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Systems Pathology l > Final Exam Review - Khan > Flashcards

Final Exam Review - Khan Flashcards

1
Q

Acute myeloid leukemia affects what age group?

A

15-39 years

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2
Q

What causes acute myeloid leukemia?

A

clonal proliferation and myeloblasts in the marrow

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3
Q

What is the most common chromosomal abnormality?

A

Acute myeloid leukemia

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4
Q

Acute myeloid leukemia with translocations between which chromosomes?

A

chromo 8 and 21 or chromos 15 and 17

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5
Q

What may be present in the cyoplasm in Acute myeloid leukemia?

A

eosinophilic, slender cytoplasmic inclusions called Auer rods

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6
Q

Chronic myelogenous leukemia (CML) peaks at what age?

A

30s and 40s, slight male predominance

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7
Q

90 % of patients with Chronic myelogenous leukemia (CML) have what chromosome?

A

(Philadelphia) Ph chromosome- (translocation of chromosome 9 to 22 BCR/ABL gene)

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8
Q

A patient presents with fatigue, anorexia, weight loss, and vague abdominal discromfort caused by hepato splenomegaly. What is the pathology?

A

Chronic myeloid leukemia (CML)

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9
Q

What is present in bone marrow with Chronic myeloid leukemia (CML)?

A

band cells (unsegmented nuclie) and meta myelocytes

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10
Q

What is often a symptom of splenic infarction?

A

acute left upper quadrant pain

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11
Q

Chronic lymphocytic leukemia (CLL) is caused by what?

A

malignant proliferation of small mature-appearing lymphoid cells
- pro lymphocytes in the blood

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12
Q

Which type of Chronic lymphocytic leukemia (CLL) has an average survival of 5 years?

A

CLL is that is positive for marker ZAP-70

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13
Q

CLL negative for ZAP-70 has a survival of more than how many years?

A

25 years

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14
Q

What can develop as Chronic lymphocytic leukemia (CLL) advances?

A
  • severe anemia
  • thombo-cytopenia
  • neutropenia
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15
Q

Chronic lymphocytic leukemia (CLL) results in what?

A

swollen lymph nodes

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16
Q

Chronic lymphocytic leukemia (CLL) may transform into what syndrome?

A

Richter’s syndrome

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17
Q

Transformation of Chronic lymphocytic leukemia (CLL) into Richter’s syndrome is characterized by what?

A
  • worsening cytopenias
  • increasing splenomegaly
  • progressive increase in the number of prolymorphocytes in the peripheral blood
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18
Q

What is the most common type of Chronic lymphocytic leukemia (CLL) transformation?

A

transformation to prolymorphocytic leukemia (15-30%)

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19
Q

Transformation in a CLL patient includes what symptoms?

A
  • fever, weight loss and muscle atrophy, and enlarged lymph nodes
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20
Q

8 % of all CLL patients will have elevated levels of what?

A

serum lactate dehydrogenase (LDH)

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21
Q

A clonal B-cell neoplasm composed of small to medium sized lymphocytes with abundant pale cytoplasm describes what?

A

Hairy cell leukemia

- hair-like cytopasmic protrusions

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22
Q

Hairy cell leukemia involves what body areas?

A

bone marrow and peripheral blood

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23
Q

What is the male to female ratio for Hairy cell leukemia?

A

5:1

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24
Q

What are lymphomas?

A

malignant proliferations of lymphocytes

- mostly affects lymph nodes but can attach any tissue/organ

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25
Q

When lymphoma involves the peripheral blood or bone marrow it is said to be what?

A

leukemic

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26
Q

Lymphomas are caused by translocations between what chromosomes?

A

9 and 22

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27
Q

B-cell, T-cell, and NK cell lymphomas are cateorized as what?

A

immature - from precursor cells; lymphoblasts

- mature are form mature effector cells

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28
Q

What are the types of low grade lymphomas?

A
  • small lymphocytic lymphoma
  • MALT lymphoma
  • Follicular lymphoma
  • mycosis fungoides
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29
Q

Low-grade lymphomas occur in what population?

A
  • older patients

- INDOLENT (incurable)

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30
Q

Low-grade lymphomas have what cell type?

A
  • small, non-destructive, mature cells
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31
Q

What are the types of high-grade lymphomas?

A
  • large cell lymphoma
  • lymphoblastic lymphoma
  • Burkitt lymphoma
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32
Q

High-grade lymphomas affect who?

A
  • children

- AGGRESSIVE

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33
Q

High-grade lymphomas have what kind of cells?

A

DESTRUCTIVE, big and ugly cells

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34
Q

What is the most common B-cell lymphoma worldwide?

A

Diffuse large B-cell lymphoma (all age groups)

- largest contributor to non-Hodgkin lymphoma

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35
Q

Diffuse large B-cell lymphoma occurs most commonly in what age group?

A

between 60-70

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36
Q

Sheets of large lymphoma cells with prominent nucleoli are present in what?

A

Diffuse large B-cell lymphoma

- immune function changes are important!

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37
Q

If tumor cells give rise to lymphadenopathy or solid tumor masses, what term is used?

A

small lymphocytic lymphoma

- if in bone marrow blood leukemia is correct Dx

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38
Q

Small lymphocytic lymphoma is caused by what?

A

B-cell neoplasm composed of a monomorphic population of predominantly small lymphocytes

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39
Q

Mantle cell lymphoma (MCL) has a median age of what?

A

60

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40
Q

Mantle cell lymphoma (MCL) is caused by what type of neoplasm?

A

a CD5 mature B-cell neoplasm (composed of a monotonous population of small to medium sized lymphocytes)

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41
Q

MALT lymphomas occur where?

A

Mucosa-associated lymphoid tissue

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42
Q

MALT lymphomas are associated with what?

A

helicobacter pylori

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43
Q

MALT lymphomas are indolent B-cell lymphomas composed of what?

A

hertogenous population of small B cells seen as extranondal sites (GI tracts, salivary glands, lungs)

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44
Q

Follicular lymphoma is caused by what type of neoplasm?

A

mature B-cell neoplasm composed of follicle center B cells (germinal center cells)

  • ranges from indolent to aggressive
  • mostly in adults
  • bone marrow involved in 40-60 % of cases
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45
Q

What is the second most common lymphoma?

A

Follicular lymphoma

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46
Q

What is the most common form of non Hodgkin lymphoma in the U.S.?

A

Follicular lymphoma

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47
Q

What happens to the normal lymph node architecture in Follicular lymphoma?

A

it is replaced by malignant lymphoid follicles in a back-to-back pattern
- will have a distinctive nodular pattern

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48
Q

Sezary’s disease is what type of lymhpoma?

A

cutaneous lymphoma (first described by Albert Sezary)

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49
Q

Sezary’s disease affects what cells?

A

T-cells

50
Q

What is the origin of Sezary’s disease?

A

peripheral CD4 T-lymphocyte

51
Q

What are the dominant symptoms of Sezary’s disease?

A
  • generalized erythroderma
  • lymphadenopathy
  • atypical T-cells in peripheral blood
  • hepatosplenoegaly
52
Q

What is one of the most rapidly growing malignancies?

A

Burkitt lymphoma

  • peak in children 4-7
  • chromosomal translocation involving 8q24
  • EXTRA nodal
53
Q

What bone is involved in Burkitt lymphoma?

A

MANDIBLE

- fascial distortion

54
Q

All variants of Burkitt lymphoma have a high risk for what?

A

CNS involvement

55
Q

Macrophages create a “starry sky” appearnce in what pathology?

A

Burkitt lymphoma

56
Q

Malignant proliferation of lymphocytes in lymph is called what?

A

Hodgkin lymphoma

57
Q

Hodgkin lymphoma is most commonly in who?

A 
younger patients (10-35 most common) 
- white males more common
58
Q

Who has a 3 times higher risk of developing Hodgkin lymphoma?

A

young adults who have had Epstein–Barr virus

59
Q

What is elevated in Hodgkin lymphoma?

A

HLA-B18

60
Q

What type of Hodgkin lymphoma is characterized by fibrous thickening of the lymph node capsule?

A

Nodular sclerosis Hodgkin lymphoma

61
Q

What type of Hodgkin lymphoma contains reed sternberg cells in a mixed inflammatory background of eosinophils, neutrophils, macrophages and plasma cells?

A

mixed cellulartiy Hodgkin lymphoma

62
Q

What type of Hodgkin lymphoma is marked by classical reed sternberg cells with nodular lymphoid infiltrate composed of small B cells?

A

Lymphocyte-rich Hodgkin lymphoma

63
Q

The presence of what cells is necessary for Dx of Hodgkin lymphoma?

A

Reed-Sternberg cell

64
Q

The “owl’s eye” correlates to what cell?

A

Reed-Sternberg cell

65
Q

What happens in macroglobulinemia?

A

abnormal plasma cells build up in the bone marrow, lymph nodes, and spleen
- too much M protein –> thick blood –> small blood vessel flow is occluded

66
Q

Woldenstrom macroglobulinemia an indolent non-Hodgkin lymphoma that causes what?

A

overproduction of monoclonal immunoglobulin M antibody (IgM)

67
Q

Symptoms of Woldenstrom macroglobulinemia include what?

A
  • weakness
  • swollen lymph nodes
  • severe fatigue
  • nose bleeds
  • weight loss
  • visual and neuro problems
68
Q

The most common cause of iron deficiency anemia in the western world is what?

A

Chronic blood loss

69
Q

Which of the fallowing is characteristic of Beta Thalassemia?

A

Excess of Alpha chains

70
Q

What cell is most commonly found in Hodgkin’s lymphoma?

A

Reed Sternberg cell

71
Q

Increased reticulocytes and presence of nucleated RBC’s in peripheral blood is indicative of what?

A

hemolytic anemia

72
Q

Acquired deficiency of clotting factors can result in what?

A

von Willebrand factor (VWF) - only one that is acquired

73
Q

What is the most common cause of Guillain-Barre syndrome?

A

Acute inflammatory demyelinating poly radiculoneuropathy (AIDP)

74
Q

Guillian-Barre syndrome is an acute what?

A

symmetric neuromuscular paralysis that often begins distally and ascends proximally

75
Q

A patient presents with ascending paralysis, respiratory distress, weakness in distal and proximal muscles, reduced deep tendon reflexes and cardiac arrhythmias. What pathology do you suspect?

A

Guillian-Barre syndrome

76
Q

A more widespread, symmetric, primarily sensory polyneuropathy is known as what?

A

Lepromatous Leprosy

77
Q

Lepromatous Leprosy preferentially involves what?

A
  • skin
  • peripheral nerves
  • anterior eyes
  • upper airways
  • testes
  • hands and feet
78
Q

Nerves develop what in Lepromatous Leprosy?

A

nerves develop segmental demyelination, remyelination and axonal degeneration with perineural fibrosis and thickening.

79
Q

Tuberculoid leprosy begins with what?

A

localized skin lesions but the neuronal involvement dominates

80
Q

What causes Lepromatous Leprosy?

A

nodular granulomatous inflammation in dermis with injuries to cutaneous and subcutaneous nerves

81
Q

What is lost with Lepromatous Leprosy?

A
  • Schwann cells
  • axons
  • myelin sheaths
    + fibrosis
82
Q

In acute pyogenic meningitis and chronic meningitis what happens to the pressure and protein level?

A

both increase

83
Q

The most common area involved in global cerebral ischemia is between what arteries?

A

between middle and anterior cerebral arteries

84
Q

Neurofibrillary tangles are not present in which CNS disease?

A

Picks disease

85
Q

What is the source of the beta-amyloid in the core of the plaque?

A

beta amyloid is derived from a precursor molecule termed beta-amyloid precursor protein (beta-APP)

86
Q

Lewy bodies are found in what disease?

A

Parkinson’s

87
Q

What are Lewy bodies?

A

intracytoplasmic, eosinophilic, round-elongated inclusions with dense core surrounded by a pale halo

88
Q

Alpha-Sy-nuclein gene is involved in what disease?

A

parkinson’s disease

- Alpha-Sy-nuclein gene –> protein that aggregates into lewy bodies which kills dopamine-producing neurons

89
Q

Are there Lew bodies in post infectious parkinsonism?

A

NO

90
Q

What are the 4 hallmark signs of Parkinsonism?

A
  1. tremors
  2. rigidity/stiffness
  3. bradykinesia especially for voluntary movements
  4. postural instability or impaired balance
91
Q

Propulsive or festinating gait is seen in what disease?

A

parkinson’s disease

92
Q

Common side effects of Levodopa + Carbidopa are?

A

Nausea/vomiting, appetite loss, rapid HR, low BP

93
Q

What is the most common cause of mental retardation in boys?

A

fragile x syndrome

94
Q

What is the most common type of adult onset muscular dystrophy?

A

myotonic dystrophy

95
Q

What is the most common type hereditary spino-cerebellar ataxia?

A

Friedreich ataxia

96
Q

What causes Friedreich ataxia?

A

autosomal recessive (mutation of frataxin-1 gene)

97
Q

Loss of neurons with mild gliosis and heart involvement occurs in what?

A

Friedreich ataxia

98
Q

Ataxia, abnormal eye movement, rigidity, and dysarthria. describes what?

A

olivopontocerebellar atrophy

99
Q

Extrapyramidal tract/autonomic nervous insufficiency with postural hypotension, anhydrosis, and poor sphincter control describes what?

A

shy drager syndrome

100
Q

Atrophy of caudate nucleus and putamen with brady kinesea and rigidity describes what?

A

Striato nigral degeneration

101
Q

Both upper and lower motor neurons are affected in what disease?

A

ALS - Lou Gehrig’s

102
Q

Patients with ALS often die from what?

A

respiratory paralysis

103
Q

Loss of anterior horn gray matter in spinal cord and

atrophy and demyelination of Anterior/lateral Corticospinal tracts from UMN loss describes what pathology?

A

ALS

104
Q

Tay sachs, neuronal ceroid lipofuscinosis and glycogen storage disease is caused by the accumulation of what?

A

metabolic products in neurons

105
Q

Oculo-cerebellar damage and presence of axonal spheroids can be seen in what syndrome?

A

shaken baby syndrome

106
Q

Spoon shaped nails, smooth tongue, or pica points to what pathology?

A

iron deficiency anemia

107
Q

With hemolytic anemia the patient may be what?

A

jaundiced

108
Q

Megaloblastic anemia can cause what?

A

swollen tongue

109
Q

Aplastic Anemia can be treated with what?

A

immnosupressive therapy or stem cell transplantation

110
Q

What is decreased in Iron Deficiency anemia?

A

reticulocytes

111
Q

Deficiency of B12 or folic acid causes what type of anemia?

A

Megalobalstic anemia

112
Q

Pernicious anemia is an autoimmune disorder caused by what?

A

antibodies against parietal cells and intrinsic factor

113
Q

Pernicious anemia leads to what?

A

sub acute combined myelin degeneration of spinal cord, brain, and peripheral nerves

114
Q

Round, purple staining nuclear fragments of DNA in the RBC’s, often seen in Megalobalstic anemia, are called what?

A

Howell-Jolly bodies

115
Q

Schilling test measures what?

A

measures B12 absorption

116
Q

Signs of destruction in hemolytc anemias are what?

A
  • increased bilirubin and LDH

- decrease haptoglobin

117
Q

Signs of production in hemolytc anemias are what?

A

increased reticulocytes and nucleated RBCs

118
Q

What pathology is characterized by a heterogenous group of inherited disorders of RBC cytoskeletons (spectrin, ankyrin, band 3 or 4) leads to unstable membranes, increased fragility and spherocytes.

A

Hereditary spherocytosis

119
Q

A G6PD deficiency will cause what?

A

accumulation of H202 and denaturation of hemoglobin (Hemolytic disease from G6PD deficiency)

120
Q

Why are infants with sicke cell hemoglobin asymptomatic for first 10 weeks?

A

because of high hemoglobin F

Systems Pathology l (7 decks)

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