Final Exam Review - Khan Flashcards
(120 cards)
1
Q
Acute myeloid leukemia affects what age group?
A
15-39 years
2
Q
What causes acute myeloid leukemia?
A
clonal proliferation and myeloblasts in the marrow
3
Q
What is the most common chromosomal abnormality?
A
Acute myeloid leukemia
4
Q
Acute myeloid leukemia with translocations between which chromosomes?
A
chromo 8 and 21 or chromos 15 and 17
5
Q
What may be present in the cyoplasm in Acute myeloid leukemia?
A
eosinophilic, slender cytoplasmic inclusions called Auer rods
6
Q
Chronic myelogenous leukemia (CML) peaks at what age?
A
30s and 40s, slight male predominance
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)
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)
9
Q
What is present in bone marrow with Chronic myeloid leukemia (CML)?
A
band cells (unsegmented nuclie) and meta myelocytes
10
Q
What is often a symptom of splenic infarction?
A
acute left upper quadrant pain
11
Q
Chronic lymphocytic leukemia (CLL) is caused by what?
A
malignant proliferation of small mature-appearing lymphoid cells
- pro lymphocytes in the blood
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
13
Q
CLL negative for ZAP-70 has a survival of more than how many years?
A
25 years
14
Q
What can develop as Chronic lymphocytic leukemia (CLL) advances?
A
- severe anemia
- thombo-cytopenia
- neutropenia
15
Q
Chronic lymphocytic leukemia (CLL) results in what?
A
swollen lymph nodes
16
Q
Chronic lymphocytic leukemia (CLL) may transform into what syndrome?
A
Richter’s syndrome
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
18
Q
What is the most common type of Chronic lymphocytic leukemia (CLL) transformation?
A
transformation to prolymorphocytic leukemia (15-30%)
19
Q
Transformation in a CLL patient includes what symptoms?
A
- fever, weight loss and muscle atrophy, and enlarged lymph nodes
20
Q
8 % of all CLL patients will have elevated levels of what?
A
serum lactate dehydrogenase (LDH)
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
22
Q
Hairy cell leukemia involves what body areas?
A
bone marrow and peripheral blood
23
Q
What is the male to female ratio for Hairy cell leukemia?
A
5:1
24
Q
What are lymphomas?
A
malignant proliferations of lymphocytes
- mostly affects lymph nodes but can attach any tissue/organ
25
When lymphoma involves the peripheral blood or bone marrow it is said to be what?
leukemic
26
Lymphomas are caused by translocations between what chromosomes?
9 and 22
27
B-cell, T-cell, and NK cell lymphomas are cateorized as what?
immature - from precursor cells; lymphoblasts
| - mature are form mature effector cells
28
What are the types of low grade lymphomas?
- small lymphocytic lymphoma
- MALT lymphoma
- Follicular lymphoma
- mycosis fungoides
29
Low-grade lymphomas occur in what population?
- older patients
| - INDOLENT (incurable)
30
Low-grade lymphomas have what cell type?
- small, non-destructive, mature cells
31
What are the types of high-grade lymphomas?
- large cell lymphoma
- lymphoblastic lymphoma
- Burkitt lymphoma
32
High-grade lymphomas affect who?
- children
| - AGGRESSIVE
33
High-grade lymphomas have what kind of cells?
DESTRUCTIVE, big and ugly cells
34
What is the most common B-cell lymphoma worldwide?
Diffuse large B-cell lymphoma (all age groups)
| - largest contributor to non-Hodgkin lymphoma
35
Diffuse large B-cell lymphoma occurs most commonly in what age group?
between 60-70
36
Sheets of large lymphoma cells with prominent nucleoli are present in what?
Diffuse large B-cell lymphoma
| - immune function changes are important!
37
If tumor cells give rise to lymphadenopathy or solid tumor masses, what term is used?
small lymphocytic lymphoma
| - if in bone marrow blood leukemia is correct Dx
38
Small lymphocytic lymphoma is caused by what?
B-cell neoplasm composed of a monomorphic population of predominantly small lymphocytes
39
Mantle cell lymphoma (MCL) has a median age of what?
60
40
Mantle cell lymphoma (MCL) is caused by what type of neoplasm?
a CD5 mature B-cell neoplasm (composed of a monotonous population of small to medium sized lymphocytes)
41
MALT lymphomas occur where?
Mucosa-associated lymphoid tissue
42
MALT lymphomas are associated with what?
helicobacter pylori
43
MALT lymphomas are indolent B-cell lymphomas composed of what?
hertogenous population of small B cells seen as extranondal sites (GI tracts, salivary glands, lungs)
44
Follicular lymphoma is caused by what type of neoplasm?
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
45
What is the second most common lymphoma?
Follicular lymphoma
46
What is the most common form of non Hodgkin lymphoma in the U.S.?
Follicular lymphoma
47
What happens to the normal lymph node architecture in Follicular lymphoma?
it is replaced by malignant lymphoid follicles in a back-to-back pattern
- will have a distinctive nodular pattern
48
Sezary's disease is what type of lymhpoma?
cutaneous lymphoma (first described by Albert Sezary)
49
Sezary's disease affects what cells?
T-cells
50
What is the origin of Sezary's disease?
peripheral CD4 T-lymphocyte
51
What are the dominant symptoms of Sezary's disease?
- generalized erythroderma
- lymphadenopathy
- atypical T-cells in peripheral blood
- hepatosplenoegaly
52
What is one of the most rapidly growing malignancies?
Burkitt lymphoma
- peak in children 4-7
- chromosomal translocation involving 8q24
- EXTRA nodal
53
What bone is involved in Burkitt lymphoma?
MANDIBLE
| - fascial distortion
54
All variants of Burkitt lymphoma have a high risk for what?
CNS involvement
55
Macrophages create a "starry sky" appearnce in what pathology?
Burkitt lymphoma
56
Malignant proliferation of lymphocytes in lymph is called what?
Hodgkin lymphoma
57
Hodgkin lymphoma is most commonly in who?
```
younger patients (10-35 most common)
- white males more common
```
58
Who has a 3 times higher risk of developing Hodgkin lymphoma?
young adults who have had Epstein–Barr virus
59
What is elevated in Hodgkin lymphoma?
HLA-B18
60
What type of Hodgkin lymphoma is characterized by fibrous thickening of the lymph node capsule?
Nodular sclerosis Hodgkin lymphoma
61
What type of Hodgkin lymphoma contains reed sternberg cells in a mixed inflammatory background of eosinophils, neutrophils, macrophages and plasma cells?
mixed cellulartiy Hodgkin lymphoma
62
What type of Hodgkin lymphoma is marked by classical reed sternberg cells with nodular lymphoid infiltrate composed of small B cells?
Lymphocyte-rich Hodgkin lymphoma
63
The presence of what cells is necessary for Dx of Hodgkin lymphoma?
Reed-Sternberg cell
64
The "owl's eye" correlates to what cell?
Reed-Sternberg cell
65
What happens in macroglobulinemia?
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
Woldenstrom macroglobulinemia an indolent non-Hodgkin lymphoma that causes what?
overproduction of monoclonal immunoglobulin M antibody (IgM)
67
Symptoms of Woldenstrom macroglobulinemia include what?
- weakness
- swollen lymph nodes
- severe fatigue
- nose bleeds
- weight loss
- visual and neuro problems
68
The most common cause of iron deficiency anemia in the western world is what?
Chronic blood loss
69
Which of the fallowing is characteristic of Beta Thalassemia?
Excess of Alpha chains
70
What cell is most commonly found in Hodgkin's lymphoma?
Reed Sternberg cell
71
Increased reticulocytes and presence of nucleated RBC's in peripheral blood is indicative of what?
hemolytic anemia
72
Acquired deficiency of clotting factors can result in what?
von Willebrand factor (VWF) - only one that is acquired
73
What is the most common cause of Guillain-Barre syndrome?
Acute inflammatory demyelinating poly radiculoneuropathy (AIDP)
74
Guillian-Barre syndrome is an acute what?
symmetric neuromuscular paralysis that often begins distally and ascends proximally
75
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?
Guillian-Barre syndrome
76
A more widespread, symmetric, primarily sensory polyneuropathy is known as what?
Lepromatous Leprosy
77
Lepromatous Leprosy preferentially involves what?
- skin
- peripheral nerves
- anterior eyes
- upper airways
- testes
- hands and feet
78
Nerves develop what in Lepromatous Leprosy?
nerves develop segmental demyelination, remyelination and axonal degeneration with perineural fibrosis and thickening.
79
Tuberculoid leprosy begins with what?
localized skin lesions but the neuronal involvement dominates
80
What causes Lepromatous Leprosy?
nodular granulomatous inflammation in dermis with injuries to cutaneous and subcutaneous nerves
81
What is lost with Lepromatous Leprosy?
- Schwann cells
- axons
- myelin sheaths
+ fibrosis
82
In acute pyogenic meningitis and chronic meningitis what happens to the pressure and protein level?
both increase
83
The most common area involved in global cerebral ischemia is between what arteries?
between middle and anterior cerebral arteries
84
Neurofibrillary tangles are not present in which CNS disease?
Picks disease
85
What is the source of the beta-amyloid in the core of the plaque?
beta amyloid is derived from a precursor molecule termed beta-amyloid precursor protein (beta-APP)
86
Lewy bodies are found in what disease?
Parkinson's
87
What are Lewy bodies?
intracytoplasmic, eosinophilic, round-elongated inclusions with dense core surrounded by a pale halo
88
Alpha-Sy-nuclein gene is involved in what disease?
parkinson's disease
| - Alpha-Sy-nuclein gene --> protein that aggregates into lewy bodies which kills dopamine-producing neurons
89
Are there Lew bodies in post infectious parkinsonism?
NO
90
What are the 4 hallmark signs of Parkinsonism?
1. tremors
2. rigidity/stiffness
3. bradykinesia especially for voluntary movements
4. postural instability or impaired balance
91
Propulsive or festinating gait is seen in what disease?
parkinson's disease
92
Common side effects of Levodopa + Carbidopa are?
Nausea/vomiting, appetite loss, rapid HR, low BP
93
What is the most common cause of mental retardation in boys?
fragile x syndrome
94
What is the most common type of adult onset muscular dystrophy?
myotonic dystrophy
95
What is the most common type hereditary spino-cerebellar ataxia?
Friedreich ataxia
96
What causes Friedreich ataxia?
autosomal recessive (mutation of frataxin-1 gene)
97
Loss of neurons with mild gliosis and heart involvement occurs in what?
Friedreich ataxia
98
Ataxia, abnormal eye movement, rigidity, and dysarthria. describes what?
olivopontocerebellar atrophy
99
Extrapyramidal tract/autonomic nervous insufficiency with postural hypotension, anhydrosis, and poor sphincter control describes what?
shy drager syndrome
100
Atrophy of caudate nucleus and putamen with brady kinesea and rigidity describes what?
Striato nigral degeneration
101
Both upper and lower motor neurons are affected in what disease?
ALS - Lou Gehrig's
102
Patients with ALS often die from what?
respiratory paralysis
103
Loss of anterior horn gray matter in spinal cord and
| atrophy and demyelination of Anterior/lateral Corticospinal tracts from UMN loss describes what pathology?
ALS
104
Tay sachs, neuronal ceroid lipofuscinosis and glycogen storage disease is caused by the accumulation of what?
metabolic products in neurons
105
Oculo-cerebellar damage and presence of axonal spheroids can be seen in what syndrome?
shaken baby syndrome
106
Spoon shaped nails, smooth tongue, or pica points to what pathology?
iron deficiency anemia
107
With hemolytic anemia the patient may be what?
jaundiced
108
Megaloblastic anemia can cause what?
swollen tongue
109
Aplastic Anemia can be treated with what?
immnosupressive therapy or stem cell transplantation
110
What is decreased in Iron Deficiency anemia?
reticulocytes
111
Deficiency of B12 or folic acid causes what type of anemia?
Megalobalstic anemia
112
Pernicious anemia is an autoimmune disorder caused by what?
antibodies against parietal cells and intrinsic factor
113
Pernicious anemia leads to what?
sub acute combined myelin degeneration of spinal cord, brain, and peripheral nerves
114
Round, purple staining nuclear fragments of DNA in the RBC’s, often seen in Megalobalstic anemia, are called what?
Howell-Jolly bodies
115
Schilling test measures what?
measures B12 absorption
116
Signs of destruction in hemolytc anemias are what?
- increased bilirubin and LDH
| - decrease haptoglobin
117
Signs of production in hemolytc anemias are what?
increased reticulocytes and nucleated RBCs
118
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.
Hereditary spherocytosis
119
A G6PD deficiency will cause what?
accumulation of H202 and denaturation of hemoglobin (Hemolytic disease from G6PD deficiency)
120
Why are infants with sicke cell hemoglobin asymptomatic for first 10 weeks?
because of high hemoglobin F
