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Flashcards in Heme Deck (254):
1

How do you tell the difference btw CML and leukemoid?

Look similar b/c increase WBC with early cells
--alkaline phosphatase is decreased in CML
--Need t(9,22) cytogenics to diagnose CML

2

pre-surg prophylax against DVT

low molecular weight heparin. helps antithrombin III

3

What do you use if u can't use heparin

Direct thrombin inhibitors
--Argatroban
--Lepirudin

4

epstein barr causes which malignancy

Burkett's lymphoma. Will showa high Ki-67 (proliferation index0

5

High AFP in a pregnant woman

Neural tube defect
--Can also be yolk sac tumor

6

Cladribine mechanism

purine analog that is incorporated into DNA causing strands to break.

Resistant to adenosine deaminase

7

Indication cladribine

hairy cell leukemia

8

Purine analogs

6-thiopurine
fludarabine
cladribine

9

Pyrimidine analogs

5-FU
capecitabine
cytarabine
gemcitabine

10

Factor V leiden factor pts at risk for

DVT-->pulmonary thromboembolism
cerebral vein thrombosis
pregnancy loss

11

Why remove spleen from SCD pts

At risk for splenic infarction

12

What causes heparin induced thrombocytopenia?

Antibodies to heparin. Results in paradoxical thrombosis or bleeding. More common with unfractionated (high molecular weight) heparin

13

What are the direct thrombin inhibitors?

hirudin, lepirudin and argatroban

14

AML translocation

t(8,21)

15

Acute promyelocytic leukemia (AML variant) translocation

t(15,17). Will look similar to AML but with more promyelocytes (Large, basophilic nucleus)

16

Burkitt lymphoma translocation

t(8,14)

17

CML translocation

t(9,22)

18

Mantle cell translocation

t(11,14)

19

CLL genetics

13q deletion

20

Describe the APL mechanism

Transfer of retinoic acid receptor alpha to the PML gene. Fusion of two gene products occurs

21

Side effect of dauno/doxorubicin

irreversible dose-dependent cardiotoxicity

22

Which drugs cause bone marrow suppression?

chloramphenicol, ziduvine, phenylbutazone

23

Which drugs cause liver necrosis?

acetaminophen and halothane

24

Which drugs cause pulmonary fibrosis?

Busulfan and bleomycin

25

Pure red cell aplasia--what do you need to look our for

rare severe hypoplasia of marrow erythroid elements. Associated with thymoma, lymphocytic leukemia, and Parvo B19

26

c-myc

Burkitt lymphoma. Chromosome 8
--It's a transcription activator of apoptosis and differentiation

27

what does t(11,14) do?

mantle cell on chromosome 11
-More Cyclin D1
-Increased G1-S

28

Bcl-2

follicular lymphoma
t(14,18)
chromosome 18
inhibits apoptosis

29

BCR-ABL

inhibits apoptosis

30

t(14,18)

follicular lymphoma

31

pathophys of follicular

Bcl2 overexpression

32

ristocetin aggregation test

measures vWF dependent platelet aggregation. Activates GP Ib-IX receptors so they can bind to vWF.

33

Does vWF cause changes in PT or PTT?

Yes--PTT (carries factor VIII)

34

Hageman factor XII

No clotting problems but prolonged PTT

35

glanzmann thrombasthenia testing

response to adenosine diphosphate (ADP), epinephrine, and collagen are decreased
--ristocetin normal

36

STARY SKY APPEARANCE

BURKITT LYMPHOMA

37

CLOSELY PACKED LYMPH NODE FOLLICLES

FOLLICULAR LYMPHOMA

38

large sheets of lymphocytes with large nuclei

Large B cell lymphoma

39

BLASTS

AML OR ALL
--ALL in kids more likely and usually B Cell rather than T cell

40

Blasts in a kid presenting with a mediastinal mass causing SVC syndrome or stridor

T cell ALL

41

B12 deficiency signs

elevated methylmalonic acid AND homocysteine

42

Folate deficiency signs

elevated homocystein

43

How to prevent hemorrhagic cystitis with chemotherapy?

hydration and mesna, which binds acrolein, a toxic metabolite

44

What membrane protein allows RBCs to carry CO2?

Chloride HCO3 antiporter
--accounts for the chloride shift. HCO3 enters, CL- leaves in tissues
--chloride levels low in venous blood

45

1/3 of platelet pool is stored in

the spleen

46

platelets contain what granules

alpha granules
dense granules

47

alpha granules

vWF
Fibrinogen

48

delta granules

ADP, calcium

49

lifespan of a platelet

8-10 days

50

granulocytes

neutrophil, eosinmophil, basophil

51

mononuclear cells

monocytes, lymphocytes

52

Normal neutrophil count

50-60

53

Normal lymphocyte count

25-33

54

hypersegmented poly

vitamin B12/folate deficiency

55

neutrophil granules

alkaline phosphatase
collagenase
lysozyme
lactoferrin
azurophilic granules

56

azurophilic granules

acid phosphatase
peroxidase
B-glucuronidase

57

produces histaminase and arylsulfatase

eosinophils

58

causes of eosinophilia

neoplastic
asthma
alelrgic
collagen vascular disease
parasites

59

basophils release

heparin
histamine
leukotrienes

60

drug: prevents mast cell degran

cromolynsodium

61

mast cells release

histamine
heparin
eosinophil chemotaxis

62

bradykinin activates

vasodilation
permeability
pain

63

other action of XIIa

activates kallikrein from prekallikrein

64

kallidrein

HMWK-->bradykinin
plasminogen-->plasmin

65

action of plasmin

degrades fibrin
activates complement cascade

66

hemophilia A

deficiency factor VIII

67

hemophilia B

deficiency factor IX

68

warfarin inhibits

epoxide reductase (reduces vitamin K)

69

Protein C

works with protein S to cleave and inactivate Va and VIIIa

70

what activates protein C?

thrombomodulin

71

antithrombin inactivates

II,VII,IX, X, XI, XII

72

what activates antithrombin?

heparin

73

what starts coagulation cascade?

platelet release of ADP and Ca2+

74

What induces GpIIb/IIIa expression?

ADP binding

75

anti-aggregation factors

PGI2 (prostacyclin)
NO

76

Ticlopidine and clopidogrel mechanism

inhibits ADP induced expression of GpIIb/IIIa

77

what cell expresses thrombomodulin?

Protein C

78

what do endothelial cells produce?

vWF
thromboplastin
tPA
PGI2

79

Acute phase reactants in plasma cause

RBC aggregation

80

Increase ESR

autoimmune dz
neoplasms
GI disease
pregnancy/infection

81

decrease ESR

sickle cell anemia
CHF
microcytosis
low fibrinogen
polycythemia

82

Acanthocyte

liver disease
abetalipoproteinemia

83

basophilic stippling

thalassemias
anemia of chronic disease
lead poisoning

Baste the tail

84

elliptocyte

hereditary elliptocytosis

85

bite cell

G6PD deficiency

86

macro-ovalocyte

megaloblastic anemia, marrow failure

87

ringed sideroblasts

excess iron storage
--sideroblastic anemia

88

schistocyte

DIC
TTP/HUS
traumatic hemolysis

89

TTP

thrombocytic thrombocytopenia purpura
--blood clots in all the small vessels
--thrombocytopenia (purpura)
--MAHA--schistocytes
--neuro sx
--kidney failure

90

spherocyte

hereditary spherocytosis
autoimmune hemolysis

91

teardrop cell

bone marrow infiltration

92

target cell

HbC
Asplenia
Liver dz
Thallassemia

HALT don't shoot the TARGET

93

heinz bodies

G6PD,
--Similar appearance in alpha-thalassemia

94

howell jolly bodies

asplenia
napthlalene (moth ball) ingestion

95

RBC smear in iron deficiency

microcytosis
hypochromia

96

plummer vinson syndrome

iron deficiency anemia
esophageal webs
atrophic glossitis (smooth tongue)

97

y4 Hb in pt with hydrops fetalis

4 gene deletion of alpha thalassemia

98

Very little alpha globin with Beta globin tetramers

HgbH disease
--caused by only one functional alpha gene
--anemia

99

1-2 gene deletion of alpha chain

no significant sx

100

cis deletion of alpha thalassemia more common in

azns

101

trans deletion of alpha thalassemia more common in

africans

102

beta thalassemia prevalent in

mediterranean population

103

increase in HbA2 (over 3.5%) in asymptomatic pt

beta thalassemia minor

104

chipmunk face, crew cut on skull Xray
--severe anemia requiring blood transfusions

beta thalassemia major (homozygote)

105

hemoglobin in beta thalassemia major

HbF (a2, y2)

106

blood smear in beta thalassemia major

anisocytosis
poikilocytosis
microcytosis
hypochromia
schistocytes

107

house with chipped paint

lead poisoning

108

first line tx for LEAD

Dimercaprol and EDTA

109

tx for lead poisoning in a kid

succimer

110

sx of lead poisoning

1. lead lines on gingivae
2. encephalopathy, erythrocyte basophilic stippling
3. abdominal pain, sideroblastic anemia
4. drops of wrist and foot

111

pt with anemia
increased iron, ferritin, normal TIBC
hereditary cause

hereditary sideroblastic anemia

112

reversible causes of sideroblastic anemia

alcohol, lead, isoniazid

113

tx hereditary sideroblastic anemia

pyridoxine B6

114

cause hereditary sideroblastic anemia

d-ALA synthase defect

115

inheritance of sideroblastic anemia

X linked

116

causes of microcytic anemia

iron deficiency
ACD
thalassemias
lead poisoning
sideroblastic anemia

117

causes of normocytic nonhemolytic anemia

ACD
aplastic anemia
chronic kidney disease

118

macrocytic megaloblastic anemia

folate deficiency
b12
orotic aciduria

119

nonmegaloblastic macrocytic anemia

liver dz
alcoholism
reticulocytosis

120

anemia
--normal MMA
--increased homocysteine

folate deficiency

121

anemia
--increased homocysteine
--increased MMA

B12 deficiency

122

causes of B12 deficiency

diphyllobothrium latum
--crohn's
vegans
PPIs

123

signs of subacute combined degeneration

peripheral neuropathy
loss of vibration and proprioception
spasticity (loss of lateral corticospinal)
dementia

124

treatment for orotic aciduria

uridine monophosphate

125

which drugs cause macrocytic anemia?

5-FU, AZT, hydroxyurea. these all interfere with DNA synthesis, but NO megaloblastic RBCs

126

anemia
--decreased haptoglobin
--increased LDH
--hemoblogin in urine

intravascular hemolysis

127

anemia
--increased LDH and high unconjugated bili

extravascular hemolysis. Means you're losing RBCs through spleen clearance, not lysis in vessels

128

findings in anemia of chronic dz

1. increased hepcidin
2. ferritin
3. decreased TIBC
4. decreased iron

129

cause of aplastic anemia

1. radiation, benzene, chloramphenicol, alkylatin agents
2. viral: parvo, EBV, HIV, HCV
3. fanconi's
4. autoimmune

130

fanconi's

proximal renal tubules[1] of the kidney in which glucose, amino acids, uric acid, phosphate and bicarbonate are passed into the urine

131

dry bone marrow type showing hypocellular marrow with fatty infiltration

aplastic anemia

132

signs of aplastic anemia

pancytopenia

133

positive osmotic fragility test

hereditary spherocytosis

134

hereditary spherocytosis prone to asplastic crisis from infection by

parvo B19

135

back pain with hemoglobinuria days later. Heiz bodies and bite cells

G6PD

136

G6PD: intravascular or extravascular hemolysis?

mostly extravascular

137

hemolytic anemia in newborn

pyruvate kinase deficiency
--low ATP=rigid RBCs

138

HbC cause

glutamic acid to lysine mutation at residue 6.

139

hemolytic anemia, pancytopenia, and venous thrombosis
--CD55/59 negative RBCs on flow

Paroxysmal nocturnal hemoglobinuria
--increased complement-mediated RBC lysis

140

how do you get PNH?

acquired mutation in stem cell
--loss of GPI anchor or decay accelerating factor (protect RBC from complement

141

tx PNH

eculizumab

142

crew cut on skull x ray with severe anemia and sudden vasoocclusive crises

sickle cell

143

mutation in sickle cell

Substitution of glutamic acid with valine at position 6

144

SCD child suddenly showing howell jolly bodies

autosplenectomy

145

kidney problems in SCD

renal papillary necrosis

146

tx sickle ell

hydroxyurea or bone marrow transplant

147

warm agglutinin type of antibody

IgG

148

cold agglutinin type of antibody

IgM

149

Warm agglutinin seen in

chronic anemia with
SLE, CLL, drugs

150

cold agglutinin seen in

chronic anemia with
-mycoplasma pneumonia
-mono
-CLL

151

pathophys MAHA

RBCs damaged b/c of narrow vessel lumen

152

cause of MAHA

DIC
TTP-HUS
SLE
Malignant HTN

153

macroangiopathic anemia

mechanical destruction RBC (valves, aortic stenosis)
--schistocytes

154

iron lab values in hemochromatosis

increased serum iron
increased ferritin
decreased TIBC
increased transferrin saturation

155

pregnancy/OCP iron tests

increased transferrin
decreased transferrin saturation
normal iron and ferritin

156

steps in heme synthesis

1. glycine+succinylCoa
2. d-ALA
3. Porphobilinogen
4. HMB
5. Uroporphyrinogen
6. coproporphytingoen
7. protoporphyrin
8. heme

157

dALA synthase requires what vitamin

b6

158

lead poisoning blocks

d-ALA dehydratase
ferrochelatase

159

sideroblastic anemia is a defect in

d-ALA synthase

160

Acute intermittent porphyria is a block in

porphobilinogen deaminase

161

Porphyria cutanea tarda is a block in

uroporphyringen decarboxylase

162

accumulated protoporph and dALA

lead poisoning

163

accumulated porphobilinogen dALA and uroporphyrin (tea colored urine)

acute intermittent porphyria

164

battery/ammunition/radiator factory worker

lead poisoning

165

painful abdomen
port wine urine
polyneuropathy
psych disturbances
precipitated by drugs

acute intermittent porphyria

166

tx AIP

glucose and heme--inhibit ALA synthase

167

blistering photosensitivity with uroporphyrin in the urine

porphyria cutanea tarda

168

defect in porphyria cutanea tarda

uroporphyrinogen decarboxylase

169

PT tests

extrinsic pathway (I, II, V, VII, X)

170

PTT tests

intrinsic pathway (everything but VII and XIII)

171

clotting results in hemophilia

Increased PTT (factor VIII and IX)

172

clotting results in vitamin K deficiency

Increased PT, PTT

173

bernard soulier labs

decreased PC
increased BT

174

glanzmann's labs

increased BT
no change in PC

175

blood smear shows no platelet clumping

glanzmanns

176

cause idiopathic thrombocytopenic purpura

anti-GpIIb/IIIa antibodies
--spleen consumes platelet/antibody complex

177

labs in ITP

decreased platelets
increased BT
increased megakaryocytes

178

TTP cause

deficiency of ADAMTS13
--cannot separate vWF multipers causing platelet aggregation and thrombosis

179

labs in TTP

decrased PC
Increased BT
schistocytes
increased LDH

180

tx vWF

DDAVP

181

labs vWF

increased BT
--if severe, increased PTT

182

inheritance vWF

autosomal dominant

183

DIC labs

1. low PC
2. high BT, PT, PTT
3. Schisto
4. D dimers
5. low fibrinogen
6. low factor V and VIII

184

mutation in prothrombin gene mutation

3' untranslated region=more prothrombin

185

pt has no increase in PTT after heparin administration

antithrombin deficiency

186

pt has thrombotic skin necrosis with hemorrhage after warfrin

protein C or S deficiency.
--cannot inactivate factors V and VIII

187

what is contained in cryoprecipitate?

fibrinogen
factor VIII
factor XIII
vWF
fibronectin

188

serum changes with blood transfusions

hypocalcemia
hyperkalemia

189

leukemoid rxn labs

increased WBC with left shift
INCREASED leukocyte alkaline phosphatase

190

CML labs

increased WBC with left shift
DECREASED leukocyte alk phos

191

typical spread of hodgkin's

localized
-single group of nodes
-spreads contiguously

192

typical spread non-hodgkin's

multiple peripheral nodes
-extranodal involvement
-does not spread through blood

193

strongest prognostic indicator in hodgkins

stage

194

hodgkin's associated with

EBV

195

non-hodgkin's associated with

HIV/immunosuppression

196

hodgkin age group

young adulhood and over 55 years. usually men

197

non-hodgkins age group

20-40 years

198

B symptoms more common in hodgkins or non-hodgkins

hodgkins

199

Reed sternberg cells are positive for

CD30 and CD15

200

Most common form of hodgkin's

nodular sclerosing

201

prognosis of nodular sclerosing

good. prognosis with lymphocyte mixed/depleted is poor

202

t(8,14)

burkitt's

203

cmyc

burkitt's

204

endemic form of burkitt's in africa

jaw lesion

205

sporadic burkitt's

pelvis/abdomen

206

most common adult NHL

diffuse large B cell

207

t(11,14)

mantle cell

208

cyclin D1

mantle cell

209

t(14,18)

follicular

210

bcl02

follicular

211

which NHLs can be seen in children

classically burkitt's
20% of diffuse large B cell lymphomas

212

CD5+ NHL

mantle cell

213

prognosis follicular lymphoma

difficult to cure
--indolent course

214

prognosis mantle cell

poor

215

adult T cell lymphoma caused by

HTLV-1

216

sx: adult T cell lymphoma

cutaneous lesions

217

adult t cell lymphoma affects people from

Japan
west africa
caribbean

218

cutaneous patches/nodules with CD4+ cells

mycosis fungoides/sezary

219

rouleaux formation

multiple myeloma

220

histology multiple myeloma

plasma cells with clock face chromatin--lots of em
--intracytoplasmic inclusions, white

221

waldenstrom's macroglobulinemia

M spike without lytic bone lesions

222

Risk of MM in MGUS

1-2% per year

223

ALL affects what age group

less than 15 years

224

mediastinal mass

T cell ALL

225

tdT and CALLA positive

ALL

226

Which ALL has the best prognosis?

t(12, 21)

227

smudge cells

small lymphocytic lymphoma/chronic lymphocytic leukemia

228

SLL and CLL typically affect what age group

>60 years

229

presentation of SLL/CLL

asymptomatic. autoimmune hemolytic anemia

230

TRAP positive

hairy cell leukemia

231

tx: hairy cell leukemia

Cladribin

232

t(15,17)

M3 type AML

233

median age of AML

65

234

auer rods

AML

235

Common presentation of M3 AML

DIC

236

tx: M3 AML

all trans retinoic acid

237

CML age group

30-60

238

t(9,22)

CML

239

blood mear in CML

increased neutrophil
metamyelocytes
basophils
splenomegaly

240

CML can transform to

AML/ALL (blast crisis)

241

AMl-M3 subtype also known as

acute promyelocytic leukemia

242

peroxidase positive cytoplasmic inclusions

auer rods

243

child with lytic bone lesions and skin rash. cells show lots of birbeck granules on EM

langerhans histiocytosis

244

pathophysiology of langerhans histiocytosis

functionally immature dendritic cells cannot stimulate T lymphocytes.

245

Cells in langerhans histiocytosis express

S-100 and CD1a

246

JAK2 positive

myeloproliferative disorder
-polycythemia vera
essential thrombocytosis
myelofibrosis

247

RBC, WBC, platelets in polycythemia vera

All increased

248

RBC, WBC, platelets in essential thrombocytosis

platelets increased

249

RBC, WBC, platelets in myelofibrosis

Low RBC. variable wbc and platelets

250

RBC, WBC, platelets in CML

low RBC lots of WBC and platelets

251

Lymphoid lineage

B cells
T cells
NK cells

252

relative polycythemia

decreased plasma volume (dehydration)

253

appropriate polycythemia

increased RBC 2ndary to O2 saturation

254

inappropriate absolute

increased RBC mass caused by
1.RCC, wilms' tumor
2. HCC
3. Hydronephrosis
4. OR ectopic EPO