Clin Med II: Green PA book info Flashcards

1
Q

What is anemia?

A

Low hemoglobin level, RBC count, or hematocrit

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2
Q

What is polycythemia?

A

Elevated hemoglobin, RBC count, or hematocrit

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3
Q

What is the normal hemoglobin concentration in males?

A

13.5-17.5 g/dl

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4
Q

What is the normal hemoglobin concentration in females?

A

12-16 g/dl

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5
Q

What is Hematocrit?

A

% of blood that is RBC

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6
Q

What is a normal hematocrit in males?

A

41-53%

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7
Q

What is a normal hematocrit in females?

A

36-46%

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8
Q

What 3 materials are needed for RBC production?

A

B12, Folate, Iron

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9
Q

What do the RBCs look like in iron deficiency anemia?

A

Hypochromic (pale) and microcytic (small)

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10
Q

What are the best first tests to assess RBC disorders?

A

CBC and peripheral blood smear to assess cell morphology

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11
Q

Where and how are RBC manufactured?

A

Bone marrow stem cells under the hormonal influence of erythropoietin secreted by the kidney

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12
Q

Why does renal failure cause anemia?

A

Lack of erythropoietin production

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13
Q

What are the most common anemias found in clinical practice?

A

Microcytic anemias

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14
Q

What is the diff dx for microcytic anemia?

A
TICS
T: Thalassemia
I: Iron deficiency
C: Chronic inflammatory block
S: Sideroblastic (eg lead toxicity)
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15
Q

What is Thalassemia syndrome?

A

Genetic underproduction of alpha or beta globin chains resulting in deficient hemoglobin synthesis and RBC hemolysis

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16
Q

What population is Thalassemia most common seen in?

A

Alpha: people of Southeast Asian or Chinese origin
Beta: African and Mediterranean populations

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17
Q

When should Thalassemia be expected in a person?

A

When they have a positive family history or a personal history of lifelong microcytic anemia with normal iron stores

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18
Q

What are the clinical features of alpha-thalassemia?

A

Patients usually have mild to no symptoms

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19
Q

When is alpha-thalassemia usually diagnosed?

A

After a non-response to treatment for suspected iron deficiency

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20
Q

When do Beta-Thalassemia major symptoms begin?

A

4-6 months old, when the switch from fetal hemoglobin to adult hemoglobin occurs

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21
Q

What are the symptoms of Beta-Thalassemia major?

A

Severe anemia, growth retardation, abnormal facial structure, pathologic fractures, osteopenia, bone deformities, hepatosplenomegaly, and jaundice

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22
Q

What diagnostic studies are used for Beta-Thalassemia major and what are the results?

A

Hematocrit: as low as 10%
Hgb electrophoresis: Hgb F and A2
Peropheral smear: Target cells, Poikilocytes, Basophilic stippling, Nucleated RBCs
Reticulocyte count: Increased
Serum iron and ferritin: normal or elevated
Hgb: 3-6 g/dL
microcytosis

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23
Q

What study confirms the diagnosis of Beta-Thalassemia major?

A

Hemoglobin electrophoresis

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24
Q

Tx for Beta-Thalassemia major?

A

Transfusions to keep Hgb at 12 g/dL

-Allogenic bone marrow transplantation being used with increasing success

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25
Q

What is the most common cause of anemia worldwide?

A

Iron deficiency

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26
Q

What are the most common causes of iron deficiency anemia in adults?

A

GI bleeds secondary to PUD, NSAID use, or cancer (likely cause), heavy menstrual cycle- but rule out GI bleed first

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27
Q

What causes iron deficiency anemia in children?

A

Low dietary intake of iron

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28
Q

What are some other causes of iron deficiency anemia?

A

Decreased absorption of iron, increased requirements, hemoglobinuria, blood donation, iron sequestration, trauma, and intravascular hemolysis

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29
Q

What are the symptoms of moderate-severe iron deficiency anemia?

A

Pallor, easy fatiguability, irritability, anorexia, tachycardia, tachypnea on exertion, poor weight gain in infants

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30
Q

What is a hallmark of iron deficiency?

A

Pica

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31
Q

What symptoms are seen with severe iron deficiency?

A

Brittle nails, cheilosis, smooth tongue, and formation of esophageal webs (Plummer-Vinson syndrome)

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32
Q

What is seen on the diagnostic studies with iron deficiency anemia?

A
  • Hgb and hematocrit: decreased
  • Peripheral smear:
    • initially: no changes in red cell size
    • later: hypochromic microcytic red cells, anisocytosis, and poikilocytosis
  • Plasma ferritin level:
33
Q

Tx for iron deficiency anemia?

A

325mg of ferrous sulfate PO 3x daily in a slowly escalating dose
-therapy should be continued for up to 6 months to replenish tissue stores

34
Q

What is the most common cause of B12 deficiency?

A

Pernicious anemia due to the lack of intrinsic factor which is necessary for B12 absorption

35
Q

What are other causes of B12 deficiency?

A

Strict vegan diet, gastric surgery, blind loop syndrome, pancreatic insufficiency, metformin, Crohn’s disease

36
Q

What can happen if B12 deficiency isn’t treated?

A

Neurological damage, yikes!

37
Q

Where does absorption of B12 occur in the body?

A

terminal ileum

38
Q

Where is B12 stored in the body?

A

liver

39
Q

What are the clinical features of B12 deficiency?

A
  • Mouth: Smooth tongue, glossitis, cheilosis
  • Neuro: stocking-glove paresthesias, loss of position, fine touch and vibratory sensation, balance problems and ataxia, dementia
40
Q

What is seen on diagnostic studies with B12 deficiency?

A
  • Peripheral smear: hypersegmented neutrophils, anisocytosis, poikilocytosis, macro-ovalocytosis
  • Serum B12: abnormally low
  • Anti-intrinsic factor antibodies
  • Serum MMA and homocysteine levels: increased
  • Serum LDL and indirect billirubin: elevated if hemolysis present
41
Q

What is the Tx for B12 deficiency?

A

Lifelong supplemental B12 (1,000 micrograms/month) given PO or IM

42
Q

What is the time frame to Tx B12 deficiency where you won’t see neuro damage?

A

within 6 months!

43
Q

How do you get G6PD deficiency?

A

It is hereditary! It is a X-linked recessive disorder

44
Q

What population is G6PD deficiency most commonly seen in?

A

American black males (10-15%) and some Mediterranean populations. Females are rarely affected!

45
Q

What can cause episodic hemolysis in G6PD deficiency?

A

Oxidative drugs (eg Aspirin, dapsone, primaquine, quinidine, sulfonamides, nitrofurantoin), fava beans, and infection

46
Q

How do patient’s with episodic hemolysis in G6PD deficiency present?

A

They are usually healthy and have no splenomegaly!

47
Q

What are the laboratory findings during hemolytic episodes in G6PD def?

A
  • Reticulocytes and serum indirect bilirubin increase
  • Peripheral smear: bite cells, Heinz bodies (denatured hemoglobin)
  • G6PD levels: low
48
Q

What is the treatment for hemolytic episodes in G6PD def?

A

Self-limited as red cells are replaced as soon as offending agent is stopped

49
Q

What is the treatment for hemolytic episodes in G6PD def?

A

Self-limited as red cells are replaced as soon as offending agent is stopped

50
Q

What is the most common cause of folic acid deficiency?

A

Poor dietary intake

-usually in alcoholics, persons with anorexia, and diet low in fruits and veggies

51
Q

What are the other causes of folic acid deficiency?

A

Defective absorption, pregnancy, chronic hemolytic anemias, alcohol abuse, folic acid antagonists (eg phenytoin, trimethoprim-sulfamenthoxazole, sulfasalazine)

52
Q

What is the daily requirement of folic acid?

A

50-100 mg/dL - usually met by balanced diet

53
Q

What conditions increase the daily requirements of folic acid?

A

Pregnancy, chronic hemolytic anemias, exfoliative skin disease

54
Q

What are the clinical features of folic acid deficiency anemia?

A

Glossitis, vague GI symptoms, no neurologic symptoms

55
Q

What lab results are pathognomonic of folic acid deficiency anemia?

A

Macro-ovalocytes and hypersegmented polymorphonuclear cells

56
Q

What are other laboratory findings with folic acid deficiency anemia?

A
  • Howell-Jolly bodies (nuclear DNA remnants)

- RBC folate level:

57
Q

What is the first line tx for folic acid deficiency anemia?

A

1mg of folic acid PO daily

58
Q

What lifestyle changes can help with folic acid def. anemia?

A

Avoid alcohol and folic acid metabolism antagonists (eg trimethoprim, seizure medications)

59
Q

Common tests and results for B12 def

A
Serum B12: low
Serum folate: normal
RBC folate: normal
Methylmalonic acid: high
Homocysteine: high
60
Q

Common tests and results for Folate def

A
Serum B12: normal or low
Serum folate: low
RBC folate: low
Methylmalonic acid: normal
Homocysteine: high
61
Q

What kind of hereditary disease is sickle cell anemia?

A

a family of autosomal recessive inherited hemoglobinopathies

62
Q

What is sickle cell anemia?

A

RBCs containing primarily Hgb S sickle under hypoxia, dehydration, acidosis, and extreme temp conditions

63
Q

What population is SC anemia most often seen in?

A

In the US. african americans (1 in 400 births), 8% carry Hgb S gene as the sickle cell trait

64
Q

When do problems with SC anemia usually start?

A

~6 months after birth when protective Hgb F levels fall to adult levels

65
Q

What do sickle cell patients present with mild-severe disease?

A

Vascular occlusions, painful crises, strokes, chest syndrome, bone infarctions, AVN, splenic sequestration, delayed growth puberty

66
Q

What can precipitate sickle cell complications?

A

Red cell dehydration, acidosis, hypoxemia, stress, menses, and temp changes

67
Q

What should patients with sickle cell anemia avoid?

A

High altitudes (above 7,000ft) and deep sea diving

68
Q

What are patients with sickle cell anemia at increased risk for?

A

Cholelithiasis, splenomegaly, leg ulcers, infections with encapsulated organs, strokes, AVN, priapism, retinopathies leading to blindness and osteomyelitis

69
Q

What may sickle cell trait result in?

A

Difficulty concentrating urine

70
Q

What will be seen on a lab test for sickle cell anemia?

A
  • Hgb S: >50%
  • Peripheral smear: sickled cells (5-50%), target cells, nucleated RBCs, Howell-Jolly bodies
  • Reticulocyte count: elevated
  • Indirect bilirubin: elevated
  • LDH: elevated
  • WBC count: elevated
  • thrombocytosis may be present
71
Q

What is the tx for sickle cell anemia?

A

Symptomatic tx of pain episodes which includes analgesics, hypotonic fluids, and rest

72
Q

How do you treat the other conditions that are seen with sickle cell anemia?

A

Stroke, sequestration, acute chest syndrome, and multiorgan failure may require transfusion or exchange transfusion

73
Q

What is polycythemia vera?

A

A slowly progressive bone marrow disorder characterized by increased numbers of RBCs and increased total blood volume

74
Q

What is diagnostic for polycythemia vera?

A

The presence of the JAK2 mutation for the primary (genetic) cause

75
Q

What does the increased red cell mass in polycythemia vera cause?

A

Hyper-viscosity, causing decreased cerebral blood flow and hypercoagulability

76
Q

What are the secondary causes of polycythemia?

A

Chronic hypoxia, cigarette smoking, living at high altitudes, renal tumors

77
Q

What are the complications of polycythemia?

A

Thrombosis can cause morbidity and mortality, bleeding, PUD, and GI bleeding

78
Q

What is the median age that polycythemia presents?

A

60 years

79
Q

What gender is polycythemia more prevalent in?

A

60% in males