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Flashcards in Hematology Deck (166):
1

What tests should be ordered initially if anemia is suspected?

1. Reticulocyte count
2. Haptoglobin
3. LDH
4. Total and direct bilirubin
5. TSH with T4
6. B12 and folate level
7. Iron studies
8. Urinalaysis with microscopic analysis
9. CBC with peripheral smear

2

A pt presenting with complaints of craving ice or dirt (Pica) suggest the pt is suffering from ...

Anemia

3

What is the best initial test for suspected anemia?

CBC with peripheral smear (focus on MCV and MCHC)

4

A pt presenting with fatigue/ loss of energy, pallor, pale conjunctiva, microcytic anemia, elevated platelets and history of blood loss most likely suffers from ...

Iron deficiency

5

What is the best initial diagnostic test and associated findings for iron deficiency?

iron studies
(low ferritin, high TIBC, low Fe, low Fe sat, elevated RDW bc new cells are progressively smaller)

6

What is the most accurate test for iron deficiency?

bone marrow biopsy (do not perform on CCS)

7

What is the treatment for iron deficiency?

ferrous sulfate orally (turns stools dark)

8

A pt presenting with fatigue/ loss of energy, pallor, pale conjunctiva, microcytic anemia and a history of rheumatoid arthritis/ end stage renal disease/ chronif infection/inflammatory or connective tissue disease most likely suffers from ...

Anemia of chronic disease

(normal/ increased iron in storage (ferritin/TIBC), but inability to process it into usable cells)

9

What is the best initial diagnostic test and associated findings for anemia of chronic disease?

iron studies
(high ferritin, low TIBC, low Fe, normal or low Fe sat)

10

What is the treatment for anemia of chronic disease?

correct the underlying disease

11

A pt presenting with possible fatigue/ loss of energy, microcytic anemia with very small MCV and the presence of target cells on smear most likely suffers from ...

Thalassemia

12

What is the most accurate diagnostic test for Thalassemia?

Hemoglobin Electrophoresis
(beta thal- elevated Hg2, HgF; alpha thal- normal)
(alpha thal best diagnosed with DNA sequencing)

13

A pt presenting with fatigue/ loss of energy, pallor, pale, conjunctiva, microcytic anemia and a history of alcoholism/ isoniazid use/ lead exposure most likely suffers from ...

Sideroblastic anemia
(iron builds up in mitochondria)

14

What is the best initial diagnostic test for sideroblastic anemia?

iron studies
(high Fe)

15

What is the most accurate diagnostic test for sideroblastic anemia?

prussian blue stain

(detect iron build up in mitochondria)

16

What is the best treatment for sideroblastic anemia?

1. major: remove toxin exposure
2. minor: pyridoxine replacement

17

What is the initial best step in management of a pt > 50 years old who presents with microcytic anemia?

Colonscopy (to rule out colon cancer)

18

What cause of anemia of chronic disease is the only form that will respond to erythropoietin?

end stage renal disease

19

A pt presenting with fatigue/ loss of energy, pallor, pale conjunctiva, neuro deficits (peripheral neuropathy), glossitis (smooth tongue), diarrhea and macrocytic anemia most likely suffers from ...

Vitamin B12 deficiency

20

What diabetic medication can result in vitamin B12 deficiency by blocking absorption of B12?

metformin

21

What is the best initial test for macrocytic anemia (vitamin B12 deficiency and folate deficiency)?

CBC with peripheral smear (look for hypersegmented neutrophils and oval cells)

(also order bilirubin, LDH and reticulocyte count)

22

A pt presenting with fatigue/ loss of energy, pallor, pale conjunctiva and macrocytic anemia with normal neuro exam most likely suffers from ..

Folate deficiency

23

What are the lab findings associated with the ineffective erythropoiesis associated with vitamin B12 deficiency? (3)

1. high LDL (RBC destruction)
2. high bilirubin (RBC destruction)
3. low reticulocyte count (RBC destruction)

24

What is the first sign/symptom to correct after starting B12 replacement therapy for vitamin B12 deficiency?

reticulocyte count ( neuro corrects last)

25

What is the most accurate test for suspected vitamin B12 deficiency?

B12 level (low; but can be falsely elevated bc it is an acute phase reactant)

26

If suspect vitamin B12 deficiency but the B12 level is normal or high, what is the next best test to detect vitamin B12 deficiency?

methylmalonic acid level (elevated in B12 deficiency)

27

What is the most accurate test for suspected folate deficiency?

folate level

28

After finding a low B12 level or elevated methylmalonic acid level in a vitamin B12 deficiency pt, what is the next best test to confirm the etiology of the B12 deficiency?

antiparietal cell antibodies and anti-intrinsic factor antibodes (to detect pernicious anemia)

(can use Schilling's test if antibodies not present)

29

What electrolyte imbalance can occur after treating vitamin B12 deficiency?

hypokalemia (low potassium)

30

A pt presents with sudden onset of weakness, fatigue, anemia, elevated indirect bilirubin, elevated reticulocyte count, elevated LDH, and decreased haptoglobin most likely suffers from ...

hemolytic anemia

31

What are the initial tests that should be performed in a pt with suspected hemolytic anemia?

1. CBC with peripheral smear
2. LDH
3. bilirubin
4. reticulocyte count
5. haptoglobin level

32

A pt presents with pain the in chest, back, thighs that is very severe in the setting of hemolytic anemia most likely suffers from...

sickle cell anemia

33

What is the best initial step in management of a sickle cell anemia pt?

oxygen, hydration (continuous NS IV), and pain meds

34

What is the next best step in management of a sickle cell anemic pt with a fever?

give antibiotics (ceftriaxone, levofloxacin, moxifloxacin) while ordering blood cultures, urinalysis, reticulocyte count, CBC and chest X-ray

35

What are the four indications for exchange transfusion in a sickle cell patient (to decrease hemoglobin S to 30-40%)?

1. eye (visual disturbance from retinal infarct)
2. lung (pulmonary infarct leading to pleuritic pain and abnormal X-ray)
3. penis (priapism from infarct of prostatic plexus veins)
4. brain (stroke)

36

A pt with sickle anemia presenting/ developing sudden drop in the hematocrit while on folate replacement most likely suffers from ...

Parvovirus B19 infection (could be folate deficiency if not on folate)

37

What is the most accurate diagnostic test for suspected parvovirus B19 infection?

DNA PCR for parvovirus

38

What is the treatment for parvovirus B19 infection in a sickle cell pt or hemoglobinopathy pt?

transfusions and IV immunoglobulins

39

What are three things that should be prescribed upon discharge for a sickle cell patient?

1. folate replacement
2. pneumococcal vaccine
3. hydroxyurea (to prevent further crisis if they happen > 4 times per year)

40

What is the mechanism of action of hydroxyurea in the treatment of sickle cell disease?

increases percentage of fetal hemoglobin (to dilute out sickle hemoglobin)

41

A pt presents with hematuria, isothenuria (inability to concentrate or dilute urine), urinary tract infections and possible visual disturbances in the setting of mild hemolytic anemia most likely suffers from ..

hemoglobin sickle cell (SC) disease
(sickle cell trait disease)

42

A pt presents with sudden onset of weakness, fatigue, anemia, elevated indirect bilirubin, elevated reticulocyte count, elevated LDH, decreased haptoglobin, spherocytes and a history of autoimmune disease/ CLL/ penicillin or sulfa drug use most likely suffers from ...

Autoimmune Hemolysis

43

What is the most accurate diagnostic test for autoimmune hemolysis?

Coombs test (detect IgG antibodies call warm antibodies)

44

What is the best initial treatment for autoimmune hemolysis?

steroids (prednisone)

45

What is the next best in management of a pt with auto immune hemolysis who is suffering from severe anemia not responsive to steroids or repeated blood transfusions?

IV immunoglobulins (to stop acute episode; predicts response to splenectomy)

46

What is the next best step in management for autoimmune hemolysis if a pt experiences recurrent episodes of hemolysis?

splenectomy

47

A pt presents with sudden onset of weakness, fatigue, anemia, elevated indirect bilirubin, elevated reticulocyte count, elevated LDH, decreased haptoglobin and has a history of syphillis, mycoplasma or EBV infection most likely suffers from ...

Cold-induced hemolysis (cold agglutinins)

(coombs negative; complement test positive)

48

What is the best treatment for cold-induced hemolysis (cold agglutinins)?

Rituximab (antibody against CD20 receptor on antibody producing cells)

49

A male pt presents with sudden onset of weakness, fatigue, severe anemia, elevated indirect bilirubin, elevated reticulocyte count, elevated LDH, decreased, haptoglobin, and has a history of fava bean ingestion/ infection/ use of oxidizine drug (sulfa, primaquine, dapsone) most likely suffers from ...

Glucose 6 Phosphate Dehydrogenase (G6PD) deficiency

50

What is the best initial test for glucose 6 phosphate dehydrogenase (G6PD) deficiency?

Heinz body (precipated hemoglobin in RBC membrane); bite cells on smear

51

What is the most accurate test for glucose 6 phosphate dehydrogenase (G6PD) deficiency?

G6PD level (about 2 months after acute disease)

52

What diagnostic test should be performed before starting treatment with dapsone?

G6PD level (to rule out glucose 6 phosphate dehydrogenase deficiency to avoid hemolysis)

53

A pt presents with sudden onset of weakness, fatigue, severe anemia, elevated indirect bilirubin, elevated reticulocyte count, elevated LDH, decreased haptoglobin and without significant history findings most likely suffers from ...

Pyruvate Kinase Deficiency
(similiar to G6PD deficiency with no known causes)

54

A pt presents with recurrent episodes of sudden onset of weakness, fatigue, anemia, elevated indirect bilirubin, elevated reticulocyte count, elevated LDH, decreased haptoglobin, spherocytes and has associated splenomegaly, bilirubin gallstones, elevated MCHC and positive family history most likely suffers from ...

Hereditary Spherocytosis

(genetic loss of ankryin & spectrin cytoskeleton of RBC membrane)

55

What is the treatment for Hereditary Spherocytosis?

splenectomy (give pneumococcal vaccine)

56

A pt presents with intravascular hemolysis (fatigue, anemia, elevated indirect bilirubin, retic count and LDH, decreased haptoglobin), elevated BUN and creatinine (renal failure), and thrombocytopenia after infection with E.Coli 0157:H7 most likely suffers from ...

Hemolytic Uremic Syndrome (HUS)

(triad: ART- autoimmune hemolysis, renal failure, thrombocytopenia)

57

A pt presents with intravascular hemolysis (fatigue, anemia, elevated indirect bilirubin, retic count and LDH, decreased haptoglobin), elevated BUN and creatinine (renal failure), thrombocytopenia, fever and neuro abnormalities , and a possible history of ticlopidine use most likely suffers from ....

Thrombocytopenic Purpura (TTP)

(pentad: FAT RN- fever, autoimmune hemolysis, thrombocytopenia, renal failure, neuro issues)

58

What should be avoided in the treatment of hemolytic uremic syndrome (HUS)? (2)

1. platelet transfusions
2. antibiotics for E.Coli

59

What is the treatment for hemolytic uremic syndrome (HUS) and thrombocytopenic purpura (TTP)?

plasmaphersis (if severe; otherwise self-limiting)

(replaces ADAMTS-13)

60

What diagnostic test is used for suspected hemolytic uremic syndrome (HUS) and thrombocyotpenic purpura (TTP)?

ADAMTS-13 level
(breaks down von Willebrand factor that causes platelets to bind into long chain)

61

A pt presenting with recurrent episodes of dark urine in the morning and pancytopenia with signs of hemolytic anemia most likely suffers from ..

Paroxysmal Nocturnal Hemoglobinuria (PNH)

62

What is the most common cause of death from paroxysmal nocturnal hemoglobinuria (PNH)?

large vessel venous thrombosis (portal vein thrombosis)

63

What is the most accurate test for paroxysmal nocturnal hemoglobinuria (PNH)?

CD55 and CD 59 antibody (decay accelerating factor)

64

What is the best initial treatment for paroxysmal nocturnal hemoglobinuria (PNH)?

steroids (prednisone)

65

What is the best treatment for transfusion dependent pts with severe paroxysmal nocturnal hemoglobinuria (PNH)?

eculizumab (inhibits C-5 and prevents complement activation)

66

A pregnant pt presenting with weakness/ fatigue, elevated liver function tests, fragments red cells on blood smear, thrombocyotpenia and normal coagulation studies most likely suffers from ...

HELLP syndrome (hemolysis, elevated liver function tests, low platelets syndrome)

67

What is the best treatment for HELLP in a pregnant pt after 34 weeks of gestations?

deliver baby

68

A pt presents with sudden onset shortness of breath with no clear reason, chest X-ray is normal, lungs are clear, blood appears brownish color, and there is a history of exposure to nitroglycerin/ amyl nitrate/ nitroprusside/ dapsone/ lidocaine/ bupivicaine/ tetracaine most likely suffers from ...

methemoglobinemia
(hemoglobin locked in oxidized state so can pick up oxygen)

69

What is the treatment for methemoglobinemia?

methylene blue

70

A pt develops shortness of breath and infilitrates on chest x-ray a few minutes after receiving a blood transfusion most likely suffers from ...

TRALI (transfusion-related acute lung injury)/ leukoagglutination reaction
(antibodies in donor blood against recipient white cells)

71

As soon as a pt receives a blood transfusion, he develops hypotension, shortness of breath, tachycardia but has normal LDH and bilirubin most likely suffers from ..

IgA deficiency

(signs of anaphylaxis)

72

What is the preventative treatment for IgA deficiency?

use donor blood from and IgA deficient donor or washed red cells

73

As soon as a pt receives a blood transfusion, he develops hypotension, shortness of breath, tachycardia, back and chest pain, dark urine, elevated LDH and bilirubin and decreased haptoglobin most likely suffers from ...

ABO incompatibility

(signs of acute hemolysis during transfusion)

74

A pt that becomes jaundice a few days after a transfusion and the hematocrit does not rise with the transfusion most likely suffers from ...

minor blood group incompatibility

(incompatibility to kell/duffy/lewis/kidd antigen or Rh incompatibility presents with delayed jaundice)

75

A pt who becomes febrile with a rise in temp of about 1 degree a few hours after transfusion without signs of hemolysis most likely suffers from ..

Febrile nonhemolytic reaction

(reaction against donor WBC antigens)

76

What is the preventative treatment for febrile non-hemolytic reaction?

using filtered blood transfusions in future to remove white cell antigens

77

A pt presenting with signs of pancytopenia (fatigue, bleeding, infections) acutely most likely suffers from ..

Acute Leukemia

78

What is the best initial test for suspected acute leukemia?

peripheral smear showing blasts

79

What is the most important prognostic finding in acute leukemia?

cytogenic abnormalities (specific karyotypic abnormlaities)

80

What is the next best step in management of a pt with high risk for relapse of acute leukemia after chemotherapy?

bone marrow transplantation

81

A pt presenting with signs of pancytopenia (fatigue, bleeding, infections) acutely with blasts and auer rods on smear most likely suffers from ...

Acute myelogenous leukemia (AML)

82

What is the best initial treatment for acute myelogenous leukemia?

idarubicin/ daunorubicin and cytosine arabinoside

83

What is the best initial treatment for M3 type (acute promyelocytic) leukemia?

all trans retinoic acid (ATRA) with idarubicin/ daunorubicin and cytosine arabinoside

84

What is the best initial treatment for acute lymphocytic leukemia (ALL)?

intrathecal methotrexate with idarubicin/ daunorubicin and cytosine arabinoside

85

What disease is highly associated with M3 type (acute promyelocytic) leukemia?

disseminated intravascular coagulation (DIC)

86

A pt presenting with shortness of breath, confusion, blurry vision with an elevated white blood cell count most likely is suffering from...

sludging of blood vessels (brain, eyes, lung) in acute leukemia

87

What is the best initial treatment for sludging associated with acute leukemia?

leukopheresis (to remove excess white cells) and hydroxyurea

88

An elderly pt presenting with pancytopenia, elevated MCV (macrocytosis), low reticulocyte count, macroovalocytes, normal B12 level, some blast and 2-lobed neutrophils (Pelger-Heut cells) most likely suffers from ...

Myelodysplasia

(can progress to acute leukemia; mild,slowly progressive preleukemia syndrome)

89

What is the best treatment for a pt with myelodysplasia and 5q minus syndrome?

lenalidomide (decreases transfusion dependence)

90

What is the major treatment for myelodysplasia?

transfusions as needed (to prevent death by infection or bleeding)

91

What myelodysplasia specific medications is the only one to increased survival?

azacitadine

92

A pt presents with elevated white cell count that is predominantly neutrophils, splenomegaly, satiety (feeling full) and a low leukocyte alkaline phosphatase most likely suffers from ...

Chronic Myelogenous Leukemia (CML)

93

What is the most accurate test for chronic myelogenous leukemia (CML)? (2)

1. philadephia chromosome by PCR of blood
2. BCR/ABL by fluorescence in situ hybridization (FISH)

94

What is the best initial treatment for chronic myelogenous leukemia (CML)?

imatinib (alternatives are dasatinib, nilotinib)

95

What is the curative treatment for chronic myelogenous leukemia (CML)?

bone marrow transplantation

96

A pt >50 y/o is found to have elevated white cell count on routine testing and the cells appear to be normal appearing lymphocytes most likely suffers from ...

Chronic lymphocytic leukemia (CLL)

97

What is the best initial test for suspected chronic lymphocytic leukemia (CLL)?

peripheral blood smear showing smudge cells (rupture nuclei of lymphocytes resembling squished jelly donuts)

98

What is the staging for chronic lymphocytic leukemia (CLL)?

stage 0: elevated white cell count only
stage 1: enlarged lymph nodes
stage 2: spleen enlargement
stage 3: anemia (abnormal IGs attack RBCS)
stage 4: low platelets (abnormal IGs attack platelets)

99

What is the best treatment for chronic lymphocytic leukemia (CLL) and when is its use indicated?

fludarabine with rituximab if stage is 2 or more

(use alemtuzumab an anti-CD52 agent if fludarabine fails)

100

What is the therapy used in chronic lymphocytic leukemia (CLL) that extends survivial?

fludarabine

101

What is the best initial therapy for hairy cell leukemia?

cladribine (2-CDA)

102

A middle aged pt (in 50s) presenting with pancytopenia (infections, bleeding, fatigue) and massive splenomegaly most likely suffers from ...

Hairy Cell Leukemia

103

A pt presenting with pancytopenia (infections, bleeding, fatigue), massive splenomeaglay, tear-drop shaped cells, JAK2 mutation most likely suffers from ...

Myelofibrosis
(fibrosis of marrow)

104

What is the best initial therapy for myelofibrosis if bone marrow transplantation is not possible?

lenalidomide/ thalidomide (with ruxolitinib- inhibits JAK2)

105

A pt presents with headache, blurred vision, dizziness, fatigue, pruritis after a hot bath/ shower, high hematocrit with low MCV in absence of hypoxia and a low erythropoietin level most likelysuffers from ...

Polycythemia Vera (Pvera)

106

What diagnostic test should be order in suspected polycythemia vera? (8)

1. CBC first (shows high hematocrit)
2. arterial blood gas second (rule out hypoxia)
3. erythropoietin level (low)
4. hematology consult
5. nuclear red cell mass test
6. JAK2 mutation
7. B12 (elevated)
8. leukocyte alkaline phosphatase (elevated)

107

What is the best initial test for polycythemia vera?

phelbotomy and daily aspirin
(can also use hydroxyurea to lower count)

108

What is therapy should be added to polycythemia vera treatment if thrombocythmia is present?

anagrelide

109

A pt presents with headache, visual disturbance, pain in hands (erythromelalgia), and elevated platelets most likely suffers from ...

Essential thrombocythemia (ET)

110

What is the treatment for essential thrombocythemia?

hydroxyurea, anagrelide and daily aspirin

111

A pt presents with bone pain causes by a fracture that occured under normal use and may have signs of renal failure/ infection most likely suffers from ...

Multiple Myeloma

112

What diagnostic test can be used to detect multiple myeloma?

1. skeletal survery (punched out osteolytic lesions)
2. serum protein electrophoresis (elevated monoconal antibody)
3. urine protein electrophoresis (bence jones protein)
4. peripheral smear (rouleaux formation of RBCs; elevated mean platelet volume)
5. calcium level (elevated)
6. beta-2 microglobulin level
7. BUN and creatinine (assess for renal failure)

113

What therapy can reverse renal dysfunction in multiple myeloma?

bortezomib

114

What diagnostic test is the best prognostic indicator for multiple myeloma?

beta-2 microglobulin level

115

What is the most accurate/ specific test for multiple myeloma?

bone marrow biopsy (high number of plasma cells)

116

What is the best initial treatment for multiple myeloma?

melphalan and steroids

(can add thalidomide, lenalidomide, bortesomib)
(treat hypercalcemia- hydration/diuresis, fractures- bisphosphonates, renal failure- hydration, anemia- epo, prophylax against infection- vaccines)

117

What is the most effective therapy for multiple myeloma and when is it indicated?

autologous stem cell bone marrow transplantation; if pt

118

An elderly pt (> 70) is found to have elevated total protein and an asymptomatic elevation of IgG on serum protein eletrophoresis most likely suffers from ..

Monoclonal Gammopathy of Unknown Significance (MGUS)

(no treatment)

119

A pt presents with blurry vision, confusion, headache, enlarged nodes and spleen and found to have elevated total protein most likely suffers from ...

Waldenstrom's Macroglobulinemia

(IgM overproduction)

120

What is the best initial tests for Waldenstrom's Macroglobulinemia?

1. serum viscosity level (elevated)
2. serum protein electrophoresis (elevated IgM level)

121

What is the best initial therapy for Waldenstrom's Macroglobulinemia?

Plasmaphersis
(can add rituximab, fludarabine, chlorambucil)

122

A pt presents with pancytopenia (fatigue, bleeding, infection) without an cytogenetic abnormalities or other possible causes most likely suffers from ...

Aplastic Anemia

(can be caused by chronic hep B/hep C)

123

What are the two possible treatments for aplastic anemia?

1. bone marrow transplant (if pt 50 and/or no match)

124

A pt presents with enlarged lymph nodes (likely cervical) and slowly develop symptoms of fever, weight loss, night sweats most likely suffers from ..

Lymphoma (Hodgkin's disease or non-Hodgkin's disease)

125

What histologic findings is specific to Hodgkin's lymphoma?

Reed-Sternberg cell (looks like owl eyes)

126

What is the best initial test for suspected lymphoma?

excisional lymph node biopsy

127

What diagnostic tests are important to perform to determine the staging of lymphoma? (3)

1. chest x-ray
2. CT scans with contrast (chest, abdomen, pelvis, head)
3. bone marrow biopsy

128

What are the stages of lymphoma?

stage 1: single lymph node
stage 2: two lymph nodes groups on 1 side of diaphragm
stage 3: lymph node involvement on both sides of diaphragm
stage 4: widespread disease

129

What is the treatment for localized (stage 1 or 2) lymphoma (lacking B symptoms)?

radiation and lower-dose chemotherapy

130

What is the treatment for advanced stages (3 or 4) lymphoma?

chemotherapy

131

What is the chemotherapy treatment for Hodgkin's Lymphoma?

ABVD (adriamycin/ doxorubicin, bleomycin, vinblastine, dacarbazine)

132

What is the chemotherapy treatment for non-Hodgkin's lymphoma?

CHOP (cyclophosphamide, hydroxyadriamycin, oncovin/ vincristine, prednisone

if anti-CD20 antigen present, add rituximab

133

A pt presents with recurrent epistaxis, gum bleeding with brushing teeth, petechia, vaginal bleeding, and bleeding is worse with aspirin and platelet count is normal but aPTT is elevated most likely suffers from ..

Von Willebrand's Disease

134

What are the most accurate tests for Von Willebrand's disease? (2)

1. von willebrand's factor
2. ristocetin cofactor assay (artificial endothelium to detect functionality of VWF)

135

What is the best initial treatment for Von Williebrand's disease?

Desmopression (DDAVP)

136

What is the next best treatment for Von Willebrand's disease if desmopression fails?

factor VIII replacement

137

What is the difference between platelet bleeding and factor type bleeding?

platelet: petechiae, epistaxis, purpura, gingiva, vagina, gums
factor: hemarthrosis, hematoma, bruises

138

A pt presents with platelet type bleeding (epistaxis, petechiae, gum bleeding, etc) and has a low platelet count (

Idiopathic Thrombocytopenic Purpura (ITP)

139

What is the best initial step in management for suspected idiopathic thrombocytopenic purprura (ITP)?

start prednisone (starting therapy before diagnosing is most important)

(if platelet count

140

What are the four diagnostic tests that should be performed in suspected idiopathic thrombocytopenic purpura (ITP)?

1. peripheral smear (large platelets)
2. sonogram (spleen size)
3. bone marrow (magakaryocytes)
4. antibodies to glycoprotein IIb/IIIa receptor

141

What is the best initial treatment for pt with idiopathic thrombocytopenic purpura (ITP) presenting with life-threatening bleeding (incranial hemorrhage, bowel bleeding)?

IV Immunoglobulin or RhoGAM

(if platelet count

142

What is the best initial treatment for pt with idiopathic thrombocytopenic purpura (ITP) with recurrent episodes of bleeding and low platelets?

Splenectomy

143

What is the best initial treatment for pt with idiopathic thrombocytopenic purpura (ITP) with recurrent episodes of bleeding and low platelets in which splenectomy did not help?

romiplostim/ eltrombopag (thrombopoietin analogs stimulate megakaryocytes)

144

A pt with renal failure presents with platelet type bleeding (petechiae, bleeding gums, epistaxis) but has normal platelet count, ristocetin test and von willebrand factor level most likely suffers from ..

Uremia-induced platelet dysfunction

(uremia prevents degranulation of platelets)

145

What is the best initial therapy for uremia-induced platelet dysfunction?

desmopressin (DDAVP), dialysis, estrogen

146

What is the mechanism of action and purpose of a mixing study?

aPTT will correct to normal with a clotting factor deficiency bc adding factor into serum; distinguishes between clotting factor deficiency and factor inhibitor antibody

147

A male child presents with joint bleeding/ hematoma and has a prolonged aPTT corrected with mixing study with normal PT most likely suffers from ...

Factor VIII deficiency

148

What is the best diagnostic test for factor VIII deficiency, factor IX deficiency, factor XI deficiency, and factor XII deficiency?

mixing study then specific factor level

(after coagulation studies)

149

A pt presents with rare bleeding with trauma or surgery and has a prolonged aPTT corrected by mixing study with normal PT most likely suffers from ...

Factor XI deficiency

150

What is the treatment for Factor VIII deficiency?

severe (

151

What is the treatment for Factor XI deficiency?

fresh frozen plasma (FFP) with bleeding episodes

152

A pt presents with joint bleeding/ hematoma, prolonged aPTT correctec by mixing study, normal PT and normal factor XI levels most likely suffers from...

Factor IX deficiency

153

A pt presenting with bleeding into joint after minor trauma, has a prolonged aPTT, normal PT and mixing study does not correct aPTT most likely suffers from ...

Factor VIII antibody

154

A pt develops a sudden drop of platelets (by at least 50%) after starting short-term anticoagulation and may have signs of thrombosis most likely suffers from ..

Heparin Induced Thrombocytopenia (HIT)

155

What are the two best initial tests for heparin-induced thrombocytopenia (HIT)?

1. platelet factor 4 antibodies
2. heparin-induced, antiplatelet antibodies

156

What is the best initial therapy for heparin-induced thrombocytopenia (HIT)?

stop heparin and use direct thrombin inhibitor (argatroban or lepirudin)

(dont switch from IV unfractionated heparin to low molecular weight)

157

A female presents with recurrent venous and/or arterial thrombosis, elevated aPTT with normal PTT, history of spontaneous abortions, and has positive VDRL most likely suffers from ...

Lupus Antiphospholipid sydrome (anticoagulant/ anticardiolipin antibodies)

158

What is the most accurate test for lupus antiphospholipid syndrome?

Russel viper venom (most accurate for lupus anticoagulant)

159

What is the treatment for lupus antiphospholipid syndrome?

heparin followed by warfarin

160

A pt presents with skin necrosis with the use of warfarin and venous thrombosis most likely suffers from ..

Protein C deficiency

161

What is the best diagnostic test for protein C deficiency?

protein C level

162

What is the treatment for protein C deficiency?

heparin followed by warfarin

163

What is the most common cause of thrombophilia presenting with venous thrombophilia?

Factor V Leiden Mutation

(mutated Factor V cant be inhibited by protein C)

164

What is the treatment for factor V leiden mutation?

heparin followed by warfarin

165

A pt presents with venous thrombosis and no change in aPTT with a bolus of heparin most likely suffers from ...

Antithrombin deficiency

166

What is the best test and treatment for antithrombin deficiency?

antithrombin III level; large amounts of heparin/ direct thrombin inhibitor followed by warfarin