The Anemias And Polycythemias

Front 1

Anemia

Back 1

A decrease in the ability of the blood to carry oxygen to the tissues.

Front 2

Anemia is a decrease of ______________________.

Back 2

Hemoglobin or erythrocytes.

Front 3

Anemia isn't a disease, but rather:

Back 3

An expression of an underlying disease or disorder.

Front 4

Adaption of anemia depends on:

Back 4

Rate of onset, severity of blood loss, and ability of the body to adapt.

Front 5

Adaptions to anemia:

Back 5

Increase in cardiac rate, increase in cardiac output, increase in circulation rate, and preferential increase in blood flow to vital organs.

Front 6

Morphological classifications of anemia

Back 6

Macrocytic/Normochromic

Normocytic/Normochromic

Microcytic/Hypochromic

Front 7

Etiological classification of anemia

Back 7

Decreased production of RBCs, increased RBC destruction or loss, and hemoglobinopathy (hemoglobin disease).

Front 8

Morphology of macrocytic anemias

Back 8

Macrocytic normochromic.

Front 9

Megaloblastic anemia

Back 9

Due to abnormal DNA synthesis (nuclear maturation defect which leads to asynchronous maturation of RBCs). Most often due to vitamin b12 or folate deficiencies.

Front 10

Non megaloblastic anemia (normoblastic)

Back 10

Cause isn't well defined; can be related to an increase in membrane lipids.

Front 11

How is b12 related to folate?

Back 11

B12 is a cofactor in the conversion of methyl tetrahydrofolate

(folic acid) to tetrahydrofolate.

Front 12

The product of folic acid to tetrahydrofolate is a cofactor needed for:

Back 12

Production of DNA thymidine.

Front 13

What does thymidine synchronize?

Back 13

The cells in the s phase of mitosis.

Front 14

What are 95% of megaloblastic anemias caused by?

Back 14

B12 or folic acid deficiencies.

Front 15

Vitamin b12 deficiencies are usually secondary to a deficiency in _____________ vs a ____________ deficiency.

Back 15

Intrinsic factor, nutritional deficiency.

Front 16

Most cases of folic acid deficiency (megaloblastic anemia) are due to ____________________.

Back 16

Nutritional deficiency.

Front 17

What kind of inherited disorders of megaloblastic anemia are rare?

Back 17

Disorders affecting the synthesis of DNA.

Front 18

What is intrinsic factor?

Back 18

Comes from parietal cells in the stomach. If absent, can't absorb folic acid.

Front 19

Sources of vitamin b12

Back 19

Liver/meat

Milk and eggs

Mollusks/clams

Fish

Fortified cereals

Front 20

Dietary sources of folic acid

Back 20

Green leafy veggies

Dried peas and beans

Mushrooms, yeast

Front 21

Where is vitamin b12 stored?

Back 21

Liver, heart, and kidneys.

Front 22

Hematological findings in megaloblastic anemia

Back 22

🔺 MCV

Normal MCH

🔻 RBC, WBC, platelets, retic count

Macrocytisis of RBCs.

Basophilic stippling

Howell jolly bodies

Schistocytes and target cells

Hyper segmented neutrophils, oval shaped RBCs.

Front 23

What is the first and last thing to be seen in megaloblastic anemia morphology?

Back 23

Hyper segmented neutrophils.

Front 24

Clinical findings in megaloblastic anemias

Back 24

Seen more in women, usually affects middle age or older, SOB, light headedness, weakness, pallor, glossitis, and megaloblastic madness.

Front 25

Megaloblastic madness

Back 25

Only seen with b12 deficiencies. Brain needs b12. Dementia can develop.

Front 26

Pernicious anemia

Back 26

Autoimmune disorder caused by a 🔻 of IF that is secreted by parietal cells of gastric mucosa.

Front 27

What % of pernicious anemia patients have antibodies against parietal cells?

Back 27

90.

Front 28

What is IF necessary for?

Back 28

Absorbing b12.

Front 29

Most common cause and what % of b12 deficiencies

Back 29

Pernicious anemia, 85%.

Front 30

Other causes of b12 deficiencies

Back 30

Partial or complete gastrectomy and gastric atrophy associated with aging.

Front 31

Lab diagnosis of pernicious anemia

Back 31

Gastric analysis, shilling test, serum vitamin b12 assay, methylmalonic acid assay.

Front 32

What is MMA?

Back 32

Serum analysis of b12 and MMA confirm the decrease of b12, but can't distinguish pernicious anemia from other causes of b12 deficiency.

Front 33

Which tests are specific for pernicious anemia?

Back 33

Gastric analysis and the shilling test, but aren't commonly performed.

Front 34

Shilling test part 1

Back 34

Back (Definition)

Front 35

Part 2 of shilling test

Back 35

Back (Definition)

Front 36

Folate deficiency can occur to one of the following reasons:

Back 36

1. Inadequate intake: seen in the poor and elderly.

2. Increased requirement: hemolytic anemias and pregnancy.

3. Malabsorption

4. Drug inhibition: oral contraceptives, long term anticoagulant use, anti seizure meds: phenobarbital, primidone, and phenytoin.

Front 37

Other causes of megaloblastic anemia

Back 37

Strict vegetarian, malabsorption syndromes (tropical sprue or chronic wasting and non tropical sprue aka gluten sensitive enteropathy), Crohn's disease, infection with diphyllobothrium latum, and chemotherapy drugs.

Front 38

Most common cause of non anemic macrocytosis and associated conditions.

Back 38

Alcoholism. Folate deficiency due to decreased dietary intake, reticylocytosis due to hemolysis or GI bleeding, liver disease, and alcohol toxicity to erythroid precursors.

Front 39

Other conditions that contribute to macrocytic anemia without megaloblastosis

Back 39

Liver disease, hemolysis or post hemorrhagic anemia, hypothyroidism, and aplastic anemia.

Front 40

Primary storage site of folate

Back 40

Liver.

Front 41

Non megaloblastic anemia hematological findings

Back 41

Normal WBC, platelet count.

🔻 RBC and H&H

MCV < 100 fl

Polychromasia, target cells, stomatocytes in liver disease, schistocytes in hemolytic anemia.

NO hyper segmented neutrophils.

Front 42

Where is most iron stored?

Back 42

Liver

Front 43

Ferritin

Back 43

Primary storage compound. Spherical protein shell that can store 4500 iron atoms.

Front 44

Hemosiderin

Back 44

Molecule of carbohydrate, lipid, protein, and iron.

Front 45

Where is hemosiderin primarily found and what is it formed by?

Back 45

Primarily in macrophages and formed by partial degradation of ferritin.

Front 46

Each gram of transferrin binds ___ mg of iron.

Back 46

1.4

Front 47

Enough transferrin is present in plasma to bind _____________ micrograms of iron/dL of plasma. This is referred to as _______________________________.

Back 47

253-435. Total iron binding capacity.

Front 48

Serum concentration is _______ micrograms/dL and ___% is complexed with transferrin.

Back 48

70-201, 95.

Front 49

Cell morphology of iron deficiency anemia

Back 49

Microcytic, hypochromic.

Front 50

Etiology of IDA in neonates

Back 50

Maternal iron deficiency, multiple births.

Front 51

Etiology of IDA in premenopausal women

Back 51

Menstrual blood loss, inadequate diet and malabsorption, pregnancy.

Front 52

Etiology of IDA in adult males and postmenopausal women

Back 52

Chronic blood loss, inadequate diet, GI tract disease: peptic ulcers, malignancies, excess aspirin ingestion, alcohol gastritis, hookworms, and hemorrhoids.

Front 53

Number one cause of IDA in adult males and postmenopausal women

Back 53

Chronic blood loss.

Front 54

Etiology of IDA in infants and children

Back 54

Growth spurts, inadequate diet, and malabsorption.

Front 55

Etiology of IDA not related to age or sex

Back 55

Inherited or acquired bleeding disorders and hemodialysis.

Front 56

What is heme iron derived from?

Back 56

Hemoglobin, the protein in RBCs that deliver oxygen to cells.

Front 57

What is heme iron found in?

Back 57

Animal foods that originally contained hemoglobin, such as red meats, fish, and poultry.

Front 58

How is iron in plant foods arranged?

Back 58

Into a chemical structure called nonheme.

Front 59

What form of iron is added to iron enriched foods?

Back 59

Nonheme.

Front 60

Which form of iron is absorbed easier?

Back 60

Heme iron.

Front 61

Most dietary iron is what type?

Back 61

Nonheme.

Front 62

Most common nutritional deficiency

Back 62

Iron deficiency.

Front 63

Beverages that complement absorption of iron

Back 63

Orange juice, vitamin c, pickles, vinegar, alcohol.

Front 64

Beverages that impede absorption of iron

Back 64

Coffee, tea, milk.

Front 65

Clinical findings of IDA

Back 65

Insidious onset: over several years or months, early stages usually show no s/s.

Fatigue, pallor, lethargy, dyspnea, cold intolerance, palpitations, pica, chelitis, and koilonychia.

Front 66

Hematological findings of IDA

Back 66

🔻 MCV & MCHC

Slight 🔺 of RDW

Target cells

🔻 serum iron and ferritin

🔺 TIBC

Platelets may be 🔺 of due to hemorrhage

Front 67

Treatment of IDA

Back 67

Treatment of underlying disorder and iron therapy through drops or pills.

Front 68

What forms are iron preparations available in and what are the side effects?

Back 68

Ferrous sulfate, ferrous gluconate, and ferrous fumerate. Side effects include: constipation, stomach discomfort, or diarrhea.

Front 69

What is seen in morphology when iron therapy is working?

Back 69

Retics or polychromasia. 3 days after treatment is started. Peaks at 10 days.

Front 70

How long does it take for a patient to get back to normal after iron therapy?

Back 70

6-10 weeks.

Front 71

Morphological classification of anemia of chronic disorders

Back 71

Normocytic/normochromic; can become normocytic/hypochromic and in long standing cases, microcytic/hypochromic.

Front 72

2nd most common anemia

Back 72

Anemia of chronic disorders.

Front 73

Etiology of anemia of chronic disorders

Back 73

Chronic infections, rheumatoid arthritis, malignancies, organ failure, and lupus.

Front 74

Contributing factors of anemia of chronic disorders

Back 74

Reduced RBC life span, uncompensated bone marrow production, decreases iron transfer from the MPS storage sites to marrow.

Front 75

What is iron blocked from? (Anemia of chronic disorders)

Back 75

Release from macrophages by hepicidin.

Front 76

What is hepicidin produced in response to?

Back 76

Interleukin 6 and other inflammatory cytokines.

Front 77

Etiology of sideroblastic anemia

Back 77

Iron loading in the mitochondria due to a defect in heme synthesis.

Front 78

Inherited sideroblastic anemia etioligy

Back 78

Sex linked and autosomal recessive.

Front 79

Idiopathic sideroblastic anemia

Back 79

Referred to as refractory anemia with ringed sideroblasts or RARS. Result of stem cell disorder.

Front 80

Secondary sideroblastic anemia

Back 80

Secondary to drugs or chemicals, alcoholism, lead poisoning, may be seen in patients who need chronic transfusion, food faddists that take megavitamins.

Front 81

Morphological classification of sideroblastic anemia: inherited and acquired secondary form

Back 81

Dimorphic population of normocytic/normochromic and microcytic/hypochromic and dual populations of macrocytes and microcytes or normocytes.

Front 82

Where are macrocytic/hypochromic cells seen in (sideroblastic anemia)?

Back 82

RARS

Front 83

Treatment of sideroblastic anemia

Back 83

1. Pyridoxine (vitamin b6) although only effective in less than 50%.

2. Phlebotomy

3. Folic acid for those with macrocytic features.

4. Secondary forms are treated by treating the underlying disease or removing the toxic agent.

Front 84

Hemochromatosis

Back 84

Disease in which body absorbs and stores more iron than needed. Since the body has no natural way of ridding excess, iron is stored in tissue, especially in liver, heart, and pancreas.

Front 85

How old are most patients when they experience s/s of hemochromatosis?

Back 85

50s or 60s.

Front 86

Two forms of hemochromatosis

Back 86

Hereditary and secondary.

Front 87

Characteristics of hemochromatosis

Back 87

Increased iron absorption in the gut and progressive iron overload.

Front 88

# of people who get hereditary hemochromatosis

Back 88

1 in 200-250. 1 in 10 Caucasians in US is a carrier.

Front 89

Most common genetic abnormality in those with a European ancestry and how many genes are responsible?

Back 89

Hereditary hemochromatosis. 5.

Front 90

Which sex is more effected with hereditary hemochromatosis?

Back 90

Males.

Front 91

Secondary causes of hemochromatosis

Back 91

Anemias with ineffective RBCS.

Chronic transfusions

Chronic liver disease

Viral hepatitis

Dietary iron overload

Mega dosing with iron supplements

Alcoholism

Front 92

Lab findings of hemochromatosis

Back 92

*Ferritin greater than 200 in premenopausal women and greater than 300 in men and postmenopausal women.

*🔺 ferritin, iron, and transferrin

*TIBC normal

Front 93

Bronze diabetes

Back 93

Can develop with hemochromatosis due to accumulation of iron in the tissues which leads to a peculiar skin discoloration. Pancreas becomes fibrotic and nonfunctional due to the iron deposits.

Front 94

Treatment of hemochromatosis

Back 94

Therapeutic phlebotomies. Reduces ferritin levels and the hematocrit level is maintained at 35%. Can also be given an iron chelating agent - Desferal. Excreted in urine.

Front 95

Anemia of blood loss morphology

Back 95

Normocytic/normochromic

Front 96

Etiology of anemia of blood loss

Back 96

Sudden blood loss. After an acute major blood loss, the H&H will stay normal for several hours due to vasoconstriction. After this time, fluid enters the circulation and the H&H and RBC 🔻.

Front 97

Anemia of chronic renal insufficiency morphology

Back 97

Normocytic/normochromic with fragmented RBCs and burr cells.

Front 98

Anemia of chronic renal insufficiency etiology

Back 98

Due to damage of the kidneys and subsequent underproduction of erythropoietin (hormone that makes RBC).

Front 99

What chemistry levels are increased with anemia of chronic renal insufficiency?

Back 99

BUN and creatinine.

Front 100

Therapy for chronic renal disease includes:

Back 100

*Renal transplant

*Dialysis

*EPO

Front 101

Dialysis: treatment of renal disease

Back 101

Can cause additional causes of anemia including blood loss, iron and folate deficiency, and hemolysis. Patient can develop a macrocytic or microcytic anemia without proper supportive therapy.

Front 102

Aplastic anemia morphology

Back 102

Normocytic/normochromic.

Front 103

Acquired aplastic anemia

Back 103

Due to damage to the pluripotential stem cells resulting in pancytopenia.

Front 104

Damage to stems cells may be due to:

Back 104

An antibody that attaches to antigens on stem cells or an immune mechanism. That causes t lymphs to suppress stem cell division.

Front 105

Common causes of acquired aplastic anemia

Back 105

Radiation, certain drugs, viruses, and exposure to certain chemicals. Most of the time the offending agent can't be identified and is idiopathic.

Front 106

Age groups aplastic anemia is found in

Back 106

2-5 years

15-25 years

Adults over 60

Front 107

Occurrence of aplastic anemia in children is mostly due to:

Back 107

Inherited forms, while most adult cases are acquired.

Front 108

There are more cases of aplastic anemia in what geographical location?

Back 108

Asia probably due to environmental and occupational factors.

Front 109

Idiopathic aplastic anemia %

Back 109

Approx 50-70%.

Front 110

Acquired form of aplastic anemia: drugs

Back 110

Approx 30% of cases.

Front 111

Acquired aplastic anemia: chemicals

Back 111

Industrial chemicals such as benzene and chemotherapy.

Front 112

Acquired form of aplastic anemia: ionizing radiation

Back 112

Industrial accidents, military nuclear tests, and radiation therapy.

Front 113

Acquired aplastic anemia: infectious agents

Back 113

Viral and bacterial infections can be followed by transient cytopenia.

Front 114

Inherited (constitutional) aplastic anemia

Back 114

Aka Fanconi anemia. Autosomal recessive disorder resulting from a heterogenous molecular defect and is characterized by abnormal chromosomal fragility.

Front 115

# effected with inherited aplastic anemia

Back 115

1 in 350,000 live birth in North America and has a heterozygote frequency in approx 1 in 300,000.

Front 116

Anemia develops in _____ of Fanconi patients, usually between the ages of ______.

Back 116

90%, 5-10.

Front 117

Clinical findings of fanconi's anemia

Back 117

🔺 hgb F

Cutaneous hyperpigmention

Hypogonadism

Skeletal disorders: mostly thumbs

Microcephaly

Mental retardation

Front 118

Treatment of inherited aplastic anemia

Back 118

Removal of drug/environmental cause and bone marrow transplant.

Front 119

Survival rates for aplastic anemia bone marrow transplants

Back 119

90% for identical twins and 35% for mismatched donors.

Front 120

Clinical findings of HH

Back 120

*arthralgia

*lethargy

*abdominal pain

*cardiac problems