Heme/Onc Flashcards

1
Q

Why are neonates prone to bleeding if not given a shot of Vitamin K at birth?

A

Enteric bacteria synthesize Vitamin K, and neonates lack these bacteria at birth.

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2
Q

Hemophilia __ is most common in what Nationality?

A

C, Ashkenazi Jews (and it is often autosomal)

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3
Q

What is the mechanism of action of Heparin?

A

Activates antithrombin

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4
Q

What lab value changes with Heparin?

A

Increase in PTT

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5
Q

Does low molecular weight heparin increase PTT?

A

No

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6
Q

What is the antidote for heparin?

A

Protamine sulfate

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7
Q

What is the mechanism of action of Warfarin?

A

Inhibits synthesis of vitamin K-dependent coagulation factors (II, VII, IX, X, proteins C and S)

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8
Q

What are the vitamin K-dependent coagulation factors?

A

Factors II, VII, IX, X, proteins C and S

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9
Q

What lab value does Warfarin affect?

A

Increases PT (INR)

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10
Q

What is the mechanism of action of tPA (tissue plasminogen activator - alteplase)?

A

Aids in conversion of plasminogen to plasmin, which breaks down fibrin.

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11
Q

How do you reverse the effects of Warfarin?

A

Rapid reversal = fresh frozen plasma

Reversal = vitamin K

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12
Q

How does tPA affect lab values?

A

Increases PT and PTT

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13
Q

What is the mechanism of action for Factor Xa inhibitors (apiXAban, riveroroXAban)?

A

Directly inhibits factor Xa.

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14
Q

Do you monitor PT/PTT with Factor Xa inhibitors?

A

NO

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15
Q

Is there a reversal agent for Factor Xa inhibitors?

A

Not yet

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16
Q

What is the mechanism of action for LMWH (enoxaprin)?

A

Mainly inhibits factor Xa

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17
Q

What is the mechanism of action for direct thrombin inhibitors (dabigatran, argatroban)?

A

Directly inhibits factor II (thrombin)

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18
Q

What anticoagulants work on IIa (thrombin)?

A

Heparin
LMWH (dalteparin, enoxaparin)
Direct thrombin inhibitors (argatrovan, dabigatran)

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19
Q

Hemophilias are __ linked clotting factor deficiencies

A

X

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20
Q

What lab value does hemophilias affect?

A

PTT (PT and bleeding times are normal)

A = factor VIII
B = factor IX
C = factor XI
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21
Q

What are the treatment options for bleeding in hemophilia?

A

Severe = transfusion

Not severe desmopressin (DDAVP) because it releases factor VIII from endothelial cells

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22
Q

What is in cryoprecipitate?

A

Mainly Factor VIII, fibrinogen (more concentrated source than FFP)

Smaller concentrations of Factor XIII, vWF, and fibronectin

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23
Q

What is vWD?

A

Von Willebrand Disease

Defect or deficiency in vWF (used to adhere platelets to site of injury) with decreased levels of factor VIII (which is carried by vWF)

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24
Q

What is the most common inherited bleeding disorder?

A

vWD

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25
Q

What is more severe, hemophilia or vWD?

A

Hemophilia

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26
Q

How does vWD often present?

A

In childhood, with recurrent and prolonged mucosal bleeding (epistaxis, gums, teeth, menorrhagia, after dental procedures)

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27
Q

How does hemophilia often present?

A

A young boy with spontaneous hemorrhage into tissues and joints, leading to arthropathy and joint destruction.

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28
Q

What is diagnostic of vWD?

A

Decreased agglutination, seen on ristocetin cofactor assay.

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29
Q

Does ASA increase the risk of bleeding in patients with vWD?

A

Yes

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30
Q

How can bleeding be controlled in vWD?

A

DDAVP (in mild/mod type 1)

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31
Q

What is the most common cause of hereditary thromboembolic disease?

A

Factor V Leiden

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32
Q

HIT: formation of blood clots and __% drop in platelet count

A

50% (rapid)

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33
Q

What conditions is APS (antiphospolipid syndrome) often associated with?

A

SLE, rheumatoid arthritis

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34
Q

How can Protein C or S deficiency present?

A

Hypercoagulable state with skin or tissue necrosis following warfarin administration

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35
Q

A patient with rapid onset dyspnea, pleuritic chest pain, hypoxia, tachycardia, increased alveolar arterial oxygen gradient

A

PE

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36
Q

What is DIC characterized by?

A

Thrombosis + Hemorrhage

Aquired coagulopathy caused by deposition of fibrin in small blood vessels, leading to thrombosis and end-organ damage.
Depletion of clotting factors and platelets lead to bleeding diathesis.

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37
Q

How can you differentiate DIC from liver disease?

A

Factor VIII is depressed in DIC (normal in liver disease)

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38
Q

What are the three causes of microangiopathic hemolytic anemia?

A
  1. HUS
  2. TTP
  3. DIC

“DICk’s HoUSe got TTPed because he caused microangiopathic hemolytic anemia”

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39
Q

What is the most immunologic disorder in women of childbearing age?

A

Idiopathic Thrombocytopenic Purpura

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40
Q

What is the differential diagnosis of thrombocytopenia?

A

HIT SHOC

HIT or HUS
ITP
TTP or Treatment (meds)
Splenomegaly
Hereditary 
Other (malignancy)
Chemotherapy
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41
Q

What is the treatment for ITP (idiopathic thrombocytopenic purpura)?

A

Platelet count > 30,000 no treatment
Platelet count < 30,000 corticosteroid or IVIG

Second line: Anti-D (Rh) immunoglobulin and rituximab … they act as “decoys” so that WBCs will recognize them instead of IgG on platelets

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42
Q

What are the causes of microcytic anemia?

A

TAILS

Thalassemia
Anemia of chronic disease
Iron deficiency 
Lead poisoning 
Sideroblastic anemia
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43
Q

What is the most common cause of acute renal failure in kids?

A

HUS (hemolytic uremic syndrome)

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44
Q

An elderly man with hypo chromic, microcytic anemia is asymptomatic. Diagnostic test?

A

Fecal occult blood test and sigmoidoscopy; suspect colorectal cancer

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45
Q

What are the precipitants of hemolytic crisis in patients with G6PD deficiency?

A

Sulfonamides, antimalarial drugs, favs beans.

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46
Q

What is the diagnostic test for hereditary spherocytosis?

A

Osmotic fragility test

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47
Q

Pure RBC aplasia

A

Diamond-Blackfan anemia

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48
Q

What anemia is associated with absent radii and thumbs, diffuse hyper pigmentation, cafe au lait spots, microcephaly, and pancytopenia?

A

Fanconi anemia

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49
Q

What medications and viruses lead to aplastic anemia?

A

Chloramphenicol, sulphonamides, radiation, HIV, chemotherapeutic agents, hepatitis, parvovirus B19, EBV

50
Q

How do you distinguish polycythemia vera from secondary polycythemia?

A

Both have increased hematocrit and RBC mass

But polycythemia vera should have normal O2 saturation and low erythropoietin levels

51
Q

What is the TTP (thrombocytopenic purpura) pentad?

A

“FAT RN”

Fever
Anemia
Thrombocytopenia
Renal dysfunction 
Neurologic abnormalities
52
Q

What is the HUS (hemolytic uremic syndrome) triad?

A

Anemia, thrombocytopenia, acute renal failure

53
Q

What is the treatment for TTP?

A

Emergent large-volume plasmapheresis, corticosteroids, anti platelet drugs

54
Q

Are platelet transfusions contraindicated in TTP?

A

YES

55
Q

Which of the following are increased in DIC?

Fibrin splint products
D-Dimer
Fibrinogen
Platelets
Hematocrit
A

Increased:
Fibrin split products and D-Dimer

Decreased:
Platelets, fibrinogen, and hematocrit

56
Q

An 8-year old boy presents with hemarthrosis and increased PTT with normal PT and bleeding time.

What is the Dx/ Txt?

A

Dx: Hemophilia A or B

Txt: Consider desmopressin (for Hemophilia A) or factor VIII or IX supplements

57
Q

A 14 year old girl presents with prolonged bleeding after dental surgery and with mensus. She has a normal PT, normal or increased PTT, increased bleeding time.

What is the Dx/Tx?

A

Dx: von Willebrand disease

Txt: Desmopressin, FFP, or cryoprecipitate

58
Q

A 60 year-old African American man presents with bone pain. What might a workup for multiple myeloma reveal?

A

Monoclonal gammopathy, Bence Jones proteinuria, and “punched out” lesions on radiographs of the skull and long bones.

59
Q

Reed-Sternberg cells

A

Hodgkin lymphoma

60
Q

A 10 year old boy presents with fever, weight loss, night sweats. Examination shows an anterior mediastinal mass. What is the expected diagnosis?

A

Non-Hodgkin lymphoma

61
Q

Microcytic anemia with:
Low serum iron
Low TIBC
Normal or increased ferritin

A

Anemia of chronic disease

62
Q

Microcytic anemia with:
Low serum iron
Low ferritin
Increased TIBC

A

Iron-Deficiency Anemia

63
Q

An 80 year old man presents with fatigue, lymphadenopathy, splenomegaly, and isolated lymphocytosis. What is the suspected diagnosis?

A

Chronic lymphocytic leukaemia (CLL)

64
Q

A patient with fatigue is found to have:
Low Hgb
Increased MCV
What are the potential causes?

A

B12 Deficiency (pernicious anemia, veg det, Crohn/GI disorders)

Folate Deficiency (alcoholics)

65
Q

What is a late, life threatening complication of chronic myelogenous leukaemia (CML)?

A

Blast crisis (fever, bone pain, splenomegaly, pancytopenia)

66
Q

Auer rods on blood smear

A

Acute myelogenous leukaemia (AML)

67
Q

AML subtype associated with DIC?

Treatment?

A

DX: M3
Txt: Retinoic acid

68
Q

Electrolyte changes in tutor lysis syndrome

A

Low Ca2+
High K+
High phosphate
High uric acid

69
Q

A 50 year old man presents with early satiety, splenomegaly, and bleeding. Cytogenetics show t(9,22). What is the diagnosis?

A

CML

70
Q

A patient on the chemotherapy service with an absolute neutrophil count (ANC) of 1000 is noted to have a fever of 38.8. what is the next best step?

A

Neutropenic fever is a MEDICAL EMERGENCY.

Start broad spectrum antibiotics.

71
Q

What is the virus associated with aplastic anemia in patients with sickle cell anemia?

A

Parvovirus B19

72
Q

A 25-year-old African American man with sick cell anemia has a sudden onset of bone pain. How do you manage this pain crisis?

A

O2, analgesia, hydration, and if severe, transfusion

73
Q

What is a significant cause of morbidity in thalassemia patients?
What is the treatment?

A

Dx: Iron overload
Txt: Deferoxamine

74
Q

What can you premedicate your patient with to try and prevent minor transfusion reactions?

A

Acetaminophen and diphenhydramine

75
Q

What is the telltale sign of porphyria?

A

Pink urine

76
Q

A college student consume alcohol and barbiturates at a party, and then has an acute episode of abdominal pain and brown urine the next day. Dx?

A

Porphyria

77
Q

Smudge cells

A

CLL

78
Q

Likely leukaemia dx based on age: <13 years

A

ALL

79
Q

Likely leukaemia dx based on age: 13-40

A

AML

80
Q

Likely leukaemia dx based on age: 40-60

A

CML

81
Q

Likely leukaemia dx based on age: >60

A

CLL

82
Q

What are the features of Multiple Myeloma?

A

CRAB

hyperCalcemia
Renal involvement
Anemia
Bone lytic lesions/Back pain

83
Q

Decreased platelets on peripheral blood film, but increased number of megakaryocytes on bone marrow aspiration and biopsy

A

ITP (immune thrombocytopenia)

84
Q

what complication are you worried about in ITP?

A

intracranial bleeding

txt: IV steroids, IVIG, platelets

85
Q

patient on heparin, day 5 patient presents with extreme pain in lower extremities, pt has non healing ulcer on tip of toe, no dorsalis pedis pulse, leg looks cyanotic

A

arterial embolism, acute limb ischemia

  • need arterial thrombosis to call it heparin-induced thrombocytopenia (no thrombosis, its heparin ASSOCIATED thrombocytopenia, and you do not need to stop heparin)
86
Q

What do these conditions have in common:

HUS
TTP
DIC

A

microangiopathic hemolytic anemias

will see schistocytes and decreased platelets on peripheral smear

87
Q

Work up for patient you think has vWD

A
PTT (prolonged)
bleeding time (prolonged)

VWF antigen
Factor VIII
ristocetin induced platelet agglutination

88
Q

Treatment for vWD

A

DDAVP (includes endothelium to release vWF and factor VIII)

89
Q

Work up for patient you think has DIC

A
CBC - platelets (low)
INR (prolonged)
PTT (prolonged) 
Fibrinogen (low)
D-Dimer (high) Fibrin degrading products (high)
90
Q

50 year old man started on warfarin therapy presents with skin necrosis, why?

A

Protein C deficiency

91
Q

What is your workup for patient with unprovoked DVT?

A

CBC, blood smear, PT, PTT, liver/ renal function, urinalysis, hemolysis markers if anemic (relics, haptoglobin)

screening that will pick up reversible causes:
malignancy work up
serology: antiphospholipid antibodies (APLA)

92
Q

Unprovoked DVT, thinking malignancy cause, what is your malignancy workup?

A
Complete hx and px
Routine blood work
Urinalysis
CXRAY
Age apron screening: mammogram, Pap, PSA, colonoscopy
93
Q

DVT DDx

A
DVT
Cellulitis
Lymphadenitis
Baker's cyst
Muscle tear
94
Q

How long do you treat someone with a provoked DVT (is post op, immobilized, OCP)?

A

3 months

95
Q

1st unprovoked DVT, treatment?

A

consider indefinite

96
Q

how many months should you wait post venous thromboembolic event prior to elective surgery?

A

3 months

97
Q

when is surgery safe for someone on anticoagulation?

A

INR < 1.5 off of warfrin
normal PTT on dabigatran
normal PTT on IV unfractionated heparin
drug specific Xa level at zero for apixaban/ rivaroxaban/ LMWH

98
Q

how do you deal with a patient with mechanical valve who has to go for surgery?

A

IV heparin or LMWH bridging should be given before and after the procedure while the INR is below 2

99
Q

what is your differential for normal MCV (80-100) anemia

A
ABCD
Acute blood loss (retics > 2-3 %)
Bone marrow failure (reltics < 2%)
Chronic disease (retics < 2%)
Destruction (hemolysis) (relics > 2-3%)
100
Q

what is the most important test to differentiate between iron def anemia and anemia of chronic disease?

A

ferritin low (IDA), TIBC high (IDA)

serum iron low in both

101
Q

what is plummer-vinson syndrome?

A

Dysphagia (esophageal)
Glossitis
Iron deficiency anemia
Stomatitis

102
Q

how do you treat lead poisoning ?

A

chelation therapy: dimercaprol and EDTA are first line

103
Q
patient with:
normocytic anemia
increased relics
decreased haptoglobin *
increased unconjugated bili
increased urobilinogen 
increased LDH
A

Hemolytic Anemia

104
Q

what are the clinical features specific to hemolytic anemia?

A

jaundice
dark urine (hemoglobinuria, bilirubin)
cholelithiasis (pigment stones)

105
Q

schistocytes in blood smear indicates what kind of anemia

A

schistocytes = hemolytic anemia (intravascular hemolysis)

vs

spherocytes (extravascular hemolysis)

106
Q

name the hemolytic anemias

A

Thalassemia - Beta Thalassemia Minor, Major, Intermediate; Alpha Thalaseemia
Sickle Cell Disease (HbAS, HbSS)
Autoimmune Hemolytic Anemia
Microangiopathic Hemolytic Anemia (TTP, HUS, DIC)
Hereditary Spherocytosis
G6PD Deficiency

107
Q

African American patient comes in with chest pain, fever, elevated WBC, pulmonary infiltrates on CXR; kid is jaundice; labs show anemia, high retics

A

Sickle cell disease presenting with Acute Chest Syndrome

caused by vaso-occlusion, infection, or pulmonary fat embolus from infarcted marrow

108
Q

African boy with jaundice; has been on septra for a UTI

A

G6PD-def

intermittent hemolysis after being exposed to an agent causing oxidative stress

109
Q

Heinz bodies on peripheral smear

A

G6PD-def

110
Q

3 major issues that cause B12 def are

A
  1. Dietary def
  2. Intrinsic factor def
  3. Malabsorption
111
Q

+ Schiling Test

A

Step 1 Normal >5% = normal B12

Step 2 If Normal test results with intrinsic factor >5% excretion = pernicious anemia

Step 2 Abnormal test result with intrinsic factor<5% excretion = intestinal causes (malabsorption)

112
Q

Hyper segmented neutrophils on peripheral smear

A
Megaloblastic anemia 
(ddx: B12 or folate def)
113
Q

Bone marrow replaced with fat

A

Aplastic anemia

114
Q

What pathogen - sickle cell patients with osteomyelitis

A

Salmonella

115
Q

19 year old, generalized lymphadenopathy, weight loss

A

HIV
Mono
Lymphoma

116
Q

What are good prognostic factors for ALL?

A
young age 
WBC <30 
T-cell phenotype 
Absence of Ph chromosome 
Early attainment of complete remission
117
Q

t (8;14) Chromosome Translocation with c-myc activation

A

Burkitt’s Lymphoma

118
Q

t(14;18) chromosome translocation with bcl-2 activation

A

Follicular Lymphoma

119
Q

complications of chemo treatment for lymphoma

A

cardiac disease: adriamycin cardiotoxic
pulmonary disease: bleomycin causes interstitial pneumonitis
infertility

120
Q

bcr-abl fusion gene

A

CML