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My Step 2 CK > Hematology > Flashcards

Hematology Flashcards

1
Q

What medication is used to prevent recurrences of sickle cell crisis in a patient with sickle cell disease?

A

hydroxyurea

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2
Q

What is the best initial treatment for parvovirus B 19 (causing aplastic anemia)?

A

immunoglobulins

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3
Q

What is a common manifestation of sickle cell trait?

A

isosthenuria (defect in the ability to concentrate urine)

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4
Q

A patient presents with recurrent episodes of hemolysis and jaundice and has a family history of anemia/ hemolysis most likely suffers from ….

A

Hereditary Spherocytosis

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5
Q

What is the most accurate test for Hereditary Spherocytosis?

A

Osmotic fragility

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6
Q

How can you distinguish microcytic anemia due to iron deficiency form anemia due to thalassemia?

A

1) RDW (increased in iron; normal in thal)

2) iron studies (abnormal in iron; normal in thal)

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7
Q

What is the initial best test in a patient with macrocytic anemia?

A

peripheral blood smear (hypersegmented neutrophils)

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8
Q

What is the next best test in a patient with macrocytic anemia and hypersegemented neutrophils?

A

folate and B12 levels

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9
Q

What medication can cause folate deficiency?

A

1) phenytoin
2) sulfa drugs
3) Methotrexate

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10
Q

What are common causes of vitamin B12 deficiency? (5)

A

1) pernicious anemia
2) pancreatic insufficiency
3) inflammation of bowel (crohns, celiac, etc)
4) diphyllohothrium latum
5) blind loop syndrome (gastric bypass)

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11
Q

What is the most common neurologic symptoms in patient with vitamin B 12 deficiency?

A

Peripheral neuropathy

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12
Q

What is the best test to differentiate macrocytic anemia due to vitamin B12 deficiency versus folate deficiency?

A

methylmalonic acid level (elevated in B12; normal in folate)

used if B12 equivocal b/s transcobolamin is acute phase reactant

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13
Q

What is the confirmatory test for pernicious anemia?

A

1) Anti-intrinsic factor

2) anti-parietal cell antibodies

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14
Q

What electrolyte disturbance is a complication of vitamin B12 and folate replacement?

A

hypokalemia (K pushed into new cells)

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15
Q

What are common non-hematology manifestations in sickle cell disease? (5)

A

1) bilirubin gallstones (due to increased indirect bilirubin)
2) increased infections (encapsulated due to autosplenectomy)
3) osteomyelitis (Salmonella)
4) retinopathy
5) stroke

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16
Q

What is the most common cause of osteomyelitis in sickle cell disease?

A

Salmonella

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17
Q

What is the best initial test for sickle cell disease?

A

peripheral smear (Howell- Jolly bodies)

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18
Q

What is the most accurate test for sickle cell disease?

A

hemoglobin electrophoresis

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19
Q

What cells are found on blood smear in sickle cell disease?

A

Howell- Jolly bodies (precipitated remnants of nuclear material in RBC)

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20
Q

What cells are found in blood smear in G6PD deficiency?

A

Bite cells

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21
Q

What is the treatment for hereditary spherocytosis?

A
  1. chronic folic acid replacement (support RBC production)

2) splenectomy (stops hemolysis)

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22
Q

A patient presents with hemolytic anemia, spherocytosis on blood smear and a positive Coombs test most likely suffers from …

A

Autoimmune (Warm or IgG) hemolysis

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23
Q

What is the most accurate diagnostic test for Autoimmune (Warm or IgG) hemolysis?

A

Coombs test (detects antibodies)

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24
Q

What distinguishes hereditary spherocytosis from autoimmune (warm or IgG) hemolysis?

A
  1. Coombs test (negative in HS; postive in IgG)

2. Family hx of anemia (positive in HS; negative in IgG)

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25
Q

What is the best initial treatment for autoimmune (warm or IgG) hemolysis?

A

Steroids

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26
Q

What treatment can be used in a patient with recurrent hemolysis from autoimmune hemolysis who are already asplenic?

A

Rituximab

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27
Q

A patient presents with numbness or mottling of nose, ears, fingers, and toes that resolves when the body part is warmed most like suffers from ….

A

Cold agglutinin disease (IgM mediated)

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28
Q

What is the most accurate test for Cold agglutinin disease?

A

cold agglutinin titer

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29
Q

What is the best treatment for cold agglutinin?

A
  1. stay warm

2. Rituximab

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30
Q

What is the inheritance pattern of G6PD deficiency?

A

X-linked recessive

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31
Q

What are common drugs that induce hemolysis in patients with G6PD deficiency? (6)

A
  1. Dapsone
  2. Quinindine
  3. Sulfa (Bactrim)
  4. primaquine
  5. nitrofurantoin
  6. fava beans
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32
Q

What is the best initial test for G6PD deficiency?

A

blood smear (bite cells and Heinz bodies w/ methylene blue stain)

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33
Q

What is the most accurate test for G6PD deficiency?

A

G6PD level 1-2 months after actue episode

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34
Q

A child presents with hemolytic anemia, schistocytes on blood smear, thrombocytopenia and renal insufficiency most likely has …

A

Hemolytic Uremic Syndrome (HUS)

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35
Q

An adult presents with hemolytic anemia, schistocytes on blood smear, thrombocytopenia, renal insufficiency, fever and neuro disorder most likely suffers from …

A

Thrombotic Thrombocytopenic Purpura

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36
Q

What is the deficient in patients with HUS and TTP?

A

metalloproteinase ADAMTS 13

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37
Q

A patient presents with episodic dark urine with first urination, pancytopenia, iron deficiency anemia most likely suffers form …

A

paroxysmal nocturnal hemoglobinuria (PNH)

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38
Q

What is the defect in paroxysmal nocturnal hemoglobuinuria (PNH)?

A

defect in PIG-A gene leading to deficient decay accelerating factor (complement regulatory protein CD55 and 59)

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39
Q

What is the pathophysiology behind paroxysmal nocturnal hemoglobinuria?

A

hypoventilation during sleep –> acidosis –> RBCs are most sensitive to complement in acidosis leading to hemolysis and thrombosis

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40
Q

What is the most accurate test for paroxysmal nocturnal hemoglobinuria?

A

CD55 and CD59 levels/ flow cytometry (decreased)

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41
Q

What is the best initial treatment for hemolysis associated with paroxysmal nocturnal hemoglobinuria?

A

prednisone

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42
Q

What is the curative treatment for paroxysmal noctural hemoglobinuria?

A

bone marrow transplant

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43
Q

What is the mechanism of action of Eculizumab and what disease can it be used in?

A

inactivates C5; paroxysmal nocturnal hemoglobinuria

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44
Q

What is the most accurate test for aplastic anemia?

A

bone marrow biopsy

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45
Q

A patient presents with fatigue, anemia, increased infections and bleeding most likely suffers from …

A

aplastic anemia (pancytopenia not due to other cause)

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46
Q

What is the curative treatment for aplastic anemia?

A

allogeneic bone marrow transplantation (BMT) (if young and matched donor)

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47
Q

What is the alternative treatment for aplastic anemia if patient is older or does not have a donor match?

A

Antithymocyte globulin (ATG) and cyclosporine

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48
Q

What is the mutation associated with polycythemia vera?

A

JAK2 protein

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49
Q

A patient presents with hypertension, splenomegaly, bleeding, thrombosis, elevated hct (>60%), oxygen sats normal, and low erythropoietin most likely suffers from …

A

polycythemia vera

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50
Q

What is the most accurate test for polycythemia vera?

A

JAK2 mutation

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51
Q

A patient presents with elevated platelets (>1 million), erythromelalgia, thrombosis and bleeding most likely suffers from …

A

Essential Thrombocytosis

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52
Q

What is the best initial therapy for Essential Thrombocytosis?

A

hydroxyurea

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53
Q

What is erythromelalgia?

A

painful red hands in essential thrombocytosis

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54
Q

A patient presents with pancytopenia, bone marrow fibrosis, hepatosplenomegaly, and teardrop-shaped cells on blood smear most likely suffers from ..

A

Myelofibrosis

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55
Q

What type of cells are present in blood smear in myelofibrosis?

A

tear-drop shaped cells

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56
Q

What are medications used in myelofibrosis?

A

thalidomide and lenalidomide (TNF inhibitors)

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57
Q

What is the best initial test for acute leukemia?

A

blood smear (blasts)

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58
Q

What is cause of death in a patient with anemia?

A

myocardial infarction

if Hct > 20

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59
Q

How much should the hematocrit increase with each unit of packed RBC?

A

3 points per unit

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60
Q

What is considered microcytic anemia?

A

MCV < 80

iron deficiency, thalassemia, sideroblastic, lead poisoning, anemia of chronic disease

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61
Q

What is considered macrocytic anemia?

A

MCV > 100 (B12, folate, liver, alcohol)

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62
Q

What is megaloblastic anemia?

A

macrocytic anemia with hypersegmented neutrophils (>4 lobes)

b12, folate

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63
Q

What are causes of normocytic anemia (MCV 80-100)?

A
  1. blood loss

2. hemolysis

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64
Q

What are common cause of folate deficiency?

A
  1. decreased dietary intake (no green vegetables, no vitamin, alcoholic)
  2. increased cell turn over (sickle cell, pregnancy, eczema)
  3. phenytoin
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65
Q

What is the Schilling’s test used for?

A

to detect cause of vitamin B 12 (pancreatic insufficiency, diphyllobothrium latum)

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66
Q

A pt with microcytic anemia along with pica, brittle nails, spoon shaped nail, and glossitis most likely suffers from …

A

iron deficiency anemia

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67
Q

What are the iron studies for a patient with iron deficiency anemia?

A

high serum iron
high TIBC
low ferritin
high RDW

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68
Q

What is the cause of anemia of chronic disease?

A

inability to use iron sequestered in stores

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69
Q

What are the iron studies for a patient with anemia of chronic disease?

A

low serum iron
low TIBC
normal-high ferritin

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70
Q

What is the treatment for thalassemia major associated hemochromatosis?

A

defuroxamine (iron chelator)

no phlebotomy b/c getting transfused regularly

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71
Q

When would you use iron supplementation and erythropoietin for anemia of chronic disease?

A

due to renal disease caused by chemotherapy/radiation for cancer

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72
Q

What is the most common cause of iron deficiency anemia?

A
  1. GI blood loss

2. menstrual blood loss

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73
Q

What is the cause of sideroblastic anemia?

A

disorder of synthesis of hemoglobin characterized by iron trapped in mitochondria

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74
Q

What drugs can cause sideroblastic anemia?

A
  1. alcohol
  2. isoniazid
  3. chloramphenicol
  4. lead poisoning
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75
Q

What are the iron studies for sideroblastic anemia?

A

high serum iron
low TIBC
high ferritin

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76
Q

What is the most accurate diagnostic test for sideroblastic anemia?

A

Prussian blue stain (ringed sideroblasts- iron trapped in mitochondria)

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77
Q

What is a medical treatment that can be helpful with sideroblastic anemia?

A

pyridoxine (B6) (if have defect of ALA synthetase)

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78
Q

Which of the microcytic anemia diseases is more likely to be associated with target cells on peripheral smear?

A

Thalassemia

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79
Q

A 6 month old baby develops severe anemia, splenomegaly, jaundice and bone deformities most likely suffers from ….

A

Beta thalassemia major (Cooley anemia)

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80
Q

What are the iron studies for thalassemia?

A

normal iron
normal TIBC
normal ferritin
normal RDW

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81
Q

What are treatment options for thalassemia?

A
  1. blood transfusion (tx iron-overload w/ deferasirox)
  2. splenectomy (decrease frequency of transfusion)
  3. bone marrow transplant
82
Q

What is the best initial test for microcytic anemia?

A

iron studies

83
Q

What is aplastic anemia?

A

failure of all 3 cell lines of bone marrow with unknown etiology (pancytopenia)

84
Q

A pt presents with bleeding, infections and fatigue most likely suffers from …

A

aplastic anemia

85
Q

What is the treatment for aplastic anemia?

A
  1. bone marrow transplant (young, healthy w/ donor)

2. immunosuppression (antithymocyte globulin, cyclosporine, prednisone)

86
Q

Which microcytic anemia is the only one to have a high serum iron?

A

sideroblastic anemia

87
Q

A pt with normocytic anemia, jaundice, dark urine, elevated LDH, elevated indirect bilirubin, elevated reticulocyte count, low haptoglobin suggests …

A

hemolytic anemia (sickle cell disease, paroxysmal nocturnal hemoglobuinuria, hereditary spherocytosis, G6PD deficiency, drug-induced hemolysis, autoimmune hemolysis)

88
Q

What is the most accurate test for thalassemia?

A

hemoglobin electrophoresis

89
Q

What is the most accurate test for pancytopenia?

A

bone marrow

90
Q

What does the bone marrow biopsy show if pancyotpenia due to aplastic anemia?

A

hypoplastic and fat filled with no abnormal cells

91
Q

A patient presenting with pancytopenia symptoms with blasts present on smear most likely suffers from …

A

acute leukemia (AML or ALL)

92
Q

A pt with pancytopenia in adult with associated auer rods and myeloperoxidase most likely suffers from …

A

AML (acute myelogenous leukemia)

93
Q

What leukemia is associated with CALLA and terminal deoxynucleotidyl (TdT)?

A

ALL (CALLA is common ALL antigen)

94
Q

Which type of acute leukemia is associated with disseminated intravascular coagulation (DIC)?

A

M3 promyelocytic leukemia

95
Q

What is the treatment for acute leukemia?

A

daunorubicin and Ara-C (cytosine arabinoside)

96
Q

What additional treatment is used for M3 promyelocytic leukemia?

A

all trans retinoic acid

97
Q

What treatment is used as prophylaxis after acute episode of acute leukemia?

A

intrathecal methotrexate (leukemia usually recurs in spine)

98
Q

If acute leukemia relapses after chemotherapy, what is the treatment?

A

bone marrow transplantation

99
Q

A pt with leukemia develops headache, SOB, confusion and brain hemorrhage most likely suffers from …

A

leucostasis (white cell sludging in vessels)

100
Q

What is the treatment for leucostasis?

A

leukapheresis

101
Q

What leukemia is associated with the philadelphia chromosome (translocation btw 9 and 22?

A

CML (chronic myelogenous leukemia)

102
Q

What is the treatment for CML?

A

Imatinib (director inhibitor of tyrosine kinase)

103
Q

How do you distiguish between CML and leukomoid reaction?

A

Leukocyte alkaline phosphatase (functional status of WBC) (low in CML)

104
Q

How do you distinguish CML and CLL?

A

CBC with differential

neutrophils- CML; lymphocytes- CLL

105
Q

What is the staging for CLL?

A

0: lymphcytosis
1: lymphadenopathy
2: splenomegaly
3: anemia
4: thrombocytopenia

106
Q

What type of cells are seen on blood smear in a pt with CLL?

A

smudge cells

107
Q

What CD antigen is associated with CLL?

A

CD19

108
Q

What is the treatment of choice for CLL?

A

fludarabine

109
Q

What is the complications for myelodysplastic syndrome?

A
  1. progression to AML
  2. bleeding
  3. infection
110
Q

What are Pelger-Huet cells and what disease are they associated with?

A

bi-lobed neutrophils; myelodysplastic syndrome

111
Q

what is the common genetic defect associated with myelodysplastic syndrome and what is the treatment used if present?

A

5q deletion; azacitidine or decitabine

112
Q

What is the treatment for polycythemia vera?

A

phlebotomy

113
Q

What is the most accurate test for polycythemia vera?

A

Janus Kinase 2 (JAK-2)

114
Q

A pt presents with elevated hematocrit without hypoxia, elevated epo or carbon monoxide poisoning most likely suffers from …

A

Polycythemia Vera

115
Q

What is the treatment for essential thrombocytopenia?

A

hydroxyurea

116
Q

What is the most accurate test for essential thrombocythemia?

A

JAK-2 (Janus Kinase-2)

117
Q

A pt presents with anemia, elevated reticulocyte count, elevated LDH, elevated indirect bilirubin and decreased haptoglobin usually suffers from …

A

hemolytic anemia

118
Q

What is the function of haptoglobin?

A

transfer free hemoglobin (decreased in hemolysis)

119
Q

What makes the urine dark in intravascular hemolytic anemia?

A

free hemoglobin (can result in renal failure and ATN)

120
Q

What is the treatment for sickle crisis in sickle cell disease?

A

fluids, opioids, and oxygen

give antibiotics if have fever

121
Q

What complications are associated with Sickle Cell disease?

A
  1. osteomyelitis (Salmonella but S. aureus most common)
  2. aspetic necrosis of femoral head
  3. bilirubin gallstones
  4. skin ulcers
  5. isosthenuria
122
Q

What is the significant clinical manifestation of sickle cell trait?

A

isosthenuria (inability to concentrate urine)

123
Q

What causes a large drop in H/H in a patient with sickle cell disease acutely?

A

Parvovirus B 19

124
Q

What is the most accurate test for Parvovirus B19?

A

PCR for Parvovirus B 19 DNA

125
Q

What is another diagnostic test that can be used to detect Parvovirus B19 other than PCR?

A

IgM antibodies against Parvovirus B 19

126
Q

What is the treatment for parvovirus B 19 in sickle disease?

A

immunoglobulins

127
Q

What is the treatment for acute chest syndrome, stroke,priapism, visual disturbances, and CNS manifestations in sickle cell patient?

A

exchange transfusion (decrease number of sickling RBC)

128
Q

What is the diagnostic test for cold agglutinin disease?

A

Coombs test

129
Q

A pt w/ hx of autoimmune diseases presents w/ hemolytic anemia and cyanotic fingers/toes/nose/ear usually in setting of cold, can have spherocytosis most likely suffers from ….

A

Cold agglutinin disease

130
Q

What is the treatment for Cold agglutinin disease?

A

Steroids (splenectomy if continue to recur)

131
Q

What is the diagnostic test for Hereditary Spherocytosis?

A

osmotic fragility test (lysis in hypotonic solutions)

132
Q

What drugs can causes drug induced hemolytic anemia?

A
  1. penicillin
  2. methyldopa
  3. quinidine
133
Q

A pt presenting with recurrent episodes of hemolytic anemia, spherocytosis, splenomegaly, elevated MCHC and family hx of anemia most likely suffers from …

A

Hereditary Spherocytosis

134
Q

What is treatment for Hereditary Spherocytosis?

A

folate therapy (splenectomy to eliminate site of hemolysis)

135
Q

What type of hemolytic anemia is associated with elevated MCHC (mean corpuscular hemoglobin concentration)?

A

Hereditary Spherocytosis

136
Q

What is the treatment for paroxsymal nocturnal hemoglobinuria (PNH)?

A

steroids and anticoagulation

137
Q

A pt presents w/ intermittent dark urine in the morning and thrombosis of major veins (hepatic vein) most likely suffers from …

A

Paroxysmal noctural hemoglobinuria (PNH)

138
Q

What is the cause of paroxysmal noctural hemoglobinuria (PNH)?

A
  1. defect in PIG-A makes susceptible to acidotic environment (hypoventilation at night)
  2. deficient in decay accelerating factor (CD55/59)
139
Q

What are the diagnostic tests used to detect paroxysmal noctural hemoglobinuria (PNH)?

A
  1. levels of CD55 and CD59
  2. sugar-water test
  3. acidified-hemolysis (Ham) test
140
Q

What is the most common cause of death in pt with paroxysmal nocturnal hemoglobinuria (PNH)?

A

hepatic vein thrombosis

141
Q

What type of cells are seen in blood smear of pt with G6PD deficiency?

A

Bite cell (removal of Heinz body)

142
Q

What is a Heinz body and what disease is it associated with?

A

precipitated hemoglobin inclusions in RBC; G6PD deficiency

143
Q

When is it best to perform a G6PD level in suspected G6PD deficiency?

A

1 week after hemolytic event

144
Q

A pt presents with acute, severe hemolytic anemia after infection or use of sulfa drug/ dapsone/quinidine/ primiquine/ fava beans most likely suffers from..

A

G6PD deficiency

145
Q

What is the function of the plasma cell?

A

produces immunoglobulins

146
Q

What are the complications of over-active plasma cells?

A
  1. excessive Immunoglobulin
  2. osteoclastic activating factor (lytic lesions in bone; elevated calcium)
  3. Bence jones protein (renal insufficiency)
  4. hyperuricemia (cells turnover)
  5. anemia (b/c bone marrow making mainly immunglobulins instead of RBCs)
  6. amyloidodsis
147
Q

A pt presents with bone pain, monocloncal immunoglobulin, renal failure, normocytic anemia, increased infections w/ Pneumococcus and H. flu most likely suffers from…

A

Multiple Myeloma

148
Q

What is the most accurate test for multiple myeloma?

A

> 10% plasma cell

149
Q

A pt has an isolated monoclonal immunoglobulin spike without symptoms most likely suffers from …

A

Monoclonal Gammopathy of Uncertain Significance (MGUS)

150
Q

What is the treatment for multiple myeloma?

A
  1. < 70 y/o & healthy: autologous stem cell transplant (prepare marrow with vincristine, adriamycin, dexamethasone)
  2. > 70 y/o & healthy: thalidomide
  3. > 70 y/o %& unhealthy: melphalan
151
Q

What treatment can be used for relapsing multiple myeloma?

A

Bortezomib (proteasome inhibitor)

152
Q

A pt presents with enlarged, painless, firm, rubbery lymph node with night sweat, weight loss, and fevers most likely suffer from ….

A

Lymphoma

153
Q

What type of cells are seen in histologic examination of lymph nodes in Hodgkin Lymphoma?

A

Reed-Sternberg cells

154
Q

What is the initial diagnostic test for lymphoma?

A

excisional lymph node biopsy

155
Q

What diagnostic test is used to determine extent of disease of lymphoma?

A

CT scan and bone marrow

156
Q

What are the 4 stages of Lymphoma?

A

1: 1 lymph node
2: 2 lymph nodes on same side of diaphragm
3: lymph nodes on both sides of diaphragm
4: widespread disease w/ extralymphatic sites (liver, bone marrow)

157
Q

What are the treatment options for the different stages?

A

1 & 2: radiation

3, 4 & B symptoms: chemotherapy

158
Q

What type of chemotherapy is used for Hodgkin Lymphoma?

A

ABVD (adriamycin, bleomycin, vinblastine, decarbazine)

159
Q

What type of chemotherapy is used for non-Hodgkin Lymphoma?

A

CHOP (cyclophosphamide, hydroxy-adriamycin, oncovin, prednisone)

160
Q

What is the side effect of adriamycin?

A

cardiotoxicity

161
Q

What is the side effect of bleomycin?

A

pulmonary fibrosis

162
Q

What is the most common side effect of CHOP chemotherapy?

A

infertility

163
Q

What is the side effect of cyclophosphamide?

A

hemorrhagic cystitis

164
Q

What is the side effect of vincristine and vinblastine?

A

peripheral neuropathy

165
Q

What is the side effect of cisplatin?

A
  1. renal toxicity

2. oto-toxicity

166
Q

What is a unique treatment for non-Hodgkin lymphoma?

A

Rituximab (anti CD20 antibody)

167
Q

What is an important task before starting treatment with Rituximab?

A

Hep B and C testing (Rituximab can induce fulminant liver injury in pts with active hep B and C)

168
Q

A pt presents with mucous bleeding, petchiae, elevated bleeding time, elevated PTT with normal PT and platelet count most likely suffers from …

A

von Willebrand disease

169
Q

What is platelet type of bleeding?

A

superficial/ mucosal (skin, epistaxis, gingival, petechiae, menorrhagia etc)

170
Q

What is factor type of bleeding?

A

deep bleeding (joint, muscles, hematoma)

171
Q

A young healthy female presents with mucosal bleeding with thrombocytopenia, elevated megakaryotes and normal PT and PTT most likely suffers from …

A

ITP (immune thrombocytopenia purpura)

172
Q

What is the only cause of isolated thrombocytopenia in healthy person with normal spleen?

A

ITP (immune thrombocytopenia purpura)

173
Q

What is the initial treatment for ITP?

A

prednisone

174
Q

What is the treatment for ITP that continues to recurrent and very low platelets < 20,000?

A

splenectomy

175
Q

If a pt with ITP has life threatening bleeding with platelets <10,000, what is the best treatment?

A

IVIG or Rhogam

b/c raise platelets the fastest but stopping macrophage from consuming platelets

176
Q

What are the treatment options for von Willebrand disease?

A
  1. DDAVP (desmospressin; mild disease)
  2. factor 8 replacement (if DDAVP not effective)
  3. avoid aspirin
177
Q

What is the pathophysiology responsible for von Willebrand disease?

A

decreased vWf –> inability for platelets to adhere to endothelial lining

178
Q

What disorder can result in elevatd PTT with history of clotting (multiple 1st trimester miscarriages, DVTs, PEs)?

A

Antiphospholipid antibody (lupus anticoagulants)

179
Q

What coagulation study is elevated with heparin treatment?

A

PTT

180
Q

What coagulation study is elevated with warfarin treatment?

A

PT

181
Q

What is the triad of symptoms associated with hemolytic uremic syndrome?

A
  1. microangiopathic hemolytic anemia
  2. uremia
  3. thrombocytopenia
182
Q

What are the 5 symptoms associated with thrombotic thrombocytopenic purpura (TTP)?

A
  1. microangiopathic hemolytic anemia
  2. uremia
  3. thrombocytopenia
  4. CNS manifestation
  5. fever
183
Q

What is the pathophysiology associated with TTP (thrombotic thrombocyotpenic purpura)?

A

deficiency of ADAMTS 13 (protein that cleaves vWf) –> platelets stick too much clogging vessels

184
Q

What is the treatment for TTP?

A

plasmapharesis

185
Q

A male pt presents with delayed bleeding in form of hematoma and hemarthrosis with elevated PTT and normal PT and platelets most likely suffers from …

A

Hemophilia (A: factor 8 deficiency; B: factor 9 deficiency)

186
Q

What is the diagnostic study used in pt with hemophilia?

A
  1. mixing study (PTT corrects -> factor deficient; PTT doesn’t correct -> antibody)
  2. factor 8 and 9 levels
187
Q

What is the diagnostic test used to determine whether von Willebrand disease is due to dysfunctional von Willebrand factor?

A

ristocetin platelet aggregation (aggregate –> functional; no aggregation –> dysfunctional)

188
Q

What is the treatment for uremia induced thrombocyopenia?

A

DDAVP (desmopressin)

189
Q

What are the treatment options for hemophilia?

A
  1. DDAVP (desmopressin causes release of subendothelial factor 8: mild disease)
  2. replace factor (severe)
190
Q

A pt with hx of sepsis/ trauma present with bleeding and hemolysis that may lead to renal failure and altered mental status most likely suffers from …

A

DIC (disseminated intravascular coagulation)

due to consumption of platelet and clotting factor

191
Q

What are the most accurate diagnostic tests for DIC?

A
  1. d-dimer
  2. fibrin split products
    (have elevated PT and PTT; decreased platelet)
192
Q

What type of leukemia is associated with DIC?

A

promyelocytic leukemia (M3)

193
Q

What is the treatment for DIC?

A

fresh frozen plasma and platelet transfusion if severe

194
Q

What factors become deficient in setting of vitamin K deficiency?

A

factor 2,7,9 and 10

195
Q

A pt presents with oozing from venapuncture sites in setting of elevated PT followed by elevated PTT most likely suffer from …

A

vitamin K deficiency

196
Q

What is treatment for vitamin K deficiency?

A
  1. vitamin K (mild)

2. fresh frozen plasma (severe)

197
Q

What are the 3 causes of elevated PT and PTT?

A
  1. DIC
  2. vitamin K deficiency
  3. liver disease
198
Q

A pt presents with GI bleeding in setting of elevated PT followed by elevated PTT, without correction after vitamin K replacement most likely suffer from …

A

Liver disease causing coagulopathy

199
Q

What is the most common cause of hypercoagulable state?

A

factor V leiden

200
Q

A pt with low- normal PTT that does not increase with heparin use most likely suffers from …

A

AT III deficiency

201
Q

What should be used in a pt that requires anticoagulation in the setting of developing HIT (heparin induced thrombocytopenia)?

A

thrombin inhibtors (argatroban and lepirudin)

My Step 2 CK (21 decks)

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