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Pathophysiology > Hematopathology (Schoenwald) > Flashcards

Hematopathology (Schoenwald) Flashcards

1
Q

What is anemia?

A

decrease in red blood cells

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2
Q

What are the 3 mechanisms that can lead to anemia?

A

blood loss- acute or chronic
decreased production of RBCs
increased destruction of RBCs (hemolysis)

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3
Q

How is anemia evaluated?

A

size of the red blood cell (MCV) and hemoglobin concentration (MCHC)

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4
Q

What are examples of blood loss that lead to anemia?

A

acute- trauma

chronic- GI malignancy, menstrual bleeding

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5
Q

What are external factors that lead to increased destruction of red blood cells?

A 
antibody-mediated transfusion reaction or erythroblastosis fetalis 
autoimmune 
trauma to RBC 
infection 
sequestration in spleen
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6
Q

What are hereditary internal factors that lead to increased destruction of RBCs?

A

cytoskeleton defect- hereditary spherocytosis
structurally abnormal hgb- sickle cell, thalassemia
enzyme deficiency- G6PD

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7
Q

What is an acquired defect that leads to increased destruction of RBCs?

A

paroxysmal nocturnal hemoglobinuria

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8
Q

What is paroxysmal nocturnal hemoglobinuria?

A

red cells are more prone to lysis by complement (complement-regulating proteins CD55 and CD59 are deficient)

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9
Q

What is the defect in paroxysmal nocturnal hemoglobinuria?

A

acquired defect in the gene for phophatidylinositol class A (PIG-A)

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10
Q

What does paroxysmal nocturnal hemoglobinuria result in?

A

venous thrombosis- hepatic, portal, mesenteric, and cerebral veins

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11
Q

What are the clinical signs of paroxysmal nocturnal hemoglobinuria?

A

episodic hemoglobinuria, 1st morning urine

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12
Q

What are examples of what can lead to decreased production of RBCs?

A

disturbance of stem cells
defective heme production
defective DNA production
destruction of bone marrow

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13
Q

What is an example of disturbance of stem cells, leading to a decreased production of RBCs?

A

aplastic anemia

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14
Q

What are examples of defective heme production, leading to a decreased production of RBCs?

A

iron deficiency

thalassemia

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15
Q

What is an example of defective DNA production, leading to a decreased production of RBCs?

A

vitamin B12 and folic acid deficiencies

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16
Q

What is an example of destruction of bone marrow, which leads to a decreased production of RBCs?

A

metastatic tumor

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17
Q

What are the sites of removal of the RBC from circulation?

A

extravascular- phagocytic systems of spleen and liver

intravascular- destruction of RBC in the vessel

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18
Q

What are complications of extravascular RBC removal?

A

hemosiderin deposition in organs

jaundice and gallstones (results from elevated unconjugated bilirubin associated with hemolysis)

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19
Q

What are complications of intravascular RBC removal?

A

acute tubular necrosis due to hemoglobinemia

jaundice, gallstones can occur due to increased unconjugated bilirubin

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20
Q

What are the lab findings in hemolysis of RBCs?

A

decreased RBCs

increased bilirubin in the circulation from the breakdown of RBCs

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21
Q

spherocytes morphology

A

hereditary spherocytosis and immune hemolysis

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22
Q

schistocytes morphology

A

TTP and HUS

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23
Q

target cells morphology

A

hemaglobinopathies

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24
Q

teardrops morphology

A

myelofibrosis

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25
Q

sickle cell morphology

A

sickle cell, thalassemia minor with sickle cell

26
Q

Rouleaux morphology

A

“stacking of coins” due to increased globes or decreased albumin (multiple myeloma)

27
Q

What is the clinical presentation of anemia?

A

pale skin, fatigue, shortness of breath, chest pain, syncope, dizziness

28
Q

What labs would you order if you suspect anemia?

A

MCV

reticulocyte count

29
Q

What is an increased reticulocyte count indicative of?

A

bone marrow is intact and able to produce RBC in acute blood loss of hemolysis

30
Q

What is a decreased reticulocyte count indicative of?

A

primary bone marrow disorder or deficiency in building blocks- iron, b12, or folate

31
Q

microcytic is an MCV…

A

less than 80

32
Q

normocytic is an MCV…

A

80-100

33
Q

macrocytic is an MCV….

A

greater than 100

34
Q

What are examples of microcytic anemias?

A

iron deficiency
thalassemia
late stage anemia of chronic disease

35
Q

What is iron deficiency anemia mostly due to in the US?

A

GI blood loss of menstrual blood loss

36
Q

Outside of the US, what is the most common cause of iron deficiency anemia?

A

poor nutrition

37
Q

Other than GI blood loss, menstruation, or poor nutrition, what else causes iron deficiency anemia?

A

malabsorption or increased demand such as in pregnancy

38
Q

What labs would you expect to see in iron deficiency anemia?

A 
decreased ferritin 
decreased serum iron 
decreased iron stores in bone marrow 
increased total iron binding capacity (TIBC) 
increased RDW 
decreased transferrin saturation
39
Q

How is transferrin saturation calculated?

A

ratio of serum iron to TIBC

40
Q

What is normal transferrin saturation?

A

normal >20%

41
Q

What value would you expect to see for transferrin saturation in iron deficiency anemia?

A

less than 1-%

42
Q

What is thalassemia?

A

deficient production of hemoglobin due to mutation or complete loss of globin chain- there are 4 copies of the alpha globulin chain and 2 copies of the beta globulin

43
Q

What is alpha thalassemia due to?

A

complete loss of one or more of the 4 copies of the alpha globulin chain gene

44
Q

What is seen in a thalassemia carrier?

A

loss of one copy of gene is silent

45
Q

What is seen in alpha thalassemia trait?

A

loss of 2 copies, asymptomatic, but MCV is low

46
Q

What is seen in alpha thalassemia that results in HbH disease?

A

loss of 3 copies- marked anemia

47
Q

What is seen in alpha thalassemia that results in hydrops fetalis?

A

loss of 4/4 copies (stillborn)

48
Q

What populations is alpha thalassemia prevalent in?

A

africans and asians

49
Q

Does alpha thalassemia usually require blood transfusions?

A

no

50
Q

What is beta thalassemia?

A

malfunction of gene due to mutation which results in complete loss of the hemoglobin protein

51
Q

What population is beta thalassemia seen in?

A

mediterranean descent

52
Q

What is the result of beta thalassemia?

A

alpha chains are insoluble and result in intravascular hemolysis and ineffective erythropoiesis

53
Q

Does major beta thalassemia require blood transfusions?

A

yes, due to severe anemia

54
Q

What is the onset of age of major beta thalassemia?

A

usually onset after 6 months of age due to loss of Hgb F and transition to Hgb A

55
Q

What sxs are seen in major beta thalassemia?

A

skeletal abnormalities due to bone marrow expansion

56
Q

Does minor beta thalassemia usually require blood transfusions?

A

no

57
Q

What is disproportionate in minor beta thalassemia?

A

Hgb and MCV disproportionate- hgb 10/ MCV 55

58
Q

What labs would you expect to see in beta thalassemia?

A

decreased MCV
decreased hgb
anisocytosis
poikilocytosis (mircocytes, targets)

59
Q

What diseases is it common to see anemia of chronic disease?

A

lung carcinoma
Hodgkin lymphoma
RA
infection- TB

60
Q

Initially, anemia of chronic disease is _____, but in late stage it can be _____.

A

normocytic, microcytic

61
Q

What labs would you expect to see in anemia of chronic disease?

A

Pathophysiology (5 decks)

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