Heme/Onc Exam Flashcards
1
Q
4 Grades of Anemia
A
Mild: 10-12 female or 10-13.5 male
Moderate: 8 and up
Severe: 6.5 and up
Life-Threatening: below 6.5
2
Q
Normal RBC life span? How do they die, and how often normally?
A
100-120d
Senescence (phagocytized in liver/spleen/marrow) and hemolysis (<0.5% per day)
3
Q
Normal CBC c diff (Hgb, Hct, rets, MCV, plt, WBC, neutros, lymphos, monos)
A
Hgb: 12-16 female/13.5-17.5 male Hct: 36-46 female/41-53 male Rets: 0.5-1.5% or 35,000-85,000 MCV: 80-100 Plt: 150,000-400,000 WBC: 4,500-11,000 Neutros: 40-60% Lymphos: 20-40% Monos: 4-11%
4
Q
What does EPO do and what triggers its release?
A
Causes proliferation/maturation of erythroid progenitors into rets and proerythroblasts
Released by KIDNEY in response to anemia and hypoxia (Hypoxia-Inducible Factors)
5
Q
What do hemopexin and haptoglobin do?
A
Bind free heme and free Hgb respectively so that they can be recycled in new RBCs
6
Q
What vitamins/minerals are needed to make an RBC?
A
Iron
Folate
B12
7
Q
Most adult Hgb is ___. What are the other two?
A
HgbA (α2β2)
HgbA2 (α2δ2) is like 2 percent
HgbF (α2γ2) is less than 1 percent
8
Q
What are some generic common Sx of anemia?
A
Fatigue
Decreased exercise tolerance
Dyspnea
Palpitations
9
Q
What are some generic common exam signs of anemia?
A
Pallor
Tachycardia
Murmurs
Jaundice/splenomegaly if hemolytic
10
Q
What use are rets in lab diagnostics of anemia?
A
Anemia –> inc EPO –> inc rets
11
Q
A LOW ret count would make me look at __.
A
MCV!
12
Q
A HIGH ret count would make me suspect ___ or ___.
A
Bleeding or hemolysis
13
Q
A low ret count with a low MCV brings what 4 DDxs to the table?
A
[this means they have a MICROCYTIC ANEMIA]: Iron-def anemia Thalassemia Anemia of chronic disease Sideroblastic anemia
14
Q
A low ret count with a normal MCV brings what 3 anemia DDxs to the table?
A
[this means they have a NORMOCYTIC ANEMIA]:
Aplastic anemia
Anemia of chronic disease
Early stages of iron-def anemia
15
Q
A low ret count with a high MCV is highly suspicious for what kind of anemia?
A
[this means they have a MACROCYTIC ANEMIA]:
Megaloblastic anemia!
(often d/t folate or B12-def)
16
Q
Describe the transformation process of an HSC to an RBC. Where does this occur?
A
HSC –> proerythroblast –> erythroblast –> normoblast –> reticulocyte –> RBC
In red bone marrow until [Hgb] hits about 34%, then in the bloodstream
17
Q
What is bilirubin?
A
The waste product of Hgb once the heme part has been recycled
18
Q
If I suspect any type of anemia, what labs do I order?
A
CBC with diff
Peripheral blood smear
19
Q
Microcytic means MCV ___; normocytic is MCV ___; and macrocytic means MCV is ___.
A
Micro <80
Normo 80-100
Macro >100
20
Q
What is the difference between an MCV and an RDW?
A
MCV is avg RBC size; RDW is how much variation there is in RBC size
21
Q
What is the difference between an MCH and an MCHC?
A
MCH is the avg wt of Hgb in an RBC
MCHC is the avg [Hgb] in an RBC (‘chromia’)
22
Q
Spherocytes on a peripheral blood smear suggest…
A
Hereditary spherocytosis
Autoimmune hemolytic anemia (will also have reticulocytosis)
23
Q
Schistocytes on a peripheral blood smear suggest…
A
DIC (will also have abnormal coag)
TTP
HUS (hemolytic-uremic syndrome)
24
Q
Dacrocytes on a peripheral blood smear suggest…
What do they look like?
A
Myelofibrosis (a type of leukemia)
Teardrops!
25
What do stomatocytes on a peripheral blood smear suggest? What does a stomatocyte look like?
Hereditary stomatocytosis
Acute EtOH intoxication
Chemo Rxs like vinblastine
Normal variant of drying if not widespread
Like a slit in the middle of the RBC
26
What do codocytes on a peripheral blood smear suggest? What does a codocyte look like?
Liver disease
HbC (should also have splenomegaly)
Thalassemias
Splenectomy - surg or autosplenectomy (d/t SCA)
Like a target!
27
What do echinocytes on a peripheral blood smear suggest? What does an echinocyte look like?
Uremia
PK-def
Like a burr - 'echino' means 'like a hedgehog' in Greek
28
You find a leptocyte on a pt's peripheral blood smear. What did it look like? What two hematological differentials are now on your DDx?
Kind of like a large, very thin target cell.
| Iron-def anemia + thalassemia
29
You find a Cabot ring on a pt's peripheral blood smear. What did it look like? What caused that?
Like a thread in the shape of a loop or an 8 inside the RBCs.
Leftover mitotic spindle from dyserythropoeisis.
30
What's a Howell-Jolly body?
Leftover nuclear fragments often d/t splenectomy (or autosplenectomy of SCA).
31
Why would basophilic stippling occur?
That's ribosomes/mitochondria chillin' around the periphery of an RBC. Could be d/t lead poisoning or sepsis.
32
Polychromasia reflects what hematologic change in the body?
Inc production of rets
33
In order to be effective, an RBC needs heme + porphyrin ring + globins. What disorders happen when each of these ingredients is taken away? What would labs show once they are past the early stages?
No heme - iron-def anemia
No porphyrin - sideroblastic anemia
No globin - thalassemias
Microcytic hypochromic! (low MCHC, low MCV)
34
What is the most common cause of iron-def anemia? What are some clues that someone has iron-def?
BLEEDING (GI/period) - 100cc lost = 50mg iron lost
PICA/phagophagia, angular cheilitis, glossitis, koilonychia
+ general anemia s/s
35
What labs should you automatically order if you suspect iron-def anemia (which, like, you always should suspect)?
SrFe
Transferrin
Ferritin
TIBC
36
So just to be clear, what will the labs of a non-early-stage iron-def anemic show?
```
LOW rets, MCV, MCHC
LOW SrFe, Transferrin, Ferritin (false high in inflam)
HIGH TIBC (can false low in inflam)
```
37
I am positive my patient has iron-def anemia. How should I treat it?
FeSO4 325mg titrating up from qhs to tid, c VitC x3-6mo
| Repeat labs in 2-3wks
38
Bad news, my patient with iron-def anemia has uncontrollable bleeding or otherwise cannot do po meds. What should I do?
Venofer (iron sucrose) IV push 2-5min
or
Ferrlecit (sod ferr gluconate) IV drip 30mins
NOT iron dextran! ==> hypersensitivity rxn!
39
Again, the 4 MICROCYTIC anemias are:
Iron-def anemia (*early stage = normocytic*)
Thalassemia
Anemia of chronic disease (*can be normocytic*)
Sideroblastic anemia
40
Why does anemia of chronic disease occur? What are the chronic diseases that can cause it?
ILs blocking response to EPO + IL-6 increasing hepcidin (vault access denied!)
Endocrine, infection, chronic inflam, CA. (things that inc IL.)
41
Is anemia of chronic disease associated with CHF, DM, COPD, or HTN?
NOOOOO.
42
Is anemia of chronic kidney disease the same as anemia of chronic disease?
Kind of, but no. CKD = dec EPO production, not dec EPO response.
43
What do the labs of a person with anemia of chronic disease look like? Think this through.
```
LOW rets, SrFe
LOW to NORMAL TIBC
NORMAL MCV, MCHC
NORMAL to HIGH Ferritin
HIGH ESR/CRP
```
44
How do you treat anemia of chronic disease?
You treat the chronic disease.
| +/- supplemental iron or EPO (aka ESA, a CSF+ESF)
45
ESAs include __, __, and __. What pt popn is best for each?
Epogen, Procrit (aka epogen alfa) = CKD pts
| Aranesp (aka darbepoetin alfa) = CA pts who are NOT getting transplants
46
What are the downsides of ESA treatment?
BBW: inc VTE, CA mortality, HTN, and a slew of other S/E like HA and myalgia
47
What monitoring is needed before and during ESA treatment?
Baseline Hgb <10; also baseline Hct and Fe
| Get Hgb qwk and stop once >12
48
What is the difference between α-thalassemia and β-thalassemia?
```
α-thalassemia = chromosome 16 deletions (up to 4)
β-thalassemia = point mutations in β-globins
```
49
Who gets α-thalassemia? What is it called when there are 3 functioning chains? 2? 1? None?
```
Chinese/SE Asians
3 good = silent carrier
2 good = α-thalassemia minor
1 good = HgbH disease
None left = hydrops fetalis (will die at birth)
```
50
HgbH disease would show what on peripheral blood smear? How do you treat this type of α-thalassemia?
Target cells and Heinz bodies
Might need transfusions or chelation. Unusually bad = splenectomy + stem cell transplant
51
In general, α-thalassemia s/s are __, __, and __, and these pts are treated with...
Fatigue, pallor, splenomegaly
| Folic acid and iron supps, genetic counseling
52
Besides a peripheral blood smear, what labs should a suspected α-thalassemia pt get? What would they show?
CBC c diff - Microcytic hypochromic with normal ferritin (this r/o's iron-def)
Hgb Electrophoresis - to determine how many αs are left
53
Homozygous β0-thalassemia (major) means no β-globins, which means...
No HgbA
| Relatively high α-chains which --> hemolysis --> severe hemolytic anemia
54
What is β+-thalassemia? What are the two types of it?
Some β-globins, some HgbA
β+/β+ or β+/β0 = moderate (intermedia)
β+/β or β+/β0 = mild (minor)
55
All β-thalassemia is microcytic hypochromic, and will show what on peripheral blood smear? What special smear finding would β-thalassemia major have?
Target cells, dacrocytes, and basophilic stippling
+ POIKILOCYTOSIS for β-thalassemia major
56
Besides a peripheral blood smear, what labs should a suspected β-thalassemia pt get? What would they show?
CBC c diff - Microcytic hypochromic with normal ferritin (this r/o's iron-def) and NORMAL RDW
Hgb Electrophoresis - to determine how many βs are left
57
Sideroblastic anemia is a microcytic hypochromic anemia associated with __, caused by genetics, drugs like __, or ___.
Copper-def (will have neutropenia)
Chloramphenicol, Linezolid; EtOHism
58
Labs of sideroblastic anemia would show...
CBC c diff - Microcytic hypochromic with HIGH SrFe, Transferrin, Ferritin, and RDW
LOW TIBC
+/- LFT/kidney fxn changes
Check lead and copper levels too
59
What four peripheral smear findings are consistent with sideroblastic anemia?
Sideroblasts, basophilic stippling, target cells, Pappenheimer bodies
60
How do you treat sideroblastic anemia?
Chelate, transfuse, give copper/B6 as needed
| If severe: bone marrow transplant
61
What treatment options are useless in sideroblastic anemia?
EPO supplementation - won't respond
| Splenectomy - C/I in congenital sideroblastic anemia
62
What are some anemias that fall under the category of intrinsic hemolytic anemia?
Hereditary sphero/elliptocytosis
G6PD-def
SCA
Thalassemia MAJOR
63
What are some anemias that fall under the category of extrinsic hemolytic anemia?
Autoimmune (IgG = warm; IgM = cold)
Drug-induced (ABx, antimalarials, antiTBs, methyl/levodopa)
Microangiopathy like DIC, TTP, HUS
Infection/burns etc.
64
What are some generic s/s of hemolytic anemia?
Pallor or jaundice/icterus
Dark urine
Petechiae, purpura, ecchymosis
Abd +ttp
65
Direct bilirubin is __.
Conjugated
66
What are the big three lab findings of hemolytic anemia?
Inc LDH
Inc bilirubin
Dec haptoglobin
67
Besides the big three findings, what 4 other lab findings are consistent with hemolytic anemia? What would be present in urine?
Inc rets, indirect bilirubin, methemalbuminemia, hemoglobinemia
Urine hemosiderin
68
What does a direct Coombs test test for?
RBCs coated with antibodies/complement
69
What does an indirect Coombs test test for?
Anti-RBC antibodies in the serum
70
How do you treat moderate to severe hereditary spherocytosis?
Splenectomy!
| + folic acid and/or transfusions
71
How do you treat G6PD?
D/C offending drug (nitrofurantion, sulfa, dapsone) if that's what's causing it
Tx an infection if that's what's causing it
72
What are the 3 most significant LAB findings for G6PD?
Spherocytes + rets on smear
Hyperchromic
Coombs neg
73
Why does PNH occur?
No CD55/59 --> complement system lyses RBCs
74
Alright there's a lot of systemic s/s of PNH but I'm down to the wire. Name three clear Sx of PNH.
Episodic hemoglobinuria
Erectile dysfxn
Thrombosis --> death
75
Main lab to diagnose PNH?
Coombs would be __.
LDH would be __.
Everything else is variable.
CD55 FLOW CYTOMETRY
neg
high
76
We give PNH pts to hematologists, but mild ones (most) don't need intervention. How do we treat moderate/severe PNH pts?
Eculizumab + steroid + transfusions PRN
77
If a PNH pt has SEVERE Sx/thrombosis, how do we treat them?
Allogenic stem cell transplant
78
G6PD is a __ disorder that causes ___, most often in what pt popn?
X-linked recessive; episodic hemolytic anemia
| AAM
79
During an episode, what would the labs of a G6PD pt show? When would you do them?
NOTHING
| @6-8wks post-hemolysis episode: high rets/bilirubin; Heinz bodies and bite cells on smear
80
What is warm AIHA?
IgG:RBC at room temp, usually idiopathic
81
What are three big Sx of AIHA?
Angina + Jaundice + Splenomegaly
82
In warm AIHA, direct Coombs would be __ and indirect Coombs would be __.
What other three lab values are good to note?
POSITIVE!!
either + or -
Inc rets + inc indirect bili + dec haptoglobin
83
How do you distinguish between AIHA and hereditary spherocytosis, since both have the same smear? (What does that same smear show?)
Direct Coombs!! + for AIHA, - for HS
| Smear for both = rets + spherocytes
84
We will give AIHA pts to a hematologist, but in general, what 4 treatment options are we looking at?
Transfusions (hard)
Therapeutic plasma pheresis
Prednisone
Splenectomy
85
Cold AIHA is called that because...
IgM:RBCs at cold temperatures which looks kinda like Raynaud's in the extremities but with hemoglobinuria when it's cold out
86
What would the Coombs for cold AIHA show? What about the smear?
Coombs + for complement, - for antibodies
| Rets on smear
87
How do you treat cold AIHA?
Rituximab/immunosuppression if bad
| Or more often just supportive stuff
88
Sickle-cell disease is a __ disorder due to a __ mutation in what? How long do RBCs with sickle-cell live?
Autosomal recessive
V6E for β-globins --> HgbS
10-20d
89
A sickle-cell crisis can be __ (like __), __ (like __), or __ (like __).
Vasoocclusive (CVA, MSK)
Hematologic (hemolytic crisis)
Infectious (PNA)
90
How do you treat adult SCA when they are not in a crisis?
Get the pneumococcal vax
Avoid triggers
Folic acid 1mg po qd
91
How do you treat SCA during a crisis?
Generous pain meds
| Supportive measures
92
What will a SCA smear show?
Sickled cells
Rets
Howell-Jolly bodies
Target cells if autosplenectomy
93
How do you treat kids with SCA?
Hydroxyurea can inc HgbF
Long-term transfusions to reduce CVAs
Allogenic HSCT
94
What are the risks associated with sickle-cell trait?
VTE
| Rhabdo/MI after heavy exercise
95
If LDH, T.Bili, and Haptoglobin are all NORMAL, what do you suspect?
Bleeding --> if not, CA, check smear
96
If LDH and T.Bili are high, but hapto is low, what do you suspect?
Hemolysis --> look at smear
97
What's the difference between macrocytic and megaloblastic anemia? (think: labs + causes)
Macro - MCV > 100 (d/t EtOHics, hypothyroid, anti-HIV/hydroxyurea)
Megalo - MCV > 115 (and d/t vit-def)
98
What is aplastic anemia?
Welp it ain't anemia
| It's pancytopenia d/t injury to pluripotent stem cells
99
Aplastic anemia is most common in...
East Asians
| People with other marrow issues
100
Aplastic anemia can be ACQUIRED:
From Rxs like...
Or from chemicals/toxins like...
Or from viruses, pregnancy, or anorexia.
Carbamazepine, phenytoin, indomethacin, gold, PTU, methimazole, sulfonamides, chloramphenicol
Benzene, solvents, and huffin' glue
101
What are two autoimmune causes of aplastic anemia?
Idiopathic autoimmune
| SLE
102
What are two congenital causes of aplastic anemia?
```
Fanconi anemia
Dyskeratosis congenita (telomere length issue in HSC)
```
103
Aplastic anemia presents as __ and __, and leads to ...
Bleeding from everywhere (=petechiae) and cardiopulm compromise
Infections = death
104
Aplastic anemia does NOT present with __, __, or __!
Hepatosplenomegaly!!
LAD
Bone pain
105
3 big findings on labs of aplastic anemia? What would a smear show?
Pancytopenia
Anemia
Low rets
Smear - no weird shapes!
106
Moderate aplastic anemia would show __ on a bone marrow Bx.
| What about severe aplastic anemia?
<30% cellularity
| <25% or <50% with really low plt, ANC, or rets
107
What do you need to diagnose someone with very severe aplastic anemia?
Severe Aplastic Anemia + ANC under 200!
108
How do you treat nonsevere aplastic anemia?
Treat the underlying cause, prevent infection, give EPO/myeloid GF
109
How do you treat severe aplastic anemia?
Allogenic HSCT!
| Or immunosuppression Horse AtG + CsA (can cause other hem/onc issues though)
110
Fanconi anemia is a __ disorder that presents with fish hands and can only truly be treated by...
Autosomal recessive
| Allogenic HSCT
111
What do megaloblastic RBCs look like? What do megaloblastic neutros look like?
Macroovalocytic
| Segmented
112
What are the most common causes of megaloblastic anemia?
Poorly prepped foods
| Folate-def in pregnancy
113
Intrinsic factor is made by __ and helps B12 get absorbed in __.
Gastric parietal cells
| The terminal ileum!
114
What two pathways need B12 to do the thing?
```
DNA synth (/RBC synth)
Myelination
```
115
Pernicious anemia is an autoimmune disorder in which antibodies attack ...
What can that eventually lead to?
Intrinsic factor and gastric parietal cells
| Gastric CA
116
B12-def is a macrocytic anemia with LOW __ and __, and HIGH __, ___, and __.
LOW B12 and rets
| HIGH MMA, homocysteine, indirect bili
117
Pernicious anemia would have LOW __, but HIGH __; also, antibodies. What special test can you do?
LOW pepsinogen
HIGH SrGastrin
Schilling test, but it kinda sucks.
118
How do we treat B12 deficiency? And don't just say 'giving B12.'
B12 100mcg SC or IM qd x 1wk, then qwk x 1mo, then qmo for life
-or-
Methylcoabalamin 1mg SL or PO qd for life
+ folic acid x1mo
119
Besides B12 level, what should you probably monitor in your B12-def pt you're treating and why?
CBC - HypoK+ risk with supplemental B12
120
Your body has enough B12 in storage for __ and enough folate in storage for __.
3yrs
| 2-3mos
121
Where does folic acid get absorbed?
Whole sm intestine
122
Normal plts are above __. Most thrombocytopenics are ASx until it's under __.
150,000
| 100,000
123
Plts come from __ cells and are stored in __. How long do they last?
Megakaryocytes (each makes like 10,000 plt)
Spleen (1/3 of all plts are in storage)
5-9d
124
What causes stored plts to be released?
| What phagocytizes plts?
Sympathetic nervous system
| Kupffer liver cells and the spleen
125
What is dilutional thrombocytopenia, and how do you prevent it?
In pts getting packed RBC - give plt to pt receiving >20 U in 24 hours.
126
Bleeding difference between thrombocytopenics and coag disorder pts?
Thrombocytopenics bleed immediately, coag issues bleed more slowly
127
Petechiae do not ...
Blanch!
128
Spontaneous bleeds occur below...
10-20,000 plts!
129
What is vWF?
| What is vWD?
A factor in plt aggregation
| An autosomal dominant bleeding disorder
130
What are the three types of vWD? Briefly describe each.
I - not enough vWF
II - the vWF is not effective
III - ZERO vWF, no Factor VIII, basically hemophilia A
131
Heart valve disease, DIC, and hypothyroidism can all cause vWD. How?
Valves - ADAMTS-13 shearing
DIC - degradation of vWF
Hypothy - dec vWF synthesis
132
What are the two treatment options for vWD?
Types I/II - desmopressin
| Type III - transfusion
133
Why is ITP called 'idiopathic' if we know why it happnes...? Why DOES it happen?
Cause the bone marrow looks normal
| Anti-IIb/IIIa antibodies attack plts and anti-IgG attack megakaryocytes (also low TPO..?)
134
Who gets ITP? What does it look like?
Peds s/p infection
| Purpuric!
135
How do you treat ITP?
IV steroid + IVIg + Anti-DIg
+/- plt transfusion right after
If emergency: splenectomy
136
What is TTP?
Like the anti-vWD: inhibited ADAMTS-13 --> inc vWF --> plt adhesion --> thrombosis
But also: using up all that plt --> thrombocytopenia
137
Besides primary ADAMTS-13 issues, how can you get TTP?
Meds: quinine, acyclovir, immunosuppressants, antiplatelets
Preg
CA/HIV
138
What is the Pentad of TTP? (3+ needed with schistocytes for Dx)
```
FAT RN
Fever
Anemia (microangiopathic hemolytic aka.. schistocytes!!)
Thrombocytopenia
Renal failure
Neuro Sx
```
139
How to treat TTP?
Pass it onnnnnnn
They will do: plasmapheresis daily!
+/- immunosuppressant/steroid
140
Hemophilia A is a __ genetic disorder caused by a deficient functional factor ___.
Hemophilia B is a __ genetic disorder caused by a deficient functional factor ___.
Hemophilia C is a __ genetic disorder caused by a deficient functional factor ___.
Recessive X-linked; VIII
Recessive X-linked; IX
Autosomal; XI
141
Moderate hemophilia means a pt has ___% of clotting factor.
1-5% (less = severe, more = mild)
142
In what three cases could a woman have symptomatic hemophilia A or B?
1) Dad has it, Mom carries it.
2) Turner Syndrome.
3) X-inactivation.
143
What labs should I order to diagnose hemophilia?
aPTT for intrinsic pathway (will be high if they have it)
| Factor VIII/IX
144
What's a common MSK Sx of hemophilia?
Target joint for hemarthrosis
145
For hemophilia ppx, we give factor VIII/IX replacement. What about for acute bleeds?
Get clotting >50%!
1 U/kg of factor VIII gtt when corrected for goal [(% desired - % actual)/2] x wt in kg
rVIIa might be better to avoid antibodies making Tx useless!
146
VitK (aka _) is a _-soluble vitamin needed to make...
Phylloquinone
Fat-soluble
II, VII, IX, X, and protC/S
147
What are the three causes of VitK-def?
Not in diet (esp. neonates)
Too much Warfarin
Bile obstruction --> can't absorb it
148
DIC is a bleeding AND clotting condition resulting from overactivated __. What is the cutaneous Sx of DIC called?
Tissue factor!
| Purpura fulminans
149
What five labs would be ELEVATED in DIC?
```
aPTT
PT
D-dimer
LFTs
Thrombin
```
150
What five labs would be DECREASED in DIC?
```
plt
Iron
Fibrinogen
AT
prot C/S
```
151
Okayyy so how do we treat DIC?
```
ABCs
plts
FFP
AT
+/- heparin
+/- protC
```
152
Polycythemia Vera is caused by a __ mutation that leads to...
JAK2
| Myeloproliferation of everything esp RBCs --> very high Hct, volume, and viscosity
153
What's the difference between primary and secondary polycythemia vera?
Primary is EPO-independent inc RBC synthesis.
154
What are the five causes of secondary polycythemia vera?
```
HEART
Hypoxemia
EPO-secreting tumors
Adrenal
Renal
Testosterone
```
155
To diagnose polycythemia vera, you need how many criteria to be met? What are the major criteria?
1 major + 2 minor or 2 major + 1 minor
Hgb > 18.5/16.5 men/women
+JAK2 mutation
156
Five ways polycythemia vera COMMONLY presents clinically...?
```
HEENT stuff (d/t high blood vol)
Thrombotic stuff
Hepatosplenomegaly
Gout
Aquagenic pruritis
```
157
What is an uncommon but pathognomic Sx of polycythemia vera?
Erythromelalgia
158
General treatment of polycythemia vera is __ and __.
lo-ASA qd
| Phlebotomy to release blood til Hct is <0.45
159
If the polycythemia vera is bad or the risk is high, treat it by...
Hydroxyurea!!!
| or interferon if that isn't possible
160
Ventilate if pt is __ or __, or for general anesthesia, or by your best judgement. Define each.
Hypoxemic: O2 <90% FiO2 (60%+)
Hypercapnic: pH <7.3 or pCO2 >50
161
ETT tube size? What Rxs can you use to place one?
7-8mm minimum
Rocuronium or succinylcholine
+
Propofol or etomidate
162
Where is the correct placement of an ETT on XR?
3-5cm ABOVE CARINA or else you might end up in R main stem
163
Complications of an ETT include ___, __, __, and more rarely, ___.
ETT migration, larynx damage, dental damage
| Tracheoesophageal fistula
164
Trach someone who...
...you don't expect to extubate within a week.
165
Name four complications of a trach.
Tracheal stenosis (usually ASx)
Accidental decannulation
Aspiration
Cuff leaks
166
Name the four most commonly used ventilator modes.
1) Volume control
2) Pressure control
3) Pressure Support
4) BiPAP (noninvasive)
167
PEEP is normally __ mmHg, normal tidal volume is ___L, and an RSBI of ___ is a go for extubation.
5-20mmHg
6-8L
<100 (RR:TV)
168
PEEP is used in most ventilation pts to __. In what two specific populations is it most useful?
Prevent alveolar collapse
| Atelectasis pts + PNA pts (to dec supplemental O2 needed)
169
In whom should we NOT use PEEP and why?
Hypovolemic pts - they already have dec cardiac output and PEEP further dec venous return --> dec CO
170
Volume control is __ or __ and is used by setting ___ on the machine.
SIMV or AC
| Set volume of air a set number of times a minute
171
In whom would I prefer AC volume control? What is its main disadvantage?
Pts who are sedated/newly intubated
| Auto-PEEP upon tachpynea
172
In whom would I prefer SIMV volume control? What is its main disadvantage?
Pts who I'm gonna start to wean because they take some of their own breaths between ventilated ones
Dyssynchrony on tachypnea
173
What do you preset in pressure control ventilation? What does that affect?
Set the driving pressure, which determines the TV
174
Pressure control is good for...
Pts who have new sutures/you want to control how much their chest expands but are sedated (it's uncomfy)
175
The ideal ventilation mode for weaning pts is __. How does it work?
Pressure Support Ventilation (PSV)
| Assists pts with their own breaths via 5-15mmH2O PRN
176
What's the main disadvantage for PSV?
No preset TV or RR = can hypoventilate if pt isn't ready
177
When is BiPAP the best option?
In impending respiratory failure
178
Process for deciding to extubate?
1) Underlying cause treated?
2) Hemodynamically stable?
3) PaO2>60 on an FiO2 40-50%, and PEEP <10?
Cool, then challenge with SBT 30'.
4) Did they pass without strugglin'?
5) Is the RSBI <100 after 30'?
179
An elevated Aa-gradient means...
Oxygen is NOT effectively transferred from the alveoli to the blood.
Low pO2 in arteries compared to inc alveolar pO2.
180
Hypoxemia with an elevated Aa-gradient that IMPROVES on FiO2 is d/t...
If it does NOT improve with FiO2, the problem is...
```
V/Q mismatch! (FYI ex: low in COPD/asthma, pulm edema; high in PE)
A shunt (ex: ARDS)
```
181
3 identifying characteristics of ARDS?
Progressing resp distress
Not improved by FiO2
Bilateral infiltrates on CXR
182
Phases of ARDS?
Exudative --> Proliferative --> maybe Fibrotic
183
How do you treat ARDS?
```
Treat the underlying cause!!!
Get paO2 to 55 using minimal settings
Get pH >7.3
Carefully use diuretics to get CVP <4, PCWP <8
Supportive stuff
```
184
Place a central line __, __, or __ using what technique?
IJ, fem, SC
| Seldinger: U/S (except SC), guidewire, confirm CXR (except for fem - use gravity test or ABG)
185
Get CVP when pt is __; normal is __ and highest during...
Supine!
0-20mmHg
Expiration
186
Place a central line with pt ___ to avoid...
Trendelenberg - venous air embolism!!
187
Three other big complications of a central line?
Thrombosis (tPA ppx)
PTX
Infection (vanco +/- zosyn while waiting on C&S)
188
Three locations of arterial lines? Why would I give that over a central line?
Rad, fem, axillary
| Unstable pts who require vasopressor support, or patients with severe HTN
189
Bad arterial waveforms like __ or __ can tip you off to a lame arterial line placement. What are some complications/drawbacks of arterial lines?
Dampened, exaggerated/fling
| Occlusions, limited ROM, infection
190
A Swan-Ganz cath can tell you directly...
RA CVP, PAP
| LA PCWP when balloon is inflated
191
Three complications of a Swan-Ganz cath?
Damaging pulm a.
Ventr. arrythmia
Infection
192
A WBC > ___ should make you think CA. What cells would be more specific to a myeloid leukemia on bone marrow Bx? What about a lymphoid leukemia?
60,000
Myeloid = PMNs
Lymphoid = lymphocytes
193
A FUO with LAD and wt loss is ___ til proven otherwise.
Lymphoma
194
AML occurs in __ pts, will show __ & __ on bone marrow Bx, and is treated by...
Old
Auer rods + >20% blasts
Chemo +/- BM transplant (M3= VitA)
195
CML occurs in __ pts, is d/t __, and is diagnosed by
Old
| 9:22 BCR-ABL (Philadelphia chromosome) - diagnosed by cytogenics
196
CML has three stages. What are they?
| How do you Tx CML?
Chronic - Accelerated (>15% blast) - Blast (>30%, extramedullary so it's becoming AML)
Imatinib + chemo +/- HSCT
197
CLL occurs in __ pts, is d/t ___, and the most common Sx are __ and ___.
Very old
Hypermutation of Ig heavy chain
LAD, Well's Syndrome (itchy eosinophilia)
198
Two diagnostic labs for CLL?
Less than 55% atypical cells + B-surface antigens CD5/CD20
199
How do you treat CLL?
Watch and wait!
| Once progressive, chemo and SCT stuff
200
ALL occurs in __ pts, has __ Sx that other leukemias don't, and is diagnosed via __.
Peds
CNS Sx
LP
201
How do you treat ALL (hint: 4 phases)?
Induction - 4-6wk inpt
Consolidation - 6-12mo
Maintenance - lo-dose chemo + prednisone 18-24mo
CNS ppx - LP/IT chemo
202
Hodgkin's lymphoma is better than NHL, and is characterized by __ & __ cells. What are the two subtypes?
Reed-Sternberg & Popcorn
| Classic & Nodular Lympho-Predominant
203
Biggest Sx of HL?
| How do you treat HL?
Painless LAD above diaphragm!
| Chemo + rad + ABVD Rxs 3-4 cycles
204
Bence-Jones proteins, lytic bone lesions, and Rouleaux formations should all make you think...
What disorder might that be preceded by?
Multiple myeloma
| MGUS
205
Lymphoplasmacytic lymphoma includes __. How is it different than multiple myeloma?
Waldenstrom's Macroglobulinemia
| No lytic bone lesions!
206
Modified Ann Arbor Staging I-IV:
I: one lymph node
II: two, on same side of diaphragm
III: two, one on each side of diaphragm
IV: an actual organ is now involved
