (ZPP)

Front 94

This is available when iron is not.

Excess protoporphyrins form & complex w/ zinc to form zinc protophyrins

Back 94

Free Erythrocyte Protoporthyrin (FEPs)

Zinc Protoporthyrin (ZPPs)

Front 95

This is the most common type of anemia

Back 95

Iron defiency anemia

Front 96

This may occur b/c of:

- ⬆ iron demands & blood loss

- Abnormal iron utilization

- Poor diet/ Malabsorption

Back 96

Iron Defiency Anemia (IDA)

Front 97

What is involved in Stage 1 Iron Defiency Anemia (IDA)?

- Stage 1 = Iron depletion

Back 97

- Asymptomatic

- BM iron stores & ferritin: ⬇

- Hemosiderin: ⬇ or absent

- TIBC & RDW: ⬆

- CBC & Morphology: Normal

Front 98

What is involved in Stage 2 Iron Defiency Anemia (IDA)?

- Stage 2 = Iron-Deficient Erythropoiesis

Back 98

- Ferritin, Iron, Hgb & Hct: ⬇

- ZPP, TIBC: ⬆

- Morphology: Microcytic

- TfRs- ⬆ on RBC surfaces

Front 99

What is involved in Stage 3 Iron Defiency Anemia?

- Stage 3 = IDA development

Back 99

- Hgb & Hct: ⬇

- Erythropoietin levels: ⬆

- Morphology: Micro/hypo

- BM: 'Ragged' erythroid precursors

- Iron: Severely ⬇

Front 100

What are the clinical symptoms of IDA?

Back 100

- Fatigue, irritability, weakness

- Headache

- Shortness of breathe, tachycardia

- Pale skin color

- Pica

Front 101

What are symptoms of severe IDA?

Back 101

- Doilonychias (Nail smoothing)

- Muscle dysfunction

- Pharyngeal webs

- Glossitis (tongue inflammation)

- Cheilitis (lip inflammation)

Front 102

What are symptoms of infants/ children w/ IDA?

Back 102

- Developmental delays (motor/ behavioral)

- Irritability, memory loss, poor growth

- Increased chance of infections

Front 103

What are the lab findings of someone with IDA?

Back 103

- Serum iron, ferritin: ⬇

- TIBC, FEP, serum soluble transferrin receptor levels: ⬆

- Morphology: Micro/hypo

- Anisocytosis, storage iron & sideroblasts: ⬇

- Ringed sideroblasts: absent

Front 104

These are used to treat this disease:

- Find primary cause

- Oral supplements (ferrous sulfate, gluconate, fumerate)

- Parenteral- IV or intramuscular

- Blood trx

Back 104

Iron Defiency Anemia (IDA)

Front 105

This is seen after therapy:

- Retics: ⬆ in 3-5 days/ max in 8-10 days

- Hgb: ⬆ in 2-3 weeks/ normal in 6 weeks

- Microcytosis: ~4 months to resolve

- Normal cell pop. ~6-10 weeks

- Iron stores: ~6 months for replenishment

- Iron therapy response: 1 g Hgb ⬆ in 1 month

Back 105

Iron Defiency Anemia (IDA)

Front 106

This is the 2nd most common form of anemia

Back 106

Anemia of Chronic Disease (ACD)

aka Anemia of Inflammation (AOI)

Front 107

This disease is common with these infections

- TB

- Chronic osteomyelitis

- Fungal infections

Back 107

Anemia of Chronic Disease (ACD)

Front 108

These neoplasms are associated w/ this disease state:

- Carcinomas

- Malignant lymphoma

- Multiple myeloma

Back 108

Anemia of Chronic Disease (ACD)

Front 109

These autoimmune disorders are associated w/ this disease:

- Lupus (SLE= Systemic lupus)

- Rheumatoid Arthritis (RA)

- Sarcoidosis

Back 109

Anemia of Chronic Disease (ACD)

Front 110

This chronic disease is said to block transfer of storage iron to erythroid precursors in BM. Possible causes:

1) Shortened RBC survival

2) BM fails to increase RBC production

3) Impaired Fe+ release from RES (Reticuloendothelial System)

Back 110

Anemia of Chronic Disease (ACD)

Front 111

This disease state allows T-cells to become activated, makes more cytokines = ⬇ iron = ⬇ available RBC production

Back 111

ACD

Front 112

- Normocytic, but may be hypo/normo

- BM RBC precursors: normal

- M:E ratio: 4:1

- Retic %: Not reflective

- WBC/Plt counts: Normal

- iron storage: Increased

- Sideroblasts: decreased- w/ very coarse iron aggregates

- Ringed sideroblasts: rare or absent

- Low Erythropoietin levels

Back 112

ACD morphology findings

Front 113

This disease shows:

- Anemia present for months

- Mild-moderate anemia

- Normo/Normo: 60-70%

- Micro/hypo: 30-40 %

- Related to underlying cause

Back 113

ACD clinical features

Front 114

This disease shows:

- Iron, TIBC, % transferrin saturation: ⬇

- TfR: Normal

- Ferritin: Normal/ ⬆ (due to trapped iron in RES cells in BM)

Back 114

ACD lab test findings

Front 115

Ways of treating this disease:

- Treat underlying cause 1st

- Need to counteract effects of cytokines & stimulate iron uptake & heme synthesis

- Blood trx (severe cases)

Back 115

ACD

Front 116

This is a rare inherited condition

- Congenital (sex-linked)

- Autosomal recessive

OR - is primary (idiopathic)- Acquired: Myelodysplasia

OR- Secondary from lead, alcohol, or drugs

Back 116

Sideroblastic Anemia

Hint 116

NEED to know if it's hereditary or acquired to treat it.

Front 117

This disease has abnormalities of heme synthesis enzymes i.e.

- Delta 5-aminolevulinic acid synthetase

- Uroporphyrinogen decarboxylase

Characterized by: Ringed Sideroblasts

Back 117

Sideroblastic Anemia

Hint 117

Front 118

What significant RBC morphology is present in lead poisioning?

Back 118

Basophilic stippling

- Mitochondrial aggregates or polyribosomes

Hint 118

Front 119

This condition will cause these clinical findings:

- People usually >50 yrs old

- Weakness, pallor, fatigue (associated w/ anemia)

Back 119

Sideroblastic anemia

Front 120

This condition will cause these morphologies:

- Moderate-Severe anemia

- Dimorphic RBC population

- RDW: Increased (Aniso)

- Hypochromia, basophilic stippling, pappenheimer bodies, target cells, poik

- Hypercellular BM w/ RBC hyperplasia

- >15% ringed sideroblasts

Back 120

Sideroblastic Anemia

Front 121

These lab findings are present in this condition:

- Iron, Ferritin, % saturation: ⬆

- TIBC & TfR: Normal or ⬇

Back 121

Sideroblastic Anemia

Front 122

This condition is acquired by lead poisoning or is hereditary from a single enzyme deficiency

- Is a RARE disease w/ light sensitivities & there are a lot of them

Back 122

Porphyrias

Front 123

This is an accumulation of excess iron in reticuloendothelial cells.

- It's Inherited or secondary to chronic anemias & their treatments

Back 123

Iron Overload

Front 124

- This is a clinical disorder from excess iron.

- It has ⬆ intestinal iron absorption that leads to excess iron in tissue from tissue damage

Back 124

Hemochromatosis

Front 125

These conditions cause excess iron to be stored in the liver, heart & pancreas- therefore- causing organ damage

Back 125

Iron Overload & Hemochromatosis

Front 126

This condition is recessive, most frequent genetic disease in caucasians (affects 1 in 300 Irish, Scottish, Welsh descents)

Back 126

Hereditary Hemochromatosis (HH)

Front 127

This rare genetic condition affects chromosome 6, short arm, HFE gene

- Is a single base substitution of HFE protein, which alters the protein conformation & interferes w/ iron absorption regulation

Back 127

Hereditary Hemochromatosis (HH)

Front 128

These symptoms are caused by gradual iron accumulation:

- Chronic liver disease

- Arthritis, diabetes

- pituitary damage, congestive heart failure, cardiac arrhythmias

Back 128

Hereditary hemochromatosis pathophysiology

Front 129

This condition absorbs 2-3x more dietary iron than normal (~3 mg/day)

Back 129

Hereditary Hemochromatosis

Front 130

Stage 1:

Genetic predisposition w/ no abnormality other than ⬆ serum transferrin saturation

Back 130

Hereditary Hemochromatosis

Hint 130

The 1st of 4 stages of this condition

Front 131

Stage 2:

2-5 g of iron overload w/o symptoms

Back 131

Hereditary Hemochromatosis

Hint 131

The 2nd of 4 stages of this condition

Front 132

Stage 3:

Iron overload w early symptoms of lethargy & joint pain

Back 132

Hereditary Hemochromatosis

Hint 132

The 3rd of 4 stages of this condition

Front 133

Stage 4:

Iron overload w/ organ damage & cirrhosis. Hepatomegaly is present in 95% of cases

Back 133

Hereditary Hemochromatosis

Hint 133

The 4th of 4 stages of this condition

Front 134

These are the early symptoms of this condition:

- Fatigue

- Joint Pain (most common)

- Bronze skin discoloration

- ED

Back 134

Hereditary Hemochromatosis

Front 135

These are the chronic symptoms of this condition:

- Enlarged liver, cirrhosis & fibrosis of liver, cardiomyopathy from iron deposits, diabetes, hypo-pituitary, -gonadism,

-parathyroidism

Back 135

Hereditary Hemochromatosis

Front 136

Lab results in this disease:

- Erythropoiesis & TIBC: Normal

- ALT, AST, Iron, ferritin, transferrin: Increased

- Hallmark: % transferrin saturation increased

>50% females & >60% Males

Back 136

Hereditary Hemochromatosis

Front 137

Analyzing the HFE gene is diagnostic in confirming this disease

Back 137

Hereditary Hemochromatosis

Front 138

The treatment of this disease is to remove excess iron

- Usually by therapeutic phlebotomy

- Or using desferrioxamine, a chelating agent

Back 138

Hereditary Hemochromatosis

Front 139

The removal of 500 mL of blood 1-2x/week until decrease in % transferrin saturation (<30%) & serum ferritin (<50 ug/L)... then is done 2-4x/year

Back 139

Therapeutic phlebotomy

Front 140

This is acquired or secondary to other anemias

- It displays: anemia, iron overload, ineffective erythropoiesis

- from trx, leads to iron storage increase (iron then can't be excreted)

- Treatment: Iron chelation therapy

Back 140

Secondary Hemochromatosis

Front 141

This is a macrocytic anemia caused by impaired DNA synthesis which causes defective nuclear maturation

Result: Megaloblasts in BM & macro-ovalocytes in blood & WBC precursors are larger

Back 141

Megaloblastic Anemia

Front 142

This condition is caused when DNA lacks bonding w/ dTTP & binds to dTDP instead

Back 142

Megaloblastic Anemia

Front 143

These deficiencies cause this disease:

- Vitamin B12

- Folic Acid

** Or combination of both

Back 143

Megaloblastic Anemia

Front 144

This condition can cause mild- severe anemia

-Symptoms: Weakness, fatigue, shortness of breath, dizzy

- Congestive heart failure may be present if severe enough

- ⬆ bilirubin = causes jaundice

Back 144

Megaloblastic anemia: Clinical manifestations

Front 145

This condition's MCV = >100 fL

- Ineffective erythropoiesis

- Hemolysis

- Ineffective granulopoiesis

Back 145

Megaloblastic Anemia

Hint 145

Look on pg. 182 for more in-depth explanation

Front 146

Bone marrow in this disease is hypercellular

M:E ratio is 1:1-1:3

- Megaloblasts present

- Giant bands & metamyelocytes

- Megakaryocytes

Back 146

Megaloblastic Anemia

Hint 146

Front 147

This condition is macrocytic/ normochromic

- Pancytopenia (RBC/WBC/Plts) may all be decreased eventually

- Macro-ovalocytes

- Hypersegmented neutrophils

- Schistocytes, teardrops, spherocytes, targets

- Howell Jolly bodies

- Basophilic stippling

- Cabot rings

- Megaloblasti nucleated RBCs

Back 147

Peripheral blood found in Megaloblastic anemia

Front 148

Lab findings of this condition:

-MCV: 100-160 fL

- MCH, RDW: ⬆

- MCHC: Normal

- Hgb: Normal or ⬇

- Abs. Retic count: ⬇

- RPI: <2.0 (Ineffective)

Back 148

Megaloblastic Anemia

Front 149

What binds to vitamin B12 so it can be stored in the liver?

Back 149

TC II: Transcobalamin

** I & III bound to 70-90%

** II bound to 10-25%

Front 150

These cause this deficiency:

- Vegan diets

- Malabsorption: Pernicious anemia, Blind loop syndrome, fish tapeworm, ileum disease

- AIDS/HIV, hemodialysis

- Drugs: Alcohol, nitrous oxide

Back 150

Causes of vitamin B12 deficiency

Front 151

What is the definition of pernicious anemia?

Back 151

It lacks the intrinsic factor (caused from parietal cell atrophy) & is the most common form of vitamin B12 deficiency

Front 152

This disease is most common in Scandinavian, English & Irish descent. More common in women @ 4:1 (>50 yrs of age)

Back 152

Pernicious anemia

Front 153

This congenital form of the disease is seen as autosomal recessive & is primarily seen in children <2 yrs old

Back 153

Pernicious Anemia

Front 154

This disease produces autoantibodies to Parietal Cells, Intrinsic Factor, thyroid tissue, & has increased association w/ other autoimmune disorders

Back 154

Pernicious Anemia

Front 155

This disease involves autoantibodies binding to 2 types of Intrinsic Factor (IF):

1) Blocks Ab's, bind to vit. B12 binding site of IF

2) Binds Ab's, to vit. B12:IF complex Ab

Back 155

Pernicious Anemia-- Immunological factors

Front 156

This disease has an insidious onset. Symptoms are:

- Weakness, lightheaded, shortness of breath, appetite loss, Lemon-yellow pallor, Glossitis, Paresthesias, problems w/ fine motor movement & leads to neurological damage

Back 156

Pernicious Anemia

Front 157

What is glossitis?

Back 157

Tongue Inflammation

Front 158

What does paresthesias mean?

Back 158

Burning/numbiness/prickling sensation

Front 159

What does Insidious mean?

Back 159

Slow onset

Front 160

The early stages of this disease include:

- Peripheral nerves affected

- Pins & needles sensation in toes 1st, then moves to all limbs

Back 160

Pernicious Anemia

Front 161

The later stages of development of this disease include:

- Posterior spinal column degeneration

- Clumsiness/ uncoordinated gait

Back 161

Pernicious Anemia

Front 162

Severe symptoms of this disease include:

- Severe weakness/ limb stiffness

- Impaired memory

- Retinal bleeding

- Thrombocytopenia possible

- Hallucinations, paranoia, stupor, severe depression

Back 162

Pernicious Anemia

Front 163

This disease's severe neurological symptoms (paranoia, hallucinations, severe depression, stupor) are reversible if they've been experiencing them <3 months. If >3 months... not very reversible

Back 163

Pernicious Anemia

Front 164

This deficiency is water soluble & has high turnover time & high rate of loss compared to vit. B12, so you see changes within a few months

Back 164

Folic Acid Deficiency

Front 165

This nutrient is found in high concentrations in green leafy veggies, dairy, cereal, liver & kidneys

Back 165

Folic Acid

Front 166

This nutrient's RDA= 50-100 ug/day.

- Absorbed through duodenum & jejunum where ~80% is absorbed

Back 166

Folic Acid

Front 167

A lack of this nutrient in the mother during pregnancy can cause the Spina Bifida birth defect

Back 167

Folic Acid Defiency

Front 168

Causes of this deficiency include:

- Decreased dietary intake (poverty, old age, alcoholism, chronic disease, malapsorption, tropical sprue, gluten sensitivity, celiac's disease)

- Increased need (Pregnancy, infancy, malignancy, contraceptives)

- Anti-malarial drugs

Back 168

Folic Acid Defiency

Front 169

What is tropical sprue?

Back 169

Associated w/ folic acid deficiency, it's an infection that causes parietal cell atrophy

- Causes: weakness, weight loss, fatty stools

Front 170

This deficiency causes same issues as vit. B12 manifestations.

- Insidious onset

- Megaloblastic anemia in BM & blood

- Do NOT see neurological issues in this (like B12 does)

Back 170

Folic Acid defiency

Front 171

You do these lab tests to diagnose this deficiency:

- CBC/Diff/RBC morphology

- Retic Index

- Lactate dehydrogenase (LD enzymes)

- Iron Studies

- BM aspirate (not often)

Back 171

Folic Acid Deficiency

Front 172

Lab findings for this deficiency:

- <200 (Normal: 200-500 ng/L)

- Gastric Auto Ab's

- Gastric atrophy test

- Serum cobalamin binding proteins

- Serum & urine methylmalonic acid (⬆100x)

- Total homocysteine: ⬆

Back 172

Vitamin B12 deficiency

Front 173

These are found in the severe form of this deficiency:

Serum folate: ⬆

RBC folate: ⬇

Back 173

Vitamin B12 deficiency

Front 174

These are lab findings for this deficiency:

- Serum & RBC folate: ⬇

- Normal ranges: 5-16 ng/mL

- Serum homocysteine

Back 174

Folic Acid deficiency

Front 175

What 3 other tests evaluate for Macrocytic Anemia?

Back 175

1) Schilling's Test

2) dU suppression test

3) Holo TC II (used more often)

Hint 175

Review on pg. 192

Front 176

This deficiency requires lifelong vitamin therapy (sometimes doses are 1000-2000 ug/day)

Back 176

Vitamin B12 deficiency

Front 177

This deficiency requires oral therapeutic dosages of 1-5 mg/ day for 2-3 weeks.

* Lifelong therapy not required

Back 177

Folic Acid deficiency

Front 178

These deficiencies show a positive response when these show:

- ⬆ retic count (3-10 days)

- Megaloblastic morphology in BM disappears (24-48 hrs)

- Hct: ⬆ (5-7 days) & normal in 4-8 weeks

- Giant metamylocytes & hypersegmented neutros are gone in 2 weeks

- Entire response: 3-6 weeks

Back 178

Megaloblastic Anemias:

- Vitamin B12

- Folic Acid

Front 179

This deficiency has a refractory or regenerative processing issue

& BM is unable to respond to cytopenia in blood

** ⬇ cells/ ⬆ fat**

Back 179

Aplastic Anemia

Front 180

This is the definition of this anemia:

- Marrow of <25% normal cells

- At least 2 cytopenias present:

- Neutrophils: <500/uL

- Platelets: <20,000/uL

- Anemia w/ corrected retic count: <1%

Back 180

Aplastic Anemia

Front 181

This disease is acquired in ~95% cases & ~40-70% is idiopathic

- Congenital: <5%--> Very rare

- Fanconi's anemia

- Dyskeratosis Congenita

Back 181

Statistics of Aplastic Anemia

Front 182

What are some secondary causes of Aplastic Anemia?

Back 182

- Chemical agents (insecticides, arsenic)

- Drugs

- Ionizing radiation (DNA breakage/genetic abnormalities)

- Infections (Acute/Chronic hepatitis)

- Miscellaneous immune based

Front 183

- Pancytopenia over time

- Malformed fingers, small head, stunted growth, mental retardation, CML development, die from hemorrhages or infection... all characteristic of what?

Back 183

Fanconi's Anemia, which is a severe, congenital form of Aplastic Anemia

Front 184

What is Dyskeratosis congenita?

Back 184

A form of congenital Aplastic Anemia--- from short telomeres & is X-linked

Front 185

This disease is insidious, occurs in all age groups

- has progressive fatigue, dyspnea, palpitations, bleeding, infection, pallor, petechiae, purpura, mucosal bleeding

Back 185

Aplastic Anemia

Front 186

These labs are needed for this condition:

- CBC w/ diff

- Retic count

- BM exam

- Liver & renal function tests

- Cultures & serological tests

Back 186

Aplastic Anemia

Hint 186

More info on pg. 197

Front 187

This is characteristic of what condition?

Back 187

This is a dry bone marrow tap-- no cells, just fat (Aplastic Anemia)

Front 188

If this disease is left untreated a poor prognosis results-- Get lethal infections or bleeding

Back 188

Aplastic Anemia

Front 189

These are the standard treatments for this disease:

- Support therapy: blood trx or steroids to stimulate hematopoiesis

- BM or stem cell transplants (<50 yrs old)

Back 189

Aplastic Anemia treatment

Front 190

This is a rare disorder where RBCs in BM are selectively destroyed

Back 190

Pure Red Cell Aplasia

Front 191

This is characterized by:

- Severe, chronic, normocytic- slightly macrocytic

- Retics: ⬇ /absent

- No hemolysis or hemorrhage

- WBCs & Plts: Normal

- BM: No RBC precursors

- Erythropoietin Levels: ⬆

Back 191

Pure Red Cell Aplasia

Front 192

Causes of this disease includes:

ACQUIRED

- Parvovirus B19 infection: 5th's Disease (Hand/foot/mouth)

- Aplastic crisis

- Immunocompromised patients

- Malnutrition, drugs, Direct toxicity, neoplasms

Back 192

Pure Red Cell Aplasia

Front 193

Causes of this disease includes:

CONGENITAL

- Anemia: Chronic,mod.-severe

- Onset: Early infancy

- Retic Count: decreased

- BM: erythroid hypoplasia & normocellular

- Head & upper limb abnormalities

- Spontaneous remission

Back 193

Pure Red Cell Aplasia

Front 194

This disease causes spontaneous remission:

- In 25% of patients

- Many respond to steroids or trx

- Increased AML incidence

- Age of onset, severity & course of disease (variations)

Back 194

Pure Red Cell Aplasia

Front 195

Type 1 of this disease includes:

- Mild-mod. macrocytic anemia

- Aniso & poik present

- BM: 1-3% erythroblasts are binucleated / chromatin bridges

- Autosomal recessive

- Skeletal defects & skin hypopigmentation

Back 195

Congenital Dyserythropoietic Anemias (CDAs)

Front 196

Type 2 of this disease includes:

- HEMPAS: Hereditary Erythroblast Multinuclearity w/ Positive Acid Serum test

** Most common CDA

- Autosomal recessive

- Mild-severe normocytic anemia

- BM: 10-50% bi- or multinucleated erythroblasts

- Strongly agglutinated by anti-i

- Ham's Test: RBCs lyse in acidified serum

Back 196

Congenital Dyserythropoietic Anemias (CDAs)

Front 197

Type 3 of this disease includes:

- Mild-mod. macrocytic anemia

- 30% gigantoblasts (multinucleated erythroblasts w/ ~12 nuclei)

- Autosomal dominant (monoclonal gammopathy)

- Sporadic occurance: mental retardation & lymphoma related

Back 197

Congenital Dyserythropoietic Anemias (CDAs)

Front 198

This is a rare, stem cell disorder characterized by recurrent episodes of hemoglobinuria & clots

- Associated w/ aplastic anemia

Back 198

Paroxysmal Nocturnal Hemoglobinuria

Front 199

This disease is caused by a mutation on the X chromosome by either insertion or deletion & causes a fringe area & INTRAvascular hemolysis

Back 199

Paroxysmal Nocturnal Hemoglobinuria

Front 200

This mutation has a complete/partial deficiency of cell surface anchor protein glycophosphatidylinositol (GPI)

- RBCs: Highly sensitive to complement mediated hemolysis

Back 200

Paroxysmal Nocturnal Hemoglobinuria

Front 201

This disease state is found on the phosphatidyl inositol glycan A (PIG-A) gene

Back 201

Paroxysmal Nocturnal Hemoglobinuria

Front 202

This disease state uses Flow Cytometry to test for presence/absence of markers

- Also cannot bind to complement so it binds to & attacks RBCs

Back 202

Paroxysmal Nocturnal Hemoglobinuria

Front 203

Clinical findings of this disease:

- Insidious onset

- Adults usually affected

- Anemia: mild-severe (from chronic hemolysis)

Back 203

PNH

Front 204

You diagnose this disease by collecting a 1st morning specimen: contains gross hematuria (25%)

- During sleep, decreased pH (acidic) causes cell lysis

Back 204

PNH

Front 205

This disease happens w/ infection, surgery, trx

- And you verify w/ microscopic exam- there will be NO intact RBCs

- Venous thrombosis (~33%)

- Leukopenia @ some pt.

Back 205

PNH

Front 206

This disease has hemosiderinuria as a constant feature & leads to IDA; hemolysis possibly leads to chronic renal failure due to tubular damage

Back 206

PNH

Front 207

Lab findings of this disease:

- Anemia: mild-severe

- Blood smear: Normal w/ possible schistocytes

-Retics: ⬆⬆ (5-10%)

- Abs. retic: Possibly ⬇

- leukopenia: ⬇b/c WBCs more complement sensitive

- Thrombocytopenia: 50-100,000 ⬇⬇⬇

- BM: Erythroid hyperplasia from chronic hemolysis

- Indirect bilirubin: ⬆

Back 207

PNH

Front 208

This disease uses Sugar Water Test (Sucrose hemolysis test):

- Sucrose med-low ionic strength

- Promotes (C3) complement binding

- Normal cells unaffected, but 10-80% RBC lysis

Back 208

PNH

Front 209

This disease uses the Ham's Test to confirm after using the Sugar water Test

Back 209

PNH

Front 210

This is the 'acidified serum lysis' test. The acidified serum activates complement via ALTERNATIVE pathway & allows C3 binding

Back 210

Confirmatory Ham's Test for PNH

Hint 210

pg. 206 in notes

Front 211

This disease lacks GPI anchor proteins & normal cells are unaffected.

Back 211

Ham's Test for PNH

Hint 211

pg. 206 in notes

Front 212

This test assesses presence or absence of GPI anchor proteins

- uses monoclonal Ab's to CD55 & CD59

- Decrease/absence of expression is indicative of this disease

Back 212

Flow Cytometry testing used for Paroxysmal Nocturnal Hemoglobinuria (PNH)

Front 213

This disease needs supportive treatment (blood trx- long term)

- BM transplant is considered a cure

- Chronic disease: many survive ~20-40 yrs after diagnosis (avg. ~10)

- Death: results from blood clots, or hemorrhage

- Some will go into remission or progress into AML

Back 213

Paroxysmal Nocturnal Hemoglobinuria (PNH)

Front 214

This level rarely exceeds 3-4 mg/dL in uncomplicated hemolytic states

Back 214

Indirect (unconjugated) bilirubin

Front 215

What is the normal level for serum haptoglobin?

Back 215

40-336 mg/dL

Hint 215

-Reflects rapid clearance by RES of Hgb/haptoglobin complex

Front 216

What other tests are done to test for hemolytic states?

Back 216

-Urine dipstick: hemoglobinuria & microscopic exam

-Urine sediment: hemosiderinuria using Prussian Blue

- Retic counts

- RPI: >2.5 indicative of hemolytic state

Hint 216

There are 4 other options to look at for finding hemolysis

Front 217

What are some forms of hereditary hemolytic anemia that is due to an intrinsic defect?

Back 217

- RBC membrane & enzyme defects

- Hemoglobinopathies

- Thalassemia

Hint 217

There are 4 main ones

* structural defects are ALWAYS hereditary

Front 218

What is 1 form of acquired hemolytic anemia that is due to an intrinsic defect?

Back 218

Paroxysmal Nocturnal Hemoglobinuria (PNH)

Front 219

What are some forms of hemolytic anemia that are caused from extracorpuscular defects?

Back 219

- Immune hemolytic anemia

- Infections

- Exposure to chemicals, toxins, physical agents

- Micro/macroangiopathic hemolytic anemia

- Spleenic sequestering

Front 220

What are some main features of RBC membrane structure?

Back 220

- membrane proteins provide strength & flexibility

- Surface area to volume ratio

- Cytoplasmic viscosity

- Elastic properties (structural/ functional skeleton integrity)

Front 221

What are the main protein components of a red blood cell?

Back 221

- SPECTRIN, ankyrin, adducin, band 4.1, band 3, band 4.2

- Hexagonal lattice shape

Hint 221

**More details on pg. 211 of notes

Front 222

This disease is autosomal dominant, common in N. European descent, usually deficient in SPECTRIN, ankyrin, Band 3 & 4.2

Back 222

Hereditary Spherocytosis (HS)

Front 223

This disease loses surface to volume ratio-- results: stomatocytes & spherocytes, cells have decreased deformability

- Protein defects weaken vertical interactions w/ skeleton & lipid bilayer

Back 223

Hereditary Spherocytosis (HS)

Front 224

This disease shows jaundice, anemia & enlarged spleen

- Acholuric jaundice: lacking bile pigment & many have pigment gallstones

- 1/4 of people asymptomatic

- Age variable

- Severe: Aplastic crisis from infection (parvovirus B19) & chronic leg ulcers

Back 224

Hereditary spherocytosis

Front 225

Lab findings of this disease:

- Increased bilirubin, retics, polychromasia, spheros, stomatos

- MCV, MCH: normal

- MCHC: >36% (~50% of cases)

- Uses Osmotic Fragility Test

Back 225

Hereditary Spherocytosis

Front 226

This tests an RBCs surface to volume ratio. It's a series of graded hypotonic salt solutions

- Water enters cells, if hgb leaks out or cells burst- determines how tolerable a cell is to swelling

Back 226

Osmotic fragility test for hereditary spherocytosis

Front 227

This hereditary disease state causes a 'shift to the right' b/c a cell lyses at higher salt concentrations than normal (since they're already full)

Back 227

Osmotic fragility test for hereditary spherocytosis

Front 228

This test measures structural & metabolic integrity w/ glucose depletion conditions

- Need to incubate RBCs 48 hrs @ 37 degrees in own plasma

- In HS, autohemolysis of 10-50%

Back 228

Autohemolysis Test for HS

Front 229

This is a rapid test similar in sensitivity & specificity to osmotic fragility

- Lysing occurs within 5 min. vs. 30 min for osmotic fragility

Back 229

Acidified Glycerol Lysis Test for HS

Front 230

This test shows SDS-PAGE... shows quantitative defects

Back 230

RBC Membrane Studies in HS

Front 231

Treatment for this disease includes:

- Splenectomy- but not recommended in children <6 yrs (can cause sepsis)

- Children instead get prophylactic antibiotics

Back 231

Hereditary Spherocytosis (HS)

Front 232

This disease is autosomal dominant & has an abnormality on the RBC membrane skeleton involving spectrin & band 4.1

- There are 3 phenotypes

Back 232

Hereditary Elliptocytosis (HE)

Front 233

This is 1 of the phenotypes of this disease:

- MOST COMMON FORM- especially in African populations

- Hereditary Pyropoikilocytosis (HPP)

- Retics: ⬆

- Haptoglobin: ⬇

Back 233

Hereditary Elliptocytosis

Front 234

This is 1 of the phenotypes of this disease:

- It's rare and is a hybrid form of HE & HS

Back 234

Spherocytic Hereditary Elliptocytosis

Front 235

This is 1 of the phenotypes of this disease:

- It has a protective effect against malaria & is common in Melanesian/ southeast asian & South African populations

Back 235

Southeast Asian Ovalocytosis (SAO)

Front 236

This is a very rare, severe hemolytic disease-- results from vertical AND horizontal defects

- Has 2 genetic defects

Back 236

Hereditary Pyropoikilocytosis (HPP)

Front 237

The 2 genetic defects of this disease are:

- Mutant alpha or beta spectrin that impairs spectrin dimers

- ⬇ synthesis of alpha spectrin (makes microspherocytes)

Back 237

Hereditary Pyropoikilocytosis (HPP)

Front 238

The lab findings of this disease:

- Microspherocytes, micropoikilocytes, fragments, maybe a few elliptocytes

- MCV: ⬇

- MCHC: ⬆

- Osmotic fragility: pre & post incubation period ⬆

- Autohemolysis Test: ⬆ & unaffected by glucose

Back 238

Hereditary Pyropoikilocytosis (HPP)

Front 239

These 2 diseases have horizontal molecular defects between proteins & skeletal membrane

- Defective spectrin dimers or band 4.1 deficiency: means weak skeleton, distorted cells, cells lose ability to retain original shape

Back 239

Hereditary Elliptocytosis AND Hereditary Pyropoikilocytosis

Front 240

This disease is very mild autosomal dominant hemolytic anemia. Has gene mutation for Band 3

- ⬆cell membrane rigidity & malaria resistance

- found in southeast Asian malarial zones

Back 240

Southeast Asian Ovalocytosis

Front 241

Lab findings of this disease:

- >30% elliptocytes

- MCV: normal or slight increase

- MCH & MCHC: normal

- Osmotic fragility: Normal

- Autohemolysis test: Normal

Back 241

Southeast Asian Ovalocytosis

Front 242

Treatments for this disease include splenectomy if it's uncompensated, otherwise there's no treatment in the compensated version

Back 242

Southeast Asian Ovalocytosis

Front 243

What are 2 disease states that have membrane cation permeability (Na+/K+) defects?

Back 243

1) Hereditary Stomatocytosis

2) Hereditary Xerocytosis

Front 244

This disease is also called hydrocytosis, have very swollen cells

- 5-50% stomatocytes, Macrocytic

- MCV: ⬆ (110-150 fL)

- RBC K+ conc. ⬇

- RBC Na+ conc. ⬆

- Osmotic fragility: ⬆

Back 244

Hereditary Spherocytosis (HST)

Front 245

This disease has many target cells, RBCs look dehydrated

- MCHC: ⬆

- Echinocytes w/ Hgb concentrated in 1 part of cells (it pools on 1 side of the cell)

- Excessively permeable to K+ so it leaks out BUT is not replaced by Na+, so the cation conc. is ⬇ (meaning water loss in cell)

- Osmotic Fragility: ⬇⬇

Back 245

Hereditary Xerocytosis (HX)

Front 246

What is the most common enzyme deficiency?

Back 246

G6PD

Front 247

This disease state affects >400 million worldwide (highest #'s in dark pigmented ethnic groups)

- X Chromosome mutation (men= full expression when inherited from mother & women= full expression when 2 mutant genes are inherited)

Back 247

G6PD deficiency of the hexose monophosphate pathway

Front 248

This disease is mainly asymptomatic & 20% of activity is considered sufficient for normal RBC function & survival

- See bite & helmet cells

- Symptoms: back pain, hemoglobinuria & jaundice

- Have sensitivity to fava beans: develop severe hemolysis after eating these beans or inhaling the plant's pollens

Back 248

G6PD defiency of the hexose monophosphate pathway

Front 249

This disease has to account for family & drug history

- RBC count: ⬇ 2-3 days after taking drugs (if drug induced)

- Retics: ⬆

- Haptoglobin: ⬇ / absent

- Normocytic/ Normochromic, Heinz bodies, bite/ helmet cells

Back 249

G6PD defiency of hexose monophosphate pathway

Front 250

Tests for this disease include:

1) Methemoglobin reduction test

2) Fluorescent spot test

Back 250

G6PD deficiency

Front 251

This test is done when RBCs fail to reduce metHgb in presence of methylene blue

Back 251

Methemoglobin reduction test in G6PD deficiency

Front 252

This test is positive in G6PD ONLY deficiency

- Mix G6PD+saponin+buffer+ blood sample put on filter paper

- G6PD converts NADP-->NADPH

- View under fluorescent light

- If deficient in G6PD: Fails to fluoresce (+ test)

- If the test fluoresces: (- test)

Back 252

Fluorescent Spot Test in G6PD deficiency

Front 253

This disease is autosomal recessive (2 variants usually inherited, 1 from each parent) & both genders equally affected

- Pennsylvania Amish has high frequency

- Decreased ability to make ATP (lack of ATP results in cell water loss, shrinkage, distorted, rigid, early destruction)

Back 253

Embden-Meyerhof pathway enzyme

PYRUVATE KINASE DEFICIENCY

Front 254

This deficiency's severity depends on mutation & are true homozygotes--> anemic & jaundice @ birth, need trx throughout life

- Anemia increases w/ infection or stress: jaundice, enlarged spleen, pigmented gallstones

- RBC 2,3-DPG levels ⬆ =⬇ oxygen affinity

Back 254

Pyruvate Kinase Deficiency

Embden- Meyerhof pathway enzyme deficiency

Front 255

Lab findings of this disease include:

- Normo/Normo, polychromasia, nRBCs, Poik & aniso

- Retics: varied

- Hgb, Hct, Haptoglobin: ⬇

- Unconjugated bilirubin: ⬆

Back 255

Pyruvate Kinase Deficiency

**Embden- Meyerhof pathway enzyme deficiency

Front 256

Lab tests of this disease:

- Fluorescent spot test: if blood lacks enzyme= NADH won't be oxidized =fluorescence lasts 45 min-1 hr. IF enzyme is present =fluorescence is gone in ~15 min

- Quantitative: 5-25% normal

- Osmotic fragility: ~normal

- Autohemolysis: No correction w/ glucose addition

Back 256

Pyruvate Kinase Deficiency

** Embden- Meyerhof pathway enzyme deficiency

Front 257

This disease state reduces ferric -->ferrous iron (+3 --> +2)

- Methemoglobinemia occurs w/ decreased enzyme activity, production exceeds reducing capacity, Hgb M disease

- Cyanosis occurs w/ increased metHgb levels

- Normal metHgb: <1%

Back 257

Methemoglobin Reductase Pathway

Front 258

This disease's congenital form is autosomal recessive & hetero zygotes are asymptomatic until drugs express them

- The acquired form is induced by drugs/toxic substances that oxidize Hgb in circulation

Back 258

Methemoglobin Reductase Pathway