Hematology/Oncology Flashcards

1
Q

compensatory mechanisms in anemia

A

increased CO
increased extraction ratio
RIGHT shift on Oxy-Hb dissociation curve (increased 2,3 BPG)
expansion of plasma volume

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2
Q

general indications for blood transfusion

A
  1. Hb < 7 g/dL

2. pt requires increased O2 carrying capacity - i.e. pt with CAD or cardiopulm dz

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3
Q

what is pseudoanemia?

A

decrease in Hb and Hct secondary to dilution (acute volume infusion or overload)

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4
Q

MCC of death due to anemia

A

myocardial ischemia

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5
Q

formula for converting Hb to Hct

A

Hb x 3 = Hct

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6
Q

how does 1 Unit of packed RBCs affect Hb and Hct levels?

A

increases Hb level by 1 point and Hct by 3 points

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7
Q

if pt has low Hb and Hct, what are the next tests to determine cause of anemia?

A

reticulocyte count

mean corpuscular volume

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8
Q

reticulocyte index > 2%

A

excessive RBC destruction and blood loss - the BM is responding to increased RBC requirements

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9
Q

reticulocyte index < 2%

A

inadequate RBC production by BM

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10
Q

how do you administer Packed RBCs?

A

mixed with Normal Saline and infuse 1 unit over 90-120 minutes; check CBCs once complete

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11
Q

what does fresh frozen plasma contain?

A

all of the clotting factors - no WBCs, RBCs or platelets

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12
Q

what is FFP given for ? (3)

A
  1. high PT/PTT
  2. coagulopathy
  3. deficiency of clotting factors
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13
Q

how do you assess response of FFP?

A

look at PT and PTT

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14
Q

what is cryoprecipitate and what is it used for?

A
  • contains factor VIII and fibrinogen

- used for: hemophilia A, DIC and vWD

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15
Q

1 unit of platelets raises platelet count by how much?

A

10 000

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16
Q

what is the ONLY time you use whole blood?

A

massive blood loss

- in a ratio of 1:1:1 of platelets:FFP:PRBCs and warmed

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17
Q

a pt who recently received a blood transfusion suddenly develops fever, chills, NV, flank pain, chest pain and dyspnea - what should you be worried about?

A

intravascular hemolysis due to ABO-mismatched blood

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18
Q

management of acute hemolytic reaction due to ABO-mismatched blood

A
  1. stop transfusion
  2. aggressive fluid replacement to avoid shock/renal failure
  3. Epi. for anaphylaxis
  4. dopamine/NE to maintain BP
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19
Q

a pt who received a blood transfusion 2 weeks ago develops mild fever, jaundice and anemia - what should you consider?

A

delayed hemolytic transfusion reaction (Extravascular hemolysis) due to mismatched minor RBC antigens

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20
Q

what is the next step in assessing anemia if the reticulocyte index is < 2?

A

exam smear and RBC indices- ie. MCV

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21
Q

differential diagnosis of microcytic anemia (MCV < 80)

A

iron deficiency
anemia of chronic disease
thalassemias
ring sideroblastic anemias

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22
Q

differential diagnosis of macrocytic anemia (MCV > 80)

A

vit B12 /folate deficiency
liver disease
stimulated erythopoiesis

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23
Q

differential diagnosis of normocytic anemia (MCV 80-100)

A
aplastic anemia
BM fibrosis
tumor
anemia of chronic disease
renal failure
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24
Q

what do you need to R/O in elderly pt with iron deficiency anemia?

A

colon cancer

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25
Q

major causes of iron deficiency anemia

A
  1. chronic blood loss - menstrual or GI loss
  2. dietary deficiency
  3. increased requirements - i.e. growth, pregnancy, lactation
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26
Q

what is the most reliable test for diagnosing iron deficiency anemia?

A

decreased serum ferritin (below 10 ng/ml)

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27
Q

what lab findings are characteristic of iron deficiency anemia?

A

low serum ferritin
low serum iron
high TIBC and low TIBC saturation
high RDW

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28
Q

what can cause a falsely elevated serum ferritin

A

malignancy and inflammatory states - ferritin is also an acute phase reactant

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29
Q

what is the gold standard for dx. iron deficiency anemia?

A

bone marrow biopsy

- rarely performed unless no

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30
Q

first step in tx. of iron deficiency anemia?

A

oral iron replacement w/ ferrous sulfate

- give trial to menstruating woman but in everyone else, attempt to determine point of chronic blood loss

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31
Q

young patient is brought in with massive hepatosplenomegaly, expansion of marrow space, growth retardation and failure to thrive; CBC reveals microcytic anemia - dx?

A

B-thalessemia major (aka. Cooley’s anemia)

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32
Q

how do you dx. B-thalassemia major?

A

hemoglobin electrophoresis –> HbF and HbA2 are elevated

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33
Q

Tx. of B-thalessemia major?

A

frequent PRBCs transfusios with desferrioxamine to prevent iron overload

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34
Q

if you suspect iron deficiency anemia, but it does not respond to iron - what should you do next?

A

Hb electrophoresis to R/O thalassemias

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35
Q

what happens if you have deletion of 2 alpha globin loci?

A

mild, microcytic hypochromic anemia
common in African-Americans
usually asx. w/ no tx. needed

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36
Q

pt comes in with hemolytic anemia, splenomegaly, and significant microcytic hypochromic anemia; Hb electrophoresis shows Hb H - what should you consider?

A

Hb H disease - mutation/deletion of 3 alpha globlin loci

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37
Q

what is Hb H

A

tetrads of B-globin chains

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38
Q

what is Hb-Barts?

A

tetrads of Y-globin chains form when there is deletion or mutationof all four alpha-globin loci resulting in fatal hydrops fetalis

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39
Q

which alpha-thalassemia requires tx and what is the tx?

A

HbH disease - tx. w/ frequent PRBC transfusions w/ desferrioxamine

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40
Q

what are the iron studies findings in thalassemia pts?

A

normal/high serum ferritin
normal/high serum iron
normal TIBC
normal/high RDW

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41
Q

what are the iron studies findings in sideroblastic anemia pts?

A

increased serum iron and ferritin
normal/low TIBC
TIBC saturation is normal/high

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42
Q

what are some acquired causes of sideroblastic anemia?

A

drugs - chloramphenicol, alcohol, INH
lead exposure
collagen vascular disease
myelodysplastic syndromes

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43
Q

what are the hereditary causes of sideroblastic anemia?

A

defect in ALA synthase

abnormal vit B6 metabolism

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44
Q

what are the iron studies findings in anemia of chronic disease?

A

low serum Fe
low TIBC
low serum transferrin
high serum ferritin

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45
Q

characteristic finding in aplastic anemia

A

BM is empty with no precursor cells leading to pancytopenia (anemia, neutropenia and thrombocytopenia)

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46
Q

causes of aplastic anemia

A
idiopathic
radiation exposure
drugs - chloramphenicol, NSAIDs, alcohol, sulfonamides, gold, carbamazepine
viral infection
chemicals - benzene, insecticides
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47
Q

how do you definitively dx. aplastic anemia?

A

bone marrow biopsy

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48
Q

what is the definitive tx. of aplastic anemia?

A

bone marrow transplantation

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49
Q

what two reactions is vit B12 necessary for?

A

conversion of homocysteine to methionine

conversion of methylmalonyl CoA to succinyl CoA

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50
Q

what is the characteristic neuropathy in vit B12 deficiency?

A

demyelination of posterior columns, lateral corticospinal tracts and spinocerebellar tracts

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51
Q

what kind of cancer are pts with pernicious anemia at higher risk for?

A

intestinal type - gastric ca.
gastric carcinoid
- perform periodic stool testing for FOB

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52
Q

diagnostic findings in vit. B12 deficiency

A
  • hypersegmented neutrophils
  • serum vit B12 level < 100
  • serum methylmalonic acid and homocysteine levels elevated
  • ab’s against IF
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53
Q

what Schilling test result implies dietary deficiency of vit B12

A

after giving radioactive Vit B12, if urine levels are > 5%

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54
Q

what do you do if in the Schilling test, urine levels are below normal (<5%)?

A

give radioactive vit B12 w/ IF –> if they go up after, then you know you have IF deficiency; if they do not go up, it is due to malabsorption

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55
Q

tx. of vit B12 deficiency

A

cyanocobalamin IM once per month

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56
Q

serum methylmalonic acid levels are only increased in what deficiency?

A

vit B12

- this test allows you to tell it apart from folate deficiency

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57
Q

what general lab findings do you see in hemolytic anemia?

A

elevated reticulocyte count
elevated LDH and bilirubin
decreased haptoglobin
decreased Hb/Hct

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58
Q

what kind of cells can be seen on PB smear in thalasemia?

A

target cells

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59
Q

what kind of cells on smear are characteristic of TTP, DIC, prosthetic heart valves and hemolytic anemias?

A

schistocytes

helmet cells

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60
Q

what characteristic clinical features (in addition to those of anemia) can be seen in hemolytic anemias?

A

jaundice

dark urine - esp. IV processes

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61
Q

what kind of cells are seen on smear in intravascular hemolysis)

A

schistocytes

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62
Q

which cells suggest extravascular hemolysis?

A

spherocytes

helmet cells

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63
Q

what types of cells characterize G6PD deficiency?

A

heinz bodies

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64
Q

what vitamin should be supplemented in hemolytic anemia?

A

folate

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65
Q

sudden drops in Hct are usually due to..

A

parvovirus B19 infection

folate deficiency - acute aplasia

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66
Q

what should you suspect in a young blank male who presents to you with painless hematuria?

A

sickle cell trait

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67
Q

what is really the only clinical feature seen in sickle cell trait?

A

isosthenuria and increased rate of UTIs

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68
Q

what does the prognosis of sickle cell disease depend on?

A

the frequency of vaso-occlusive crises

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69
Q

MC clinical manifestation of sickle cell disease vasoocclusive crises

A

painful crises involving bone - multiple sites

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70
Q

what is usually the first manifestation of sickle cell disease?

A

hand-foot syndrome (dactylitis) - painful swelling of dorsa of hands and feet seen in infancy

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71
Q

splenic sequestration crises

A

in sickle cell dz, sudden pooling of blood into spleen results in rapid development of splenomegaly and hypovolemic shock; potentially fatal

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72
Q

what is priapism due to in sickle cell crises?

A

infarction of prostatic plexus of veins

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73
Q

tx. that can potentially prevent recurrence of priapism in sickle cell dz

A

hydralazine
nifedipine OR
antiandrogen (stilbestrol)

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74
Q

what types of infections are sickle cell pts susceptible to?

A

encapsulated bacterias - SHiNS

salmonella osteomyelitis

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75
Q

screening test for sickle cell trait/disease

A

sickle cell prep - blood smear with chemical added to remove O2 from hemoglobin - looks for sickled cells

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76
Q

diagnostic test for sickle cell dz

A

hemoglobin electrophoresis

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77
Q

what should sickle cell pts receive prophylactically until age 6?

A

penicillin

folic acid supplments in definitely

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78
Q

which drug is used to enhance HbF levels and reduce incidence of painful crises in sickle cell dz?

A

hydroxyurea

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79
Q

which conditions in sickle cell dz warrant consideration for blood transfusion?

A

acute chest syndrome
priapism unresponsive to other measures
cardiac decompensation
stroke

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80
Q

causes of spherocytosis

A
hereditary
G6PD deficiency
ABO incompatability
hyperthermia
autoimmune hemolytic anemia
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81
Q

diagnostic test for hereditary spherocytosis

A

osmotic fragility test - RBC burst in hypotonic saline

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82
Q

lab findings in hereditary spherocytosis

A

elevated reticulocyte count
elevated MCHC
direct Coombs test result is negative

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83
Q

what is the Tx. for hereditary spherocytosis?

A

splenectomy

- vaccinate against pneumo, H.flu and meningococcus several weeks before

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84
Q

what nationality of people are primarily affected by G6PD deficiency?

A

African-Americans - mild form

Mediterranean descent - severe form

85
Q

precipitants of hemolytic episode in G6PD deficiency?

A
sulfonamides/sulfur containing drugs - TMP/SMX
primaquine (antimalarials)
nitrofurantoin
dimercaprol
fava beans
infections (MCC)
86
Q

CF of G6PD deficient pts

A

hemolytic episodes marked by jaundice and dark urine that is induced by either an infection or a drug

87
Q

characteristic cells seen in G6PD pts

A

Heinz bodies - Hb precipitates

Bite cells

88
Q

how do you diagnose G6PD deficiency?

A

measurement of G6PD levels - do not do this during a crises but rather when pt is asymptomatic

89
Q

what is the “Warm” autoimmune hemolytic anemia?

A

IgG ab that binds to RBC mbs resulting in extravascular hemolysis in the spleen

90
Q

what is “cold” autoimmune hemolytic anemia?

A

IgM ab binds to RBC mb at temp. between 0-5 C producing complement activation and intravascular hemolysis primarily in liver

91
Q

which infections are associated with cold AIHA?

A

mycoplasma pneumonia

infectious mononucleosis

92
Q

cold agglutinin disease

A

cyanosis of ears, nose, fingers and toes in cold temperatures

93
Q

how do you diagnose autoimmune hemolytic anemia?

A

Direct Coomb’s test

94
Q

Tx. of warm AIHA

A

steroids - prednisone in divided doses

- splenectomy if pt doesnt respond to steroids

95
Q

Tx. of cold AIHA

A

avoid exposure to cold

RBC transfusion if absolutely necessary

96
Q

what is the deficiency in paroxysmal nocturnal hemoglobinuria?

A

deficiency of anchor proteins that link complement inactivating proteins to RBC mbs resulting in unusual susceptibility to complement mediated lysis of RBCs, WBCs and platelets

97
Q

in addition to anemia (pancytopenia) and hemolysis, what CF can be seen in paroxysmal nocturnal hemoglobinuria?

A

thrombosis of venous systems ex. Budd Chiari

98
Q

most sensitive and specific test for PNH

A

flow cytometry of anchored cell surface proteins - CD55 and CD59 } DAF

99
Q

Ham’s test

A

test for PNH - cells incubated in acidic serum, triggers alternative complement pathway resulting in lysis of PNH cells

100
Q

sugar water test

A

test for PNH - PNH cells lyse in sucrose

101
Q

Tx of PNH

A

glucocorticoids - prednisone (DOC)

- alternates: BM transplant, eculizumab

102
Q

CF of idiopathic thrombocytopenic purpura

A

minimal cutaneous and mucosal bleeding
low platelet counts
generally healthy

103
Q

what do you suspect in a young female whose CBC shows a platelet count of 15 000; she reports no symptoms.

A

idiopathic thrombocytopenic purpura

104
Q

definite dx. of ITP

A

BM biopsy - shows increased megakaryocytes

105
Q

first line tx. in ITP

A

steroids -prednisone

106
Q

how can you tx. pts with ITP who have platelet count < 10 000 or develop a life threatening bleed?

A

IVIG

107
Q

two new drugs approved for ITP in splenectomy-resistant pts?

A

romiplastim

eltrombopag

108
Q

Type 1 HIT

A

heparin causes platelet aggregation within 48 hrs of starting heparin tx; no tx is needed

109
Q

Type 2 HIT

A

heparin induces antibody-mediated injury to platelet; seen in 3-12 days after heparin tx; d/c heparin immediately

110
Q

pt comes in with signs of hemolytic anemia, thrombocytopenia, a rising Creatinine level, fever and fluctuating neurologic signs - what should you consider?

A

TTP

111
Q

How do you Tx. TTP?

A

plasmaphoresis (large volume)

112
Q

decrease in platelet count by 50% after heparin suggests ?

A

HIT

113
Q

main complication of HIT

A

venous thrombosis with DVT and PE

114
Q

diagnostic tests for HIT

A

antiplatelet factor IV antibody

serotonin release assay

115
Q

Tx. of HIT

A

stop heparin

116
Q

disorder of platelet adhesion due to deficiency of platelet glycoprotein GP1b-IX

A

Bernard Soulier disease

117
Q

disorder of platelet aggregation due to deficiency in platelet glycoprotein GPIIb/IIIa

A

Glanzmann’s thrombasthenia

118
Q

what is the role of vWF?

A
  1. mediates adhesion of platelets to injured vessel wall (GP1b/IX and subendothelium)
  2. carrier of factor VIII in blood
119
Q

MC inherited bleeding disorder

A

vW disease

120
Q

how can you diagnose vW disease?

A

prolonged bleeding time but normal platelets
aPTT may be prolonged
decreased plasma vWF and factor VIII
reduced ristocetin-induced platelet aggregation

121
Q

how can you tell vW dz apart from hemophilia A?

A

bleeding in vWD is much milder than in hemophilia, factor VIII levels are not reduced as much and spontaneous hemarthrosis do not occur

122
Q

first line tx. for vWD

A

desmopressin (DDAVP) - induces endothelial cells to secrete vWF

123
Q

what do you do if pt with vWD does not respond to DDAVP?

A

try factor VIII concentrates

- also given during major trauma or surgery

124
Q

how much factor VIII do you need before signs of bleeding appear?

A

> 10% of normal factor VIII and patients are asymptomatic

125
Q

lab findings in hemophilia A

A

prolonged PTT
low factor VIII levels
- everything else is normal

126
Q

mixing study results in hemophilia A

A

if plasma from a hemophiliac pt is mixed with normal plasma, the PTT normalizes –> this is indicative of a factor deficiency

127
Q

how can you prevent recurrent hemarthroses in hemophilia?

A

prophylactic factor VIII concentrates

synovectomy / radiosynovectomy

128
Q

Tx of acute hemarthrosis in hemophilia

A

analgesia - codeine +/- acetaminophen (avoid NSAIDS and aspirin)
immobilization, ice packs etc

129
Q

mainstay of therapy for hemophilia

A

factor VIII concentrate

- for acute bleeding episodes and before surgery/dental work

130
Q

what drug may be helpful in mild hemophilia A?

A

DDAVP - can increase factor VIII level four-fold

131
Q

what is the defect in hemophilia B?

A

factor IX

132
Q

(1) normal PT
(2) normal PTT
(3) normal bleeding time

A

(1) 11-15 sec
(2) 25-40 sec
(3) 2-7 min

133
Q

lab findings in DIC

A
Increased:
- PT, PTT, bleeding time and thrombin time
- high d-dimers
Decreased:
- fibrinogen level
- platelet count
134
Q

coagulopathy due to liver disease

A

PT and PTT are elevated

- all else is normal

135
Q

coagulopathy due to vitamin K deficiency

A

PT is initially prolonged, PTT prolongation follows

136
Q

how do you Tx. DIC?

A

must take care of underlying cause

- things like FFP, platelet transfusions and cryoprecipitate can buy you time

137
Q

how can you confirm the diagnosis of vitamin K deficiency?

A

correction of PT/PTT after admin for vit. K

138
Q

how do you correct vitamin K deficiency coagulopathy?

A

oral or parenteral vitamin K

FFP if severe bleeding is present

139
Q

what type of bleeding is most common in coagulopathy due to liver disease?

A

GI bleeding - ex. varices

140
Q

tx for coagulopathy due to liver disease if there is prolonged PT/PTT or bleeding..

A

FFP

141
Q

what is the role of protein C

A

inhibitor of factors V and VIII

- deficiency leads to unregulated fibrin synthesis

142
Q

what is the role of protein S

A

protein S is a cofactor for protein C - so a deficiency leads to decreased protein C activity

143
Q

factor V leiden mutation

A

activated protein C resistance - protein C can no longer inactivate factor V

144
Q

MOA of heparin

A

potentiates antithrombin III to inhibit clotting factors IIa and Xa; prolongs PTT (intrinsic pathway)

145
Q

adverse effects of heparin

A
bleeding
transient alopecia
osteoporosis
HIT
rebound hypercoagulability
146
Q

C/I to heparin

A
previous HIT
active bleeding
recent surgery on eyes, spine or brain
hemophilia/thrombocytopenia
severe HTN
147
Q

how can you reverse the effects of heparin?

A

standard hep has short half life, so its effects stop w/in like 4 hours; but if you need to you can give PROTAMINE SULFATE

148
Q

benefits of LMWH

A

given subcutaneously (not IV)
no monitoring needed
less side effects
- better for outpatient use

149
Q

MOA of LMWH

A

inhibit factor Xa (less IIa and platelet inhibition)

150
Q

what is the therapeutic INR you want on warfarin?

A

2-3

151
Q

adverse effects of warfarin

A

hemorrhage
skin necrosis
teratogenic
should not give to alcoholics or pts prone to falls (risk of IC bleed)

152
Q

mechanism of warfarin skin necrosis

A

inhibits protein C and S first, leading to hypercoagulable state with thrombus formation in first few days of admin; MC in pts with congenital protein C and S deficiency to start

153
Q

what do you do if your INR is too high while pt is on warfarin?

A

lower/omit dose; if very high, give vitamin K

- if severe bleeding, give pt FFP

154
Q

MOA of clopidogrel

A

blocks binding of ADP to platelet receptor P2Y12; reduces platelet activation and aggregation

155
Q

indications for use of clopidogrel

A

pretreatment for pts before PCI (600 mg)
after PCI w/ stend placement for 12 mths (75 mg)
peripheral artery disease
acute coronary syndromes

156
Q

if a patient is taking clopidogrel, which other drugs should you avoid?

A

PPIs

157
Q

what should you do in any women over age 30 presenting with a breast mass?

A

biopsy - conservative approach

158
Q

a young woman presents with a round, moveable breast mass which changes in size over course of menstrual cycle - what is this most likely?

A

fibroadenoma

159
Q

a woman presents with bilateral breast pain that cycles with hormonal changes…

A

fibrocystic changes

160
Q

what should you always order before beginning tamoxifen therapy for breast cancer?

A

echocardiogram - high risk of cardiotoxicity (esp. if EF < 55%)

161
Q

diagnostic criteria of MGUS

A

IgG spike < 3 g/dL
< 10% plasma cells in BM
Bence-Jones proteinuria < 1 g/24 hrs
no end organ damage

162
Q

a pt presents with low Hb, high calcium, high serum protein and poor renal function - what should you suspect?

A

multiple myeloma

163
Q

CRAB mnemonic for multiple myeloma

A

hyperCalcemia
Renal failure
Anemia
lytic Bone lesions

164
Q

diagnostic criteria for mutliple myeloma

A
  • > 10% plasma cells in BM
  • one of the following:
  • M protein in serum or urine
  • lytic bone lesions
165
Q

what does a peripheral smear look like in someone with multiple myeloma?

A

RBCs in rouleaux formation

166
Q

lab findings in multiple myeloma

A
hypercalcemia
elevated total protein, but lower albumin
elevated ESR
elevated BUN/Cr
pancytopenia may be present
167
Q

Tx. of choice for mutliple myeloma

A

autologous hematopoietic stem cell transplantation

168
Q

how do you tx. multiple myeloma pts who are not transplant candidates?

A

systemic chemotx with alkylating agents: melphalon, prednisone, thalidomide

169
Q

a pt comes in fatigue, weight loss, LAD, splenomegaly, anemia; lab tests show IgM > 5 g/dL and Bence Jones proteinuria - dx?

A

Waldenstrom’s macroglobulinemia

170
Q

Tx. of hyperviscosity syndromes

A

plasmaphoresis

chemotherapy

171
Q

pt presents with painless LAD of the cervical LNs along with fever, night sweats and recent weight loss; he also notes pruritus - dx to consider?

A

Hodgkin’s lymphoma

172
Q

how do you diagnose Hodgkin’s lymphoma?

A

LN biopsy showing Reed-Sternberg cells surrounded by inflammatory cells

173
Q

what tests do you do to determine staging in HL?

A

CT scan, CXR and BM biopsy

174
Q

tx. of stage 1, 2 and 3A Hodgkin’s lymphoma

A

radiation therapy only

175
Q

tx. of stage 3B and 4 Hodgkin lymphoma

A

chemotherapy = ABVD

- adriamycin, bleomycin, vincristine, dacarbazine

176
Q

what lab test can sometimes correspond with disease activity in Hodgkin’s lymphoma?

A

elevated ESR

increased LDH = adverse prognosis

177
Q

RFs for NHL

A
HIV/AIDs
immunosuppression
viral infections - EBV, HTLV1, HCV
h.pylori gastritis
autoimmune disease
178
Q

what type of NHL presents are peripheral LAD and a t(14:18) translocation

A

follicular NHL

179
Q

what infection and what translocation are associated with Burkitt’s lymphoma?

A

African variety = EBV infection

t(8:14)

180
Q

pt presents with eczematoid skin lesions that progress to generalize erythroderma; his lymphocytes are cribiform shape

A

mycosis fungoides (T cell lymphoma of skin)

181
Q

Sezary syndrome

A

disseminated form of mycosis fungoides

182
Q

what are indirect indicators of tumor burden in NHL?

A

serum LDH and B2-microglobulin

183
Q

chemotherapy used in NHL

A

CHOP - multiple courses

  • cyclophosphamide
  • hydroxydaunomycin (doxorubicin)
  • oncovin (vincristine)
  • prednisone
184
Q

RFs for AML

A

radiation exposure
myeloproliferative syndromes
Down’s syndrome
chemotherapy - alkylating agents

185
Q

poor prognostic indicators in ALL

A

age 9
WBC count > 100 000/mm3
CNS involvement

186
Q

testicular involvement is common in what hematologic malignancy?

A

ALL

187
Q

which leukemia presents with an anterior mediastinal mass?

A

T cell ALL

188
Q

pt recently tx with chemotherapy for acute leukemia presents with hyperkalemia, hyperphosphatemia and hyperuricemia - dx?

A

tumor lysis syndrome - rapid cell death with release of intracellular contents

189
Q

what study is needed for diagnosis of leukemia?

A

BM biopsy

190
Q

chemotx. for AML

A

cystosine arabinoside AND either daunorubicin or idarubicin

191
Q

chemotx. for ALL

A

vincristine
predniosone
daunorubicin

192
Q

in which leukemia should you give prophylaxis for CNS involvement?

A

ALL - give MTX intrathecally

193
Q

when does BMT come into play in tx. of acute leukemia?

A

any pt who relapses after chemotherapy

194
Q

what disease is characterized by monoclonal proliferation of lymphocytes that are morphologically mature but functionally defective?

A

CLL

- usually in pts > 60

195
Q

an elderly man comes in complaining he frequently gets respiratory and skin infections that are long lasting and difficult to tx. On exam you notice, painless LAD - what test should you do?

A

CBC - to diagnose CLL

196
Q

lab findings in CLL

A

WBC 50,000-200,000 (80-90% lymphocytes)
smudge smells on PB smear
may have pancytopenia

197
Q

what test is used to confirm CLL?

A

flow cytometry of peripheral blood - shows clonal population of B cells (CD19)

198
Q

how do you tx. CLL?

A

usually observe patients until they become symptomatic (may take years)
Chemo = RFC (rituximab, fludarabine, cyclophosphamide)

199
Q

standard of care for CML

A

imatinib

- tyrosine kinase inhibitors (also: dasatinib, nilotinib)

200
Q

Lab findings in CML

A

WBCs 50,000-200,000 (left shift toward granulocytes)
eosinophilia/basophilia
leukemic cells in peripheral blood
decreased leukocyte ALP

201
Q

pt comes in complaining of severe pruritus after a hot bath or shower - what should you consider?

A

polycythemia vera

202
Q

CF of polycythemia vera

A

hyperviscosity sx
thrombotic phenomena
bleeding
splenomegaly/hepatomegaly

203
Q

lab findings in polycythemia vera

A

elevated RBC count, hemoglobin/Hct > 50
reduced serum EPO levels
elevated vit B12 levels
thrombocytosis/ leukocytosis

204
Q

Tx. of polycythemia

A

repeated phlebotomy

205
Q

major criteria for dx. polycythemia vera

A
  1. elevated RBC mass (men > 36, women > 32)
  2. arterial O2 sat > 92%
  3. splenomegaly
206
Q

minor criteria for dx. polycythemia vera

A
  1. thrombocytosis (plts > 400,000)
  2. leukocytosis (WBC > 120,000)
  3. leukocyte ALP > 100
  4. serum vit B12 > 900
207
Q

essential thrombocythemia

A

Platelets > 600,000/mm3 with findings of splenomegaly, pseudohyperkalemia, elevated bleeding time and erythromelalgia

208
Q

BM biopsy finding in essential thrombocytemia

A

increased number of megakaryocytes

209
Q

Tx. of essential thrombocytemia

A

antiplatelet agents - anagrelide, aspirin

hydroxyurea if severe