Heme/Onc Flashcards
(120 cards)
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.
2
Q
Hemophilia __ is most common in what Nationality?
A
C, Ashkenazi Jews (and it is often autosomal)
3
Q
What is the mechanism of action of Heparin?
A
Activates antithrombin
4
Q
What lab value changes with Heparin?
A
Increase in PTT
5
Q
Does low molecular weight heparin increase PTT?
A
No
6
Q
What is the antidote for heparin?
A
Protamine sulfate
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)
8
Q
What are the vitamin K-dependent coagulation factors?
A
Factors II, VII, IX, X, proteins C and S
9
Q
What lab value does Warfarin affect?
A
Increases PT (INR)
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.
11
Q
How do you reverse the effects of Warfarin?
A
Rapid reversal = fresh frozen plasma
Reversal = vitamin K
12
Q
How does tPA affect lab values?
A
Increases PT and PTT
13
Q
What is the mechanism of action for Factor Xa inhibitors (apiXAban, riveroroXAban)?
A
Directly inhibits factor Xa.
14
Q
Do you monitor PT/PTT with Factor Xa inhibitors?
A
NO
15
Q
Is there a reversal agent for Factor Xa inhibitors?
A
Not yet
16
Q
What is the mechanism of action for LMWH (enoxaprin)?
A
Mainly inhibits factor Xa
17
Q
What is the mechanism of action for direct thrombin inhibitors (dabigatran, argatroban)?
A
Directly inhibits factor II (thrombin)
18
Q
What anticoagulants work on IIa (thrombin)?
A
Heparin
LMWH (dalteparin, enoxaparin)
Direct thrombin inhibitors (argatrovan, dabigatran)
19
Q
Hemophilias are __ linked clotting factor deficiencies
A
X
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
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
22
Q
What is in cryoprecipitate?
A
Mainly Factor VIII, fibrinogen (more concentrated source than FFP)
Smaller concentrations of Factor XIII, vWF, and fibronectin
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)
24
Q
What is the most common inherited bleeding disorder?
A
vWD
25
What is more severe, hemophilia or vWD?
Hemophilia
26
How does vWD often present?
In childhood, with recurrent and prolonged mucosal bleeding (epistaxis, gums, teeth, menorrhagia, after dental procedures)
27
How does hemophilia often present?
A young boy with spontaneous hemorrhage into tissues and joints, leading to arthropathy and joint destruction.
28
What is diagnostic of vWD?
Decreased agglutination, seen on ristocetin cofactor assay.
29
Does ASA increase the risk of bleeding in patients with vWD?
Yes
30
How can bleeding be controlled in vWD?
DDAVP (in mild/mod type 1)
31
What is the most common cause of hereditary thromboembolic disease?
Factor V Leiden
32
HIT: formation of blood clots and __% drop in platelet count
50% (rapid)
33
What conditions is APS (antiphospolipid syndrome) often associated with?
SLE, rheumatoid arthritis
34
How can Protein C or S deficiency present?
Hypercoagulable state with skin or tissue necrosis following warfarin administration
35
A patient with rapid onset dyspnea, pleuritic chest pain, hypoxia, tachycardia, increased alveolar arterial oxygen gradient
PE
36
What is DIC characterized by?
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.
37
How can you differentiate DIC from liver disease?
Factor VIII is depressed in DIC (normal in liver disease)
38
What are the three causes of microangiopathic hemolytic anemia?
1. HUS
2. TTP
3. DIC
"DICk's HoUSe got TTPed because he caused microangiopathic hemolytic anemia"
39
What is the most immunologic disorder in women of childbearing age?
Idiopathic Thrombocytopenic Purpura
40
What is the differential diagnosis of thrombocytopenia?
HIT SHOC
```
HIT or HUS
ITP
TTP or Treatment (meds)
Splenomegaly
Hereditary
Other (malignancy)
Chemotherapy
```
41
What is the treatment for ITP (idiopathic thrombocytopenic purpura)?
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
42
What are the causes of microcytic anemia?
TAILS
```
Thalassemia
Anemia of chronic disease
Iron deficiency
Lead poisoning
Sideroblastic anemia
```
43
What is the most common cause of acute renal failure in kids?
HUS (hemolytic uremic syndrome)
44
An elderly man with hypo chromic, microcytic anemia is asymptomatic. Diagnostic test?
Fecal occult blood test and sigmoidoscopy; suspect colorectal cancer
45
What are the precipitants of hemolytic crisis in patients with G6PD deficiency?
Sulfonamides, antimalarial drugs, favs beans.
46
What is the diagnostic test for hereditary spherocytosis?
Osmotic fragility test
47
Pure RBC aplasia
Diamond-Blackfan anemia
48
What anemia is associated with absent radii and thumbs, diffuse hyper pigmentation, cafe au lait spots, microcephaly, and pancytopenia?
Fanconi anemia
49
What medications and viruses lead to aplastic anemia?
Chloramphenicol, sulphonamides, radiation, HIV, chemotherapeutic agents, hepatitis, parvovirus B19, EBV
50
How do you distinguish polycythemia vera from secondary polycythemia?
Both have increased hematocrit and RBC mass
But polycythemia vera should have normal O2 saturation and low erythropoietin levels
51
What is the TTP (thrombocytopenic purpura) pentad?
"FAT RN"
```
Fever
Anemia
Thrombocytopenia
Renal dysfunction
Neurologic abnormalities
```
52
What is the HUS (hemolytic uremic syndrome) triad?
Anemia, thrombocytopenia, acute renal failure
53
What is the treatment for TTP?
Emergent large-volume plasmapheresis, corticosteroids, anti platelet drugs
54
Are platelet transfusions contraindicated in TTP?
YES
55
Which of the following are increased in DIC?
```
Fibrin splint products
D-Dimer
Fibrinogen
Platelets
Hematocrit
```
Increased:
Fibrin split products and D-Dimer
Decreased:
Platelets, fibrinogen, and hematocrit
56
An 8-year old boy presents with hemarthrosis and increased PTT with normal PT and bleeding time.
What is the Dx/ Txt?
Dx: Hemophilia A or B
Txt: Consider desmopressin (for Hemophilia A) or factor VIII or IX supplements
57
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?
Dx: von Willebrand disease
Txt: Desmopressin, FFP, or cryoprecipitate
58
A 60 year-old African American man presents with bone pain. What might a workup for multiple myeloma reveal?
Monoclonal gammopathy, Bence Jones proteinuria, and "punched out" lesions on radiographs of the skull and long bones.
59
Reed-Sternberg cells
Hodgkin lymphoma
60
A 10 year old boy presents with fever, weight loss, night sweats. Examination shows an anterior mediastinal mass. What is the expected diagnosis?
Non-Hodgkin lymphoma
61
Microcytic anemia with:
Low serum iron
Low TIBC
Normal or increased ferritin
Anemia of chronic disease
62
Microcytic anemia with:
Low serum iron
Low ferritin
Increased TIBC
Iron-Deficiency Anemia
63
An 80 year old man presents with fatigue, lymphadenopathy, splenomegaly, and isolated lymphocytosis. What is the suspected diagnosis?
Chronic lymphocytic leukaemia (CLL)
64
A patient with fatigue is found to have:
Low Hgb
Increased MCV
What are the potential causes?
B12 Deficiency (pernicious anemia, veg det, Crohn/GI disorders)
Folate Deficiency (alcoholics)
65
What is a late, life threatening complication of chronic myelogenous leukaemia (CML)?
Blast crisis (fever, bone pain, splenomegaly, pancytopenia)
66
Auer rods on blood smear
Acute myelogenous leukaemia (AML)
67
AML subtype associated with DIC?
| Treatment?
DX: M3
Txt: Retinoic acid
68
Electrolyte changes in tutor lysis syndrome
Low Ca2+
High K+
High phosphate
High uric acid
69
A 50 year old man presents with early satiety, splenomegaly, and bleeding. Cytogenetics show t(9,22). What is the diagnosis?
CML
70
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?
Neutropenic fever is a MEDICAL EMERGENCY.
| Start broad spectrum antibiotics.
71
What is the virus associated with aplastic anemia in patients with sickle cell anemia?
Parvovirus B19
72
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?
O2, analgesia, hydration, and if severe, transfusion
73
What is a significant cause of morbidity in thalassemia patients?
What is the treatment?
Dx: Iron overload
Txt: Deferoxamine
74
What can you premedicate your patient with to try and prevent minor transfusion reactions?
Acetaminophen and diphenhydramine
75
What is the telltale sign of porphyria?
Pink urine
76
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?
Porphyria
77
Smudge cells
CLL
78
Likely leukaemia dx based on age: <13 years
ALL
79
Likely leukaemia dx based on age: 13-40
AML
80
Likely leukaemia dx based on age: 40-60
CML
81
Likely leukaemia dx based on age: >60
CLL
82
What are the features of Multiple Myeloma?
CRAB
hyperCalcemia
Renal involvement
Anemia
Bone lytic lesions/Back pain
83
Decreased platelets on peripheral blood film, but increased number of megakaryocytes on bone marrow aspiration and biopsy
ITP (immune thrombocytopenia)
84
what complication are you worried about in ITP?
intracranial bleeding
txt: IV steroids, IVIG, platelets
85
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
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
What do these conditions have in common:
HUS
TTP
DIC
microangiopathic hemolytic anemias
will see schistocytes and decreased platelets on peripheral smear
87
Work up for patient you think has vWD
```
PTT (prolonged)
bleeding time (prolonged)
```
VWF antigen
Factor VIII
ristocetin induced platelet agglutination
88
Treatment for vWD
DDAVP (includes endothelium to release vWF and factor VIII)
89
Work up for patient you think has DIC
```
CBC - platelets (low)
INR (prolonged)
PTT (prolonged)
Fibrinogen (low)
D-Dimer (high) Fibrin degrading products (high)
```
90
50 year old man started on warfarin therapy presents with skin necrosis, why?
Protein C deficiency
91
What is your workup for patient with unprovoked DVT?
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
Unprovoked DVT, thinking malignancy cause, what is your malignancy workup?
```
Complete hx and px
Routine blood work
Urinalysis
CXRAY
Age apron screening: mammogram, Pap, PSA, colonoscopy
```
93
DVT DDx
```
DVT
Cellulitis
Lymphadenitis
Baker's cyst
Muscle tear
```
94
How long do you treat someone with a provoked DVT (is post op, immobilized, OCP)?
3 months
95
1st unprovoked DVT, treatment?
consider indefinite
96
how many months should you wait post venous thromboembolic event prior to elective surgery?
3 months
97
when is surgery safe for someone on anticoagulation?
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
how do you deal with a patient with mechanical valve who has to go for surgery?
IV heparin or LMWH bridging should be given before and after the procedure while the INR is below 2
99
what is your differential for normal MCV (80-100) anemia
```
ABCD
Acute blood loss (retics > 2-3 %)
Bone marrow failure (reltics < 2%)
Chronic disease (retics < 2%)
Destruction (hemolysis) (relics > 2-3%)
```
100
what is the most important test to differentiate between iron def anemia and anemia of chronic disease?
ferritin low (IDA), TIBC high (IDA)
serum iron low in both
101
what is plummer-vinson syndrome?
Dysphagia (esophageal)
Glossitis
Iron deficiency anemia
Stomatitis
102
how do you treat lead poisoning ?
chelation therapy: dimercaprol and EDTA are first line
103
```
patient with:
normocytic anemia
increased relics
decreased haptoglobin *
increased unconjugated bili
increased urobilinogen
increased LDH
```
Hemolytic Anemia
104
what are the clinical features specific to hemolytic anemia?
jaundice
dark urine (hemoglobinuria, bilirubin)
cholelithiasis (pigment stones)
105
schistocytes in blood smear indicates what kind of anemia
schistocytes = hemolytic anemia (intravascular hemolysis)
vs
spherocytes (extravascular hemolysis)
106
name the hemolytic anemias
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
African American patient comes in with chest pain, fever, elevated WBC, pulmonary infiltrates on CXR; kid is jaundice; labs show anemia, high retics
Sickle cell disease presenting with Acute Chest Syndrome
| caused by vaso-occlusion, infection, or pulmonary fat embolus from infarcted marrow
108
African boy with jaundice; has been on septra for a UTI
G6PD-def
| intermittent hemolysis after being exposed to an agent causing oxidative stress
109
Heinz bodies on peripheral smear
G6PD-def
110
3 major issues that cause B12 def are
1. Dietary def
2. Intrinsic factor def
3. Malabsorption
111
+ Schiling Test
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
Hyper segmented neutrophils on peripheral smear
```
Megaloblastic anemia
(ddx: B12 or folate def)
```
113
Bone marrow replaced with fat
Aplastic anemia
114
What pathogen - sickle cell patients with osteomyelitis
Salmonella
115
19 year old, generalized lymphadenopathy, weight loss
HIV
Mono
Lymphoma
116
What are good prognostic factors for ALL?
```
young age
WBC <30
T-cell phenotype
Absence of Ph chromosome
Early attainment of complete remission
```
117
t (8;14) Chromosome Translocation with c-myc activation
Burkitt's Lymphoma
118
t(14;18) chromosome translocation with bcl-2 activation
Follicular Lymphoma
119
complications of chemo treatment for lymphoma
cardiac disease: adriamycin cardiotoxic
pulmonary disease: bleomycin causes interstitial pneumonitis
infertility
120
bcr-abl fusion gene
CML
