Pathology Flashcards
(199 cards)
1
Q
Where is vWF stored?
A
W-P bodies of endothelium
2
Q
What is stored in W-P bodies?
A
1) vWF
2) p-selectins
3
Q
What does vWF bind?
A
SEC and platelets
4
Q
What receptor doe platelets use to connect to fibrinogen?
A
GpIIb/IIIa
5
Q
Petechiae occurs with (quantitative/qualitative) platelet disorders
A
quantitative (thrombocytopenia)
6
Q
Autoimmune production of IgG against platelet antigens is called ____
A
ITP
7
Q
In ITP, autoimmune antibodies are made in the _____. Red cells are consumed in the _______
A
Antibodies made in spleen
Red cells consumed in the spleen
8
Q
Dx: Child with ITP
Rx: ________
A
usually self-limiting, possible platelet transfusion
9
Q
Dx: Adult with chronic ITP, low platelet crisis
Rx: ______
A
IVIG
10
Q
Dx: Adult with chronic ITP, low platelet crisis
Surgical option?
A
Splenectomy
11
Q
Pathologic platelet micro-thrombi in small vessels is called _____
A
Microangiopathic Hemolytic Anemia
12
Q
TTP is caused by a deficient enzyme _____
A
ADAMTS13
13
Q
ADAMTS13 normally cleaves _____ multimers into monomers
A
vWF
14
Q
The lack of ADAMTS13 in TTP is usually due to an underlying ________
A
autoimmune Dz
15
Q
Hemolytic Uremic Syndorme primarily affects the _______ (organ)
A
kidney
16
Q
The usual causative agent for HUS is ______
A
E. coli O157:H7
17
Q
E. coli O157:H7 has the virulence factor ______ that damages renal endothelial cells
A
verotoxin
18
Q
Name 2 Microangiopathic Hemolytic Anemias:
A
- TTP (thrombotic thrombocytopenic purpura)
2. HUS (Hemolytic Uremic Syndrome)
19
Q
In Microangiopathic Hemolytic Anemias, bleed time (increases/decreases) and PT/PTT is ______
A
increases
PT/PTT is normal
20
Q
How do you treat HUS and TTP?
A
- plasmapheresis
2. corticosteroids
21
Q
In Bernard-Soulier, there is a genetic deficiency in ______
A
GP1b
22
Q
In Bernard-Soulier, what function is impaired?
A
platelet adhesion
23
Q
Platelets without GP1b can’t bind ____
A
vWF
Bernard-Soulier Syndrome
24
Q
Blood smear of Bernard-Soulier Syndrome patient shows _______
A
enlarged platelets
25
In Glanzmann's Thrombasthenia there is a genetic deficiency in ________
GIIb/IIIa
26
ASA blocks cyclooxygenase preventing platelets from making ____
TXA2
27
In vWF disease (PT/PTT) is increased
PTT
28
Which test is abnormal in vWF disease?
1. ristocetin test
| 2. and PTT
29
What enzyme does coumadin block?
epoxide reductase
30
where is vitamin K generated?
gut bacteria
31
You can follow the extent of liver failure by measuring (PT/PTT)
PT
32
The best screening test for DIC is _______
elevated D dimer
33
A serine protease that converts plasminogen to plasmin
tPA
34
Dissolves fibrin clots:
1) cleaves fibrin
2) clears serum fibrinogen
3. destroys clot factors
plasmin
35
Lab findings: increased PT and PTT, increased bleed time, increased fibrinogen splits, no D-dimer
Dx: ?
overactive plasmin
36
Lines of Zahn: their presence implies thrombosis at a site of rapid blood flow that happened with the patient (alive/dead)
alive!
37
Coagulation can be kicked off by either ____ or ___
SEC or TF
38
Blocks platelet aggregation (TXA2/ PGI2)
PGI2 blocks
39
Promotes platelet aggregation (TXA2/ PGI2)
TXA2 promotes
40
on the surface of endothelial cells, serves as a cofactor for thrombin
TM: thrombomodulin
41
Name 3 endothelial damages that increase clot risk
1. atherosclerosis
2. vasculitis
3. elevated homocystine
42
Vitamin k is required for which blood protein factors to work?
2, 7, 9, 10, C and S
43
Which has the shortest half-life? (2, 7, 9, 10, C or S)
C and S! hence the need for HEP therapy along with coumadin
44
Heparin binds and activates _____
ATIII
45
What are the 4 types of micorcytic anemias?
1. sideroblastic
2. thalasemia
3. iron deficient
4. anemia of chronic Dz
46
anemia, dysphagia w/ esophageal web, red beefy tounge is ____ _____ disease
plummer-vinson
47
what acute phase reactant sequesters Iron?
hepcidin
48
what does TIBC stand for?
total iron binding capacity
49
Hx: bone marrow suppression w chemo and anemia
| how can you treat the anemia?
erythropoietin
50
Protoporphyrin is attached to iron in what cell compartment?
mitochondria
51
iron laden mitochondria occurs in _______ anemia
sideroblastic
52
alcoholism can lead to a ________ anemia
sideroblastic
53
intracellular protein that stores iron and releases it in a controlled fashion, commonl measured in labs
ferritin
54
decreased synthesis of globin chains is called ______
thalassemia
55
which leads to a worse thalasemia, cis or trans deletion?
cis
56
Where is the cis deletion thalassemia more commonly seen? (asia/africa)
asia
57
4 beta chains in a thalassemia is called Hb__
HbH
58
a tetramer of gamma chains in hemoglobin is called Hb_____
Hb barts
59
Beta thalassemia leads to (microcytic/macrocytic) anemia
microcytic
60
Massive erythroid hyperplasia such as in SS and beta thal leads to what appearance on skull radiograph?
"crew cut"
| due to massive erythroid hyperplasia
61
why is a person w/ beta thalassemia at risk for hemochromotosis?
frequent transfusion
62
Electrophoresis: no HbA, increased HbA2 and HbF
Dx: ________
beta thalassemia major
63
Macrocytic anemia is usually due to a deficiency in ______ of _______
1. folate
| 2. vitamin B12
64
Three diseases that can lead to a macrocytic anemia are:
1. alcoholism
2. liver disease
3. chemo drugs: 5-FU, methotrexate and folate def.
65
R binder is produced by the ______, then complexed to vit. B12 for absorption
salivary glads
66
intrinsic factor that binds B12 is produced by the _____ cells o the stomach
parietal
67
B12 is stored in large volume in the _______ (organ)
liver
68
Pernicious anemia is due to _________ destroying the parietal cells of the stomach
autoimmune disease
69
With low serum B12, serum homocystine and _______ build up, leading to spinal cord damage
methylmalonic acid, can damage the spinal cord
70
Hemoglobinemia with hemoglobinuria and hemosiderinuria (intravascular/extravascular) destruction of RBCs
intravascular destruction
71
brown urine", with chronic intravascular hemolysis is called _________
hemosiderinuria
72
what 3 tethering molecules are deficient in Hereditary spherocytosis?
1. spectrin
2. ankrin
3. band 3.1
73
what virus infects erythrocyte pre-cursors?
Parvovirus B19
74
Treatment for Hereditary spherocytosis is _______
splenectomy
75
Howell-Jolly bodies indicate _______
splenic dysfunction, (such as post splenectomy)
76
Three risk factors for RBC sickling are:
1. acidosis
2. dehydration
3. hypoxemia
77
Swollen hands and feet due to vaso-occlusive infarts of bones is called _______
dactylitis
78
in kids with SS, there is increased risk of infection with _____ _____
encapsulated bacteria
79
What lab test can allow identification of HbS?
Metabisulfite
80
What two molecules linked by GPI prevent complement form destroying Red cells?
1. DAF: decay accelerating factor
| 2. MIRL: Membrane inhibitor of reactive lysis
81
Nightime shallow breathing and acidosis lead to activation of ______
complement
82
Lack of CD55 indicates the disease ______
CD55 is DAF,
| indicates PNH: paroxysmal nocturnal hemoglobinuria
83
Patients with PNH, paroxysmal nocturnal hemoglobinuria have an increased risk for which caner?
AML, acute myeloid leukemia
84
G6PD deficiency renders cells susceptible to _______ _______
oxidative stress
85
Red cells as Bite cells are seen with _________
G6PD deficiency
86
Warm agglutinin is seem with (IgG/IgM) mediated immune hemolytic anemia
IgG
87
The two autoimmune disease, 1. ITP and 2. immune hemolytic anemia can be treated with ____
IV IG
88
RBC are bound by antibodies in the colder extremities with (IgG/IgM) immune hemolytic anemia
IgM
89
deficiency of all types of blood cells due to lack of blood cell progenitors is called _____ _____
aplastic anemia
90
A pathologic process that replaces bone marrow with fibrosis, tumor, granuloma etc, is called a ________ proces
myelophthisic process
91
Where is most of the marginated pool of neutrophils?
the lung endothelium
92
A left shift with immature neutrophile means a decrease in the _____ receptor or CD ____
Fc receptor = CD 16
93
Basophelia is associated with (ALL/APL/CML)
CML
94
Which cells are enlarged 'monocytes' in Mononucleosis?
CD8+ T-cells
| Mononucleosis is a misnomer
95
Mononucleosis increases the risk for ______(organ) rupture
splenic
96
acute leukemia is a neoplastic proliferation of ______, greater than 20% in Bone marrow
blasts
97
You can specifically identify a Lymphoblast by the presence of (TdT/ MPO) in the nucleus
TDT
98
You can specifically identify a myeloidblast by the presence of (TdT/ MPO) as crystals of auer rods
MPO: myeloperoxidase crystalizes as auer rods
99
What are the two types of acute leukemia?
AML: Acute myeloid leukemia
ALL: Acute lymphoblastic leukemia
100
ALL is common in children with ______ syndrome after the age of 5
downs
101
The more common B-ALL usually has the translocation t(__:__)
t(9:22)
102
T-ALL usually presents as a T_____ (organ) mass in a T___ aged person
Thymic mass in a Teenaged
103
Hairy Cell Leukemia is a proliferation of (B cells/ T cells)
B cells
104
In CML, the predominant cell is the (RBC/ basophil/ neutrophil/ eosinophil/ monocyte/ megakaryocyte)
Basophil
105
CML is driven by the translocation t(__:__)
t(9:22)
106
A patient with CML can transform to (AML/ ALL)
ALL or AML! the mutation is in the Hematopoietic stem cell
107
1. Polycythemia Vera,
2. Essential Thrombocythemia and
3. Myelofibrosis are driven by a mutation in ______
JAK2 kinase
108
Budd-Chiari syndrome with thrombosis in the portal vein can be cause by this blood cell proliferation _______
Polycythemia vera
109
In Polycythemia vera there are increased red cells AND ____ cells, which can degranulate in heat causing itching after bathing
mast cells
110
low SaO2, increased EPO, high red count (lung Dz/ Renal cell carcinoma/ polycythemia vera)
lung Dz
111
normal SaO2, increased EPO, high red count (lung Dz/ Renal cell carcinoma/ polycythemia vera)
Renal cell carcinoma
112
normal SaO2, low EPO, high red count (lung Dz/ Renal cell carcinoma/ polycythemia vera)
polycythemia vera
113
In myelofibrosis, megakaryocytes overproduce _____ resulting in fibrosis of the marrow space
PDGF
114
follicular lymphoma is characterized by the translocation [ t(14:18)/ t(11:14)/ t(8:14) ]
t(14:18)
Ig heavy chain: 14
Bcl-2: 18
115
over-expression of Bcl-2 will (promote/ inhibit) apoptosis
inhibit
116
Tingible body macrophages that eat up apoptotic B cells are (present/ absent) in follicular lymphona
absent! lack of B- cell apoptosis
117
mantle cell lymphoma is drive by translocation [ t(14:18)/ t(11:14)/ t(8:14) ]
t(11:14)
Ig heavy chain: 14
Cyclin D1: 11
118
Which B cell proliferation is associated with chronic inflammatory states like hashimotos and sjogrens (Follicular/ Mantle cell/ Marginal zone) Lymphome
Marginal zone lymphoma
119
Burkitt lymphoma is driven by [ t(14:18)/ t(11:14)/ t(8:14) ]
t(8:14)
Ig heavy chain: 14
c-myc: 8
120
Reed–Sternberg cells are CD__ and CD__ positive and not CD20+
CD15+ and CD30+
121
In Multiple myeloma, neoplastic plasma cells can activate the RANK receptor leading to ____-___ lesions on x-ray
punched out lesions
122
An M-spike on Serum protein electrophoresis may indicate the neoplastic proliferative disorder ______ _______
Multiple myeloma
123
Free light chain excreted in the urine with multiple myeloma is called ____-_____ protiens
Bence-Jones
124
Lytic bone lesions, hypercalcemia, M-spike, AL-amyloid and Bence-Jones protiens all indicate ______ _____
Multiple myeloma
125
An M-spike with IgM, absent lytic bone lesions, generalized LAD and increased bleeding are all characterisitcs of _____ _____
Waldenstrom macroglobulinemia
126
A Neoplastic proliferation with Birckeck, tennis racket granules and CD1a+ and S100+ cells is _______ _______
Langerhans Histiocytosis
127
Benign proliferation of langerhans cells, presents with pathologic fracture, skin not involved.
Eosinophilic granuloma, will have inflammatory eosinophils
128
What are the 3 Langerhans cell histiocytosis?
1. Eosinophilic Granuloma (benign)
2. Letter Siwe disease (malignant, >2y.o.)
3. Hand-Schuller-Christian disease (malignant >3y.o.)
129
Are there blood vessels in the epidermis?
no, they stop at the dermis
130
Which layer of the epidermis has desmosoms that look like spinous processes?
the stratum spinosum
131
vitamin A derivatives are used to treat acne by (killing P. acnes/ reducing keratin production)
reducing keratin production
132
Benzoyl peroxidase is used to treat acne by (killing P. acnes/ reducing keratin production)
antimicrobial, kill P. acnes
133
Caused by excessive keratinocyte proliferation (psoriasis/ eczema)
psoriasis
134
usually on flexor surfaces (psoriasis/ eczema)
eczema = atopic dermatitis
135
usually on extensor surfaces (psoriasis/ eczema)
psoriasis
136
A type I hypersensitivity rxn associated with asthma and rhinitis (psoriasis/ eczema)
eczema = atopic dermatitis
137
Associated with HLA-C, lesions arise more frequently with trauma (psoriasis/ eczema)
psoriasis
138
diffuse epidermal hyperplasia is called ________
Acanthosis
139
perakeratosis, with excess keratin and retention of nucleii is (psoriasis/ eczema)
psoriasis
140
pinpoint bleeds with elongated dermal papilla (psoriasis/ eczema)
psoriasis
141
What are the p's of lichen Planus?
```
pruritic
planar
polygonal
purple
papules
```
142
Autoimmune destruction of desmosomes leading to blisters (Pemphigus Vulgaris/ Bullous Pemphigoid/ Dermatitis Herpetiformis)
Pemphigus Vulgaris
143
Autoimmune destruction of hemi-desmosomes at basement membrane (Pemphigus Vulgaris/ Bullous Pemphigoid/ Dermatitis Herpetiformis)
Bullous Pemphigoid
144
IgG mediated (Pemphigus Vulgaris/ Bullous Pemphigoid/ Dermatitis Herpetiformis)
Bullous Pemphigoid and Pemphigus Vulgaris
145
Bullae or blisters rupture more easily (Pemphigus Vulgaris/ Bullous Pemphigoid/ Dermatitis Herpetiformis)
Pemphigus Vulgaris, blisters in stratum spinosum, not at basal layer
146
Mediated by IgA at tips of dermal papillae (Pemphigus Vulgaris/ Bullous Pemphigoid/ Dermatitis Herpetiformis)
Dermatitis Herpetiformis
147
Strong association with celiac disease (Bullous Pemphigoid/ Dermatitis Herpetiformis/ Erythema Multiforme)
Dermatitis Herpetiformis
148
Resolves with a gluten free diet(Bullous Pemphigoid/ Dermatitis Herpetiformis/ Erythema Multiforme)
Dermatitis Herpetiformis
149
Most commonly seen with HSV infection (Bullous Pemphigoid/ Dermatitis Herpetiformis/ Erythema Multiforme)
Erythema Multiforme
150
Multiple erythmentaous rashes with white middle targetoid appearance (Bullous Pemphigoid/ Dermatitis Herpetiformis/ Erythema Multiforme)
Erythema Multiforme
151
Rank from least severe to most severe (Steven Johnson syndrome/ Erythema Multiforme/ Toxic epidermal necrolysis )
Erythema Multiforme, Steven Johnson syndrome, TEN. the are all the same, just different levels of involvement
152
A benign proliferation of squamous cells with a " stuck on appearance" (Acanthosis Nigricans/ Seborrheic keratosis)
seborrheic keratosis
153
The Lesser-Trelat sign is when multiple of these arise sugesting an underlying GI carcinoma (Acanthosis Nigricans/ Seborrheic Keratosis/ Basal cell carcinoma)
seborrheic keratosis
154
Epidermal hyperplasia with darkening of the skin and a "velvet-like" appearance (Acanthosis Nigricans/ Seborrheic Dermatosis/ Basal cell carcinoma)
Acanthosis Nigricans
155
Associated with insulin resistance, diabetes, or underlying malignancy
(Acanthosis Nigricans/ Seborrheic Dermatosis/ Basal cell carcinoma)
Acanthosis Nigricans
156
Risk factors include prolonged exposure to sunlight, albinism and xeroderma pigmentosum (Acanthosis Nigricans/ Squamous cell carcinoma/ Basal cell carcinoma)
Basal cell carcinoma
157
appears as a nodule with a central area of ulceration and surrounded by telangectasia, especially on the upper lip. (Acanthosis Nigricans/ Squamous cell carcinoma/ Basal cell carcinoma)
Basal cell carcinoma
158
Risk factors include sunlight exposure, immunosupressive thereapy, arsnic poisoning and chronic inflammation. (Melanoma/ Squamous cell carcinoma/ Basal cell carcinoma)
Squamous cell carcinoma
159
Usually presents on the lower lip (Melanoma/ Squamous cell carcinoma/ Basal cell carcinoma)
Squamous cell carcinoma
160
Actinic keratosis is a precursor to (Melanoma/ Squamous cell carcinoma/ Basal cell carcinoma)
squamous cell carcinoma
161
Melanocytes are derived from which neural layer (ecterderm/ mesoder/ endoderm/ neural crest)
neural creast
162
vitiligo is (autoimmune/congenital)
autoimmune
163
Albinism is (autoimmune/congenital)
congenital
164
Due to defect in the enzyme tyrosinase (vitiligo/ albinism)
albinism
165
A freckle is darker than the surrounding sking due to a localized increase in (melanocytes/ melanosomes)
melanosomes, the number of melanocytes remains the same
166
A mask like hyperpigmentation of the cheeks is called _____
melasma
167
Which will have hair growing from it? (melanoma/ nevus)
Nevus, benign
168
Which has the worst prognosis? (Melanoma/ Squamous cell carcinoma/ Basal cell carcinoma)
melanoma
| Squamous cell and basal are very fixable
169
Melanomas: Which two have a good prognosis (Lentigo Maligna Melanoma/ Superficial Spreading Melaona/ Nodular Melanoma/ Acral Lentiginous melanoma)
Lentigo Maligna Melanoma and
| Superficial Spreading Melaona
170
Melanomas: which has an early vertical phase, therefore a poor prognosis?
(Lentigo Maligna Melanoma/ Superficial Spreading Melaona/ Nodular Melanoma/ Acral Lentiginous melanoma)
Nodular Melanoma
171
Melanomas: Which arises on palms and soles?
| Lentigo Maligna Melanoma/ Superficial Spreading Melaona/ Nodular Melanoma/ Acral Lentiginous melanoma
Acral Lentiginous Melanoma
172
Which is only along the dermal + epidermal junction with no invasion? (Lentigo Maligna Melanoma/ Superficial Spreading Melaona/ Nodular Melanoma/ Acral Lentiginous melanoma)
Lentigo Maligna Melanoma
173
Which only invades the dermis superficially and has a good prognosis? (Lentigo Maligna Melanoma/ Superficial Spreading Melaona/ Nodular Melanoma/ Acral Lentiginous melanoma)
Superficial spreading melanoma
174
Painless separation of the nail from the nailbed is called _______
onycholysis
175
Do petechia blanch with pressure? (yes/no)
no. They are bleeding into the skin
176
When RBC's are of unequal size it is called _______
anisocytosis
177
the luebering rapoport pathway is used to make ______
2,3, BPG
178
which has HLA on their membrane (RBCs/ platelets)
Platelets have HLA
179
Decreased serum ferritin is diagnostic of ______ deficiency
iron deficiency
180
Increase in ferritin during inflammation is due to increased IL-__
IL-6
181
Ferritin is primarily in (serum/macrophages) while transferrin is in (serum/macrophages)
ferritin is in macrophages
| transferrin is in serum
182
TIBC correlates with (ferritin/ serum transferrin)
serum transferin
183
decreased ferritin stores leads to (increased/decreased) transferrin synthesis
increased, therefore TIBC increases
184
The master iron regulator protein is called (hepcidin/ haptoglobin)
hepcidin
185
Which is directly absorbed in the duodenum? (Fe3+/Fe2+)
| Which is transported by transferrin in the blood? (Fe3+/Fe2+)
Absorbed: Fe2+
Transported: Fe3+
186
In iron deficiency anemia, serum ferritin is (down/up)
| In ACD serum ferritin is (down/up)
Iron deficiency, ferritin down
| ACD, ferritin is up
187
An acute phase reactant that complexes with Hb to be degraded by macrophages is (hepcidin/ haptoglobin)
haptoglobin
188
Which indicates loss of function of splenic macrophages? (Heinz bodies/ Howell-Jolly bodies)
Howell-Jolly bodies
189
Which is caused by oxidative stress that damages hemoglobin? (Heinz bodies/ Howell-Jolly bodies)
Heinz bodies
190
Which is associated with SS disease? (Heinz bodies/ Howell-Jolly bodies)
Howell-Jolly bodies
191
Which is associated with G6PD deficiency? (Heinz bodies/ Howell-Jolly bodies)
Heinz bodies
192
Hereditary spherocytosis is inherited by (AD/AR/XR) pattern
AD
193
Sickle cell anemia is inherited by (AD/AR/XR) pattern
AR
194
G6PD deficiency is inherited by (AD/AR/XR) pattern
XR
195
"smudge cells" appear in (ALL/CLL/AML/CML/APL/HCL)
CLL: chronic lymphocytic leukemia
196
TRAP stain is positive in (ALL/CLL/AML/CML/APL/HCL)
HCL: Hairy cell leukemia
197
Auer rods are present in (ALL/CLL/AML/CML/APL/HCL)
APL: Acute promyelocytic anemia
198
The most common type of Hodgkin's lymphoma is (Nodular sclerosis/ Mixed cellularity/ Lymphocyte depleted/ Lymphocyte rich)
Nodular sclerosis
199
The hodgkin's lymphone most associated with EBV is (Nodular sclerosis/ Mixed cellularity/ Lymphocyte depleted/ Lymphocyte rich)
1. mixed cellularity: 60-70%
2. Lymphocyte rich: 40%
3. Lymphocyte depleted: many
