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SM Flashcards

1
Q

Correcting sample turbidity for determining falser high Hb

A

Replace plasma with saline

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2
Q

Red cell agglutination results in

A

Falsely decrease RBC count

Falsely high MCV

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3
Q

MCV

A

Volume of packed RBCs/Red cell count

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4
Q

Roeleaux formation (stacked coins) means

A

High protein level

inflammation or malignancy

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5
Q

Plasmapheresis

A

Gets rid of IgM in serum to decrease viscosity

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6
Q

Which antibody causes cold agglutination

A

IgM against RBC

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7
Q

Causes of cold agglutination

A

Mycoplasma, EBV, lymphoma, non-lymphoid malignancy

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8
Q

Central pallor of RBC should be

A

<1/3 the diameter

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9
Q

Microcytic, hypochromic RBC

A

Iron deficiency

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10
Q

Microangiopathic hemolytic anemia

A

DIC, TTP, HUS, HELLP syndrome

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11
Q

Haptoglobin

A

Sign of hemolysis

Binds to free Hb

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12
Q

Autoimmune hemolytic anemia is secondary to what and causes what on blood smear

A

CLL and spherocytes

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13
Q

When shouldn’t you give rasburicase (recombinant urate oxidase used to prevent tumor lysis in CLL tx)

A

If patient has G6PD deficiency

Results in bite cells

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14
Q

Immature erythroid blood cells can artificially elevate

A

WBC count

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15
Q

Decrease Hgb and Hct but increase RBC count

A

Thalassemia

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16
Q

CBC parameters that don’t change between the sexes

A

MCV and RDW

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17
Q

Fick equation

A

Blood flow x Hgb x (A SaO2 - V SaO2)

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18
Q

Typical P50 for O2

A

27 mmHg

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19
Q

Shift O2 dissociation curve to the right

A

Decreased pH

Increased [CO2], 2,3 BPG, temperature

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20
Q

Mentzer index

A

MCV/RBC count

If <13, thalassemia is more likely than iron deficiency

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21
Q

Burr cell (Echinocytes)

A

Liver disease and chronic kidney disease

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22
Q

Mutation in uroporphyrinogen 3 cosynthase

A

Congenital eryhtopoietic porphyria (CEP)

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23
Q

Clinical manifestations of CEP

A 
Photosensitivity
Anemia
Splenomegaly
Hypertrichosis
Decreased RBC lifespan and anemia
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24
Q

Autosomal dominant mutation in RBC membrane proteins

A

Hereditary Spherocytosis

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25
Acanthocytes (spur cells)
Anorexia | Severe nutritional deficiency
26
Target cells (codocyte)
Macro - liver disease | Micro - Thalassemia, hemoglobin E, C disease
27
Results in pancytopenia and macrocytois
B12/folate deficiency
28
Beta locus for Hb located on
chromosome 11
29
Alpha locus for Hb located on
chromosome 16
30
Genetics of SCD
glutamic acid replaced by valine at the 6th codon of the beta gene
31
Gardos channel on RBC
Ca2+ in; K+ and water out | Causes sickling in SCD
32
KCl co-transporter
Activated by decreased pH (acidosis) K+, Cl- and water leave the cell Causes sickling in SCD
33
Cell fee Hb is a sink for what and causes what
NO | Vasomotor dysregulation
34
Most common location of vaso-occlusions in SCD
Post-capillary venules due to lowest O2 tension
35
Most common cause of death in children with SCD worldwide
Infection | Encapsulated - S pneumoniae, H influenzae, S aureus, Salmonella
36
Viral infection that causes aplastic crisis in SCD
parvovirus B19 (5th disease, slapped cheeks)
37
Infection leading to acute chest syndrome in SCD
Mycoplasma, chylamydia, S pneumoniae
38
SCD tx
``` Penicillin prophylaxis Hydroxyurea -> HbF Transfusions Stem cell transplantation Pain management ```
39
Most important determinant of intact RBC membrane
Cell surface/volume ratio
40
Non-spherocytic hemolytic anemia (CNSHA)
RBC enzymopathies
41
Drug that triggers acute hemolytic anemia in G6PD deficiency
Sulfa drugs
42
PKLR mutations
Pyruvate kinase deficiency | Leads to increase 2,3 BPG as compensation
43
Drugs that cause warm antibody hemolytic anemia
methyldopa, penicillin, quinidine
44
Hapten induced drug absorption
High dose penicillin Cephalosporins, tetracycline Drug binding to RBC is a critical step
45
Immune complex drug anemia
Quinidine, quinine, sulfonamides, isoniazid | Neoantigen - Coomb's test would be negative
46
Autoimmune drug anemia
methyldopa, levodopa, procainamide Drug stimulates production of autoantibody So doesn't matter if you stop the drug
47
Acute cold-antibody hemolytic anemia
Mycoplasma and EBV
48
Anti-I
mycoplasma
49
Anti-i
EBV, lymphoma
50
Transfusion can make hemolysis worse in | Also corticosteroids aren't effective in
Cold agglutinins
51
Direct Coomb's test positive
Warm agglutinins | Rh hemolytic disease
52
Indirect Coomb's test positive
Cold agglutinins | ABO hemolytic disease
53
Mutation in PIG-A responsible for GPI-anchored protein deficiencies in
PNH
54
PNH
Cells deficiency in membrane inhibitor of lysis, CD59, leading to complement -mediated lysis of RBC. Causes pancytopenia Can evolve - aplastic anemia, myelodysplasia, leukemia
55
Results in thrombosis in portal vein, cerebral vessels and cutaneous veins
PNH
56
Erythropoiesis production time
5 days
57
EPO interacts with
Epo-R on MEP
58
EPO is useful when
Renal insufficiency Post chemo MDS Early aplastic anemia
59
EPO not useful when
``` Pernicious anemia Aplastic anemia (late) ```
60
Interacts with c-mpl receptor on MEP
Thrombopoietin | Not useful clinically
61
Granulopoiesis production time
5-7 days
62
Other names for M-CSF
CSF-1, c-fms
63
Primes granulocytes for activation
GM-CSF
64
Used to shorten post chemo neutropenia
GM-CSF and G-CSF
65
SE of GM-CSF
Fever, eosinophilia
66
Example of class 1 cytokine receptors
IL3-R, IL5-R, GM-CSF-R | EPO-R, G-CSF-R
67
Example of class 3 cytokine receptors
VEGF-R
68
DNA repair defect of cross-link repairs
Fanconi anemia
69
Telomere defect
Dyskeratosis congenita
70
Mutation on SDBS gene
Shwachman-Diamond syndrome
71
Accumulation of mutations in HSC genome
MDS
72
Source of HSC in clinical use
Bone marrow Cord blood Cytokine mobilized
73
Committed progenitor cells, BFU-E and CFU-E, are
EPO dependent
74
Single amino acid substitution caused by point mutation activating cryptic mRNA splice site
Hemoglobin E disease
75
Acute hemolysis is more prominent in
Alpha thalassemia and HbH disease compared to beta-thalassemia
76
What to balance in thalassemia tx
Appropriate transfusions and adequate iron chelation
77
HbH clinical features
Iron overload Hepatosplenomegaly Cholelothiasis Anemia during pregnancy
78
Agents available for iron chelation
DFO, Deferiprone, Deferasirox
79
Inhibitors of platelet coagulation generated by endothelium
Prostacyclin, NO
80
Antithrombotic mechanisms
Antithromin 3 Protein C and S TFPI Fibrinolytic system
81
Primary hemostasis
Vasoconstriction and aggregated platelets
82
Secondary hemostasis
Fibrin formation for more permanent clot
83
Burst of thrombin generation
Thrombin activates 5, 8, 11
84
Factors inhibited by warfarin
2, 7, 9, 10 | These are all vitamin K dependent
85
Inhibitors of thrombin
Antithrombin and thrombomodulin
86
Inhibitors of Va and VIIIa
Protein C and Protein S
87
TFPI inhibits
Xa and TF-VIIa
88
Protease next 2 inactivates
Free factor 11a
89
Plasmin leads to fragments of cross-linked fibrin
D-dimers | Can be elevated even with clot present
90
PAI-1
Inhibitor of tPA (plasminogen activator)
91
Hepzyme
Neutralizes heparin which is present when patient has a central venous catheter
92
HHT is also known. as
Osler-Weber-Rendu syndrome
93
Mutation in Endogeni or ALK1 gene leading to dysregulated TGF-beta
HHT
94
Cryoprecipitate
Used to replace factor 8 and fibrinogen and vWF
95
1 Bethesda unit
Amount of antibody that neutralizes 50% of factor 8 or 9 in normal plasma after 2 hrs of incubation
96
Competitively inhibits binding of plasminogen and t-PA (used post-surgically)
Amicar/transexamic acid
97
Factor deficiency that doesn't affect PT or PTT
Factor 13 | Crosslinks fibrins
98
Riastap
Plasma-derived fibrinogen concentrate
99
Why should you transfuse carefully in long-standing anemia?
TACO | Transfusion-associated circulatory overload
100
Indications for giving EPO
Renal disease | Chemotherapy
101
Random donor platelets that require 5 units for adult dose
Whole-blood platelet unit
102
The way most platelet donation is done in the USA | Single-donor platelets
Plateletpheresis
103
Prevents anti-Rho antibodies from form when given D+ RBC
Rh immune globulin
104
Plasma has less --- than FFP
Factor 8
105
Factor 7 1/2 life
4-6 hrs | Shortest
106
Factor 8 1/2 life
12 hrs
107
Other factor 1/2 lives
1-2 days
108
Fibrinogen 1/2 life
2-4 days
109
Factors available on US market
7, 8, 9, 10, 13, fibrinogen
110
Factors not available on US market
Factor 5 and 11
111
INR target for warfarin tx
2-3
112
Ig to ABO carbohydrate
IgM
113
Donor plasma that typically causes TRALI
multiparous women
114
Normal lifespan of platelets
9-10 days
115
TPO is produced by what and binds which receptor
Produced by the liver constitutively and binds the c-Mpl receptor on platelets, MK cells, stem cells
116
Platelet secondary hemostasis function
Provide phospholipid and calcium as co-factors for coagulation cascade. Stores factor 5 and 8
117
Platelet proliferation receptor acts through
JAK-STAT
118
Platelet differentiation acts through
MAP kinase
119
Aspirin inhibits
Platelet function
120
Platelet count where spontaneous bleeding occurs
50k
121
NAIT
Neonatal alloantibody immune thrombocytopenia
122
Congenital platelet disorders
Bernard Soulier | Wiscott-Aldrich syndrome
123
When do we see HIT
4-10 days after initiation of heparin
124
Thrombocytopenia in HIT
Decrease platelet count of 50% or more | Count can still be normal
125
HIT - venous or are arterial thrombosis?
2/3 venous
126
Direct thrombin inhibitors
Argatroban, Bivalrudin
127
Triad of symptoms for thrombotic microangiopathies (TMAs)
Thrombocytopenia Intravascular hemolysis (see schistocytes) Renal failure
128
Pathophysiology of TTP
Decreased ADAMTS13 leading to increase vWF multimers
129
Treatment for TTP
Plasmapheresis - replace ADAMTS13 | Rituximab - reduces relapse
130
Treatment for atypical HUS
``` Plasmapheresis Eculizumab (anti C5 ab) ```
131
Glanzmanns Thrombasthenia
Platelets dysfunctional due to lack of G2b/3a (binds fibrinogen) Platelet count is normal
132
Bernard Soulier
Defect in G1b-V-X (vWF receptor) on platelets Large platelets Moderate to severe bleeding
133
Wiskott Aldrich
Only condition that causes microthrombocytopenia Defect in WAS gene X-linked Immune deficiency, eczema, microthrombocytopenia
134
Congenital bone marrow failure syndromes
Dyskeratosis congenita Fanconi's anemia Osteopetrosis
135
Acquired bone marrow failure syndromes
Aplastic anemia | Myelofibrosis
136
IRIDA
Iron refractory iron deficiency anemia | Caused by inactivation of TMPPSS6
137
IL-6 receptor antagonist
Tocilizumab | Decreases hepcidin in anemia of inflammation
138
Infections associated with hemochromatosis
Listeria, yersinia, vibrio
139
Causes of macrocytosis that's not B12 or folate
``` Alcohol Liver disease Hypothyroidism Bone marrow failure Reticulocytosis Drugs (hydroxyurea, anti-retrovirals) ```
140
Most common cause of B12 deficiency in the elderly
Atrophic gastritis with deficient HCl secretion
141
Mechanism behind Trousseau syndrome (migratory thrombophlebitis)
Tissue factor by tumor Procoagulants by tumor Compression of vessels by tumor
142
Tumors with highest rates of VTE
primary brain tumors, pancreatic, stomach, lung
143
Protein C inactivates
Factor 5 and 8
144
Protein S binds to
C4b
145
What conditions increase C4b and therefore decrease free Protein S
Estrogen&pregnancy, Sickle cell, HIV
146
Chemo drug that decreases antithrombin
I-asparaginase
147
Autoantibodies of APS
Lupus anticoagulant Anti-B2 glycoprotein Anti-cardiolipin
148
Direct thrombin inhibitors
Dabigatran, argatroban, Desirudin, bicalirudin
149
Direct factor 10a inhibitors
Rivaroxaban, apixaban, edoxaban
150
Drugs that increase warfarin sensitivity
``` Antibiotics: erythromycin, fluconazole, INH Cardiac: amiodarone, propranolol Anti-inflammatories: piroxicam GI: cimetidine, omeprazole Herbs: ginkgo, garlic ```
151
Drugs that increase warfarin resistance
Antibiotics: nafcillin, rifampin Anticonvulsants Other: sucralfate, cholestyrame Herbs: St Johns Wort
152
Used to monitor unfractionated heparin
aPTT | Can also use UFH anti-Xa level
153
Unfractionated heparin side effects
Bleeding, HIT, osteoporosis, hyperkalemia
154
Tx for HIT
Argatroban
155
Reversal agent for dabigatran
Idarucizumab
156
Reversal agent for rivaroxaban, apixaban
Andexanet alpha
157
Anticoagulation meds not good for pregnancy
DOACs and warfarin
158
Where is vitamin K absorbed and what is required?
Terminal ileum | Bile acids
159
Used to treat fibrinogen disorders
Cryoprecipitate
160
Most common cause of chronic DIC
Malignancy
161
Most common manifestation of anti-phospholipid syndrome
Venous thrombosis
162
Most important for colorectal cancer staging
Depth of invasion and lymph nods
163
Main considerations for PCa
Unilateral vs. bilateral disease Extraprostatic extension Seminal vehicle invasion
164
Increases risk of bilateral breast cancer
Lobular carcinoma in situ
165
Invasive ductal carcinoma
Most common breast cancer Hard and stellate appearance Cords or solid nests of malignant cells invading stroma
166
Invasive lobular Ca
Lack of cohesion, strands of infiltrating small tumor cells in single-file E-cadherin assent Mets: serosal surfaces, bone marrow, meninges
167
Most important for staging breast cancer
Tumor size, multifocality, lymph node
168
HTLV-1
ATLL
169
EBV
Burkitt lymphoma, NK/T cell neoplasms
170
HHV8
Kaposi's sarcoma, Castleman's disease, primary effusion lymphoma
171
Merkel cell polyomavirus (MCV)
Merkel cell carcinoma
172
Flower shaped lymphocytes
ATLL
173
Oncoprotein of HTLV1 that CD8 cells recognize and allows them to suppress
Tax protein
174
Associated with post-transplant lymphoproliferative disease
EBV
175
Viral proteins of EBV
LMP-1 and EBNA-2
176
Associated with t(8:14) - translocation of myc gene
Burkitt lymphoma
177
Starry sky pattern in germinal center
Burkitt lymphoma
178
Neutrophil maturation
Blast-> promyelocyte -> myelocyte -> metamyelocyte -> band -> neutrophil
179
CD56 and CD16
NK cells
180
Myeloid sarcoma
Myeloid neoplasms involving the tissue
181
Mantle cell lymphoma is actually a
leukemia
182
What morphology is only seen in myeloid lineage
Auer rods
183
Phenotypic markers of immaturity
CD34, CD117, TdT, CD1a
184
Location of naive B cells waiting to be stimulate
Mantle zone
185
CD23 and CD21
Follicular dendritic cells
186
CD10
Germinal center B cells
187
t(14:18) - expression of BCL-2/IgH
Follicular lymphoma | DLBLC
188
Most common B cell lymphomas
DLBCL | Follicular lymphoma
189
Phenotype for follicular lymphoma
CD10, CD20, CD21, BCL6 (in grade 3)
190
B symptoms
Fever, weight loss, night sweats
191
Germinal center markers
CD10, BCL6, MUM1
192
Genetics of DLBLC
BCL6 over expression t(14:18) C-Myc rearrangment
193
Precursor to CLL
B cell lymphocytosis
194
Markers of CLL/SLL
Kappa restricted, CD5, CD23, negative FMC
195
ZAP70 causes
inferior outcomes for CLL/SLL
196
Genetic deletion in blank causes CLL/SLL
chromosome 13q
197
CLL/SLL may transform to
DLBLC or Hodgkin lymphoma
198
t(11:14); CCND1-IgH
Mantle cell lymphoma
199
BCL1 (cyclin D)
Mantle cell lymphoma | Product of t(11:14) translocation
200
Anaplastic large cell lymphoma
t(2:5) -> ALK CD30+ Large hallmark cells with horse shoe shaped nuclei
201
Exranodal NK/T-cell lymphoma
EBV CD56 Cytoplasmic CD3
202
Popcorn cells
Nodular lymphocyte predominant Hodgkins
203
Reed-Sternberg cells
Hodgkin lymphoma | CD30, CD15, PAX5
204
Tx for ET
Aspirin | IFN/HU
205
Tx for PV
Phlebotomy and aspirin
206
Ruxolitinib
Jak 2 inhibitor | Treatment of myeloproliferative disorders
207
Leukoerythroblastosis
Myelofibrosis
208
Left shift on blood smear
CML
209
Most common cause of stem cell transplant
AML
210
Each unit of RBCs has how much iron
200-250 mg
211
If someone has G6PD deficiency, when might G6PD levels be normal
During period of acute attack because levels may be normal in reticulocytes
212
Causes of aplastic crisis
``` Folate deficiency Other vitamin deficiencies Aplastic anemia Marrow replacement (malignancy) ```
213
Parvovirus B-19 can cause
Chronic hemolytic anemia | Aplastic anemia
214
Most common porphyria in adults with chronic blistering cutaneous
Phorphyria cutanea tarda | Test plasma or urine porphyrins
215
2nd most common porphyria in adults with acute neurovisceral symptoms
Acute intermittent porphyria (AIP) | Test urine prophobilinogen
216
Most common porphyria in children acute non blistering cutaneous symptoms
Erythropoietic protoporphyria | Test erythrocyte protoporphyrin
217
Increase neutrophils
CML
218
AML risk factors
Prior chemo or radiation MDS, MPS Benzene, cigarette smoking Down syndrome, Fanconi anemia
219
Precursor myeloid markers
CD34, CD117
220
Granulocytic markers
CD13, CD33, MPO
221
Monocytic markers
CD14, CD64
222
Microgranular variant of APL
bi-loved nuclei | Auer rods: rare
223
Rydapt (midostaurin)
FDA approved for AML with FLT3-ITD
224
Targeted therapy in AML
FLT-3 | IDH2
225
MDS patients typically die from
Complications related to cytopenia
226
Genetic syndromes with increase risk of MDS
Fanconi anemia, dyskeratosis congenita
227
Treatment for MDS with isolated del(5q)
Lenalidomide
228
Marker for stem cells
CD34
229
Increased risk of B-ALL in children with?
Down syndrome
230
Atypic megakaryrocytes
MDS del (5q)
231
Kappa:lambda
2:1
232
Most common plasma cell disorder
MGUS
233
Multiple myeloma end organ damage
``` CRAB HyperCalcemia Renal insufficiency Anemia Bone lesions ```
234
Dutcher bodies in multiple myeloma
Ig in nucleus
235
Russel bodies in multiple myeloma
Ig in cytoplasm
236
Normal plasma cells
CD19+ , CD56-
237
Neoplastic plasma cells
CD19- , CD56+
238
Leads to bone destruction in multiple myeloma
MIP1a -> RANKL
239
Myeloma cells dependent on ---- for growth, survival, Ig production
IL-6
240
Myeloma Ig created can be
abnormal and nephrotoxic
241
Rouleaux formation
Multiple myeloma
242
Most common cause of death in multiple myeloma
Infection
243
Daratumumab
targets CD38 cells to kill multiple myeloma cells directly
244
Waldenstrom's macroglobulinemia
IgM monoclonal gammopathy in the presence of lymphoplasmacytic lymphoma
245
Plummer-Vinson syndrome
Iron deficiency Post-cricoid dysphagia Esophageal webs
246
Basophilic oval nuclei
Descriptive for HPV
247
Most common bone sarcoma
Osteosarcoma
248
Vinyl chloride is associated with
Hepatic angiosarcoma
249
No role for radiation in which sarcomas?
Abdominal or visceral
250
Bimodal and associated with Paget disease
Osteosarcoma
251
Arise in metaphysic of bone Codman triangle-periosteal reaction Lytic and blastic change
Osteosarcoma
252
Affect the shaft of the bone in an infiltrative "onion skinning" pattern
Ewing sarcoma
253
Tx for Giant Cell Tumor of Bone
Denosumab
254
Preventing tumor lysis syndrome
Hydration Allopurinol Rasburicase
255
Established drug for FAP
Sulindac | NSAID causing regression of adenomas
256
Anti-CTLA4 antibody
Ipilimumab
257
Cytokines leading to tumor regression
IL-12, IL-2, IFN-g, TNF-a
258
Cytokines leading to tumor progression
IL-4, IL-5, IL-10, TGF-b
259
Anti PD-1 antibody
Nivolumab | Pembrolizumab
260
Adverse events with immunotherapy
Diarrhea/colitis Rash and/or pruritus Hepatitis Endocrine dysfunction
261
Which cancer requires no biopsy to confirm diagnosis
HCC | AFP > 500 is diagnostic
262
Common sites of lung mets
Brain, bone, liver, adrenal
263
Alectinib
Treats EML4/Alk translocation lung adenocarcinoma
264
Cancers that commonly mets to the brain
Lung, breast, melanoma
265
Brain mets that commonly bleed
Renal cell, melanoma, thyroid cancer, choriocarcinoma

Heme/Oncology (2 decks)

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