Hematology Flashcards
(127 cards)
1
Q
most common inherited bleeding disorder
A
Von Willebrand Disease
2
Q
What is the most likely diagnosis: Epistaxis Ecchymosis Menorrhagia Normal platelet count and PT/INR History of bleeding after aspirin or NSAID use
A
Von Willebrand Disease
3
Q
What is the best initial test
-Von Willebrand disease
A
Evaluate bleeding
-CBC, PT, PTT
Investigate Von Willebrand
- Von Willebrand factor ristocetin cofactor activity (vWF:RCo)
- Von Willebrand factor antigen (vWF:Ag)
- Factor VIII activity
4
Q
What is the treatment of choice
-Von Willebrand Disease
A
Desmopressin if symptomatic
5
Q
What is 2nd line tx
-Von Willebrand Disease
A
Factor VIII
Von Willebrand factor
6
Q
Hemophilia most commonly affects (M or F)
and it is __-linked __
A
Males
x-linked recessive
7
Q
What is the most likely diagnosis:
Male child
Joint bleeding
May give history of prolonged bleeding with circumcision, dental repair, or surgery
PT, platelets, and platelet function is typically normal
PTT is prolonged
A
Hemophilia
8
Q
What is the most accurate test
Hemophilia
A
Specific assay for the disease (factor VIII, IX, or XI)
9
Q
What is the treatment of choice
Hemophilia
Mild bleeding;
Moderate/severe bleeding
A
Mild bleeding: desmopressin
Moderate/severe bleeding: give specific factor missing
10
Q
Secondary ITP most commonly from:
A
- Systemic disease: adult
- ITP most commonly chronic in adults
- Viral: Children
- ITP most commonly resolves in children - Common medications to induce ITP: quinidine, gold, penicillin, procainamide, TMP-SMX
11
Q
Common medications to induce ITP:
A
quinidine gold penicillin procainamide TMP-SMX
12
Q
What is the most likely diagnosis: Young woman Isolated low platelets Normal sized spleen Giant platelets
A
immune thrombocytopenic purpura
13
Q
Next best step
Immune Thrombocytopenic Purpura
A
CBC
14
Q
Best initial step
Immune Thrombocytopenic Purpura
A
CBC
15
Q
What is the most accurate test
Immune Thrombocytopenic Purpura
A
Bone marrow biopsy
16
Q
What is tx of choice
Immune thrombocytopenic purpura
A
- Platelets >30,000: observation
- Platelets <20,000: steroids (dexamethasone/prednisone)
- Platelets <10,000: Add IVIG for bump in platelets
17
Q
What is 2nd line tx
immune thrombocytopenic purpura
A
1.IVIG if steroids fail or contraindicated
Also used if need rapid increase in platelets (works in a few days)
2.Splenectomy
3.Rituximab
4.Immune modulators (azathioprine or cyclosporine) or thrombopoietin receptor agonists.
5. Severe bleeding is treated with platelet transfusion, IVIG, and steroids
18
Q
Most common cause of drug induced
Thrombotic Thrombocytopenic purpura
A
quinine
19
Q
Thrombotic Thrombocytopenic purpura
is most common in which patients
A
women
young adult
black
20
Q
What is the most likely diagnosis:
Hemolysis
Fragmented RBCs (schistocytes/helmet cells)
Renal dysfunction (increased creatinine/proteinuria)
Neuro and GI symptoms may be thrown in as well
Neurological dysfunction
low platelets
A
Thrombotic Thrombocytopenic Purpura
21
Q
Most accurate test
Thrombotic Thrombocytopenic purpura
A
Reduced plasma ADAMTS13 activity
22
Q
Treatment of choice
Thrombotic Thrombocytopenic purpura
A
- plasma exchange
- Steroids
- Rituximab
23
Q
Iron Deficiency anemia MC secondary to
A
blood loss
-GI or menses
24
Q
What is the most likely diagnosis Will give blood loss in vignette Heavy menses or GI bleed Decreased MCV Decreased iron/ferritin Increased TIBC/RDW
A
Iron deficiency anemia
25
Next best step
| Fe deficiency anemia
look for source of bleeding
26
Best initial test
| Fe deficiency anemia
CBC
27
Most accurate test
| Fe deficiency anemia
Bone marrow biopsy
28
tx of choice
| Fe deficiency anemia
ferrous sulfate
29
```
What is the most likely diagnosis
Will give chronic disease in vignette
Decreased or Normal MCV
Decreased iron
Normal ferritin
Decreased TIBC
```
Anemia of chronic disease
30
Best initial test
| anemia of chronic disease
CBC
31
What is the most accurate test
| anemia of chronic disease
bone marrow biopsy
32
what is the treatment of choice
| anemia of chronic disease
1. address underlying issue
| 2. erythropoietin in ESRD
33
Alpha thalassemia is common in ____ populations
Asian
34
Beta thalassemia is common in ___ populations
African descent
35
What is the most likely diagnosis
Elevated iron and ferritin
Decreased MCV
Target cells or tear drop cells on smear
thalassemia
36
how does minor thalassemia present
asymptomatic
37
how does Beta major thalassemia present
- symptoms start 4-6mo
- bone marrow deformity
- severe anemia
- pathologic fractures
38
how does alpha major thalassemia present
hydrops fetalis
| -causes fetal death
39
Target cells or tear drops on smear
thalassemia
40
what is best initial test
| -thalassemia
CBC
41
What is the most accurate test
| -thalassemia
electrophoresis
42
what is the treatment of choice
thalassemia
-trait
-major
trait: observation
major: transfusion & Fe chelation w/ deferoxamine
43
what is second line tx for thalassemia major
splenectomy
44
What is the most likely diagnosis
Hypersegmented neutrophils
Increased MCV
Elevated homocysteine
folate/b12 deficiency
45
hypersegmented neutrophils
| & elevated homocysteine
folate/b12 deficiency
46
peripheral neuropathy and neurologic symptoms associated with which anemia
B12
47
increased methylmalonic acid (MMA)
B12 deficiency/ anemia
48
medications that can cause B12 deficiency
1. metformin
2. PPI
3. cimetidine
49
Medications that can cause folate deficiency
1. methotrexate
2. trimethoprim
3. phenytoin
4. Etoh
50
Best initial test for B12 anemia
1. get B12 level
| 2. Get MME (methylmalonic acid)
51
treatment of choice
| B12 deficiency
1. B12 replacement PO or IM
| - if severe symptoms of preg IM preferred
52
treatment of choice
| folate deficiency
folate replacement
53
polycythemia vera
| presentation
- itching after hot showers
- elevated hematocrit
- Jak2 mutation
- typically asymptomatic
- hx of HTN or thrombotic event
54
Best initial test
| -polycythemia vera
CBC
55
Most accurate test
| -polycythemia vera
Jak2 mutation
56
tx of choice
| polycythemia vera
phlebotomy
| ASA
57
2nd line tx
| polycythemia vera
high risk: hydroxyurea
| -over 60 w/ thrombosis
58
which gender is more commonly affected in thrombocytosis
females
59
thrombocytosis
| MC secondary causes
1. chronic inflammation
2. infection
3. malignancy
60
erythromelalgia: burning of hands &feet
thrombocytosis
61
What is the most likely diagnosis
Look for symptoms of clotting and bleeding
Erythromelalgia: burning of hands and feet
Recurrent 1st trimester fetal loss in women
Elevated platelet count (can be up to 1,000,000 or more)
thrombocytosis
62
Best initial test
| thrombocytosis
CBC
63
Most accurate test
| thrombocytosis
Bone Marrow Biopsy
64
Tx of choice
| -thrombocytosis
hydroxyurea
65
Hemochromatosis
| -MC affects which population
50-60
| male
66
```
What is the most likely diagnosis
Male in 50s/60s
Elevated LFTs
Bronzing skin (hyperpigmentation)
Diabetes
Cirrhosis
```
Hemochromatosis
67
hemochromatosis
| -presentation
- 50-60yo
- elevated LFT
- Bronzing sign (hyperpigmentation)
- DM
- Cirrhosis
68
Best initial test
| -hemochromatosis
-ferritin & iron
69
most accurate test
| -hemochromatosis
-liver biopsy
70
tx of choice
| hemochromatosis
-phlebotomy
71
2nd line tx
| -hemochromatosis
-deferoxamine (Fe chelation)
72
Sickle cell
| -most commonly affected pop
African americans
73
```
What is the most likely diagnosis
African American patient
Acute chest, back, and/or extremity pain
Lung infiltrates might be shown
Fever
Hemolysis on lab work
```
sickle cell
74
helmet cell & schistocytes
Thrombotic Thrombopcytopenia purpura
75
next best step
| -sickle cell anemia
-if pt presents w/ fever, start antibiotics
76
Best initial test
| -sickle cell anemia
peripheral smear:
- sickled RBC (NOT seen with trait)
- Howell-Jolly bodies
77
Howell-Jolly bodies
sickle cell anemia
78
Most accurate test
| -sickle cell
HgB electrophoresis
79
Tx of choice
| -sickle cell anemia
1. folic acid supplements
2. hydroxyurea (prevents acute syndrome)
3. Acute pain crisis: hydration, O2, analgesia (may require opiods)
4. IF fever >101 give ceftriaxone
80
Most Common leukemia in adults
CLL
81
```
What is the most likely diagnosis
Patient over 50 complaining of fatigue
Elevated WBC
Cervical lymphadenopathy
Peripheral smear shows smudge cells and small mature lymphocytes
Anemia and thrombocytopenia
```
CLL
82
smudge cells & immature lymphocytes
CLL
83
best initial test for CLL
CBC
84
Most accurate test for CLL
1. flow cytometry
| 2. bone marrow biopsy
85
tx of choice for
| CLL
symptomatic & active:
1. fludarabine
2. cyclophosphamide
3. rituximab
86
```
What is the most likely diagnosis
Patient over 50 complaining of pruritis after hot showers
Fatigue
Weight loss
Abdominal fullness
Splenomegaly
Extremely high WBC >100,000
Philadelphia chromosome
translocation mutation
```
CML
87
Phildelphia chromosome is what kind of mutation and between which chromosomes
translocation
| BCR (chromosome 22) and ABL (Chromosome 9)
88
Best initial test for
| CML
CBC
89
Most accurate test for
| CML
BCR-ABL1 fusion gene
| Also known as philadelphia chromosome
90
Initial tx of choice
| CML
Tyrosine Kinase inhibitors
1. imatinib
2. ibrutinib
91
2nd line tx
| CML
bone marrow transplant
92
AML is more common in which pts
1. Down Syndrome
2. Li-Fraumeni
3. Fanconi
93
Patient over 60 presenting with pancytopenia
-Low hgb: fatigue
-Low wbc: infection
-Low platelets: bleeding
> 20% blasts on peripheral smear
Auer Rods and Phi bodies on bone marrow biopsy
AML
94
Auer Rods & phi bodies
AML
95
Best initial test
| AML
CBC peripheral smear
96
most accurate test
| AML
flow cytometry
97
treatment of choice AML
1. chemo
2. If patient has acute promyelocytic leukemia (APL) subtype, then add all-trans retinoic acid (ATRA) aka Vit A
Used to be called M3 subtype
98
2nd line tx
| AML
bone marrow transplant
99
MC pediatric anemia
ALL
100
```
What is the most likely diagnosis
5 year old patient presenting with a limp
Pancytopenia
Low hgb: fatigue
Low wbc: infection
Low platelets: bleeding
>20% blasts
```
ALL
101
Best initial test ALL
CBC peripheral smear
102
most accurate test
| ALL
flow cytometry
103
tx of choice
| ALL
chemo
104
2nd line tx
| ALL
bone marrow transplant
105
is non-hodgkin or hodgkin more common
non-hodgkin
106
What is the most likely diagnosis
Older patient presenting with fever, night sweats, and weight loss
Painless lymphadenopathy
Normal CBC
lymphoma
107
reed-sternberg cells
hodgkin lymphoma
108
pain with alcohol ingestion
hodgkin lymphoma
109
Best test lymphoma
excisional biopsy of lymph nodes
110
What is the treatment of choice
| Non-Hodgkin Stage 1, 2, 3 and 4
R-CHOP (gold standard chemotherapy)
Rituximab, cyclophosphamide, doxorubicin
vincristine
prednisone
111
tx Hodgkin Stage 1 and 2:
| Favorable disease:
ABVD (gold standard chemotherapy)
doxorubicin, bleomycin, vinblastine, dacarbazine
Unfavorable disease: ABVD + local radiation
112
tx Hodgkin Stage 3 and 4
ABVD plus radiation
113
multiple myeloma is MC in which population
African Americans
| >70
114
What is the most likely diagnosis
70 year old African American patient complaining of bone pain
Pathological fractures
Renal failure
Peripheral smear shows rouleaux formation
Hypercalcemia
Anemia
multiple myeloma
115
Peripheral smear shows rouleaux formation
multiple myeloma
116
multiple myeloma
What is the best initial test
if sure:
if unsure:
If unsure: X-Ray of the area with bone pain to look for lytic lesions
If sure about MM: CT, MRI, or PET as they are better than x-ray
117
Most accurate test
| multiple myeloma
bone marrow biopsy
118
treatment of choice
| multiple myeloma
Chemotherapy and immunomodulators
| Bortezomib, lenalidomide or cyclophosphamide, and dexamethasone
119
2nd line tx
| multiple myeloma
Autologous hematopoietic cell transplantation
120
G6PD MC in which pop
American black males and some Mediterranean populations.
121
G6PD commonly exacerbated by
1. Medications
- Aspirin
- Primaquine
- Dapsone
- Quinidine
- Nitrofurantoin
- Sulfonamides
2. Fava bean ingestion
3. infection
122
G6PD presentation
Intermittent episodes of hemolysis
- Elevated reticulocyte count
- Elevated Indirect bilirubin
- Elevated LDH
- Decreased haptoglobin
Jaundice and darkening of the urine
2dary to increased bilirubin
G6PD levels will be low
Asymptomatic in between episodes
123
Peripheral smear may reveal bite cells and Heinz bodies
G6PD
124
repeat testing of G6PD in ___ mo to confirm event
3
125
G6PD initial test
peripheral smear
126
most accurate test
| G6PD
G6PD activity levels
127
treatment of choice
| G6PD
avoid oxidative stress/triggers
