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Systems Pathology 2 > Quiz 3 > Flashcards

Quiz 3 Flashcards

1
Q

Formation of blood cellular components

A

hematopoiesis

pg 4

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2
Q

Where does hematopoiesis occur in children?

A

tibia and femur

pg 4

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3
Q

Where does hematopoiesis occur in adults?

A

pelvis, cranium, vertebral bodies, sternum, ribs

pg 4

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4
Q

Formation of blood cellular components outside of the medullary cavity

A

extramedullary hematopoiesis

pg 4

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5
Q

Where does extramedullary hematopoiesis occur?

A

spleen, liver, lymph nodes, thymus

pg 4

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6
Q

Categories of Anemia

A

1) blood loss
2) increased RBC destruction
3) decreased RBC production
pg 8

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7
Q

Effects of hypoxia from anemia

A

increased erythropoietin from kidneys, pallor, fatigue, weakness
pg 9

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8
Q

Anemia due to trauma, GI or gynecologic issues

A

anemia of blood loss

pg 10

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9
Q

Anemia of blood loss is ____(hypo, normo, or hyper -chromic/cytic)

A

normocytic/normochromic

pg 10

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10
Q

Anemia of blood loss may lead to ____ shock

A

hypovolemic shock

pg 10

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11
Q

Anemia due to the decreased life span of RBC

A

hemolytic anemia

pg 11

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12
Q

Decreased life span of RBC

A

hemolysis

pg 11

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13
Q

2 types of defects resulting in hemolytic anemia

A

intracorpuscular defects
extracorpuscular defects
pg 11

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14
Q

Hereditary defect resulting in hemolytic anemia

A

intracorpuscular defect
(abnormal membranes or disordered Hb synthesis)
pg 11

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15
Q

Acquired defect resulting in hemolytic anemia

A

extracorpuscular defect
(RBC trauma, infections, antibodies)
pg 11

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16
Q

Where does intravascular hemolysis occur?

A

peripheral circulation

pg 12

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17
Q

Where does extravascular hemolysis occur?

A

liver and spleen

pg 12

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18
Q

Increased Hb in urine

A

hemoglobinuria

pg 12

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19
Q

Components of intravascular hemolysis

A

damaged heart valve, toxins or heat; hemoglobinuria; jaundice and gallstones
pg 12

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20
Q

Components of extravascular hemolysis

A

RBC damage, antibody opsonization; splenomegaly

pg 12

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21
Q

Which is more common, extravascular or intravascular hemolysis?

A

extravascular hemolysis

pg 12

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22
Q

Clinical term for gallstones

A

cholelithiasis

pg 14

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23
Q

% of population affected by cholelithiasis?

A

10-20%

pg 15

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24
Q

Cholelithiasis are more commonly ____(asymptomatic or symptomatic)

A

asymptomatic 80%

pg 15

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25
Q

Which is more common: cholesterol stones or pigmented stones?

A

cholesterol stones

pg 15

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26
Q

Risk factors for cholelithiasis

A

family history, obesity, diabetes, hemolytic anemia, estrogen
pg 16

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27
Q

Abnormally shaped RBCs

A

poikilocyte

pg 17

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28
Q

Irregularly shaped RBCs, from hemolysis

A

schistocyte

pg 17

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29
Q

Teardrop shaped RBCs

A

dacrocyte

pg 17

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30
Q

RBC membrane defect(autosomal dominant)

A

hereditary spherocytosis

pg 21

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31
Q

Hereditary Spherocytosis may result in ____ ____ which may be made worse by the presence of parvovirus B19

A

aplastic crisis
inability to make RBCs
pg 21

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32
Q

Beta-globin mutation(autosomal recessive)

A

sickle cell anemia

pg 24

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33
Q

Sickle cell trait is ____zygous

A

heterozygous

pg 24

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34
Q

Sickle cell anemia/disease is ____zygous

A

homozygous

pg 24

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35
Q

Sickle cell trait effects what % of African Americans?

A

8%

pg 24

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36
Q

In what areas do RBCs sickle in those with sickle cell anemia?

A

sites of blood stasis; bone marrow(MC), spleen, site of infection or inflammation, and/or during dehydration
pg 25

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37
Q

Which has a worse prognoses; heterozygous or homozygous sickle cell?

A

homozygous; 50% survive beyond 5th decade

pg 27

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38
Q

Lethal results of sickle cell anemia

A

1) acute chest syndrome
2) stroke
pg 27

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39
Q

Effects of sickle cell anemia

A

thrombosis, fever, malaise, chronic low-level pain, splenomegaly/infarction, gallstones, priapism, stunted growth, osseous distortion
pg 28

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40
Q

Possible H-shaped vertebra are characteristic of which disease?

A

Sickle cell anemia (10% of patients)

pg 31

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41
Q

Hemoglobinopathy of mutated alpha or beta-globin genes

A

Thalassemia

pg 33

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42
Q

Hemoglobinopathy decreasing beta-globin chains

A

Beta-thalassemia

ph 34

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43
Q

Beta-thalassemia with 1 allele mutation

A

minor

pg 34

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44
Q

Beta-thalassemia with 2 allele mutation

A

major

pg 34

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45
Q

Which beta-thalassemia presents with subtle hemolysis?

A

minor

pg 34

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46
Q

Which beta-thalassemia presents with severe hemolysis, severe anemia, extramedullary hematopoiesis, and splenomegaly?

A

major

pg 34

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47
Q

What is monitored in those with beta-thalassemia minor?

A

serum ferritin

pg 35

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48
Q

Treatment for those with beta-thalassemia major

A

repeated transfusions and iron chelation
or bone marrow transplant (high risk)
pg 36

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49
Q

Life expectance for those with beta-thalassemia major

A

~20 years

pg 36

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50
Q

Eventually cause of death for those treated for beta-thalassemia major

A

iron overload, hemochromatosis, lethal dilated cardiomyopathy
pg 36

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51
Q

Radiological findings in those with beta-thalassemia major

A

“hair on end” or “lace-like trabeculation”

pg 37

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52
Q

A notable facies of beta-thalassemia major

A

chipmunk facies

pg 38

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53
Q

Hemoglobinopathy with mutated alpha-globin gene

A

alpha-thalassemia

pg 41

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54
Q

Which is more severe: alpha or beta-thalassemia?

A

beta-thalassemia

pg 41

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55
Q

What determines the severity of alpha-thalassemia?

A 
# of altered genes
pg 41
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56
Q

1 deletion of alpha-globin

A

silent carrier of alpha–thalassemia

pg 42

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57
Q

4 deletions of alpha-globins

A

no O2 capacity, lethal hydrops fetalis

pg 42

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58
Q

Deficiency results in a decrease in GSH

A

Glucose-6-phosphate dehydrogenase deficiency

pg 43

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59
Q

GDPD is typically asymptomatic until exposed to…

A

1) infections
2) fava beans
3) ADRs
pg 45

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60
Q

Symptoms/signs of G6PD

A

acute onset, fatigue, pallor, splenomegaly, back/abdominal pain, hemosiderinuria
pg 47

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61
Q

Risks of G6PD

A

males, African descent, areas of endemic malaria

pg 47

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62
Q

Microscopic findings of G6PD

A

bite cells and Heinz bodies

pg 46

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63
Q

Dark urine upon waking along with an acquired PIGA gene mutation

A

paroxysmal nocturnal hemoglobinuria

pg 48

64
Q

PIGA gene mutation results in…

A

deactivated complement inhibitors

pg 49

65
Q

Features of paroxysmal nocturnal hemoglobinuria

A

complement-mediated hemolysis, mild/chronic low-level anemia, increased risk for venous thrombosis
pg 49

66
Q

Treatment for paroxysmal nocturnal hemoglobinuria

A

antibodies that inhibit MAC, marrow transplant(risky)

pg 49

67
Q

Repetitive physical trauma to RBCs

A

traumatic hemolysis

pg 51

68
Q

Causes of traumatic hemolysis

A

prosthetic heart valves, narrowing of vessels (microangiopathic hemolytic anemia)
pg 51

69
Q

Schistocytes

A

“burr cells”
“helmet cells”
pg 52

70
Q

Disease caused by plasmodium falciparum

A

malaria

pg 54

71
Q

MC area affected by malaria

A

Asia and Africa

pg 54

72
Q

“Blackwater fever”

A

dark urine associated with malaria

pg 54

73
Q

Features of malaria

A

episodic shaking, chills, fever, vomiting sweating jaundice, splenomegaly, HA, joint pain, hemoglobinuria, renal failure
pg 55

74
Q

Malaria with CNS involvement

A

cerebral malaria

pg 55

75
Q

Additional features of cerebral malaria

A

seizures convulsions, coma/death (within days)

pg 55

76
Q

Treatment for malaria

A

chloroquine, primaquine, chemotherapy

pg 55

77
Q

Anemias of diminished erythropoiesis

A

1) iron deficiency anemia
2) megaloblastic anemias
3) anemia of chronic disease
4) aplastic anemia
5) myelopththisic anemia
pg 56

78
Q

World’s MC nutritional deficiency and cause of anemia

A

iron deficiency

pg 57

79
Q

____(men or women) store more iron

A

Men

pg 57

80
Q

Where is iron stored in the body?

A

liver, spleen, marrow, skeletal muscle

pg 57

81
Q

Hb makes up __% of body’s iron

A

80%

pg 57

82
Q

Iron is transported by…

A

transferrin (serum ferritin)

pg 57

83
Q

Conditions creating poor bioavailability of iron

A

decrease in dietary intake, chronic blood loss (GI or gynecological), malabsorption, increased metabolic demands
pg 58

84
Q

Features of iron deficiency anemia

A

fatigue, pale skin, lack of energy, impaired cognition, decreased immunity, fingernail “spooning”, pica
pg 59

85
Q

Appetite for substances of non-nutritive value

A

pica

pg 59

86
Q

A craving for ice is indicative of what deficiency?

A

iron

pg 59

87
Q

MC cause of iron deficiency in the US

A

chronic blood loss

pg 60

88
Q

Deficiency in folate or vitamin B12 results in what type of anemia?

A

megaloblastic anemia

pg 61

89
Q

Folate and vitamin B12 are required for…

A

DNA synthesis

pg 61

90
Q

Features of folate deficiency anemia

A

fatigue, weakness, sore tongue, macrocytes in peripheral blood
pg 62

91
Q

Vitamin B12 is needed for…

A

DNA synthesis and maintenance of PNS and spinal cord

pg 63

92
Q

MC cause of vitamin B12 deficiency anemia

A

chronic malabsorption

pg 63

93
Q

Features of vitamin B12 deficiency anemia

A

fatigue, pallor, weakness, dyspnea, demyelination of PNS and CNS, numbness, tingling, burning, decreased proprioception, ataxia
pg 64

94
Q

Treatment of vitamin B12 deficiency anemia

A

injected vitamin B12

pg 64

95
Q

Anemia with inflammation decreasing erythropoiesis

A

anemia of chronic disease

pg 65

96
Q

Anemia of chronic disease is MC among what population?

A

hospitalized
(infections, autoimmunity, cancers)
pg 65

97
Q

Features of anemia of chronic disease

A

similar to iron deficiency anemia + decreased iron-binding capacity and increased iron storage in marrow
pg 65

98
Q

Anemia with suppression of myeloid stem cells, “bone marrow failure”, pancytopenia

A

aplastic anemia

pg 67

99
Q

Features of aplastic anemia

A

anemia (weakness, pallor, dyspnea), Thrombocytopenia(petechiae), granulocytopenia (infections)
pg 68/69

100
Q

In which anemia is splenomegaly “characteristically absent”?

A

aplastic anemia

pg 69

101
Q

Treatment of aplastic anemia

A

immunosuppressive meds, regular transfusions, marrow transplant (risky)
pg 69

102
Q

Anemia with extensive marrow infiltration

A

myelophthisic anemia

pg 70

103
Q

MC cause of myelophthisic anemia

A

metastasis to bone (lung breast, prostate)

pg 70

104
Q

Causes of myelophthisic anemia

A

met to bone, granulomatous disease, lipid-storage disease

pg 70

105
Q

Features of myelophthisic anemia

A

anemia, thrombocytopenia, dacrocytes

pg 70

106
Q

Treatment for myelophthisic anemia

A

marrow transplant

pg 70

107
Q

Where are leukocytes produced?

A

marrow

pg 71

108
Q

Leukocytes make up __% of total blood volume

A

1%

pg 71

109
Q

Increase in WBCs

A

leukocytosis

pg 71

110
Q

Decrease in WBCs

A

leukopenia

pg 71

111
Q

Granulocytes

A

neutrophils, eosinophils, basophils

pg 72

112
Q

Agranulocytes

A

monocytes, lymphocytes

pg 72

113
Q

Categories of WBCs disorders

A

1) reactive
2) neoplastic
pg 73

114
Q

Decrease in granulocytes

A

neutropenia <500 cell/microL

pg 74

115
Q

SEVERE neutropenia

A

agranulocytosis <100/microL

pg 74

116
Q

Infection causes by Epstein-Barr virus (HHV-4)

A

infectious mononucleosis

pg 76

117
Q

Features of infectious mononucleosis

A

reactive lymphocytosis, fever, pharyngitis, fatigue, splenomegaly and generalized lymphadenitis
pg 76

118
Q

Microscopic finding of infectious mononucleosis

A

“atypical lymphocytes”

pg 77

119
Q

Infectious mononucleosis is most commonly spread via…

A

saliva

pg 78

120
Q

Disease caused by bartonella henselae

A

cat-scratch disease

pg 80

121
Q

MC population affected by cat-scratch disease

A

pediatrics (90%)

pg 80

122
Q

Features of cat-scratch disease

A

cervical and axillary lymphadenopathy, splenomegaly, pharyngitis, malaise, anorexia, irregular stellate necrotizing granulomas
pg 80

123
Q

Neoplastic proliferations of WBCs

A

1) lymphoid neoplasms
2) myeloid neoplasms
3) histiocytic neoplasms
pg 81

124
Q

Classifications of lymphoid neoplasms

A

1) leukemia
2) lymphoma
pg 84

125
Q

WBC cancer involving marrow or blood

A

leukemia

pg 84

126
Q

WBC cancer involving lymphatic tissue

A

lymphoma

pg 84

127
Q

Sudden onset with abnormal lymphoblasts suppressing marrow

A

acute leukemia

pg 85

128
Q

Categories of acute leukemia

A

1) acute lymphoblastic leukemia
2) acute myeloid leukemia
pg 85

129
Q

MC presenting feature of acute leukemia

A

anemia

pg 85

130
Q

Aggressive cancer of lymphoblasts, arrested maturation of B cells or T cells

A

acute lymphoblastic leukemia

pg 88

131
Q

MC pediatric leukemia

A

acute lymphblastic leukemia (80%)

pg 88

132
Q

Treatment of acute lymphblastic leukemia

A

chemotherapy
80% cure rate
pg 88

133
Q

Cancer of B cells, MC leukemia of adulthood

A

chronic lymphocytic leukemia

pg 89

134
Q

MC age of diagnosis of chronic lymphocytic leukemia

A

60 years

pg 89

135
Q

Features of chronic lymphocytic leukemia

A

pancytopenia, anemia/fatigue. cachexia, generalized lymphadenopathy, hepatosplenomegaly, suppression of B cells
pg 90

136
Q

Prognosis of chronic lymphocytic leukemia

A

4-6 years
if it becomes aggressive ~1 year
pg 90

137
Q

B cell cancer with a nodular appearance

A

Follicular lymphoma

pg 91

138
Q

Lymphocytes with cleaved nuclei

A

centrocytes

pg 91

139
Q

Centrocytes are associated with which B cell cancer

A

follicular lymphoma

pg 91

140
Q

Follicular lymphoma makes up what % of non-Hodgkin’s lymphoma?

A

40%

pg 91

141
Q

Features of follicular lymphoma

A

painless generalized lymphadenopathy, adults >50years

pg 92

142
Q

40% of cases of follicular lymphoma progress into…

A

diffuse B cell lymphoma

pg 93

143
Q

t(14;18), BCL2 gene is associated with which B cell cancer

A

follicular lymphoma

pg 93

144
Q

Cancer of B cells with t(11;14)

A

mantle cell lymphoma

pg 94

145
Q

Population affected by mantle cell lymphoma

A

males, >50 years

pg 94

146
Q

Cancer of B cells, MC lymphoma of adulthood

A

diffuse large B cell lymphoma

pg 95

147
Q

Population affected by diffuse large B cell lymphoma

A

elderly, 60 years

pg 95

148
Q

Risks for diffuse large B cell lymphoma

A

EBV infection, immunosuppression

pg 95

149
Q

Features of diffuse large B cell lymphoma

A

aggressive, rapidly-enlarging mass(es), commonly extranodally
pg 96

150
Q

Treatment of diffuse large B cell lymphoma

A

high-dose chemotherapy
(~80% enter remission, 50% cured)
pg 96

151
Q

Cancer of B cells, classically affect African children, “starry ski” histology”

A

Burkitt lymphoma

pg 97

152
Q

Risks for Burkitt lymphoma

A

EBV infection, mutated MYC gene via t(8;14)

pg 97

153
Q

Population MC affected by Burkitt lymphoma

A

Africans

pg 99

154
Q

Fastest growing human tumor

A

Burkitt lymphoma

pg 99

155
Q

Burkitt lymphoma MC develops…

A

extranodally
Africa- maxilla and mandible
USA- abdomen, GI, ovaries
pg 99

156
Q

Prognosis of Burkitt lymphoma

A

great with aggressive chemotherapy

pg 99

Systems Pathology 2 (8 decks)

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