Hematology Flashcards
1
Q
Howell-Jolly Bodies are an indication of:
A
decreased spleen function or splenectomy
2
Q
Episodic hemolytic anemia associated with sulfa drugs, fave beans, and infections is caused by ___________.
A
G6PD deficiency
3
Q
On hemoglobin electrophoresis, sickle cell anemia shows:
A
Hgb S
4
Q
What enzyme do you expect to be elevated in the blood in the setting of RBC destruction?
A
LDH
5
Q
PBS: auto agglutination / clumping of RBC
A
autoimmune hemolytic anemia
6
Q
Elevated direct (conjugated) bilirubin might be seen along with ___________(physical sign) in RBC destruction.
A
dark urine
7
Q
Increased RBC destruction overwhelms the livers UGT enzyme, causing _________ (abnormal lab value) and ___________(physical sign)?
A
elevated indirect bilirubin (T bili)
jaundice
8
Q
Microcytic anemia with normal or elevated serum iron, no response to iron tx might indicate __________.
A
Alpha thalassemia
9
Q
Beta thalassemia abnormalities on hemoglobin electrophoresis are:
A
low HgbA
high HgbF
high HgbA2
10
Q
most common presenting complications of neutropenia
A
bacterial/fungal infections of the skin and/or oral cavity
11
Q
Which nutrient deficiency can present w/ pancytopenia?
A
B12
12
Q
What are “bands”?
A
immature neutrophils
may be present in infection
pre-segmented
Add Segs/Neutr + Bands to get ANC.
13
Q
Elevated basophils may be seen with_____
A
CML, anaphylaxis, hypersensitivity ran
14
Q
GCS-F is associated with increased risk of __________ in patients with severe congenital neutropenia.
A
MDS/AML
15
Q
mild
moderate
severe
neutropenia
A
1000-1500
500-1000
<500
16
Q
Ddx for monocytosis
A
infection (TB)
malignancy
rheumatologic disease
severe congenital neutropenia
17
Q
How do we calculate ANC?
A
(WBC number x 1000) x (neutrophil%/100)
18
Q
Which ethnicities are more likely to have ethnic neutropenia?
A
African, Jewish, Arab
mild-moderate ANC with BENIGN history
19
Q
Name some medications that can cause neutropenia.
A
Antibiotics (Bactrim, penicillins), anticonvulsants, psychotropics
20
Q
Neutrophils may also be reported as __________ or __________.
A
Segmented neutrophils (SEG%) Granulocytes (GRAN%)
21
Q
Someone with a rheumatologist disease will have low __________ and high _________.
A
lymphocytes
monocytes
22
Q
What will neutrophils look like in the setting of B12 deficiency?
A
hypersegmented >5 segments
23
Q
_______________ is characterized by neutropenia and pancreatic dysfunction (steatorrhea,
failure to thrive) and may progress to pancytopenia
A
Schwachman-Diamond syndrome
24
Q
Eosinophilia is defined as AEC > ______.
A
500
25
Differential for lymphocytosis.
infection (esp viral)
| leukemia
26
Plan of action for patient with severe neutropenia in the ER
1) Triage
2) Labs: CBC w/ diff, blood culture
3) Start broad spectrum abs
3) Thorough hx/physical: spleen
4) consult specialist, if not available - consider GCSF
27
2 alpha + 2 beta globin chains = Hemoglobin ______
A
28
2 alpha + 2 gamma globin chains= Hemoglobin _______
F
29
A newborn screen reported as FAS is
sickle cell trait
30
4 beta globin chains = Hemoglobin _________
H
31
4 gamma globin chains = Hemoglobin _________
Barts
32
A newborn screen Hb profile with 20-50% of Hb as Hb Barts indicates __________.
Alpha thalassemia (3 gene deletion)
33
2 alpha + 2 delta globin chains = Hemoglobin ___________
A2
34
A newborn screen reported as FS is
sickle cell disease/anemia
35
A newborn screen Hb profile with HbA2 > 3.5% would indicate ________________.
Beta thalassemia trait (B, Bo)
36
A patient with sickle cell anemia has a new pulmonary infiltrate +/- fever, dyspnea, pain, hypoxia, and elevated WBC. This is likely ____________. What do you do?
```
Acute Chest Syndrome.
Admit
Ceftriaxone + macrolide
incentive spirometry
cautious IVF
```
37
A newborn screen with Hb parts of 3-8% would indicate _____________
alpha thalassemia trait (2 gene deletion)
38
An undesired painful erection in a patient with sickle cell anemia is called _________.
Priapism
39
Differentiate between beta thalassemia major, intermedia, and trait.
Based on clinical symptoms. Major requires regular transfusions, intermedia requires occasional transfusions, trait is asymptomatic.
40
A newborn screen reported as FSA is
sickle beta thalassemia
41
A patient with sickle cell anemia presents with headache, nausea, hemiparesis. What do you do?
```
Transfuse immediately (preferably exchange transfusion)
MRI (but don't wait for results to transfuse)
```
42
A newborn screen Hb profile with 100% HbF at birth would indicate ____________.
Beta thalassemia major. (Bo, Bo)
Later in life there would be HbF and HbA2
43
A 2 year old with sickle cell anemia presents with worsened anemia, elevated relic, platelets <150,000, abdominal distention and tender splenomegaly. This is likely ___________.
```
Acute splenic sequestration
6 mo- 3 yr with high recurrence
monitor for hypovolemia
transfuse
?splenectomy
```
44
A newborn screen reported as FA is
normal
45
Treatment for priapism is:
analgesic
hydration
aspiration + irrigation of corporeal bodies
ppx: alpha-adrenergic agonists (sildenafil)
46
What is the term for inability to concentrate urine, which results in nocturia and polyuria?
hyposthenuria
47
Patients with sickle cell anemia who have a transcranial doppler ultrasound >200 cm/sec should be initiated on what therapy to reduce risk of stroke?
chronic transfusions
| goal keep 70% blood volume as donor blood
48
In a patient with G6PD deficiency, what are potential triggers of a hemolytic episode?
sulfa drugs
fava beans
moth balls
infection
49
Clotting factors in intrinsic pathway
12, 11, 9, 3, 10
50
vit K dependent clotting factors
10, 9 7 2
51
Clotting factor in extrinsic pathway
7
52
Protein C & Protein S are ____
?
53
________ measures extrinsic pathway.
PT
54
________measures intrinsic pathway
PTT
55
Hemophelia A is a deficiency in Factor ______
8
56
In a mixing study, if an inhibitor is present the factor activity is going to be __________ and PTT will be ________.
decreased
increased
Indicates: Lupus anticoagulant, Heparin, or Specific inhibitor
57
In a mixing study, if no inhibitor is present, the factor activity is going to be ______% with a normal PTT.
50%
(only need 30% activity to normalize PTT)
```
Indicates:
Factor deficiency (including vWF)
```
58
Prolonged PTT can be seen in :
von Willibrand disease
hemophilia
aspirin
59
Which lab could indicate any of the following?
Factor 7 deficiency
Vitamin K deficiency
Warfarin
Abnormal PT
60
Normal PT and PTT but still have bleeding disorder, consider Factor ______ deficiency
13
61
Abnormal PT and PTT (both) may indicate:
Vitamin K deficiency
DIC
Liver Disease
No/abnormal fibrinogen
62
____________ is reduced in liver dysfunction and DIC
fibrinogen
63
_____________ carries and stabilizes factor 8
von Willibrand factor
64
vWF deficiency presents with _______
mucocutaneous bleeding
| menorhhagia (more common in women)
65
VWf deficiency etiology
autosomal dominant inheritance
| MC inherited bleeding d/o
66
DDAVP (arginine vasopressin)
Stimate: releases stored vWF from endothelial cells
67
Humate-P
Factor 8 product that also contains vWF
68
Amicar (aminocaproic acid)
effective for clotting mucosal bleeding
69
Name 4 things that can increase vWF levels, leading to a hyper-coagulable state.
pregnancy
oral contraceptives
stress
surgery
70
in Hemophilia, >60% of bleeds occur in ________ and >30% occur in ________.
Joints
| Muscle
71
Symptoms of a joint/muscle bleed
pain (tingling, burning, tenderness, heat)
disuse/immobility (measure ROM)
abnormal appearance. (measure circumference)
72
Factor 8 replacement
half life 8-12 hours
| each unit/kg increases serum level by 2% activity
73
Factor 9 replacement
half life 12-24 hours
| each unit/kg increases serum level by 1% activity
74
PPX for severe hemophilia
infuse factor replacement 2-3 x/week to keep trough levels at 2-3% (from severe to moderate- no spontaneous bleeding)
very expensive
requires IV access
75
life threatening bleeds in severe hemophilia
intracranial
retropharyngeal
iliopsoas
76
How to treat patients with hemophilia that have developed inhibitors?
give factor 7
| give higher doses of 8 or 9
77
DIC presentation
bleeding
petechiae
purpura
78
DIC pathophys
generalized activation of coagulation system due to exposure of blood to tissue factor (trauma), infection, or malignancy.
79
How to treat DIC?
```
underlying cause
hemodynamic/ventilatory support
hydration
RBC transfusion
If bleeding: platelets, FFT, cryoprecipitate
```
80
Virchow's triad (risk factors for thrombosis)
alterations in blood flow (stasis)
vascular endothelial injury
alteration in constituents of blood
81
secondary hyper coagulable states
```
surgery
stasis
pregnancy
OCP
malignancy
inflammation
```
82
thrombophilia
factor V Leiden
Protein S deficiency
Protein C deficiency
antithrombin deficiency
83
Therapy for venous thromboembolism (DVT, PE)
heparin --> warfarin
| goal: PTT 2x upper limit of normal
84
Warfarin target
INR 2.5
85
Widespread microthrombi + severe thrombocytopenia =
Disseminated Intravascular Coagulation DIC
86
cryoprecipitate
replaces fibrinogen
87
Inherited thrombophilias
Factor V Leiden
Protein C or S deficiency
antithrombin deficiency
88
The TTP pentad
```
thrombocytopenia
microangiopathic hemolytic anemia
neurologic signs
renal impairment
fever
```
89
Diagnostic test for TTP
| give both names
ADAMTS13 activity below normal range
| VonWillebrand Cleaving Protease
90
Low ADAMTS13 with an inhibitor (antibody) indicates _________ TTP.
acquired
91
Low ADAMTS13 withOUT an inhibitor indicates __________ TTP.
congenital
92
Tx for TTP
plasmapheresis
| replaces ADAMTS and removes any antibody
93
Do not give __________ to a patient with TTP.
platelets
94
Triad for Hemolytic Uremic Syndrome
Microangiopathic hemolytic anemia
Thrombocytopenia
Renal impairment
Often: bloody diarrhea because MC cause of HUS is shiga-toxin producing E coli
95
atypical HUS (aHUS) is a mutation in genes that _______
protect us from complement over-activation
96
epidemiology of HUS vs aHUS
HUS children
| aHUS teens/adults
97
Eculizumab
monoclonal antibody for complement
| used to treat aHUS
98
Labs that distinguish DIC from HUS and TTP are:
PT/PTT are normal in HUS and TTP
99
Most common cause of acute renal failure in children
HUS
100
Philadelphia chromosome is seen in 95% of cases of ___________
CML.
It can also be found in ALL.
101
Acute leukemia will show __________ on bone marrow biopsy.
hypercellularity with >20% blasts
102
Patients with bone marrow failure may progress to develop which leukemia?
AML
103
Which is more likely to have CNS involvement? AML or ALL
ALL
104
A bone marrow biopsy of AML will show ___________.
Auer rods
105
Which form of leukemia typically has elevated WBC, platelets, and basophils?
CML
106
How is CLL diagnosed?
>5000 clonal B lymphocytes on flow cytometry
107
What are the three phases of CML?
Chronic phase
Accelerated phase
Blast crisis
108
Which form of leukemia can present with bone pain?
ALL
109
Which form of leukemia is not responsive to chemotherapy?
CLL
110
Which form of leukemia is characterized by clonal proliferation of lymphoid precursors?
ALL
111
Which form of leukemia is characterized by clonal proliferation of mature myeloid cells
CML
112
Smudge cells on PBS are suggestive of_________.
CLL
113
Which cancer is caused by monoclonal proliferation of plasma cells?
Multiple myeloma
114
What treats Philadelphia positive (BCR-ABL) leukemia?
Imatinib (tyrosine kinase inhibitor)
115
Signs/Symptoms
Calcium elevation
Renal failure
Anemia (normocytic)
Bone pain & pathologic fractures
116
Multiple myeloma epidemiology
blacks:whites 2:1
median age 70 yrs
familial
117
PBS for multiple myeloma will show _______________
Rouleaux formation (stacked RBC)
118
Urinalysis for multiple myeloma should include a test for _______________, which is responsible for the associated renal failure.
Bence Jones protein
119
Protein electrophoresis (serum or urine) in multiple myeloma will show a spike in ______________.
monoclonal M protein
120
BMB for multiple myeloma will have ____________________.
>10% plasma cells
121
Tx for Multiple Myeloma
```
autologous transplant
chemo if not a transplant candidate
bisphosphonates (IV zoledronic acid)
thromboprophylaxis
prophylactic abs
```
122
Skeletal lytic lesions (punched out, soap bubble appearance) on Xray is characteristic of ________________.
Multiple myeloma
123
Hodgkin's lymphoma excision biopsy shows:
Reed Sternberg Cells which looks like "owl eyes"
124
Ann Arbor staging
I: one nodal site
II: two nodal sites same side of diaphragm
III: nodal sites both sides of diaphragm
IV: extra nodal sites
add "B" if there are "B symptoms"
125
Treatment for Hodgkins lymphoma stage I, II, IIIA
radiation
126
Treatment for Hodgkins lymphoma stage IIIB, IV
combination chemotherapy
127
Tx for diffuse B cell Non Hodgkins lymphoma
R-CHOP
128
Name some common types of Non Hodgkins lymphoma
```
Diffuse B cell
Follicular
Mantle cell
Small cell
Burkitt's
T cell
```
129
Lymphoma is commonly associated with _______ virus
EBV
130
Signs of Burkitt's lymphoma
jaw pain/mass
| abdominal pain
