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BL Unit 1 > BL Ambruso lecture review > Flashcards

BL Ambruso lecture review Flashcards

1
Q

Define anemia.

A

Insufficient red cell mass to adequately deliver O2 to peripheral tissues.

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2
Q

What is hematocrit?

A

% volume of red cells in blood

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3
Q

What factors influence blood reference ranges? (hint consider your patient)

A

age, gender, geography

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4
Q

What are the 3 routine measurements in the diagnosis of anemia?

A

CBC, peripheral smear, retic count/index

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5
Q

What 3 questions should you ask in classifying anemia? (hint - questions are from a familiar slide)

A

1.) Is anemia associated with other hematologic abnormalities? 2.) Is there an appropriate retic response? 3.) What are the red blood cell indices?

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6
Q

What are some of the physical exam signs and symptoms of anemia?

A

pallor, tachycardia, shortness of breath, tachypnea, dyspnea, systolic murmur.

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7
Q

At which ph (low or high) is iron more soluble?

A

Low (acidic) pH

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8
Q

T or F: Iron is actively excreted from the body?

A

FALSE

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9
Q

What factors positively influence iron absorption?

A

pH, Vitamin C, phytates and oxalates, and EPO production

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10
Q

Describe the iron cycle.

A

Iron is absorbed through the intestines into the plasma. Transferrin transports the iron the the liver for storage or the bone marrow where it is incorporated into the RBCs. When RBCs die, the reticoluendothelial macs release it back into the intestine.

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11
Q

What does hepcidin do?

A

Decreases iron absorption esp during inflammation/infection

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12
Q

In which population is iron deficiency most common?

A

Adolescent females (11%)

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13
Q

T or F: MCV will be increased in iron deficiency anemia?

A

FALSE

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14
Q

T or F: TIBC will be increased in iron deficiency anemia?

A

TRUE

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15
Q

What is the differential diagnoses for iron deficiency anemia (hint - 4 things)?

A

Anemia of chronic inflammation/infection, anemia of chronic disease, thalassemia, sideroblastic anemia

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16
Q

Which gene abnormality will cause increased iron absorption?

A

HLA-H gene - a protein produced by duodenal crypt cells and reticuoloendothelial cells which acts as a co-factor for iron absorption.

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17
Q

What are the consequence of iron overload?

A

Organ damage (heart, liver, endocrine).

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18
Q

How is iron overload treated?

A

Therapeutic phlebotomy (blood-letting) and iron chelators.

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19
Q

Which cytokines are important in the pathophysiology of the anemia of neoplasms/sepsis?

A

TNF and INFBeta

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20
Q

Which cytokines are important in the pathophysiology of the anemia of chronic infection/inflammation?

A

IL-1 and INFgamma

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21
Q

How does lead cause anemia?

A

Inhibits heme and protoporphyrin synthesis

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22
Q

How does renal insufficiency cause anemia?

A

Decreased EPO sythesis

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23
Q

How doe endocrine disorders cause anemai?

A

Can by caused by hyperthyroidism and adrenal insufficiency.

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24
Q

How is lead poisoning treated?

A

Removal of source of lead and possibly chelation therapy.

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25
Q

How is anemia of chronic disease treated?

A

Treat underlying disease and iron deficiency. Also, EPO is used in some cases.

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26
Q

How is anemia of endocrine disorders treated?

A

Hormone replacement therapy and co-morbid conditions

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27
Q

How is renal deficiency anemia treated?

A

EPO

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28
Q

Why are B12 and folate important for hematopoiesis?

A

Important for conversion of homocysteine to methionine, purine and pyrimidine synthesis, and DNA synthesis

29
Q

Where is folate absorbed?

A

In the jejunum

30
Q

Where is B12 absorbed?

A

In the ileum

31
Q

What are the primary causes of folate deficiency?

A

Dietary, malabsorption, mutations in metabolism, increased demands, increased loss

32
Q

What are the primary causes of B12 deficiency?

A

Malabsorption,

33
Q

What smear findings are pathonomic of B12 or folate deficiency?

A

Macrocytic, hypochromic anemia with hypersegmented neutrophils

34
Q

What anemia causes abnormal neurological findings?

A

B12 deficiency?

35
Q

What is another name for B12?

A

Cobalamin

36
Q

Which tests are good at determining B12 vs folate deficiency?

A

Serum folate and serum B12, methylmalonic acid, and deoxyuridine test

37
Q

What is the Schilling test?

A

Tests for B12 deficiency. Ingest radiolabeled B12 followed by injection of B12. If radiolabeled B12 comes out in urine (5-35%), then oral B12 is absorbed. If less than that, oral B12 is not absorbed.

38
Q

Which hormone assists in B12 absorption?

A

Intrinsic factor

39
Q

Where is intrinsic factor produced?

A

Stomach by gastric parietal cells

40
Q

How long does it take for folate deficiency to appear?

A

Days to weeks

41
Q

How long does it take for B12 deficiency to appear?

A

Months to years

42
Q

What are some general signs to the body of red cell age?

A

Changes in extracellular markers, decrease in enzyme activity, oxidative injury, changes in calcium balance.

43
Q

What lab results are evidence of increased hemolysis?

A

Inc bilirubin, inc LDH, dec haptoglobin, hemosiderin in urine

44
Q

What is the reticulocyte in hemolysis versus iron deficiency?

A

Hemolytic anemia will result in increased retic count, while iron deficiency will result in decreased retic count.

45
Q

Describe lab results in hereditary spherocytosis.

A

Dec MCV, increased osmotic fragility

46
Q

Which proteins are implicated in hereditary spherocytosis?

A

Ankyrin, specrin, band 3

47
Q

What is the inheritance pattern of hereditary spherocytosis?

A

75% autosomal dominant, 25% autosomal recessive

48
Q

Removal of which organ helps to relieve symptoms of hereditary spherocytosis?

A

Spleen

49
Q

What other organ is commonly removed in hemolytic diseases?

A

Gallbladder

50
Q

What is the inheritance pattern of G6PD deficiency?

A

X linked recessive

51
Q

In which regions of the world is G6PD deficiency most common?

A

Malaria belt - subtropical Easter hemisphere

52
Q

T of F: G6PD primarily results in intravascular hemolysis?

A

False; it results in extravascular hemolysis.

53
Q

What is the most common cause of acute anemia in G6PD deficiency?

A

Oxidant stress: fava beans, drugs

54
Q

Which supplement is often given for hemolytic diseases?

A

Folate. It helps when people have increased need for RBC production.

55
Q

What might be seen in the peripheral smear of G6PD

A

Microcspherocytosis, blister cells, bite cells

56
Q

Which immune components are reactive in cold autoimmune hemolytic anemia?

A

Complement and IgG

57
Q

Which immune component is reactive in warm autoimmune hemolytic anemia?

A

IgG

58
Q

Which test is used for warm and cold autoimmune hemolytic anemia?

A

Coombs test- indirect and direct

59
Q

What is the function of the spleen?

A

Clearance of intravascular particles, produces IgM agglutinins especially for encapsulated organisms.

60
Q

What should done to manage a splenectomy?

A

Because asplenic patients have difficulty fighting certain infections, asplenic patients should receive vaccinations, prophylactic penicillin, and seek physician assistance for fever.

61
Q

What infectious diseases are regularly tested for?

A

Syphilis, Hep ABC, HIV.

62
Q

Describe warm agglutination reaction.

A

Strong at at 37 C, extravascular hemolysis, strongly activates IgG and complement, usually resolves with clearance of infection.

63
Q

Describe cold agglutination reaction.

A

Strong at 4 C, intravascular hemolysis, strongly activates IgG and complement

64
Q

What other syndrome can cause cold agglutination?

A

Donath Landsteiner syndrome - 4 C, mostly intravascular hemolysis, strongly activates complement, usually resolves with clearance of infection.

65
Q

How much blood can you usually take out of one patient at one time?

A

450-500 mL whole blood

66
Q

Which blood components must be crossmatch compatible?

A

fresh frozen plasma and cryoprecipitate. Crossmatch is required for all other blood products including platelet concentrate.

67
Q

What is the most common reason for transfusion reaction?

A

Clerical error in label or handoff of blood products.

68
Q

T or F: Acute hemolytic transfusion reactions are not a big deal.

A

False; Mortality rate is high at 40%, any patient with a reaction should be managed aggressively with heparin, fluids, and diuretics.

69
Q

Name the less severe transfusion reactions (8 things).

A

Delayed hemolytic transfusion reaction, febrile reaction, allergic reaction, transfusion-related lung injury, dilutional coagulopathy, bacterial contamination, graft-versus-host disease, iron overload

BL Unit 1 (5 decks)

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