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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
What is the most common cause of anemia worldwide?
Iron deficiency
26
What are the most common causes of iron deficiency anemia in adults?
GI bleeds secondary to PUD, NSAID use, or cancer (likely cause), heavy menstrual cycle- but rule out GI bleed first
27
What causes iron deficiency anemia in children?
Low dietary intake of iron
28
What are some other causes of iron deficiency anemia?
Decreased absorption of iron, increased requirements, hemoglobinuria, blood donation, iron sequestration, trauma, and intravascular hemolysis
29
What are the symptoms of moderate-severe iron deficiency anemia?
Pallor, easy fatiguability, irritability, anorexia, tachycardia, tachypnea on exertion, poor weight gain in infants
30
What is a hallmark of iron deficiency?
Pica
31
What symptoms are seen with severe iron deficiency?
Brittle nails, cheilosis, smooth tongue, and formation of esophageal webs (Plummer-Vinson syndrome)
32
What is seen on the diagnostic studies with iron deficiency anemia?
- 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
Tx for iron deficiency anemia?
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
What is the most common cause of B12 deficiency?
Pernicious anemia due to the lack of intrinsic factor which is necessary for B12 absorption
35
What are other causes of B12 deficiency?
Strict vegan diet, gastric surgery, blind loop syndrome, pancreatic insufficiency, metformin, Crohn's disease
36
What can happen if B12 deficiency isn't treated?
Neurological damage, yikes!
37
Where does absorption of B12 occur in the body?
terminal ileum
38
Where is B12 stored in the body?
liver
39
What are the clinical features of B12 deficiency?
- Mouth: Smooth tongue, glossitis, cheilosis - Neuro: stocking-glove paresthesias, loss of position, fine touch and vibratory sensation, balance problems and ataxia, dementia
40
What is seen on diagnostic studies with B12 deficiency?
- 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
What is the Tx for B12 deficiency?
Lifelong supplemental B12 (1,000 micrograms/month) given PO or IM
42
What is the time frame to Tx B12 deficiency where you won't see neuro damage?
within 6 months!
43
How do you get G6PD deficiency?
It is hereditary! It is a X-linked recessive disorder
44
What population is G6PD deficiency most commonly seen in?
American black males (10-15%) and some Mediterranean populations. Females are rarely affected!
45
What can cause episodic hemolysis in G6PD deficiency?
Oxidative drugs (eg Aspirin, dapsone, primaquine, quinidine, sulfonamides, nitrofurantoin), fava beans, and infection
46
How do patient's with episodic hemolysis in G6PD deficiency present?
They are usually healthy and have no splenomegaly!
47
What are the laboratory findings during hemolytic episodes in G6PD def?
- Reticulocytes and serum indirect bilirubin increase - Peripheral smear: bite cells, Heinz bodies (denatured hemoglobin) - G6PD levels: low
48
What is the treatment for hemolytic episodes in G6PD def?
Self-limited as red cells are replaced as soon as offending agent is stopped
49
What is the treatment for hemolytic episodes in G6PD def?
Self-limited as red cells are replaced as soon as offending agent is stopped
50
What is the most common cause of folic acid deficiency?
Poor dietary intake | -usually in alcoholics, persons with anorexia, and diet low in fruits and veggies
51
What are the other causes of folic acid deficiency?
Defective absorption, pregnancy, chronic hemolytic anemias, alcohol abuse, folic acid antagonists (eg phenytoin, trimethoprim-sulfamenthoxazole, sulfasalazine)
52
What is the daily requirement of folic acid?
50-100 mg/dL - usually met by balanced diet
53
What conditions increase the daily requirements of folic acid?
Pregnancy, chronic hemolytic anemias, exfoliative skin disease
54
What are the clinical features of folic acid deficiency anemia?
Glossitis, vague GI symptoms, no neurologic symptoms
55
What lab results are pathognomonic of folic acid deficiency anemia?
Macro-ovalocytes and hypersegmented polymorphonuclear cells
56
What are other laboratory findings with folic acid deficiency anemia?
- Howell-Jolly bodies (nuclear DNA remnants) | - RBC folate level:
57
What is the first line tx for folic acid deficiency anemia?
1mg of folic acid PO daily
58
What lifestyle changes can help with folic acid def. anemia?
Avoid alcohol and folic acid metabolism antagonists (eg trimethoprim, seizure medications)
59
Common tests and results for B12 def
``` Serum B12: low Serum folate: normal RBC folate: normal Methylmalonic acid: high Homocysteine: high ```
60
Common tests and results for Folate def
``` Serum B12: normal or low Serum folate: low RBC folate: low Methylmalonic acid: normal Homocysteine: high ```
61
What kind of hereditary disease is sickle cell anemia?
a family of autosomal recessive inherited hemoglobinopathies
62
What is sickle cell anemia?
RBCs containing primarily Hgb S sickle under hypoxia, dehydration, acidosis, and extreme temp conditions
63
What population is SC anemia most often seen in?
In the US. african americans (1 in 400 births), 8% carry Hgb S gene as the sickle cell trait
64
When do problems with SC anemia usually start?
~6 months after birth when protective Hgb F levels fall to adult levels
65
What do sickle cell patients present with mild-severe disease?
Vascular occlusions, painful crises, strokes, chest syndrome, bone infarctions, AVN, splenic sequestration, delayed growth puberty
66
What can precipitate sickle cell complications?
Red cell dehydration, acidosis, hypoxemia, stress, menses, and temp changes
67
What should patients with sickle cell anemia avoid?
High altitudes (above 7,000ft) and deep sea diving
68
What are patients with sickle cell anemia at increased risk for?
Cholelithiasis, splenomegaly, leg ulcers, infections with encapsulated organs, strokes, AVN, priapism, retinopathies leading to blindness and osteomyelitis
69
What may sickle cell trait result in?
Difficulty concentrating urine
70
What will be seen on a lab test for sickle cell anemia?
- 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
What is the tx for sickle cell anemia?
Symptomatic tx of pain episodes which includes analgesics, hypotonic fluids, and rest
72
How do you treat the other conditions that are seen with sickle cell anemia?
Stroke, sequestration, acute chest syndrome, and multiorgan failure may require transfusion or exchange transfusion
73
What is polycythemia vera?
A slowly progressive bone marrow disorder characterized by increased numbers of RBCs and increased total blood volume
74
What is diagnostic for polycythemia vera?
The presence of the JAK2 mutation for the primary (genetic) cause
75
What does the increased red cell mass in polycythemia vera cause?
Hyper-viscosity, causing decreased cerebral blood flow and hypercoagulability
76
What are the secondary causes of polycythemia?
Chronic hypoxia, cigarette smoking, living at high altitudes, renal tumors
77
What are the complications of polycythemia?
Thrombosis can cause morbidity and mortality, bleeding, PUD, and GI bleeding
78
What is the median age that polycythemia presents?
60 years
79
What gender is polycythemia more prevalent in?
60% in males

Heme/Onc (4 decks)

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