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Step2CK > HemeOnc Step Up > Flashcards

HemeOnc Step Up Flashcards

1
Q

what happens to O2 curve in alkalosis

A

decreased body temp, increased HbF shfit to left

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2
Q

what happens to O2 curve in acidosis

A

increased body temp
high altitude
exercise shift curve to right

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3
Q

what is a left shift fo O2 curve

A

increased Hb affinity

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4
Q

what is a right shift O2 curve

A

decreased affinity for O2. easier off loading

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5
Q

immediate Tx for CO poisoning

A

100% O2 mask

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6
Q

signs CO poisoning

A

mental status changes, cherry red lips, hypoxia despite normal pulse ox readings

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7
Q

causes of microcytic acnemia

A 
iron deficiency
lead poisoning
chronic disease
sideroblastic
thalassemias
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8
Q

causes of normocytic anemia

A

hemolytic
chronic disease
hypovolemia

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9
Q

causes of macrocytic anemia

A

folate def
vit B12 def
liver disease
alcohol abuse

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10
Q

signs of hemolytic anemia

A

brown urine
HSM
jaundice

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11
Q

labs in hemolytic anemia

A

decrease Hb and Hct
increased reticulocyte count from turnover
increased bilirubin (indirect) from lysis
increased LDH
normal MCV!!!!
decreased serum haptoglobin

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12
Q

most common form anemia

A

iron deficiency

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13
Q

Coombs direct test

A

Coombs reagant mixed with RBC. if agglutinates then IgG and c’ are present (warm and cold agglutinins.

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14
Q

Coombs indirect test

A

patient serum mixed with type O RBCs
then mixed with coombs reagant
if agglutinate– anti-RBC Ab in serum
Rh alloimmunization

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15
Q

schistocytes

A

seen in hemolytic anemia

RBC fragments

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16
Q

angular cheilitis

A

irritaiton of lips and corners of mouth

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17
Q

blood smear shows burr ceslls and schistocytes

A

Drug induced hemolytic anemia

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18
Q

wha drugs cause hemolytic anemia

A

penicillin, methyldopa

quinidine

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19
Q

warm reacting Ab are what

A

IgG

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20
Q

cold reacting Ab are what

A

IgM

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21
Q

Tx immune hemolytic anemia

A

corticosteroids

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22
Q

Coombs test is + when

A

drug induced hemolytic anemia and immune

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23
Q

finding in hereditary spherocytosis

A

HSM

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24
Q

Tx hereditary spherocytosis

A

splenectomy

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25
Q

what induced G6PD hemolytic anemia

A

ingestion fava beans high dose ASA, sulfa drugs, dapsone, quinine, quinidine, primaquine, nitrofurantoin

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26
Q

bite cells and heinz bodies

A

G6PD def

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27
Q

labs in Fe def anemia

A 
decreased Hb and Hct
decreased MCV
decreased or normal reticulocytes
decreased ferritin
decreased Fe
increased transferrin- TIBC
low Fe:TIBC ratio
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28
Q

labs in anemia of chronic disease

A

decreased Fe
normal or increased ferritin
decreased TIBC(transferrin)
hypochromic microcytic or normocytic

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29
Q

labs in lead poisoning anemia

A

inc Fe or normal
inc or normal ferritin
normal or decreased TIBC
stippled microcytic RBC

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30
Q

labs in sideroblastic anemia

A

inc Fe
inc ferritin
dec TIBC (transferrin)
ringed sideroblasts in bone marrow

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31
Q

labs in thalassemias

A 
inc or normal Fe
inc or normal ferritin
normal TIBC
increased Fe:TIBC ratio
target cells in alpha
basophilic stippling in beta
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32
Q

signs of lead poisoning

A

mental developmental delayse, gingival lead lines, peripheral neuropathy (motor)
pallor weakness, abdominal pain

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33
Q

basophilic stippling

A

lead poisoning

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34
Q

Tx for lead poisoning

A

EDTA or simercaptosuccinic acid DMSA for chelation

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35
Q

severe lead toxication Tx

A

dimercaprol

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36
Q

most common cause megaloblastic anema

A

folate def

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37
Q

what drugs inhibit folate metabolism

A

MTX, trimethoprim and phenytoin

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38
Q

signs of folate def

A

diarrhea, sore tongue, palor, tachy, tachypnea, inc pulse P, not neuro Sx

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39
Q

labs in folate def

A

dec Hb and HCt
increased MCV
dec folate
decreased reticulocytes

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40
Q

hypersegmented neutrophils

A

folate def and B12 def

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41
Q

populations with inadequate folate

A

alcoholics and elderly

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42
Q

pernicious anemia

A

autoimmune anemia from no IF so cannot absorb B12

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43
Q

infection with pernicious anemia

A

diphyllobothrium latum

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44
Q

Sx B12 def

A

DOE, memory loss, tachy, inc PP, symmetric paresthesias, loss vibration, ataxia, dementia

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45
Q

labs in B12 def

A

macrocytic
dec Hb Hct
increased MCV
dec B12

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46
Q

which diet causes B12 def

A

strict vegetarian

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47
Q

which megaloblastic anemia deficiency occurs first

A

folate because have lots of B12 stores in body

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48
Q

associated findings in anemia of chronic disease

A

iron trapping in macrophages
decreased EPO
increased hepcidin (inhibits iron absorption)

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49
Q

labs in anemia chronic disease

A 
mild dec Hb and Hct
normal or dec MCV
dec Fe
dec transferrin!!! TIBC
normal or increased ferritin
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50
Q

aplastic anemia in sickle cell patient

A

parvoB19

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51
Q

what is aplastic anemia

A

pancytopenia in bone marrow failure

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52
Q

what drugs cause aplastic anemia

A

chloramphenicol, sulfonamides, phenytoin, chemo Rx

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53
Q

Signs Sx aplastic anemia

A

fatigue weakness, persistent infections, poor clotting, easy bruising, pallor. petechiae, tachy, tachypnea, systolic murmur. inc PP

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54
Q

labs in aplastic anemia

A

dec Hb HCt dec WBC dec platelets

BM shows hypocellularity and fatty infiltrate!!!

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55
Q

Tx for long term survival aplastic anemia

A

BM transplant

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56
Q

what causes sideroblastic anemia

A

defective heme synthesis

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57
Q

iron panel in sideroblastic anemia

A

dec Hb Hct
increased ferritin
increased Fe
decreased TIBC(transferrin)

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58
Q

blood smear in sideroblastic anemia

A

multiple sizes of RBCs

ringed sideroblasts– RBC surrounded by iron granules that stain blue

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59
Q

Tx sideroblastic anemia

A

B6 in hereditary
give EPO in acquired cases

phlebotomy for iron overload
chelation with deferoxamine

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60
Q

sideroblastic anemia puts one at risk for

A

Acute leukemia

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61
Q

what causes thalassemias

A

Hb defects in alpha or beta globin

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62
Q

alpha thalassemia common in what ethnicities

A

asian or african

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63
Q

alpha thalassemia minima

A

1 abnormal alpha allele

asymptomatic

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64
Q

alpha thalassemia minor

A

reduced alpha globin
mild anemia
microcytic anemia and target cells

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65
Q

Hemoglobin H disease

A

3 abnormal alpha alleles

chronic hemolytic anmia, splenomegaly, microcytic RBcs, HbH in blood, decreased lifespan

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66
Q

What causes hydrops fetalis

A 
4 abnormal alpha chains
Hb Barts (no alpha)
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67
Q

beta thalassemia minor

A

1 abnormal beta allele

mild anemia, increased Hb A2

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68
Q

beta thalassemia major

A 
2 abnormal beta alleles
no beta globin production
asymptomatic until decline fetal Ab
growth retardation, develoipmental delays, bony abnormalities, HSM, anemia, increase A2 and F
microcytic anemia
die without tranfusions
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69
Q

what ethnicity has beta thalassemias

A

mediterranean

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70
Q

labs in thalassemias

A

decreased MCV
increased reticulotyes
in Hb Bart (cannot release O2)
increased HbA2 or F in beta thalssemia

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71
Q

Dx thalassemia

A

Hb electrophoresis

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72
Q

which thalassemia has RBCs of variable size and shapes with target cells

A

beta

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73
Q

find micorcytic anemia on blood smear

next step

A

rule out thalassemia before giving Fe because can cause overload

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74
Q

complications thalassemias

A

chronic iron overload from transfusions

damages heart and liver

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75
Q

sickle cell is defect in what

A

beta chain of Hb causing HbS- poorly soluble when deoxygenated
defective chain

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76
Q

what causes sickle shape

A

acidosis, hypoxia and dehydration

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77
Q

labs in sickle cell

A 
dec Hct
increased reticulocyte
increased PMN
decreased haptoglobin
increased indirect bili
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78
Q

Hb in sickle cell

A

HbS, no HbA

may have Hb F

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79
Q

imaging findings sickle cell

A

codfish vertebrae

lung infltrates in acute chest syndrome

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80
Q

blood smear sickle cell

A

target cells, nucleated RBCs, deoxygenation of blood produces sickle cells

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81
Q

when do HbF levels decrease

A

after 6 mo old

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82
Q

Tx sickle cell

A

hydration
supp O2
analgesics during crisis
hydroxyurea

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83
Q

how does hydroxyurea help in sickle cell

A

increases HbF

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84
Q

what vaccine do you give for sickle cell patients

A

pneumococcal and meningococcal

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85
Q

sickle cell patients are susceptible to what

A 
salmonella osteomyelitis
strep pneumo
H flu
N meningitis
Klebsiella
(encapsulated)
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86
Q

what is acute chest syndrome

A

acute pneumonia, pulm infarction and embolus

in sickle cell

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87
Q

what organs are most susceptible to ischemia in sickle cell patients

A

kidney heart and retina

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88
Q

what can cause iatrogenic lymphopenia

A

after chemo
radiation
diseases with increased cortisol

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89
Q

what diseases have eosinophilia

A 
addison
neoplasm
asthma
allergic drug reactions
collagen vascular diseases
transplant rejections
parasitic infections
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90
Q

what drugs cause neutropenia

A

clozapine, antithyroid medicaitons, sulfasalazine, methimazole, TMP-SMX, chemo and aplastic anemia

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91
Q

what can help with neutropenia

A

granulocyte colony stimulating factor, corticosteroids

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92
Q

Type I HS

A

anaphylactic IgE

release of mast cell contents

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93
Q

Tx type I HS

A

antihistamines, leukotriene inhibitors, bronchodilators, corticosteroids

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94
Q

Type II HS

A

IgM and IgG reacting with complement cascade

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95
Q

examples of type II HS

A

drug induced or immune hemolytic anemia

hemolytic disease of the newborn

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96
Q

Tx type II HS

A

anti inflammatories or immunosupressive agents

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97
Q

type III HS

A

IgM and IgG IC that deposit into tissue and start C’ cascade

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98
Q

types of HS III

A

arthus reaction
serum sickness
glomerulonephritis

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99
Q

Tx type III HS

A

anti inflammatories

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100
Q

type IV HS

A

T cells and macropahges which secrete lymphokines that induce macrophages to destroy tissue

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101
Q

types of HS IV

A

transplant rejection
allergic contact (poison ivy)
dermatitis
PPD testing

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102
Q

Tx type IV HS

A

corticosteroids or immunosuppressants

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103
Q

Tx for anaphylaxis

A 
subcut epinephrine
intubation
antihistamines
bronchodilators
recumbent positioning
IV hydration
vasopressors for hypotension
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104
Q

what calculates platelet function

A

bleeding time

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105
Q

what induces the vasoconstriction and platelet plug at sites of vascular injury

A

ADP

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106
Q

coagulation cascade makes what

A

fibrin clot

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107
Q

PTT reflects what

A

intrinsic pathway

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108
Q

PT reflects what

A

extrinsic pathyway

induced by tissue factor

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109
Q

if have increased PT or INR then suspect what

A

decrease function Factors II VII IX and X

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110
Q

heparins effect

A

enhances antithrombin III

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111
Q

problem if start warfarin before LMWH

A

short period hypercoagulability from inhibition proteins C and S

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112
Q

what is considered thrombocyopenia

A
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113
Q

labs for thrombocytopenia

A

increased BT

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114
Q

which clotting factors do not decreased with liver failure

A

vWF and factor VIII

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115
Q

inheritance vWD

A

auto dominant

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116
Q

labs in vWD

A 
increased PTT
increased BT
decreased factor VIII
decreased vWF
decreased ristocetin cofactor activity
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117
Q

Tx for vWD

A

desmopressin for minor bleeding

vWF concentrate and factor VIII concentrate before surgery

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118
Q

how does ASA work

A

inhibits cyclooxygenase and supress thromboxane A2

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119
Q

what are thiopyridines and how do they work

A

clopidogrel and ticlopidine

block ADP R to suppress fibrinogen binding to injury and platelet adhesion

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120
Q

What are the GIIb/IIIa inhibitors

A

abciximab
tirofiban
epitifibatide

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121
Q

how do GIIb/IIIa inhibitors work

A

inhibit platelet aggregatino

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Perfectly
122
Q

What are the adenosine reuptake inhibitors and how do they work

A

dipyridamole

inhibit activity adenosine deaminase and phosphodiesterase to inhibit aggregation

123
Q

how does heparin work

A

binds antithrombin to increase activity and prevent clot formation

124
Q

adverse effects heparin

A

hemorrhage
HS
thrombocytopenia
narrow therapeutic window

125
Q

how does LMWH work

A

binds to factor Xa to prevent clot formation

126
Q

What are the direct thrombin inhibitors

A

lepirudin

argatroban

127
Q

direct thrombin inhibitors suppress acitvity of what factors

A

V IX and XIII and aggregation

128
Q

what are the direct Xa inhibitors

A

apixaban

rivaroxaban

129
Q

how do the direct Xa inhibitors work

A

no activity against thrombin

highly selective inhibition factor Xa which is in the common pathway

130
Q

how does warfarin work

A

antagonize K dependent carboxylation of II VII IX and X

131
Q

labs in Vit K def

A

increased PT and increased INR

132
Q

Tx vit K def

A

oral or IM vit K and FFP

133
Q

inheritance hemophilia

A

X linked recessive

134
Q

hemophilia A

A

factor VIII

135
Q

hemophilia B

A

factor IX

136
Q

labs in hemophilia

A

increased PTT
normal PT
normal BT
decareased VIII or IX

137
Q

Tx for hemophilias

A

replacement of factors
desmopressin can help in hemophilia A
transufsions needed

138
Q

thrombocytopenia with splenomegaly and normal Bone marrow

A

splenic platlet sequestration

139
Q

Dx HIT

A

diffuse thrombus formation
sudden decrease platlets
+ serotonin release assay
+ Heparin induced platelet aggregation assay

140
Q

What is Immune thrombocytopenia ITP

A

autoimmune B cell directed production of antiplatelet Ab

141
Q

platelet count in ITP

A
142
Q

Tx for ITP

A

adults need corticosteroids
delayed splenectomy
IV Ig, plasmapheresis or recombinant VIIa

143
Q

What causes TTP-HUS

A

diffuse platelet aggregation due to AutoAb against preventative enzyme
assoc with endothelial injury and Ecoli infection

144
Q

Dx TTP-HUS

A

hemolytic anemia, acute renal failure, thrombocytopenia without severe bleeding
sometimes fever

145
Q

Tx TTP-HUS

A

corticosteroids, plasmapheresis, FFP

146
Q

Tx antiphospholipid syndrome

A

anticoagulation with heparin warfarin and hydroxychloroquine

147
Q

Dx HELLP

A 
during pregnancy
HTN
increased LFTs
decreased Hb
schistocytes
148
Q

labs in DIC

A

dec platelets
increased PT and PTT
decreased fibrinogen

149
Q

blood smear DIC

A

schistocytes with few platelets

150
Q

Tx DIC

A 
underlying disorder
platelets
FFP
cryoprecipitate
heparin
151
Q

Dx for Systemic inflammatory response synrome

A

2:

  • temp >38.3 or 90
  • RR >20 or PaCO212000 or 10% bands
152
Q

community acquired sepsis pathogens

A 
strep
taph
E coli
Klebsiella
Pseudomonas
N meningitis
153
Q

Nosocomial pathogens causing sepsis

A

Staph, gram neg bacilli anaerobes, pseudomonas and candida

154
Q

sepsis in IV drug user

A

staph

155
Q

what mosquito caries malaria

A

anopheles

156
Q

Sx malaria

A

chills, diaphoresis, HA, myalgias, fatigue, nausea, abdominal pain, vomiting, diarrhea, periodic fever, splenomegaly

157
Q

Dx malaria

A

PCR

158
Q

stain used in blood smear malaria

A

giemsa

159
Q

Tx malaria

A

chloroquine, primaquine, quinine

atavoquone and megloquine used in the R cases of P falciparum

160
Q

labs in mono

A 
\+ heterophile (monospot)
\+ EBV serology
elevated LFTs
increased WBCs
hemolytic anemia
thrombocytopenia
161
Q

blood smear in mono

A

increased lymphocytes

162
Q

Tx mono

A

supportive

163
Q

complications mono

A

splenic rupture
rare aplastic anemia
DIC, tTP
fulminant liver failure

164
Q

what type of virus is HIV 1 and 2

A

RNA retro

165
Q

risk factors HIV in US

A

homosexual, bisexual
IV drug user
blood transfusions before 1980s
high risk sexual behavior

166
Q

acute Sx hIV

A

flu like with myalgias nausea and vomiting, diarrhea
sore throat weight loss
mucosal ulcers fever lymphadenopathy

167
Q

late Sx HIV

A

opportunistic infections and AIDS illnesses

weight loss night sweats and dementia

168
Q

needle stick with HIV + patient next step

A

prophylactic tenofovir and emtricitabine and raltegravir

169
Q

Testing post needle stick for HIV

A

immediately, 6 weeks, 3 mo and 6 mo

170
Q

opportunistic infection CD4

A

herpes zoster or simplex

parasitic diarrhea from isospora, strongyloides and crytptosporidium

171
Q

Tx parasitic diarrhea in HIV patientq

A

antiretroviral and metronidazole

TMP SMX and paromomycin

172
Q

opportunistic infection CD4

A

kaposi sarcoma

coccidiomycosis

173
Q

Tx kaposi sarcoma

A

topical alitretinoin, chemo, laser therapy and radiation

174
Q

Tx coccidiomycosis

A

fluconazole, itraconazole, amphotericin B

175
Q

opportunistic infections CD4

A 
AIDS dementia
bacterial pneumonia
candida esophagitis
cervical CA
PCP pneumonia
TB
176
Q

Dx of AIDs dementia

A

declining mental status
generalized neuro Sx
elevated B2 microglobulin in CSF
cerebral atrophy on CT or MRI

177
Q

dysphagia and odynophagia in HIV patient

A

candida esophagitis

178
Q

Tx candida esophagitis

A

cephalosporins, beta lactams or macrolides

179
Q

Dx PCP

A

bilateral infiltrates on CXR
icreased LDH
sputum gram stain

180
Q

Tx PCP

A

TMP SMX

corticosteroids

181
Q

Dx TB in HIV

A

acid fast bacilli
cavitary defects
hilar adenopathy
+ PPD

182
Q

Tx TB

A

RIPE

183
Q

opportunistic infections CD4

A

histoplasmosis

184
Q

Tx histoplasmosis

A

amphotericin B or itraconazole

185
Q

opportunistic infections CD4

A

cerebral toxo
lymphoma
Progressive multifocal leukoencephalopathy
wasting syndrome

186
Q

Dx toxo in HIV

A

+ toxo IgG Ab

ring enhacing lesion on CT or MRI

187
Q

Tx cerebral toxo

A

pyrimethamine
sulfadiazine
clindamycin

188
Q

Tx lymphoma (CNS or non hodgkin)

A

chemo radiation

189
Q

Dx lymphoma in HIV

A

CT or MRI shows lesion

Bx confirms

190
Q

focal neurologic Sx in HIV

A

cerebral toxo or lymphoma

191
Q

signs of progressive multifocal leukoencephalopathy

A

ataxia, motor deficits, mental status changes

192
Q

Dx PML

A

positive PCR for JC virus

193
Q

opportunistic infections CD4

A

cryptococcul meningitis
CMV
myocobacterium

194
Q

Dx cryptococcal meningitis

A

elevated P on LP
yeast on India ink stain of CSF
+ cryptococcal Ag in CSF or serum

195
Q

Tx cryptococcal meningitis

A

amphotericin B, fluconazole

196
Q

Sx CMV in HIV

A

vision loss and esophagitis and diarrhea

197
Q

Dx CMV

A

viral titer, yellow infiltrates with hemorrhage on fundoscopic exam

198
Q

Tx CMV in HIV

A

ganciclovir, foscarnet, valganciclovir

199
Q

signs MAC

A

fatigue, weight loss, fever, diarrhea, abdominal pain, lymphadenopathy, HSM

200
Q

Dx MAC

A

blood cultures

201
Q

Tx MAC

A

clarithromycin, azithromycin, ethambutol, rifabutin, rifampin

202
Q

labs for HIV

A

ELISA if + repeat

then western blot because high specificity

203
Q

viral load indicates what in HIV

A

rate of degree progression

useful in detection of acute infection

204
Q

HAART

A

2 NRTI
+ Protease inhibitor or NNRTI
low dose ritonavir can be added to increase Protease inhibitor

205
Q

Antibiotic prophylaxis in HIV

A

TMP SMX when CD4

206
Q

what are the NRTIS

A 
abacavir
emtricitabine
lamivudine
zidovudine
tenofovir
207
Q

What are the NNRTIs

A

efavirenz
etravirine
rilpivirine

208
Q

what are the protease inhibitors

A

navirs

209
Q

what are the integrase inhibitors

A

gravirs

210
Q

what is the fusino inhibitor in HIV meds

A

enfuvirtide

211
Q

what is the CCR5 antagonist in HIV Rx

A

maraviroc

212
Q

how do NRTIs work

A

inhibit producitno viral genome and preven incorporation of viral dNA into host genome

213
Q

adverse effects NRTIs

A

lactic acidosis
lipodystrophy
pancreatitis
HS ractions

214
Q

adverse zidovudine

A

bone marrow toxicity

215
Q

how do NNRTIs work

A

inhibit reverse transcriptase activity to prevent replication

216
Q

adverse effects NNRTIs

A

rash

217
Q

adverse efavirenz

A

neropsychiatric effects and is teratogenic

218
Q

how do protease inhibitors work

A

interfere with viral replication to cause nonfunctional viruses

219
Q

adverse effects protesase inhibitors

A

hyperglycemia
hyper TG
GI toxicity

220
Q

side effect of atazanavir

A

hyperbili

221
Q

how do integrase inhibitors work

A

inhibit final step of integration viral DNA into host

222
Q

adverse effects integrase inhibitors

A

neutropenia, pancreatitis, hepatotoxicity

hyperglycemia

223
Q

how do fusion inhibitors work

A

bind to gp41 inhibiting viral ability to fuse to CD4 membrane and enter cell

224
Q

how do CCR5 antagonist work

A

inhibit CCR5 coreceptor on virus blocking entry to host cell

225
Q

Tx pregnant HIV + woman

A

Tx to keep viral load low during pregnancy
zidovudine during labor
and again after birth at 6 weeks

226
Q

can HIV + breast feed

A

no

227
Q

65 year old with burning in hands and feet and intense itching after showering
large retinal veins and splenomegaly

A

polycythemia vera

228
Q

labs in PCV

A

inc Hb Hct inc RBC mass inc WBC normal platelets
dec EPO
hyperceullular marrow

229
Q

Tx PCV

A

serial phlebotomy
antihistamines
ASA
hydroxyura (BM suppression)

230
Q

complications PCV

A

thombus formation, leukemia (acute or chonic myelogenous) stroke

231
Q

msot common cause of inc RBC production

A

chronic hypoxia

232
Q

MM

A

proliferation plasma cells

233
Q

monoclonal gammopathy of undetermined significance puts one at risk for

A

MM

234
Q

what is produced in MM

A

abnormal M protein from IgG and IgA heavy chaings and kappa gamma light chains (bence jones proteins)

235
Q

Signs Sx MM

A

back pain, radicular pain, weakness, fatigue, weight loss, constipation, fractures, frequent infections, pallor and bone tenderness

236
Q

labs in MM

A 
dec Hb HCt WBC
inc  BUn and Cr
Inc Ca
high M protein and bence jones proteins
increased plasma cells on BM Bx
237
Q

imaging MM

A

punched out lesions on long bones and skull

238
Q

Tx MM

A

radiation, cehmo, bone marrow transplant

repair fractures Tx infections

239
Q

complications MM

A

renal failure, recurrent infections, hyperca, spinal cord compression
poor prognosis 2-3 year survival

240
Q

most common CA in children

A

ALL

241
Q

ALL

A

children 2-5 years old
whites>blacks
proliferation of lymphoid line- B cell precursors

242
Q

labs in ALL

A

dec Hb Hct decreased platelets WBCs

increased uric acid, increased LDH

243
Q

bone marrow Bx in ALL

A

abundant blasts

9:22 BCR-ABL phildelphia chromosome

244
Q

signs Sx ALL

A

bone pain, infections, fatigue, DOE, easy bruising, fever, pallor, purpura, HSM and lymphadenopathy

245
Q

Tx ALL

A

chemo and BM transplant

246
Q

complications ALL

A

adulta have bad prognosis. kids have better

presence of philadelphia chromosome= worse prognosis

247
Q

AML

A

both children and adults

248
Q

Signs Sx AML

A

fatigue, easy bruising, DOE, frequent infections, arthralgias, fever, pallor, HSM, muscosal bleeding, ocular hemorrhages

249
Q

labs in AML

A

dec Hb Hct

dec platelets WBCs

250
Q

bone marrow Bx in AML

A

blasts of myeloid origin and stain with myeloperoxidase

251
Q

blood smear in AML

A

myeloblasts with notched nuclei and AUER rods

252
Q

Tx AML

A

chemo and BM transplant

253
Q

complications AML

A

relapse common
DIC
long term survivial poor

254
Q

CLL

A

proliferation mature B cells

elderly patients >65

255
Q

Signs Sx CLL

A

fatigue, infecitons, night sweats, fever, lymphadenopathy, HSM

256
Q

labs in CLL

A

increased WBC >100,000

257
Q

blood smear CLL

A

small lymphocytes with smudge cells

258
Q

Tx CLL

A

supportive chemo tadiation for bulky masses

splenectomy for splenomegaly

259
Q

complications CLL

A

malignant B cells may for auto Ab leading to severe hemolytic anemia.

260
Q

CML

A

mature meyloid cell proliferation in middle aged adults

radiation exposure

261
Q

what occurs with CML after stable for a couple eyars

A

blast crisis- usually fatal

262
Q

signs Sx CML

A

fatigue, weight loss, night sweats, splenomegaly,

263
Q

labs in CML

A

WBC >100,000 with high neutrophils

decreased leukocyte alkaline phosphatase

264
Q

BM Bx in CML

A

granulocyte hyperplasia with cytogenic analysis showing philadelphia chromosome

265
Q

Tx for CML

A

chemo with imatinib

BM transplant in younger patients

266
Q

complications CML

A

blast crisis

267
Q

philedelphia chromosome

A

almost always CML

5% ALL

268
Q

Hair cell leukemia

A

proliferation B cells in middle aged men

269
Q

Signs Sx hairy cell leukemia

A

fatigue, infections, abdominal fullness, massive splenomegaly and no lymphadenopathy
NO fever or night sweats

270
Q

labs in hairy cell leukemia

A

dec Hb Hct platelets and WBCs

BM show lymphocytes

271
Q

Tx hairy cell leukemia

A

chemo

272
Q

blood smear hairy cell leukemia

A

lymphocytes with hairy projections of cytoplasm

273
Q

purpose of radiation therapy

A

necrose tumor cells and decrease tumor size

274
Q

what are some radiation induced malignancies

A

thyroid
CML
sarcomas

275
Q

adverse of chemoTx in general

A

bone marrow suppression, alopecia, GI upset, infertility, neurotoxicity, hepatotoxicity, skin changes, pulm fibrosis, cardiomyopathy, renal toxicity

276
Q

which drugs cause free radical damage and cytotoxic alkylization of DNA and RNA

A 
clyclophosphamide, chlorambucil, ifosfamide, mechlorethamine
carmustine and streptozocin
busulfan
thitepa andhexamtheylmelamine
dacarbazine
277
Q

which drugs inhibit spindle proteins and stop mitosis

A

vinca alkaloids like cinblastine and vincristine and etoposide

278
Q

which drugs cause cytotoxic polymerization

A

taxanes like paclitaxel and docetaxel

279
Q

which chemo drugs interfere with enzyme regulation or dNA regulation

A

cytarabine, 5-fluoreuracil, MTX, mercaptopurine

carboplatin and cisplatin

280
Q

risk factors for hemolytic disease of newborn

A

abortion, amniocentesis, third trimester bleeding

281
Q

Tx for hemolytic disease of newborn

A

RhoD Ig within 72 hours of Rh+ fetus or anytime blood may have mixed

282
Q

fanconi anemia

A

autosomal recessive bone marrow failure

283
Q

signs of fanconi anemia

A

fatigue, DOE, infections
short stature, abnormal skin pigments(cafe au lait and hypopigmented areas)
horseshoe kidney and thumb abnormalities

284
Q

labs in fanconi anemia

A

dec Hb Hct platelets and WBC
increased AFP
BM show hypocellularity

285
Q

Tx fanconi anemia

A

Antibiotics, tranfusions, stem cell transplants, growth factors, androgens, corticosteroids

286
Q

complications fanconi anemia

A

death as child or leukemia

287
Q

diamond blackfan anemia

A

congenital pure red anemia from defect in erythroid progenitor cells

288
Q

Signs Sx diamond blackfan

A

faitue dyspnea, cyanosis, pallor

craniofacial abnormalties, thumb abnormalities, heart murmurs, mental retardation and hypogonadism

289
Q

labs in diamond blackfan

A

dec Hb Hct reticulocyte count
inc MCV
decreased activity in BM but with increased EPO

290
Q

Tx for diamond blackfan anemia

A

transfusions, corticosteroids, BM transplant

291
Q

Neuroblastoma

A

neural crest cell tumor in adrenal glands or sympathetic ganglia

292
Q

risk factors Neuroblastoma

A

NF

tuberous sclerosis, pheo, beckwith wiedemann syndrome, turners, low maternal folate

293
Q

signs Sx neuroblastoma

A

abdominal distention with pain, weight loss, malaise, bone pain,, diarrhea, abdominal maa, HTN, horners, proptosis, movement disorders, hepatomegaly, fever, periorbital bruising!

294
Q

labs in neuroblastoma

A

inc VMA and homovanillic acid in 24 hour urine collection

295
Q

radiology findings in neuroblastoma

A

CT locate adrenal or ganaglion tumor

296
Q

Tx neuroblastoma

A

surgical resection, chemo radiation

297
Q

complications neuroblastoma

A

poor prognosis if present afeter 1 year of life

298
Q

mets of neuroblastoma

A

bone and brain

299
Q

rhabdomyosarcoma

A

tumor striated mm in children

300
Q

signs Sx rhabdomyosarcoma

A

painful soft tissue mass with swelling

301
Q

Dx rhabdomyosarcom

A

Bx and CT shows extent

302
Q

Tx rhabdomyosarcoma

A

surgical debulking, radiatoin, chemo

303
Q

most common soft tissue sarcoma in children

A

rhabdomyosarcoma

Step2CK (26 decks)

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