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First Aid Chapters > FA: Heme Onc > Flashcards

FA: Heme Onc Flashcards

1
Q

how long do RBCs live?

A

120 days

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2
Q

what do RBCs use for energy

A

glucose. 90% glycolysis and 10% from HMP shunt

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3
Q

important membrane antiporter in RBCs

A

HCO3- antiporter which allows RBCs to export HCO3- and transport Co2 from the periphery to the lungs for elimination

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4
Q

erythrocytosis

A

too many red blood cells

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5
Q

anisocytosis

A

variation in size of RBCs

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6
Q

Poikilocytosis

A

varying shapes of RBCs

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7
Q

reticulocyte

A

immature RBC, marker of erythroid proliferation

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8
Q

lifespan of platelet

A

8-10 days

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9
Q

job of platelets

A

primary hemostasis

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10
Q

dense granules in platelets contain

A

ADP and calclium

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11
Q

alpha granules in platelets contain

A

Von Willibrand Factor and fibrinogen

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12
Q

thrombocytopenia

A

low platelet level. this or decreased platelet function results in petechiae

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13
Q

which receptor on platelet attaches to VWF

A

GpIb

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14
Q

which receptor on the platelet attaches to fibrinogen

A

GP2b/3a

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15
Q

what are three granulocytes

A

neutrophils, eosinophils and basophils

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16
Q

normal WBC

A

4,000-10,000

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17
Q

pneumonic for WBC differential

A 
Neutrophils Love Making Everyone Better
Neutrophils
Lymphocytes
Monocytes
Eosinophils
Basophils
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18
Q

normal percentage of Neutrophils

A

54-62%

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19
Q

normal percentage of Lymphocytes

A

25-33

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20
Q

normal percentage of monocytes

A

3-7

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21
Q

normal percentage of eosinophils

A

1-3

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22
Q

normal percentage of basophils

A

0-1

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23
Q

cell with a multi lobed nucleus

A

this is a neutrophil

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24
Q

specific granules of neutrophils

A

contain ALP, collagenase, lysozyme, and lactoferrin

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25
Q

azurophilic granules of neutrophils

A

contain proteinases, acid phosphatase, myeloperoxidase, and beta glucoronidase

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26
Q

hypersegmented polys

A

this is a neutrophil with more than 5 lobes. seen in vitamin B12 and folate deficiency

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27
Q

band cell

A

immature neutrophil

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28
Q

when do you see increased band cells

A

infections (bacterial) and CML

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29
Q

Monocyte appearance on histology

A

kidney shaped nucleus. blue. frosted glass cytoplasm

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30
Q

function of a monocyte

A

differentiates into macrophages in the tissues

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31
Q

what activates macrophages

A

gamma interferon

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32
Q

macrophages are antigen presenting cells with MHC type…

A

MHC type II

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33
Q

what is the cell surface marker for macrophages

A

CD14

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34
Q

function of eosinohpil

A

anti helminthic infections.. highly phagocytic for antigen-antibody complexes

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35
Q

how do eosinophils fight off helminths

A

major basic protein.

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36
Q

how to recognize eos

A

bi-lobed nucleus

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37
Q

two things produced by eosinophils

A

histaminase and arylsulfatase. limit reaction during mast cell degranulation.

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38
Q

function of basophils

A

allergic reactions.

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39
Q

contents of basophil granules

A

basophilic granules with heparin, histamine and leukotrienes.

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40
Q

if you see isolated basophilia what should you worry about?

A

CML

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41
Q

mast cell function

A

mediates allergic reactions. can bind to the Fc portion of IgE to membrane and cause IgE cross linking.

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42
Q

what does degranulation of mast cells release

A

histamine, heparin, and eosinophil chemotactactic factors

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43
Q

what prevents mast cell degranulation

A

cromolyn sodium

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44
Q

function of denritic cell

A

antigen presenting cell, high phagocytic

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45
Q

what type of MHC does dendritic cell have

A

MHC type II and Fc receptor on surface.

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46
Q

what do we call dendritic cells in the skin

A

Langerhans cells

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47
Q

Three types of lymphocytes

A

B cells, T cells and NK cells

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48
Q

what type of immunity are NK cells part of

A

innate immunity

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49
Q

where do B cells get made

A

stem cells in bone marrow

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50
Q

where do B cells mature

A

bone marrow

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51
Q

which part of lymph node can B cells be found in?

A

follicle and white pulp of spleen

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52
Q

cell of humoral immunity

A

B cell

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53
Q

cell of cellular immunity

A

T cell

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54
Q

where do T cells mature

A

thymus gland

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55
Q

CD8 T cells

A

cytotoxic T cells- express CD8 and recognizing MHC 1

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56
Q

CD4 T cells

A

helper T cells, express CD4 and recognize MHC2

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57
Q

CD28 marker

A

this is for regulatory T cells which is a costimulatory signal necessary for T cell activation

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58
Q

what cell is effected in multiple myeloma

A

plasma cell disease

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59
Q

do anti A and anti B IgM antibodies cross the placenta

A

NO

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60
Q

do anti Rho IgG cross the placenta

A

Yes

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61
Q

universal recipient of RBCs

A

AB

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62
Q

universal donor of RBCs

A

O

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63
Q

universal donor of plasma

A

AB (these people’s plasma have no antibodies in it)

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64
Q

universal recipient of plasma

A

O (because they have A and B antibodies already in their plasma)

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65
Q

hemophilia A has a deficiency in

A

factor 8

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66
Q

hemophilia B has a deficiency in

A

factor 9

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67
Q

factor 2

A

prothrombin

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68
Q

factor 2a

A

thrombin

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69
Q

what does kalikrein activate

A

bradykinin

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70
Q

what is the function of bradykinin

A

increase vasodilation, permeability and pain.

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71
Q

famous enzyme that inactivates bradykinin

A

ACE. hence why ACE inhibitors cause cough

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72
Q

what enzyme does warfarin inhibit

A

epoxide reductase which reduces vitamin K so it can be used as an activator of the factors 2,7,9,10

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73
Q

what activates protein C

A

thrombin thrombomodulin complex in the endothelial cells

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74
Q

what activates plasminogen

A

tpa

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75
Q

what is the function of protein C and protein S

A

cleave and inactive factor 5 and 8

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76
Q

function of antithrombin III

A

inhibits activated forms of factor 2, 7,9,10, 11, 12

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77
Q

how does heparin work

A

it increases the activity of antithrombin III

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78
Q

what are the major targets of antithrombin

A

factor 2 (thrombin) and factor 10a

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79
Q

what does plasmin do

A

responsible for fibriolysis. cleaves the fibrin mesh and destorys the coagulation factors. it is activated by tpa

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80
Q

factor V leiden mutation

A

this produces a factor 5 that is not sensitive to the work of protein C

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81
Q

thing that helps with platelet adhesion

A

VMF

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82
Q

thing that helps with platelet aggregation

A

ADP which induces Gp2b/3a and TXA2 which attracts more platelets which causes fibrinogen to be able to attach and cause aggregation

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83
Q

how does aspirin prevent the clot from forming

A

prevents TXA2 production so you do not attract the platelets to the area for aggregation

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84
Q

Ticlopidine and Clopidogrel mechanism

A

inhibit ADP induced G2b/3a expression so you can’t form the clot. basically blocks the ADP receptor so it can’t be activated

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85
Q

abciximab mechanism

A

inhibits g2b/3a directly

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86
Q

ristocetin mechanism

A

activates vMF to bind Gp1b. used for diagnostic test- if there is vWF disease, normal platelet aggregation is not seen.

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87
Q

Bernard Soulier syndrome

A

deficiency in Gp1b

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88
Q

Glanzmann thrombasthenia

A

deficiency in Gp2b/3a

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89
Q

what is the SED rate

A

increase in fibrinogen which is an acute phase reactant so the RBCs aggregate more causing them to settle more because RBC aggregates have a higher density than plasma.

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90
Q

low SED rate?

A

polycythemia, sickle cell anemia, CHP, microcytosis, hypofibrinogenemia

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91
Q

acanthocyte (spur cell) of the RBC

A

liver disease, abetalipoproteinemia- cholesterol dysregulation

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92
Q

basophilic stippling of the RBC

A

microcytic anemias- anemia of chronic disease, alcohol, lead poisoning and thalassemias (not iron def)

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93
Q

bite cell

A

G6PD deficiency

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94
Q

Elliptocyte

A

RBC showing hereditary elliptocytosis

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95
Q

macro-ovalocyte

A

megaloblastic anemia and marrow failure

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96
Q

hypersegmented PMN

A

megaloblastic anemia

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97
Q

ringed sideroblast

A

sideroblastic anemia - defect in heme production

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98
Q

schistocyte or helmet cell

A

DIC, TTP, HUS, traumatic hemolysis like with mechanical valve prosthesis

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99
Q

sickle cell

A

sickle cell anemia

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100
Q

spherocyte

A

hereditary spherocytosis- spectrin mutation. also seen in autoimmune hemolysis

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101
Q

teardrop cell

A

bone marrow infiltration like myelofibrosis.

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102
Q

target cell

A

Hemoglobin C disease, asplenia, liver disease, thalassemia

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103
Q

Heinz Bodies

A

this is from oxidation of hemoglobin sulhydryl groups- denatured hemoglobin precipitates and phagocytic damage to RBC leads to bite cells. visualized with crystal violet. seen in G6PD

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104
Q

Heinz body like inclusions

A

these can be seen in alpha thalassemia from the precipitated hemoglobin

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105
Q

howell jolly bodies mechanism

A

basophilic nuclear remnants found in RBCs that would have normally been removed by the spleen. seen in patients with functional hyposplenia and asplenia

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106
Q

other causes of megaloblastic anemia aside from B12 and folate deficiency

A

orotic aciduria.

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107
Q

type of anemia with iron deficiency anemia

A

microcytosis and hypochromia

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108
Q

plummer vinson syndrome

A

triad of iron deficiency anemia, esophageal webs, and atrophic glossitis

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109
Q

cis alpha thal deletion population

A

asian population

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110
Q

trans alpha thal deletions

A

present in African populations

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111
Q

hemoglobin barts

A

gamma x4. incompatible with life

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112
Q

4 allele deletion in alpha thal

A

incompatible with life. hemoglobin barts. causes hydrops fetalis

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113
Q

3 allele deletion in apha thal

A

hemoglobin H. excess beta joins together and you get Beta 4 which is hemoglobin H

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114
Q

1-2 allele deletion in alpha thal

A

not clinically significant

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115
Q

alpha thal… what is the issue

A

this is a deletion in the alpha globin gene

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116
Q

beta thal.. what is the issue

A

this is a mutation in the splice sites which reduces beta thal synthesis.

117
Q

population where you see beta thal

A

Mediterranean populations

118
Q

Beta thal minor

A

heterozygote. beta chain is underproduced. usually asymptomatic. diagnosis is confirmed with increased HA2 on electorphoresis (>3.5%)

119
Q

beta thal major

A

beta chain is absent leading to severe anemia. requires blood transfusions which can give secondary hemochromatosis.

120
Q

classic deformity seen with beta thal major

A

extramedullary hematopoiesis leads to hepatosplenomgaly. increased risk of parvo virus aplastic crisis. marrow expansion shows crew cut on skull x ray and chipmunk face.

121
Q

how does lead poisoning cause anemia

A

inhibits ferochelatase and ALA dehydrogenase. this decreases heme synthesis and increases RBC protoporphyrin. also inhibits rRNA degradation causing RBCs to retain aggregates of rRNA (basophilic stipling).

122
Q

burton lines

A

these are lead lines on gingivae and also seen in metaphysis of bones.

123
Q

first line treatment of lead poisoning

A

dimercaprol and EDTA . can also use succimer for chelation for kids.

124
Q

sideroblastic anemia

A

defect in heme synthesis

125
Q

mutation for hereditary sideroblastic anemia

A

X linked defect- delta- ALA synthase gene mutation.

126
Q

reversible causes of sideroblastic anemia

A

ALCOHOL, lead, B6 deficiency, copper deficiency and isoniazid

127
Q

treatment for sideroblastic anemia

A

B6 (pyroxidine)

128
Q

labs in sideroblastic anemia

A

increased iron, normal TIBC, increased feritin.

129
Q

what does the term megaloblastic anemia mean

A

this means there is impaired DNA synthesis. maturation of the cells in the bone marrow is delayed relative to maturation of cytoplasm.

130
Q

what test to distinsguish between folate and B12 deficiency

A

MMA

131
Q

3 drugs that cause folate deficiency and megaloblastic anemia

A

methotrexate, trimethoprim, phenytoin.

132
Q

should there be any neurologic symptoms in folate deficiency?

A

no because these symptoms are from the B12 deficiency and its role in fatty acid pathways and myelin synthesis

133
Q

what bug likes to eat B12

A

diphyllobothrium latum which is a fish tapeworm

134
Q

hypersegmented neutrophils, glossitis and orotic acid in urine

A

orotic aciduria. inability to convert orotic acid to UMP for the denovo pyrimidine synthesis.

135
Q

defect in orotic aciduria

A

UMP synthase

136
Q

how to distinguish orotic aciduria from ornithine transcarbamylase deficiency

A

both have increased orotic acid in the urine but orotic aciduria does NOT have increased ammonia.

137
Q

how to treat orotic aciduria

A

uridine monophosphate to bypass mutated enzyme

138
Q

what is a non megaloblastic macrocytic anemia

A

this is an anemia in which DNA synthesis is not impaired. causes include liver disease, alcoholism, reticulocytosis, drugs (5FU, zidovudine, hydroxyurea)

139
Q

if you see decreased haptoglobin

A

think intravascular hemolysis with normocytic anemia

140
Q

causes of intravascular hemolysis with normocytic anemia

A

paroxsymal noctural hemoglobinuria, mechanical destruction, microangiopathic hemolytic anemia

141
Q

extravascular hemolysis findings

A

spherocytes, increased LDH, increased unconjugated bilirubin, jaundice

142
Q

cause of extravascular hemolysis

A

hereditary spherocytosis

143
Q

what type of hemolysis shows increased urobilinogen in urine?

A

intravascular because this is conjugated.

144
Q

fatty infiltration of the bone marrow

A

aplastic anemia

145
Q

causes of G6PD crisis

A

sulfa drugs, antimalarials, infections, fava beans

146
Q

lab findings of Heinz bodies and bite cells

A

G6PD deficiency

147
Q

hemolytic anemia in a newborn

A

pyruvate kinase deficiency. decrease in ATP leads to a firm or rigid RBC

148
Q

defect in HgC defect

A

glutamic acid to lysine mutation on residue 6 of Beta globin

149
Q

mutation in paroxysmal nocturnal hemoglobinuria

A

impaired synthesis of GPI anchor for decay accelerating factor that protects RBCs form complement destruction. intravascular hemolysis

150
Q

labs to diagnose paroxysmal nocturnal hemoglobinuria

A

CD55 and CD59 negative RBCs. coombs negative

151
Q

treatment for paroxysmal nocturnal hemoglobinuria

A

eculizumab

152
Q

mutation in sickle cell anemia

A

B chain mutation- has valine instead of glutamic acid

153
Q

what precipitates sickling

A

low O2, dehydration, acidosis.

154
Q

crew cut on Xray

A

you can see this here and with the thalassemias

155
Q

type of osteomyelitis seen in sickle cell

A

salmonella

156
Q

most common cause of death from sickle cell

A

acute chest syndrome

157
Q

treatemtn for sickel cell

A

hydroxyurea to increase the hemologbin F. bone marrow transplantation also

158
Q

coombs test is positive in what disease

A

autoimmune hemolytic anemia

159
Q

warm agglutinin

A

IgG chronic anemia seen in SLE, CLL, or with drugs like alpha methyldopa

160
Q

cold agglutinin

A

IgM acute anemia triggered by cold. seen in CLL, mycoplasma pneumonia, infections like mono

161
Q

direct coombs test

A

put anti Ig antibody into the patient’s serum and see if the red blood cells are coated with Immunoglobuin.

162
Q

indirect coombs test

A

take the patient’s serum and add normal RBCs. see if they clump together or agglutinate.

163
Q

microangiopathic hemolytic anemia mechanism

A

RBCs are damaged when passing through obstructed or narrowed vessel lumina. seen in DIC, HUS, TTP, SLE

164
Q

what is classic finding in microangiopathic hemolytic anemia

A

schistocytes or helpmet cells

165
Q

what diseases are associated with microangiopathic anemia?

A

prosthetic heart valves and aortic stenosis

166
Q

pregnancy and lab values for iron tests

A

increased TIBC and decreased saturation of iron. all else is the same.

167
Q

Neutropenia

A

absolute neutrophil count of <1500

168
Q

lymphopenia

A

absolute lymphocyte count <1500

169
Q

eosinopenia

A

cushing syndrome, corticosteroids.

170
Q

corticosteroids and white count

A

corticoisteriods cause neutrophilia, but eosinopenia, and lymphopena.

171
Q

how do corticosteroids increase neutrophil count

A

they do this by decreasing the activation of neutrophil adhesion molecules so they cannot migrate out of the vasculature and into the tissue.

172
Q

how do corticosteroids decrease lymphocytes and eosinophils

A

steroids sequester eosinophils in the lymph nodes and cause apoptosis of lymphocytes

173
Q

what is a porphyria

A

this is a hereditary or acquired defect in heme synthesis that leads to the accumulation of heme precursors.

174
Q

how does lead posioning affect heme

A

can’t produce heme. affects two enzymes- ferochelatase which adds the Fe to the Heme. it also causes a problem with the enzyme delta ALA deaminase

175
Q

what is the mutation in a sideroblastic anemia (X linked)

A

mutation in the first enzyme in heme production which is the rate limiting step- this is delta ALA synthase.

176
Q

what substrates accumulate in lead poisoning

A

protophorphyrin and delta ALA. it cannot turn the delta ALA into porphobilinogen.

177
Q

which step in heme production requires B6

A

the first (rate limiting) step requires B6. this is the conversion of glycine and succinyl CoA into delta ALA. the enzyme that does this is ALA synthatase

178
Q

which adults would get lead poisoning

A

battery/ammunition/radiator factory. headache, memory loss and demylination.

179
Q

enzyme mutation in acute intermittent porphyria

A

porphobilinogen deaminase

180
Q

what builds up in acute intermittent porphyria

A

porphobilinogen, delta ALA, coporphobilinogen in the urine

181
Q

symptoms of acute intermittent porphyria

A

painful abdomen, port wine colored urine, poyneuropathy, psychological disturances, precipitated by drugs, alcohol and starvation

182
Q

treatment of acute intermittent porphyria

A

glucose and heme. heme inhibits ALA synthetase

183
Q

porphyria cutanea tarda symptoms

A

blistering cutaneous lesions that are photosensitive. most common porphyria.

184
Q

enzyme deficient in porphyria cutanea tarda

A

uroporphyrinogen decarboxylase

185
Q

what builds up in porphyria cutanea tarda

A

uroporphyrin leading to tea colored urine.

186
Q

what diagnostic test would show hemophilia

A

PTT

187
Q

hemophilia A defect

A

factor 8

188
Q

hemophilia B defect

A

factor 9

189
Q

what lab tests show bleeding problem from vitamin K deficiency

A

increased PT PTT

190
Q

what test shows platelet problem

A

bleeding time

191
Q

beranard soulier syndrome

A

defect in platelet plug formation. decrease in Gp1b so you cannot attach the platelets to VMF. get a decrease in platelet count with this

192
Q

Glanzmann thrombasthenia

A

increase in bleeding time but normal platelet count. decrease in Gp2b/3a- can’t get platelet-platelet aggregation. blood smear shows NO platelet clumping

193
Q

immune thrombocytopenia

A

anti gp2b/3a antibodies produced. splenic macrophage consumes them. decreased platelet survival. may be triggered by viral illness. labs show increased megakaryocyte count.

194
Q

TTP

A

thrombotic thrombocytopenic purpura

195
Q

TTP mechanism

A

inhibition or deficiency of ADAMTS13 (vMF metalloprotease) which leads to a decrease in degradation of VMF multimers. large multimers are around- the platelets increase adhesion and aggregation and you get thrombosis. decreased platelet survival.

196
Q

renal symptoms, fever, neurological symptoms, thrombocytopenia, and microangiopathic hemolytic uremia

A

these are symptoms of TTP

197
Q

von Willebrand disease

A

problem with both primary platelet plug formation and the intrinsic coagulation cascade

198
Q

what lab values are increase in von Willebrand disease

A

definiteily bleeding time but also potentially PTT because of its association with factor 8

199
Q

vWF and factor 8

A

vWF serves to carry or protect factor 8

200
Q

most common inherited bleeding disorder

A

vWF diseae

201
Q

ristocetin cofactor assay

A

used to diagnose vWF. add this with the patients blood and you will get no agglutination because the ristocetin is an antibiotic that causes the Gp1b to come out and the blood to clump. this only happens with vWF around.

202
Q

treatment for vWF disease

A

DDAVP- releases vWF stored in endothelium

203
Q

what is DIC

A

widespread activation of clotting leads to a deficiency in clotting factors which creates a bleeding state

204
Q

lab findings in DIC

A

schistocytes, increased fibrin split products, decreased fibrinogen, decreased factor 5 and 8

205
Q

most common cause of inherited hypercoagulopathy in whites

A

Factor V Leiden disease

206
Q

how do you find antithrombin deficiency

A

give heparin and see no change in PTT. on its own it won’t cause an issue with the PTT.

207
Q

acquired forms of antithrombin deficiency

A

can be lost in the urine- renal failure and nephrotic syndrome.

208
Q

what deficiency increases the risk of thrombotic skin necrosis with hemorrage after warfarin

A

protein c and s deficiency. mostly protein C deficiency though for skin and other tissue necrosis.

209
Q

when to give packed RBCs

A

acute blood loss, severe anemia

210
Q

when to give platelets

A

stop significant bleeding from thrombocytopenia

211
Q

when to give fresh frozen plasma

A

DIC, cirrhosis, wardarin overdose, exchage transfusion in TTP/HUS

212
Q

when to give cryoprecipitate

A

treat coagulation factor deficiencies involving fibrinogen and factor 8

213
Q

what does cryoprecipitate contain

A

fibrinogen, factor 8, factor 13, vWF, and fibronectin

214
Q

potential side effects from blood transfusion

A

hyperkalemia, hypocalcemia, and iron overload

215
Q

leukemia

A

lymphoic or myeloid neoplasm with involvement of the bone marrow. tumor cells found in peripheral blood

216
Q

lymphoma

A

discrete tumor mass arising from lymph nodes.

217
Q

leukemoid reaction

A

inflammatory reaction to infection. increased WBC with increase PMNs. increased ALP etc.

218
Q

diagnosis with reed sternberg cells

A

Hodgkin lymphoma

219
Q

reed sternberg cell markers

A

CD15 and CD30. two nuclei often- two eyes- owl eye cells

220
Q

which form of hodgkin lymphoma is most common

A

nodular sclerosing type.

221
Q

which form of hodgkin lymphoma is best prognosis

A

lymphocyte rich kind

222
Q

which form of hodgkin lymphoma has worst prognosis

A

lymphocyte mixed or lymphocyte depleted form

223
Q

8;14 translocation

A

burkitt lymphoma

224
Q

chromosome 14?

A

Ig heavy chain

225
Q

chromosome 8?

A

myc gene (cmyc specifically here)

226
Q

most common form of non hodgkin lymphoma in adults

A

diffuse large b cell lymphoma

227
Q

14;18 translocation

A

diffuse large b cell lymphoma and follicular lymphoma

228
Q

11;14 translocation

A

mantle cell lymphoma

229
Q

chromosome 11?

A

cyclin -d

230
Q

chromosome 14?

A

IG heavy chain

231
Q

cell marker for mantle cell lymphoma

A

CD5

232
Q

chromsome 18?

A

bcl2

233
Q

painless waxing and waning of lymphadenopathy

A

follicular lymphoma

234
Q

HTLV1 infection

A

assocaited with IV drug use. causes adult T cell lymphoma.

235
Q

lytic bone lesions with hypercalcemia in Japanese person, West Africa or Carribean descent

A

think about adult T cell lymphoma

236
Q

cell marker for mycosis fungiodes and Sezary syndrome

A

CD4+

237
Q

most common primary tumor arising in the bone in those > 40 yrs

A

multiple myeloma

238
Q

bence joines protein

A

immunoglobulin light chain in urine in multiple myeloma

239
Q

rouleaux formation

A

this is RBCs stacked like poker chips in multiple myeloma

240
Q

if M spike is IgM and no lytic bone lesions

A

think about Waldestrom macroglobulinemia

241
Q

which immunoglobulin mostly elevated in multiple myeloma

A

IgG and IgA

242
Q

multiple myelomas disorders assocaited with it

A

primary amyloidosis

243
Q

myelodisplastic syndrome

A

stem cell problem with ineffective hematopoiesis.

244
Q

pseudo pelger huet anomaly

A

neutrophils with bi-lobed nuclei - two nuclear mases with a thin filament of chromatin- that is commonly seen after chemotherapy

245
Q

leukemia

A

unregulated growth of leukocytes in bone marow.

246
Q

cancer that can present as a mediastinal mass

A

ALL.

247
Q

age group of ALL

A

<15 yrs

248
Q

leukemia assocaited with down’s syndrome

A

ALL

249
Q

12;21 translocation

A

type of ALL with a better prognosis

250
Q

TdT+ marker and CD10+

A

TdT is a marker of pre T and B cells and CD10 is pre B cells only. seen in ALL

251
Q

what is another name for small lymphocytic lymphoma

A

CLL

252
Q

ages of CLL

A

> 60yrs

253
Q

B cell markers seen in CLL

A

CD5+ and CD20+

254
Q

smudge cells in blood smear

A

CLL

255
Q

what cell is the issue in hairy cell leukemia

A

B cells

256
Q

TRAP stain

A

used for hairy cell leukemia

257
Q

dry tap on aspiration

A

think about hairy cell leukemia causes marrow fibrosis.

258
Q

treatment of hairy cell leukemia

A

cladribine (2CDA) which is an adenosine analog- inhibits adenosine deaminase

259
Q

disease with auer rods

A

AML

260
Q

down syndrome

A

AML can also be seen in down synrome (as with ALL)

261
Q

15;17 translocation

A

M3 type of AML which is often called acute promyelocytic leukemia.

262
Q

treatment of M3 type AML

A

all trans retinoic acid (vitamin A) leading to differentiation of myeloblasts.

263
Q

common presentation of M3 type of AML

A

DIC

264
Q

9;22 chromosome translocation

A

this is the philadelphia chromosome- CML.

265
Q

chromosome 9 is …

A

bcr

266
Q

chromosome 22 is..

A

abl

267
Q

disease with increased neutrophils, metamyelocytes and basophils

A

CML

268
Q

disease with very low leukocyte alkaline phosphatase

A

CML

269
Q

how to differentiate CML from leukemoid reaction?

A

leukemooid: incr. LAP
CML: decr. LAP

270
Q

treatment for CML

A

imatinib

271
Q

lytic bone lesions in a child with skin rash or recurrent otitis media with a mass involving the mastoid bone

A

could be langerhans cell histiocytosis

272
Q

what is langerhans cell histiocytosis

A

proliferative disorders of dendritic cells

273
Q

cell marker for langerhand cell histiocytosis

A

S100 and CD1a

274
Q

what type of ganules are seen in langerhan cells

A

birbeck or tennis racket granules

275
Q

mutation often seen in myeloproliferative disorders

A

Jak2 growth signaling problem.

276
Q

polycythemia vera

A

crit >55%. non hereditary mutation in Jak2 gene.

277
Q

patients who are very itchy after their shower

A

polycythema vera

278
Q

symptoms of erythromelagia

A

this is severe burning pain and reddish or bluish discoloration due to episodic blood clots in the vessels of the extremities. sign of polycythemia vera

279
Q

cause of secondary polycythemia vera

A

EPO increase

280
Q

essential thrombocytosis

A

increase in the number of platelets. bone marrow contains thse shuge megakaryocytes

281
Q

myelofibrosis

A

fibrotic obliteration of bone marrow with tear drop cells (RBCs) and immature form of myeloid line.

282
Q

which is the one myeloprolfierative disorder that does not have a JAK2 mutation

A

CML

283
Q

what is relative polycythemia

A

this is just a decrease in plasma volume. like from dehydration and burns. makes it look like a high crit.

284
Q

what is appropriate absolute polycythemia

A

this is when you would expect the body to make more RBCs. for example, lung disease, congenital heart disease, high altitude- when its hypoxic. increase in RBC mass and increase in EPO levels to do this and decrease in oxygen saturation

285
Q

inappropriate absolute polycythemia

A

no change in O2 saturation. this is when you have something making EPO like a renal cell carcinoma, wilms tumor, cyst, hepatocellular carcinoma, hydronephrosis. its all about ectopic EPO. no hypoxia

286
Q

what does a renal cell carcinoma produce

A

EPO

287
Q

what would EPO levels be like in polycythemia vera

A

decrease (negative feedback).

288
Q

what would plasma volume and RBC count be in polycythemia vera

A

increase plasma volume but BIG increase in RBC count

289
Q

why does warfarin cause skin necrosis

A

from removal of protein C (it inhibits this too)

First Aid Chapters (8 decks)

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