Heme/Onc Flashcards

1
Q

name the important neutrophil chemotactic agents

A
C5a
IL-8
LTB4
kvllikrein
platelet-activating factor
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2
Q

lipid A from bacterial LPS binds what on macrophages to induce septic shock

A

CD 14

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3
Q

what causes eosinophilia

A
Neoplasm
Asthma
Allergic RXN
Chronic adrenal insufficniey
parasites
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4
Q

what lymphocyte mediate cell immunity

A

T cells

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5
Q

what lymphocyte mediates humoral immune response

A

b cells

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6
Q

cell wth clock face chromatin distribution and eccentric nucleus with well developed golgi

A

plasma cell

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7
Q

what makes oxidized vitamin k into reduced vitamin k so it can activate the coagulation factors

A

epoxide reductase

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8
Q

antithrombin inhibits wha factors

A
2
7
9
10
11
12
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9
Q

inhibit ADP induced expression of GpIIb/IIIa

A

Ticlopidine
clopidogrel
Prasugrel

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10
Q

inhibit GpIIb/IIIa directly

A

Abciximab, Eptifibatide, Tirofiban

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11
Q

what activates vWF to bind to GpIb

A

Ristocetin

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12
Q

spur cells are seen in

A

Abetalipoproteinemia and Liver disease

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13
Q

what test tests for autoimmune hemolytic anemia because the body produces AB to RBC

A

direct coombs test

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14
Q

a test that detects antibodies to RBC unbound in the patients serum. used in pregnant women and before blood transfusions

A

indirect coombs test

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15
Q

cis deletion of alpha globulin in alpha thalassemia means what

A

deletions are on the same chromosome

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16
Q

cis deletion of A thalamseima is seen in

A

Asians

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17
Q

trans deletion of A thalassemia is seen in

A

Black people

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18
Q

b thalesemia is present in what people

A

mediteraniean

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19
Q

what is protective against major beta thalassemia

A

HbF until 6 months

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20
Q

b thalassemia major patients have an increased chance of what problem

A

parvovirus B19 induced aplastic crisis

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21
Q

what is the chelation for lead poison used in children

A

Succimer

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22
Q

sideroblastic anemia has a defect in what

A

ALA synthase gene

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23
Q

treatment for sideroblastic anemia

A

ALA synthase or pyridoxine

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24
Q

historical test used to diagnose B12 def.

A

Schilling test ( tells if the problem is malabsorption or dietary insufficiency)

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25
defect in UMP synthase that causes failure to thrive in children and megaloblastic anemia. No hyperammonemia is present
Orotic Aciduria
26
important physical symptom of Diamond Blackfan syndrome
triphalangeal thumb
27
haptoglobin are increased in what kind of hemolysis
extravascular hemolysis
28
haptoglobin are decreases in what kind of hemolysis
intravascular hemolysis ( paroxysmal nocturnal hemoglobunbria)
29
hemolytic anemia of the newborn because of decreased levels of ATP is seen because of
Pyruvate kinase deficiency
30
what is the terminal complement inhibitor that treats Paroxysmal Nocturnal Hemoglobinuria
Eculizumab
31
Cold IGM complement induced autoimmune hemolytic anemia can be caused by
Mycoplasma Pneumonia Mono Cll
32
acute intermittent porphyria is caused by a deficiency in what
porphobilinogen deaminase
33
porphyria cutanea tarde is a deficiency in what
uroprphyrinogen decarboxylase
34
what usually cause HUS in children
SHiga toxin producing E. Coli (O157:H7)
35
skin necrosis and hemorrhage after administration of warfarin is seen when
there is protein C and S deficiency
36
what should be given if you want to replace coagulation factors fibrinogen and factor 8
cryopercipitate
37
8:14t
burritos
38
14:18 t
follicular lymphoma
39
11:14 t
mantle cell lymphoma
40
starry sky appearance of lymphocytes associated with EBV and a jaw lesion in the african type
Burritos lymphoma
41
most common type of non hodgkin lymphoma in adults
diffuse large b cell lymphoma
42
waxing, winning lymphadenopathy lymphoma
follicular lymphoma
43
verry aggressive with patients mainly presenting in the alter stages lymphoma
mantel cell lymphoma
44
AIDS defining illness that needs to be differentiated from toxoplasma
primary central nervous system lymphoma
45
associated with IV drug abuse which presents with cutneoys lesions and lytic bone lesions and hypercalcemia
adult t cell lymphioma
46
skin patches are seen with atypical CD4 cells with cerebriform nuclei which may progress to Sezary Syndrome
Mycosis Fungicides
47
hyper viscosity syndrome associated with an Mspike and Raynaud phenomenon
Waldenstrom Macroglobulinemia
48
bone lytic lesions, anemia, renal involvement and hypercalcemia
multiple myeloma
49
monoclonal plasma cell expansions that is asymptomatic that could lead to multiple myeloma
MGUS
50
neutrophils with bilobed nuclei usually seen after chemo
Pseudo-Pelter Huet Anomaly
51
what is the ALL with a better prognosis
12:21
52
t cell All can present how
like a mediastinal mass like in SVC syndrome
53
when SLL/CLL progresses into aggressive diffuse large be cell lymphoma
Richter Transformation
54
marrow fibrosis that causes dry tap on aspiration and spleenomegaly
hairy cell leukemia
55
hairy cell leukemia stains with what
TRAP
56
tx for hair cell leukemia
pentostatin | cladribine
57
AML that responds t retinoid acid
15:17
58
philadelphia chromsome 9:22 BCR-ABL represent
CML
59
what is Imatinib
BCR-ABL tyrosine kinase inhibitor that treats CML
60
lytic bone lesions in kids with skin rash and recurrent otits media . a mass involving the mastoid bone
Langerhans cell histocytosis
61
what is characteristic on the EM of langerhans cell histocytosis
tennis rackets, Birbeck granules
62
when is there a decrease in EPO seen in a polycythemia
vera
63
innaprpriate absolute polycythemia its seen when
there is ectopic EPO secretion (renal cell carcinoma)
64
when is there appropriate absolute polycythemia
high altitude
65
when is there relative polycythemia
dehydration and burns
66
name the two LMW heparin
Enoxaparin | Dalteparin
67
What kind of virus is parvovirus b 19
Non envelopes single stranded DNA
68
What kind of virus is EBV
Envelopes double stranded Dna
69
What kind off virus is hep c
Envelopes single stranded positive sense rna
70
What kind of virus is hep e
Non envelopes single stranded positive sense RNA
71
What kind of virus is HIV
Enveloped single stranded positive sense RNA
72
What kind of virus is parvovirus b 19
Non envelopes single stranded DNA
73
What kind of virus is EBV
Envelopes double stranded Dna
74
What kind off virus is hep c
Envelopes single stranded positive sense rna
75
What kind of virus is hep e
Non envelopes single stranded positive sense RNA
76
What kind of virus is HIV
Enveloped single stranded positive sense RNA
77
In mono there is hyperplasia of what part of the lymph node
Paracortex (t cell)
78
Peri arterial lymphatic sheath is the part that is distended when
There is a T cell infection like mono ( this is the area of T cells)
79
What does mono spot test detect
IgM Heterophile antibodies
80
name the six protooncogenes
``` RAS ABL HER2 EGFR MYC BRAF ```
81
Name six tumor suppressor genes
``` WT1 VHL RB Tp53 APC BRCA ```
82
protooncogene associated with hairy cell leukemia
BRAF
83
what do the BRCA genes do
they repair breaks in DNA
84
zinc containing enzymes that are used in wound healing and remodeling to break down collagen, laminin, and fibronectin
Metalloprotineases
85
CD 55 is used for what? and is missing in what
it is used to inactivate complement and it is missing in Paroxysmal Nocturnal Hemoblobinguria
86
hemolytic anemia, low haptons, pancytopenia and thrombosis of things like the mesenteric vein are associated with what disease
Paroxysmal Nocturnal Hemoglobinuria
87
explain hemosiderosis seen in PNH
there is a continuous destruction of RBC which leads to free iron being deposited in the kidney
88
cast nephropathy is seen in what disease
multiple myeloma
89
what diseases cause osteolytic bone metastasis
``` Multiple myeloma non small cell lung cancer non hodgkin lymphoma renal cell carcinoma melanoma ```
90
what diseases cause osteoblastic bone metastasis
prostate cancer small cell lung cancer hodgkin lymphoma
91
cancer associated with high parathyroid hormone related peptide (PTH rP) and hypercalcemia as well as osteolytic bone metastasis
non small cell lung cancer
92
diffuse medium sized lymphocytes and high proliferation index represented by the high Ki-67 fraction approaching 100% is seen in what disease
Burritk's Lymphoma
93
how can giving packed RBC to a patient who lost a lot of blood cause hypocalcemia
packed blood contains citrate which can chelate calcium and cause hypocalcemia (parathesias) * usually seen after a massive transfusion
94
what infections induces heterophil antibodies that react with antigens from animal RBCs (sheep-PaulBunnell test or horse - Monospot test)
EBV infections
95
what are two malignancies that EBV is associated with
BurrKits lymphoma | Nasopharyngeal Carcinoma
96
the absence of factor 8 and 9 won't allow for the activation of factor 10 and subsequently won't allow for the activation of prothrombin to thrombin so clots won't be formed. What can you give to someone with hemophilia who won't quit bleeding
thrombin
97
what turns fibrinogen into fibrin, main part of a clot
thrombin
98
waxing and waning lymphadenopathy are common in what cancer
follicular lymphoma
99
what cells clonal expand because of EBV infected B cells
Cytotoxic T cells CD 8+
100
CD14 is a marker for what
macrophages, this is where LPS binds to activate them
101
decreased ferritin, increased transferrin and low MCV are seen in
IRON deficiency anemia
102
RECAM-1 is used for
transmigration
103
ICAM-1 is used for
tight adhesion
104
Leukocyte Adhesion Deficiency type 1 is what
when there is an absence of CD 18 so that interns Mac-1 and LFA1 aren't synthesized so there is no tight adhesion, crawling or trasnmigration
105
symptoms of LAD 1
skin infections with no puss, delayed detachment of umbilical cord and poor wound healing
106
small rings called trophozoites found on RBC is common in what disease
malaria p. falciparum
107
main treatment for malaria
chloroquine
108
treatment for malaria in chloroquine resistant areas
mefloquine
109
ivermectin treats>
Onchocerciasis "River Blindness"
110
Nifurtimox treats
Chagas Disease
111
Pentamide is used for what
prophylaxis of Pneumocystis Jirovecii in HIV patients | - african sleeping sickness and leishmaniasis
112
what is needed to kill P vivid and P oval liver hypnozoites
Primaquine
113
most cases of HUS are preceded by what
diarrhea from Shiga toxin producing bacteria (Shigella and Ecoli 0157:H7)
114
systemic autoimmune disease characterized by proximal muscle weakness resembling polio but with the additional inflammatory features that involved to skin
Dermatomyositis
115
Gottronsq Papules and Heliotrope rash are seen in what disease
Dermatomyositis
116
non enveloped single stranded DNA virus that can cause arthralgia in the hands, knees, wrist and feet without a rash
Parvovirus
117
Why is haptoglobin decreased in hemolytic anemia due to prosthetic valves
Because haptoglobin binds to free hemoglobin and with the increased destruction of red blood cells, there is more free hemoglobin binding to the haptoglobin making the number of haptoglobin decease
118
Repeated splenic infections seen in sickle cell disease makes what happen to the spleen
Fibrosis and atrophy
119
Splenic sequestration can cause what to happen to the spleen
Splenic congestion
120
Patients with sickle cell disease are lily to develop what because of high RBC turnover
Folic acid deficiency
121
Parvovirus in a pregnant woman can cause what in the fetus
interruption of erythropoiesis causing profound anemia which causes CHF. This CHF causes pleural effusion, lung hypoplasia and ascites
122
what kind of viruses are VZV, CMV and HSv
enveloped dsDNA
123
what kind od virus is rubella
enveloped ss RNA
124
What can VZV in a pregnant woman do to a fetus
cause limb hypoplasia
125
indirect bilirubin is the same as
unconjugated billirubin
126
what is the most specific red cell index for spherocytosis
increased mean corpuscular hemoglobin concentration
127
what is caused by a defective binding of the red cell cytoskeleton to the plasma membrane due to mutations involving ankyrin, band 3 and spectrum proteins
hereditary spherocytosis
128
pappenheimer bodies in RBCS are associates with what
sideroblastic anemia
129
increased osmotic fragility on acidified glycerol lysis test ( hemoglobin is released when the patients RBC are incubated with glycerol) is seen in
hereditary sphereocytosis
130
pigmented gallstones are a complication of what
hemolytic anemia like in hereditary spherocytosis
131
two factors that drive angiogensesis
VEGF | FGF
132
what can interferon-y do
INDIRECTLY PROMOTE neovascularization by activating macrophages (these release VEGF)
133
benzene is found in what
gasoline and tobacco smoke
134
what disease affects erythrocyte precursors and causes red cell aplasia ( anapestic crisis) in patients who already have a preexisting blood disease like nickel cell, or hereditary spherocytosis
Parvovirus B19
135
neuroblastoma has an over expression of what marker
n-myc
136
what is the function of the c-myc gene
transcription activation which controls cell proliferation, differentiation and apoptosis
137
cyclin D1 promotes what
g1--> s phase
138
what gives the toughness and rigidity to keratin protein
the disulfide bonds between the cysteine molecules
139
what gives keratin its small structure and ability to coil tightly and form many hydrogen bonds
alanine and glycine molecules
140
what are the two markers used for neuroendocrine tumors
Chromogranin A and neuron specific enolase
141
what is the intermediate filament seen in mesenchymal tissue
vimentin
142
what pathology is associated with an inversion of the short arm of chromosome 2 that creates a fusion gene between EML4 ( echinoderm microtubule associated protein like 4) and ALK ( analytic lymphoma kinase) that causes active tyrosine kinase that causes malignancy
non small cell lung cancer
143
treatment for the EML4-ALK fusion protein that causes malignancy in non small cell lung cancer
Crizotinib (tyrosine kinase inhibitor)
144
EML4-ALK non small cell lung cancer pathophysiology is the most similar to what cancer
CML ( it is also a fusion protein)
145
hemolytic anemia that presents after the administration of TMP-SMX is usually due to what disease
G6PD deficiency
146
increased vulnerability od RBC to oxidative stress manifesting as hemolytic anemia that is induced by - infection - drugs ( bacterium, dapsone, antimalarials, nitrofurantoin) - fava beans
G6PDD
147
x linked disorders mainly seen in men in which presides of oxidative stress cause extra/intravascualr hemolysis. Haptoglobin are low and urine is dark read. Fave bean, and sulfa drugs canc cause this
G6PD D
148
what should be checked before administering dapsone so that you do not cause hemolytic anemia in your patient
G6PD levels
149
how does peripheral tolerance occur
by T cell anergy ( inactivation of t cells that react to self)
150
central tolerance happens where
in the fetal thymus during negative selction
151
where does isotoype switching of b cell occurs ( when they switch from IgM to something else by making antibodies)
in the germinal centers in the lymph nodes
152
interaction of the CD40 receptor on B cells and the CD40 ligand on activated T cells cause what
isotope switching
153
where are heterophiles antibodies found
in patients with EBV mono
154
when are ringed sideroblast found
in myelodysplastic syndrome
155
the hEr2 oncogene encodes for what
185-kD transmembrane glycoprotein that has intracellular tyrosine kinase activity
156
toll like receptors release what
cytokines
157
what is clathrin
an intracellular cytosolic protein the mediated endocytosis
158
autoimmune destruction of platelets by anti platelet antibodies likely IgG agains GpIIb/IIIa
Immune thrombocytopenia purpura
159
main clue for immune thrombocytopenia purport
isolated thrombocytopenia
160
small lymphoid cells with increased nuclei to cytoplasmic ration and cleaved nuclei are seen in what cancer
follicular lymphoma
161
what is the disease that presents as a benign leukocytosis that happens in response to an underlying condition like infection, hemorrhage, malignancy or acute hemolyisi.
Leukemiod reaction
162
Dhole bodies are basophilic granules inside of neutrophils seen in
leukemiod reaction
163
where dose positive selection happen
in the cortex of the thymus
164
where does negative selection occur
in the medulla of the thymus
165
interaction of the developing t cell with thyme medullary epithelial and dendritic cells in seen in
negative selection
166
eczematous nipple lesion is associated with what kind of disease
pagets
167
what causes black gall stone
chronic hemolysis
168
what causes brown gall stones because of the release of microbial B-glucuronidases
biliary tract infection
169
what are two things that the splenic red pulp is important for
1. destroying aged RBCs | 2. Clearing out circulating bacteria
170
what are the three main encapsulated bacteria that can present as a problem for individuals with no spleen
Strep Pneumo H. Influenza N. Meningitides
171
what causes macrophages and t cells to be activated in viral infected cells
interferon a and b
172
pro carcinogens are metabolized by what
P450 monooxygenase
173
what makes bilirubin into soluble bilirubin
glucuronide transferase
174
PT prolongation seen in liver dysfunction is due to what
Factor 7 running out from the extrinsic pathway (shortest half life)
175
pure red cell aplasia is associated with what?
thymic tumor (thymoma)
176
MAIN MECHANISMS OF IONIZING RADIATION
double strand breaks in the DNA | free radical formation
177
hypophosphorylated RB is what
active
178
hyperphosphorylated RB is what
inactive
179
explain the pathophysiology of Thrombotic Thrombocytopenia Purpura
there is a decrease in ADAM13 activity which causes a larger vWF multiuser that causes microvascular platelet rich thrombi
180
name the clinic features of Thrombotic Thrombocytopneic Purpura
hemolytic anemia low haptoglobin thrombocytopenia schistocytes
181
how to manage TTP
plasma exchange
182
what is the normal role of ADAM13
it cleaves vWF into smaller pieces so that clotting won't occur
183
IgA immune complex deposition and palpable purpura renal disease and arthritis
Hence Schonlein Puprpura
184
frequent nose bleeds and HEAVY menstrual periods is a good sign of what disease
vWF deficiency
185
deep tissue blessing into muscle and joints, g.i. bleeding is usually a sign of what disease
Factor 8 deficiency
186
clot instability and recurrent bleeding after surgery is a sign of what disease
Factor 13 deficiency
187
vWF is a protective carrier for what
factor 8
188
vWF binds to what
collagen
189
prolonged PTT and bleeding time is seen in what problem
vWF deficiency