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Flashcards in Hematology Deck (268):
1

all forms of anemia lead to

fatigue/loss of energy

2

pt with severe anemia will have

SOB, lightheadedness, or confusion

3

diseases with similar presentations to anemia:

- hypoxia
- CO poisoning
- methemoglobinemia
- ischemic heart disease

4

craving for ice or dirt, think

anemia

5

physical exam findings on anemic patient

- pallor
- flow murmur
- pale conjunctiva

6

physical exam findings in hemolytic anemia

- jaundice
- scleral icterus

7

in severe anemia, what is needed to exclude ischemia?

EKG

8

best INITIAL test for anemia

CBC with peripheral smear

9

additional initial tests for anemia

- reticulocyte count
- haptoglobin
- LDH
- total and direct bilirubin
- TSH and free T4
- B12/folate levels
- iron studies
- UA w/ microanalysis

10

categorization of anemia is based on

MCV (mean corpuscular volume)

11

what value may indicate if there's a problem with the synthesis of Hb?

MCHC (mean corpuscular hemoglobin concentration)

12

anemia categorized based on MCV is as follows:

- microcytic
- macrocytic
- normocytic

13

based on MCHC anemia can be further categorized as follows:

- hypochromic
- hyperchromic
- normochromic

14

- blood loss
- THROMBOCYTOSIS

what is the diagnosis?

IDA

15

- RA
- ESRD
- any chronic infectious, inflammatory, or connective tissue disease

what is the diagnosis?

ACD

16

- very small MCV w/ few or no symptoms
- TARGET cells

what is the diagnosis?

thalassemia

17

- alcoholic
- INH
- lead exposure

what is the diagnosis?

sideroblastic anemia

18

best INITIAL test for IDA, ACD, thalassemia, or sideroblastic anemia

iron studies

19

results of iron studies in IDA

- low ferritin
- high TIBC (total iron binding capacity)
- low iron
- low iron saturation
- elevated RDW (red cell distribution width)

20

results of iron studies in ACD

- HIGH ferritin (acute phase reactant)
- low TIBC (total iron binding capacity)
- low iron
- normal or low iron saturation

21

results of iron studies in thalassemia

NORMAL

22

results of iron studies in sideroblastic anemia

HIGH IRON

23

MOST ACCURATE test for IDA

bone marrow biopsy (don't do this on CCS exam)

24

MOST ACCURATE test for thalassemia

Hb electrophoresis

(beta: elevated HbA2, and HbF)
(alpha: normal)

25

MOST ACCURATE test for sideroblastic anemia

Prussian blue stain

26

best INITIAL treatment for IDA

ferrous sulfate PO

27

best INITIAL treatment for ACD

correct underlying cause

28

best INITIAL treatment for thalassemia

no treatment for trait

29

best INITIAL treatment for sideroblastic anemia

minor: pyridoxine (vitamin B6)
major: remove toxin exposure

30

MOST ACCURATE test for ALPHA thalassemia

DNA sequencing

31

name the 4 types of microcytic anemia

1. IDA
2. ACD
3. thalassemia
4. sideroblastic anemia

32

causes of macrocytic anemia

- vitamin B12 deficiency
- folate deficiency

33

which medication blocks B12 absorption?

metformin

34

B12 deficiency causes:

- hypersegmented neutrophils
- peripheral neuropathy (can cause any neuro sx)
- glossitis (smooth tongue)
- diarrhea

35

folate deficiency causes:

- hypersegmented neutrophils only

36

labs seen in B12 defiicincy

- DECREASED reticulocyte count
- elevated bilirubin
- elevated LDH

37

mechanism of elevated indirect bilirubin and LDH in B12 deficiency

"ineffective erythropoiesis"

RBCs are destroyed as they leave bone marrow

38

MOST ACCURATE tests for B12 and folate deficiencies

B12 and folate levels

39

next step if B12 deficiency is SUSPECTED, but B12 level is NORMAL

methylmalonic acid level

40

after finding low B12 level or elevated methylmalonic acid level, next best step to confirm etiology of B12 deficiency

antiparietal cell Ab and anti-intrinsic factor Ab

41

treatment for B12 and folate deficiencies

replace

42

first thing that improves after B12 replacement therapy

reticulocytes

43

last thing that improves after B12 replacement therapy

neurological abnormalities

44

name the hemolytic anemias (9)

1. sickle cell anemia
2. Hb sickle cell disease
3. autoimmune hemolysis
4. cold-induced hemolysis (cold agglutinins)
5. glucose-6-phosphate dehydrogenase (G6PD) deficiency
6. pyruvate kinase deficiency
7. hereditary spherocytosis
8. hemolytic uremic syndrome (HUS), and thrombotic thrombocytopenic purpura (TTP)
9. paroxysmal nocturnal hemoglobinuria (PNH)

45

all forms of hemolytic anemia present with

SUDDEN ONSET of weakness and fatigue

46

diagnostic testing for hemolysis shows the following

- elevated indirect bilirubin
- elevated reticulocyte count
- elevated LDH
- decreased haptoglobin
- spherocytes on smear

47

in hemolysis, why is indirect bilirubin elevated, and haptoglobin decreased?

- limited capacity of liver to glucuronidate indirect to DIRECT bilirubin
- haptoglobin is a transport for indirect bilirubin which is quickly used up

48

what electrolyte should be monitored after treating vitamin B12 deficiency?

K+ (can be low)

49

- very severe chest, back, and thigh pain

sickle cell anemia

50

best INITIAL treatment for sickle cell anemia

- OXYGEN
- HYDRATION
- PAIN MEDICATIONS
- ABX (ceftriaxone, levofloxacin, or moxifloxacin)

51

why is FEVER an EMERGENCY in a pt w/ sickle cell anemia?

NO SPLEEN

52

tests that should be ordered for pt with sickle anemia

- blood cultures
- UA
- reticulocyte count
- CBC
- CXR

53

if fever is present after treatment for sickle cell anemia, then?

physical examination

54

possible physical exam findings in sickle cell anemia:

- HEENT
- CVS
- respiratory
- abdomen
- extremities
- neurological

HEENT- retinal infarction
CV- flow murmur from anemia
respiratory- rales, or consolidation from infection or infarction
abdomen- splenomegaly in children, absence of spleen in adults
extremities- skin ulcers, aseptic necrosis of hip (found on MRI)
neurological- stroke (current or previous)

55

when is exchange transfusion done in sickle cell anemia?

- eye: visual disturbance from retinal infarction
- lung: pulmonary infarction leading to pleuritic chest pain and abnormal CXR
- penis: priapism from infarction of prostatic plexus of veins
- brian: stroke

56

what is the goal of exchange transfusion?

decrease Hb S to 30-40%

57

- sudden drop in hematocrit in pt with hemoglobinopathy
- low reticulocyte count

- parvovirus B19
- folate deficiency

58

MOST ACCURATE test for parvovirus B19

PCR for parvovirus DNA

59

treatment for sickle cell crisis d/t parvovirus B19

transfusions and IVIG

60

sickle cell anemia patients should be discharged on:

1. folate
2. Pneumococcal vaccine
3. hydroxyurea, if crises occur > 4x/year

61

MOA of hydroxyurea

increases % of Hb F

62

sickle cell disease manifestations

- visual disturbance (frequent)
- hematuria
- isosthenuria (inability to concentrate/dilute urine)
- UTIs

63

sickle cell trait manifestations

- hematuria
- isosthenuria
- splenic vein thrombosis can occur with severe hypoxia

64

look for other autoimmune diseases in history, or medications

autoimmune hemolysis

65

diagnostic tests for autoimmune hemolysis

- elevated LDH
- elevated indirect bilirubin
- elevated reticulocyte count
- decreased haptoglobin

66

peripheral smear will show what in autoimmune hemolysis

spherocytes

67

MOST ACCURATE diagnostic test for autoimmune hemolysis

Coombs test

68

best INITIAL treatment for autoimmune hemolysis

steroids

69

treatment if RECURRENT episodes of hemolysis

rituximab

70

MOST EFFECTIVE treatment if RECURRENT episodes of hemolysis

splenectomy

71

mechanism of spherocytes in autoimmune hemolysis

Abs attack RBC membranes which are biconcave turning them into spheres

72

in autoimmune hemolysis, steroids and splenectomy only work on?

IgG Abs "warm antibodies"

73

if severe hemolysis does not respond to steroids or repeated blood transfusions use

IVIG

74

a response to IVIG predicts a response to

splenectomy

75

cold-induced hemolysis (cold agglutinins); look for what in the history?

- mycoplasma
- Epstein-Barr virus

76

in cold-induced hemolysis Coombs test will be?

NEGATIVE

77

in cold-induced hemolysis complement test will be?

POSITIVE

78

treatment for in cold-induced hemolysis

rituximab

79

will steroids, splenectomy, or IVIG work for cold-induced hemolysis?

NO

80

MOA of rituximab

MAB against CD20R on lymphocytes which make Abs

81

syphilis causes cold agglutinins with?

IVIG

82

- sudden onset hemolysis
- X-linked
- oxidant stress causes acute hemolysis
- MC oxidant stress = infection
- sulfa, primaquine, dapsone, fava beans

glucose-6-phosphate dehydrogenase (G6PD) deficiency

83

best INITIAL test for glucose-6-phosphate dehydrogenase (G6PD) deficiency

- Heinz body test
- bite cells

84

Heinz bodies are

collection of oxidized, precipitated Hb embedded in RBC membrane

85

bite cells are

pieces of RBC membrane removed by spleen

86

MOST ACCURATE test for glucose-6-phosphate dehydrogenase (G6PD) deficiency

G6PD LEVEL, BUT ONLY AFTER 2 MONTHS HAVE PASSED from acute episode (otherwise, may be falsely normal)

87

treatment for glucose-6-phosphate dehydrogenase (G6PD) deficiency

avoid oxidant stress

88

- sudden onset hemolysis
- NOT provoked (unclear cause)

pyruvate kinase deficiency

89

- recurrent episodes of hemolysis
- splenomegaly
- bilirubin gallstones
- elevated mean corpuscular Hb concentration (MCHC)

hereditary spherocytosis

90

MOST ACCURATE test for hereditary spherocytosis

osmotic fragility test

91

treatment for hereditary spherocytosis

splenectomy

92

mechanism of hereditary spherocytosis

genetic loss of ankyrin and spectrin in RBC membrane

93

hemolytic uremic syndrome (HUS) diagnosis is based on

IT'S IN THE NAME!!!

- intravascular hemolysis (Autoimmune hemolysis)
- elevated BUN and creatinine (Renal failure)
- thrombocytopenia (Thrombocytopenia)

94

thrombotic thrombocytopenic purpura (TTP) diagnosis is based on

- intravascular hemolysis
- elevated BUN and creatinine
- thrombocytopenia

AND

- fever
- neurological abnormalities

95

never use what in HUS or TTP?

platelets

96

what is LOW in TTP?

ADAMTS-13 level

97

treatment for HUS/TTP

- some cases resolve on their own
- severe cases: plasmapheresis

98

what makes HUS worse?

antibiotics

99

mechanism of HUS/TTP

ADAMTS-13 = metalloproteinase that breaks down VWF to RELEASE platelets from one another

if VWF is NOT dissolved, platelets form abnormally long strands and RBCs break down as they pass through

100

- pancytopenia
- recurrent episodes of dark urine, especially in morning
- MCC of death = large vessel venous thrombosis

paroxysmal nocturnal hemoglobinuria (PNH)

101

paroxysmal nocturnal hemoglobinuria (PNH) can transform into

- aplastic anemia
- acute myelogenous leukemia (AML)

102

MOST ACCURATE test for paroxysmal nocturnal hemoglobinuria (PNH)

CD55 and CD59 Abs (aka, decay accelerating factor)

103

best INITIAL treatment for paroxysmal nocturnal hemoglobinuria (PNH)

glucocorticoids

104

treatment for transfusion-dependent pts with severe paroxysmal nocturnal hemoglobinuria (PNH)

eculizumab

105

MOA of eculizumab

inhibits C-5 and prevents complement activation

106

how can HELLP syndrome be distinguished from DIC?

HELLP has NORMAL coagulation studies

107

- SOB for no clear reason
- clear lungs on exam
- normal CXR

methemoglobinemia

108

methemoglobinemia is

Hb locked in OXIDIZED state that cannot pick up O2

109

possible drugs that can cause methemoglobinemia

- nitroglycerin
- amyl nitrate
- nitroprusside
- dapsone
- any anesthetic (ends in -CAINE; lidocaine/bupivacaine/tetracaine)

110

in methemoglobinemia, what color is the blood?

BROWN

111

treatment for methemoglobinemia

methylene blue

112

transfusion reactions: case 1

- 20 minutes after a pt receives a blood transfusion, the pt becomes SOB
- there are transient infiltrates on CXR
- all symptoms resolve spontaneously

transfusion-related acute lung injury (TRALI), or "leukoagglutination reaction"


113

transfusion reactions: case 2

- as soon as a pt receives a transfusion, he becomes HYPOTENSIVE, SOB, TACHYCARDIC
- LDH, and bilirubin levels are NORMAL

IgA deficiency

114

transfusion reactions: case 3

- during a transfusion, a pt becomes hypotensive and tachycardic
- has back and chest pain
- has dark urine
- LDH, and bilirubin are ELEVATED
- haptoglobin is LOW

ABO incompatibility

115

transfusion reactions: case 4

- a few days after a transfusion, pt becomes jaundiced
- hematocrit does NOT rise with transfusion
- generally without symptoms

minor blood group incompatibility

116

transfusion reactions: case 5

- a few hours after a transfusion, pt becomes FEBRILE with a rise in temperature of about 1 degree
- no evidence of hemolysis

febrile nonhemolytic reaction

117

- present w/ signs of pancytopenia:
1. fatigue
2. bleeding
3. infections d/t functional immunodeficiency

acute leukemia

118

best INITIAL test for acute leukemia

peripheral smear showing blasts

119

Auer rods are associated with?

acute myeloid leukemia (AML)

120

most important prognostic finding in acute leukemia

cytogenetic abnormalities (specific karyotype abnormalities)

121

if pt is a high risk for relapse of acute leukemia after chemotherapy, pt should have what after chemotherapy induces remission?

bone marrow transplantation

122

best INITIAL treatment for acute leukemia

chemotherapy

- idarubicin, or daunorubicin

AND

- cytosine arabinoside

123

what treatment should be added for acute promyelocytic leukemia (M3 AML) to the initial treatment?

all trans retinoic acid (ATRA)

124

what treatment should be added for acute lymphocytic leukemia (ALL) to the initial treatment?

intrathecal methotrexate

125

M3, acute promyelocytic leukemia is associated with

disseminated intravascular coagulation (DIC)

126

- SOB, confusion, and blurry vision
- white cell count > 100,000

leukostasis = sludging of blood vessels of brain, eyes, and lungs

127

treatment for leukostasis

leukapheresis

128

what should be added in a pt with leukostasis to lower the white cell count?

hydroxyurea

129

- elderly pt w/ pancytopenia
- elevated MCV
- low reticulocyte count
- macroovalocytes
- "Pelger-Huet cell" (neutrophil w/ 2 lobes)
- NORMAL B12 level
- small # of blasts (not enough to be considered acute leukemia)

myelodysplasia

130

mild, slow progressive preleukemia syndrome

myelodysplasia

131

myelodysplasia may progress to

acute leukemia

132

MCC of death in myelodysplasia

infection or bleeding

133

main treatment for myelodysplasia

transfusions as needed

134

only thing that increases survival in myelodysplasia

azacytidine

135

decreases transfusion dependence in myelodysplasia

lenalidomide

136

name the myeloproliferative disorders (6)

1. chronic myelogenous leukemia (CML)
2. chronic lymphocytic leukemia (CLL)
3. hairy cell leukemia
4. myelofibrosis
5. polycythemia vera
6. essential thrombocythemia

137

- elevated white cell count
- predominantly NEUTROPHILS
- splenomegaly (common)

chronic myelogenous leukemia (CML)

138

of all forms of myeloproliferative disorders, which has the highest risk of transforming into acute leukemia?

untreated chronic myelogenous leukemia (CML)

139

mechanism of early satiety in CML and CLL

d/t splenomegaly compressing stomach

140

diagnostic test for chronic myelogenous leukemia (CML)

elevated neutrophil count with LOW LAP score

141

MOST ACCURATE test for chronic myelogenous leukemia (CML)

Philadelphia chromosome by PCR,

or

BCR/ABL by fluorescence in situ hybridization (FISH)

142

best INITIAL treatment for chronic myelogenous leukemia (CML)

imatinib

143

curative treatment for chronic myelogenous leukemia (CML)

bone marrow transplantation

144

- pts > 50 y/o w/ elevated white cell count described as "normal appearing lymphocytes"
- often asymptomatic

chronic lymphocytic leukemia (CLL)

145

best INITIAL test for chronic lymphocytic leukemia (CLL)

peripheral blood smear shows "SMUDGE" cells

(ruptured nuclei of lymphocytes)

146

stages for chronic lymphocytic leukemia (CLL)

stage 0 = elevated white cell count alone
stage 1 = enlarged lymph nodes
stage 2 = spleen enlargement
stage 3 = anemia
stage 4 = low platelets

147

mechanism of infection and hemolysis in chronic lymphocytic leukemia (CLL)

- lymphocytes produce abnormal/insufficient immunoglobulins
- insufficient immunoglobulins = infection
- abnormal immunoglobulins = thrombocytopenia/hemolyis

148

treatment for chronic lymphocytic leukemia (CLL)

based on disease stage

149

treatment for chronic lymphocytic leukemia (CLL): stages 0 and 1

no treatment needed

150

treatment for chronic lymphocytic leukemia (CLL): stages 2-4

fludarabine, and rituximab and cyclophosphamide

151

which treatment is most likely to extend survival in treatment for chronic lymphocytic leukemia (CLL)?

fludarabine

152

- middle-aged pt (50's)
- pancytopenia
- massive splenomegaly

hairy cell leukemia

153

MOST ACCURATE test for hairy cell leukemia

smear showing HAIRY cells and immunophenotyping

154

best INITIAL treatment for hairy cell leukemia

cladribine (2-CDA)

155

- pancytopenia
- splenomegaly
- NORMAL TRAP level

myelofibrosis

156

key feature seen on PBS in myelofibrosis

teardrop-shaped cells

157

finding on bone marrow biopsy in myelofibrosis

fibrosis and JAK2 mutation

158

best INITIAL treatment for myelofibrosis if bone marrow transplant is NOT possible

lenalidomide/thalidomide

159

curative treatment for myelofibrosis

bone marrow transplantation

160

inhibits Janus kinase

ruxolitinib

161

- headache
- blurred vision
- dizziness
- fatigue
- pruritis, after hot bath/shower
- splenomegaly

polycythemia vera

162

key to diagnosis in polycythemia vera

MARKEDLY HIGH hematocrit in the ABSENCE of hypoxia with a LOW MCV

(erythropoietin level will be low)

163

diagnostic tests for polycythemia vera

- CBC
- ABG to r/o hypoxia
- erythropoietin level (low)
- JAK2 mutation (97% sensitive)

164

best INITIAL treatment for polycythemia vera

phlebotomy

165

used to lower the cell count in polycythemia vera

hydroxyurea

166

treatment for thrombocytopenia in polycythemia vera

anagrelide

167

other treatment that should be given for polycythemia vera

aspirin

168

- markedly elevated platelet count
- presents w/ headache, visual disturbance, and pain in hands

essential thrombocythemia

169

MCC of death in essential thrombocythemia

thrombosis and bleeding

170

treatment for essential thrombocythemia

hydroxyurea

171

treatment for essential thrombocythemia if pt is thrombosing

aspirin

172

what other lab values are elevated in polycythemia vera?

- B12
- LAP (leukocyte alkaline phosphatase) score

173

name the plasma cell disorders (4)

1. multiple myeloma
2. monoclonal gammopathy of unknown significance (MGUS)
3. Waldenstrom's macrogammaglobulinemia
4. aplastic anemia

174

- bone pain caused by fracture occurring under normal use (most frequent presentation)

multiple myeloma

175

MCC of death from MM

- infection: pts effectively immunodeficient
- renal failure

176

initial tests for MM

- skeletal survey
- serum protein electrophoresis (SPEP)
- urine protein electrophoresis (UPEP)
- peripheral smear
- elevated Ca++ level
- B2 microglobulin level
- elevated BUN/creatinine

177

skeletal survey findings in MM

punched out osteolytic lesions

(osteoblastic lesions suggest metastatic prostate cancer)

178

serum protein electrophoresis (SPEP) findings in MM

elevated monoclonal Ab (usually IgG) levels

(20% are IgA)

179

urine protein electrophoresis (UPEP) findings in MM

Bence-Jones protein

180

peripheral smear findings in MM

"rouleaux" formation of blood cells

(mean platelet volume (MPV) is elevated bc cells stick together)

181

cause of elevated Ca++ level in MM

d/t osteoLYTIC lesions

182

prognostic indicator in MM

B2 microglobulin level

183

mechanism of renal failure in MM

- hypercalcemia = nephrocalcinosis
- hyperuricemia = directly toxic to kidney tubules
- Bence-Jones protein clog up glomeruli and are also toxic to kidney tubules
- amyloid occurs in myeloma

184

reverses renal dysfunction in MM

bortezomib

185

single MOST SPECIFIC test in MM

bone marrow biopsy (detects high numbers of plasma cells (10%))

186

treatment for MM

melphalan and steroids

187

most effective treatment for MM

autologous stem cell bone marrow transplantation

(pts

188

other treatment considerations for MM:

- hypercalcemia
- bone fractures
- renal failure
- anemia
- infection

- hydration/diuresis
- bisphosphonates
- hydration
- erythropoietin
- vaccinations

189

MGUS presents w/

ASYMPTOMATIC elevation of IgG on SPEP

190

treatment for MGUS

none

191

Waldenstrom's macrogammaglobulinemia presents w/

hyperviscosity from IgM overproduction

192

how does Waldenstrom's macrogammaglobulinemia present?

- blurred vision
- confusion
- headache
- enlarged LN's/splenomegaly

193

best INITIAL test for Waldenstrom's macrogammaglobulinemia

- serum viscosity level (will be markedly increased)

AND

- SPEP (elevated IgM level)

194

best INITIAL treatment for Waldenstrom's macrogammaglobulinemia, if SYMPTOMATIC

plasmapheresis

195

further treatment for Waldenstrom's macrogammaglobulinemia

- rituximab
- fludarabine
- chlorambucil

196

hemolytic uremic syndrome (HUS) is associated w/

E. coli 0157:H7

197

- ticlopidine predisposes to this

thrombotic thrombocytopenic purpura (TTP)

198

- pancytopenia w/ no identified etiology

aplastic anemia

199

best possible treatment for aplastic anemia

bone marrow transplantation

200

if bone marrow transplantation is not possible, treatment for aplastic anemia

antithymocyte globulin and cyclosporine

201

- enlarged LNs (most commonly cervical area)
- +/- "B" symptoms (fever, weight loss, night sweats)

lymphoma

202

lymphoma: starts at the neck and spreads centrifugally away from center

Hodgkin's lymphoma (HD)

203

lymphoma: widespread disease

non-Hodgkin's lymphoma (NHL)

204

best INITIAL test for HD and NHL

excisional LN biopsy

205

the major difference between HD and NHL, is HD has

Reed-Sternberg cells

206

tests needed for staging HD and NHL

- CXR
- CT scans w/ contrast of chest, abdomen, pelvis, and head
- bone marrow biopsy

207

staging of HD and NHL

stage 1: single LN group
stage 2: 2 LN groups on ONE side of diaphragm
stage 3: LNs on BOTH sides of diaphragm
stage 4: widespread disease

208

HD presents in what stages?

80-90% in stages 1 or 2

209

NHL presents in what stages?

80-90% in stages 3 or 4

210

lymphoma treatment: localized disease (stages 1 and 2) WITHOUT "B" symptoms

radiation and lower-dose chemotherapy

211

lymphoma treatment: advanced disease (stages 3 and 4)

chemotherapy ONLY

212

chemotherapy treatment for HD

ABVD

- Adriamycin (doxorubicin)
- bleomycin
- vinblastine
- dacarbazine

213

chemotherapy treatment for NHL

CHOP

- cyclophosphamide
- hydroxyadriamycin
- Oncovin (vincristine)
- prednisone

214

if anti-CD20 Ag is present in NHL, what should be added?

rituximab (adds efficacy to CHOP)

215

name the coagulation disorders (4ish)

1. von Willebrand's disease (VWD)
2. idiopathic thrombocytopenic purpura (ITP)
3. uremia-induced platelet dysfunction
4. clotting factor deficiencies (factors 8, 9, 11, 12)

216

- bleeding from platelet dysfunction (superficial bleeding: skin and mucosal surfaces)
- epistaxis
- bleeding worse w/ aspirin use
- NORMAL platelet count
- elevated aPTT in up to 50% of pts

von Willebrand's disease (VWD)

217

MOST ACCURATE tests for von Willebrand's disease (VWD)

- ristocetin cofactor assay
- von Willebrand's factor (VWF) level

218

first-line treatment for von Willebrand's disease (VWD)

desmopressin

219

MOA of desmopressin

releases subendothelial stores of VWF and factor 8

220

treatment for von Willebrand's disease (VWD) if desmopressin is ineffective

factor 8 replacement

221

- platelet-type bleeding
- platelet count

idiopathic thrombocytopenic purpura (ITP)

222

diagnostic tests for idiopathic thrombocytopenic purpura (ITP)

- peripheral smear
- sonogram
- bone marrow
- Abs to glycoprotein 2b/3a receptor

223

finding of peripheral smear in idiopathic thrombocytopenic purpura (ITP)

large platelets

224

finding of sonogram in idiopathic thrombocytopenic purpura (ITP)

normal spleen size

225

finding of bone marrow biopsy in idiopathic thrombocytopenic purpura (ITP)

increased # of megakaryocytes

226

most important step in idiopathic thrombocytopenic purpura (ITP)

initiating treatment

227

- platelet count of 5,000
- epistaxis and petechiae
- intracranial hemorrhage and melena

what is the best initial step?

IVIG

228

idiopathic thrombocytopenic purpura (ITP) treatment:

platelet count > 50,000

NO treatment

229

idiopathic thrombocytopenic purpura (ITP) treatment:

platelet count

prednisone

230

idiopathic thrombocytopenic purpura (ITP) treatment:

platelet count

IVIG aka Rhogam

231

idiopathic thrombocytopenic purpura (ITP) treatment:

RECURRENT episodes

splenectomy

232

idiopathic thrombocytopenic purpura (ITP) treatment:

no response to splenectomy

- romiplostim
- eltrombopag

233

uremia prevents platelets from working properly by

preventing degranulation

234

- renal failure
- normal platelet count w/ platelet-type bleeding

uremia-induced platelet dysfunction

235

the ristocetin test and VWF level will be what in uremia-induced platelet dysfunction

NORMAL

236

best INITIAL treatment for uremia-induced platelet dysfunction

desmopressin, dialysis, and estrogen

237

the first test to determine difference between clotting factor deficiency and a factor inhibitor Ab

mixing study

(aPTT will correct to normal w/ clotting factor deficiency)

238

- woman present w/ bleeding into her thigh after minor trauma
- aPTT is prolonged, and PT is normal
- mixing study does NOT correct the aPTT

what is the diagnosis?

factor 8 Ab

239

clotting factor deficiencies:

joint bleeding or hematoma in a MALE child

factor 8 deficiency

240

treatment for factor 8 deficiency:

minor and major (

- desmopressin

- factor 8 replacement

241

clotting factor deficiencies:

- joint bleeding or hematoma
- less common than factor 8 deficiency

factor 9 deficiency

242

treatment for factor 9 deficiency

factor 9 replacement

243

clotting factor deficiencies:

rare bleeding w/ trauma or surgery

factor 11 deficiency

244

factor 11 deficiency treatment

fresh frozen plasma w/ bleeding episodes

245

clotting factor deficiencies:

no bleeding

factor 12 deficiency

246

treatment for factor 12 deficiency

no treatment needed

247

best INITIAL test for all the clotting factor deficiencies

mixing study

248

test after mixing study for all the clotting factor deficiencies

check the specific factor

249

how does heparin-induced thrombocytopenia present?

drop in platelets of at least 50%

250

when does heparin-induced thrombocytopenia occur?

a few days AFTER start of heparin

251

is HIT exclusive to heparin only?

NO!! any form of heparin; this is an allergic reaction

252

MC clinical manifestation of HIT

THROMBOSIS

253

best INITIAL tests for HIT

- platelet factor 4 Abs
- heparin-induced antiplatelet Abs

254

best INITIAL therapy for HIT

- STOP THE HEPARIN
- use a direct thrombin inhibitor (argatroban, lepirudin, fondaparinux)

255

thrombophilia/hypercoagulable states:

- venous or arterial thrombosis
- elevated aPTT w/ normal PT
- spontaneous abortion
- false positive VDRL

lupus antiphospholipid syndrome

- lupus anticoagulant
- anticardiolipin Abs

256

best INITIAL test for lupus antiphospholipid syndrome

mixing study

257

MOST ACCURATE test for lupus anticoagulant

Russel viper venom test

258

treatment for lupus antiphospholipid syndrome

heparin followed by warfarin

259

thrombophilia/hypercoagulable states:

- skin necrosis w/ warfarin use
- venous thrombosis

protein C deficiency

260

test for protein C deficiency

protein C level

261

treatment for protein C deficiency

heparin followed by warfarin

262

thrombophilia/hypercoagulable states:

- MCC of thrombophilia
- venous thrombosis

factor V Leiden thrombophilia

263

test for factor V Leiden thrombophilia

factor V Leiden mutation test

264

treatment for factor V Leiden thrombophilia

heparin followed by warfarin

265

thrombophilia/hypercoagulable states:

- no change in aPTT with IV heparin bolus
- venous thrombosis

antithrombin deficiency

266

test for antithrombin deficiency

antithrombin 3 level

267

treatment for antithrombin 3 deficiency

large amounts of heparin or direct thrombin inhibitor followed by warfarin

268

mechanism of factor 5 thrombophilia

mutated factor 5 cannot be inactivated by protein C

(factor 5 mutation functions like protein C deficiency)