Hematology Flashcards
1
Q
Fanconi Anemia
A
- usually pancytopenias
- absent/abnormal thumbs
- other congenital anomalies
2
Q
Diamond-Blackfan Anemia
A
macrocytic anemia usually in first year of life
lots of congenital anomalies
3
Q
Transient Erythroblastopenia of Childhood (TEC)
A
self limited normocytic anemia between age 1-3
4
Q
MDS can transform to
A
AML
5
Q
Treatment of CML
A
Tyrosine kinase inhibitors (TKIs)
imatinib, dasatinib, and nilotinib
6
Q
Polycythemia Vera
A
JAK 2 + low EPO
transform to AML
Rx phlebotomy, ASA, hydroxurea
7
Q
Essential Thrombocytosis
A
1/2 of ppl will have JAK2 mutation
asymptomatic or vasomotor symptoms (HA, parathesias)
8
Q
Common cause of reactive thrombocytosis
A
IDA
9
Q
Essential Thrombocytosis Complications
A
thrombosis
hemorrhage
acquired VWD and increased bleeding risk
10
Q
Treatment of Essential Thrombocytosis
A
hydroxyurea if >1,000,000
ASA for vasomotor/high risk
11
Q
Primary Myelofibrosis Presentation
A
Usually symptomatic on presentation
- Cytokine-related hypercatabolic symptoms, such as fatigue, weight loss, fever, and chills
- Splenomegaly, abd distension
- extramedullary hematopoiesis
12
Q
Primary Myelofibrosis Treatment
A
BMT
if JAK2 mutation, can use Ruxolitinib
13
Q
Causes of Eosinophilia
A
CHINA C- collagen vascular disease H- Helminth infection (Strongyloides) I- idiopathic N- neoplasm; lymphomas and myeloproliferative A- allergy, atopy, asthma
14
Q
Medications Associated with Eosinophilia
A
carbamazepine, sulfonamides
15
Q
Hypereosinophilic Syndrome
A
eosinophils >1500
end-organ damage attributed to eosinophilia, typically affecting the skin, lungs, heart, gastrointestinal tract, and brain
16
Q
Hypereosinophilic Syndrome Treatment
A
steroids
17
Q
Definition of Acute Leukemias
A
more than 20% blasts
18
Q
Acute promyelocytic leukemia
A
chromosomal translocation t(15;17)
treatment with all-trans retinoic acid
19
Q
AML treatment
A
induction therapy with an anthracycline (such as daunorubicin) and infusional cytarabine.
20
Q
ALL in adults
A
tends to be B cells
can have Philadelphia chromosome
can have CNS involvement
21
Q
Anemia associated with CKD
A
Use darbopoetin/EPA for Hgb <10 in those with HD; do not increase to >11.5
However iron stores must be normal for EPA to work, so if low, administer iron prior to EPA
22
Q
Screenings for adult survivors of leukemia
A
lipid profile
a1c/glucose
ECHO if anthracycline received
23
Q
Elevated RBC association
A
thalassema
24
Q
Corrected retic count
A
Retic % x hgb/normal hgb
normal hgb: 40 for women, 45 for men
25
MGUS
M protein less than 3
or less than 10% plasma cells on bone marrow
no end organ damage!
26
Multiple Myeloma - CRAB
Hypercalcemia
Renal Disease
Anemia
Bone Disease
27
Lenalidomide and pomalidomide adverse effects
VTE
28
Bortezomib AE
peripheral neuropathy
| herpes zoster reactivation
29
Light chain amyloidosis
cardiac involvement is common
30
Waldenström macroglobulinemia (WM) i
indolent B cell lymphoma
secrete IgM
can develop hyperviscosity syndrome
31
Type I cryoglobulinemia
monoclonal immunoglobulin- usually IgM, associated with plasma cell dyscrasia
asymptomatic or hyperviscosity, thrombosis
32
Type II or III cryoglobulinemias
mixed
| associated with hep C, connective tissue diseases
33
Treatment of skeletal lesions in multiple myeloma
Zoledronic acid and pamidronate
34
Treatment of acquired hypogammaglobulinemia associated with multiple myeloma
IVIG
| will present with recurrent infections
35
Evaluating for bone lesions in smoldering multiple myeloma
Whole body MRI
36
Diagnosis of amyloidosis
if whole body symptoms- consider fat bad biopsy (less invasive)
otherwise, consideration of renal biopsy
37
5 Causes of Microcytic Hypoproliferative Anemia
```
F- IDA
L - Lead poisoning
A - ACD
T - thalassemia
S - sideroblastic anemia
```
38
IDA Symptoms (board question style)
PICA
Restless Leg Syndrome
Dysphagia due to esophageal web or stricture/Plummer-Vinson Syndrome
39
IDA Lab Tests
decreased Iron
Increased TIBC and transferrin
decreased transferrin sat
decreased ferritin
40
IDA- Oral Fe Treatment will work in
6 weeks; therapy for 3-6 months
41
Beta Thalassemia's
Minor: 1 normal allele, microcytic anemia
Intermedia: ?
Major: deletion of both alelles; severe hemolytic microcytic anemia
42
Alpha Thalassemia's
Minima: no abnormalities
Minor/Trait: microcytosis and/or anemia
Hemoglobin H: microcytic anemia w/ hemolysis
Hydrops Fatalis
43
Causes of Sideroblastic Anemia
Lead, Alcohol, Linezolid
| MDS
44
Symptoms of Lead Poisoning
abdominal pain, motor neuropathy
45
Lead Poisoning Treatment
EDTA chelation
46
soluble transferrin receptor
elevated in IDA, normal in ACD
47
ACD Labs, differences from IDA
normal or elevated ferritin
| normal or decreased TIBC
48
Causes of Normocytic Anemia
ACD, Renal Failure
Thyroid Disease
Aplastic Anemias, Pure Red Cell Aplasias
MDS, MM, etc
49
Teardrop cells
MDS
50
Methylmalonic acid and homocysteine
Both elevated in B12
| Only homocysteine elevated in folate
51
Differential for Macrocytic Anemia
B12, folate, alcohol
MDS
Liver disease, hypothyroidism
52
Extravascular Hemolysis
- red cells removed by liver an spleen
- spherocytes on smear
- urine negative for Hgb and hemosiderin
53
Intravascular Hemolysis
- red cells lyse while in circulation
| - urine positive for Hgb and hemosiderin
54
What is elevated reticulocyte count?
Greater than 2%
55
Spherocytes differential
Dat + AIHA
| Dat - hereditary spherocytosis
56
Bite Cells
G6PD
57
Goal Ferritin level in Anemia of Renal Disease
>100
| transferrin saturation >20%
58
Pernicious Anemia Antibodies
parietal cells > intrinsic factor Ab
59
What can be determined on electrophoresis
alpha thal- Hemoglobin H
Sickle cell disease
all Beta thals
60
Treatment of Thalassemias
folate supplementation
| avoid iron as easily overloaded
61
Congenital Hemolysis Syndromes
Sickle cells
hereditary spherocytosis
G6PD def
thals
62
G6PD Def Inheritance
X-linked; primarily in males
63
Drugs Use associated with TTP
cocacine, ectasy, oxomorphone
64
Primary Hemostasis
interaction between platelets, von Willebrand factor (vWF), and the vessel wall
65
Secondary Hemostasis
activation of coagulation factors that eventually lead to fibrin clot formation
66
PT evaluates
more sensitive to the effects of the vitamin K–dependent factors (II, VII, and X)
67
PTT evaluates
factors VIII, IX, XI, and XII.
68
Differential for prolonged PT
Factor VII deficiency
Vitamin K deficiency & Warfarin
Liver disease, DIC
69
Differential for prolonged PTT
Von Willebrand Disease
Def of VIII, IX, XI, XII
Heparin Exposure
70
Von Willebrand Factor Function
promotes platelet adhesion
| functions as a protective carrier protein for factor VIII
71
VWF Evaluation
normal or prolonged PTT
prolonged closure time on the PFA-100
reduction in von Willebrand antigen (quantitative analysis) - less than 30%
reduced vWF ristocetin cofactor activity
72
Treatment of VWD
Type 1 - desmopression
| Type 2b, 3 - factor concentrates
73
Active Bleeding in Liver Disease- treatment
treat to fibrinogen >100 and platelets >75
74
Acquired von Willebrand Disease Causes
high circulatory shear stress (valvular heart disease, hypertrophic cardiomyopathy, circulatory assist devices, and extracorporeal membrane-oxygenation system
75
Acquired Hemophilia
autoantibody directed against factor VII
| often associated with pregnancy, autoimmune diseases, malignancy
76
DIC Lab Findings
prolonged PT, PTT
low fibrinogen, low platelets
elevated D-dimer
77
How to test for Thrombophilia
Stop anticoagulation for at least two weeks
78
What to consider for heparin resistance
antithrombin defiency
79
Most Common Causes of Thrombophilia
Factor V Leiden
Prothrombin G20210A Gene Mutation
Antithrombin Defiency
80
Protein C and Protein S act as
natural anticoagulants
81
Warfarin Induced Skin Necrosis, what commonly causes this...
Protein C deficiency
82
Antiphospholipid Syndrome Lab Abnormality
elevated PTT
83
Additional Evaluation of Budd-Chiari
usually associated with myeloproliferative neoplasm
| consider screening including JAK2 mutation
84
Dabigatran MOA
direct thrombin inhibitor
85
NOAC Mechanisms
factor Xa inhibitors
86
Monitoring unfractionated heparin
PTT
87
Monitoring LMWH
Factor Xa if needed
88
Bridging/Overlapping Warfarin when initiating for acute DVT/PE
overlap for at least 5 days and until INR >2 for 24 hours
89
Heparin Antidote
protamine sulfate
90
Supratherapeutic INR while on warfarin - asymptomatic
4.5 - 10: Withhold
| >10: Vitamin K 2.5mg
91
Supratherapeutic INR while on warfarin - symptomatic
vitamin k + four factor PCC
92
Idarucizumab
monoclonal antibody to reverse dabigatran
93
Therapetuic Level of Heparin, way to estimate...
1.5-2.5 x PTT
94
Chronic ITP Treatment During Pregnancy
Monitory unless platelets <30K
95
ALL Risk Factor
EBV, HIV/T-CL
96
CLL Risk Factors
Agent orange, first degree relatives
97
AML Symptoms
gingivial hyperplasia but no LAD or splenomegaly
98
AML Treatment
cytarabine + idarubicin
99
Vincristine side effects
neurotoxicity
100
Vinblastine side effects
blasts the bone marrow --> pancytopenic
101
Bleomycin side effects
lung toxicity/IPF
102
ALL treatment
1) BCR/ABL (t9:22) with imatinib
2) CD 20+ rituximab
3) CVAD (cyclophos, vincristine, Adriamycin/doxorubicin, dexameth)
103
ALL treatment for pediatrics includes
asaparaginase
104
Hairy Cell Leukemia specifics
older pts,
CDIIc+, dry tap
treat w/ Cladribine (2-Cda)
105
Indolent B Cell Lymphomas
Follicular
MALT
CLL
Hairy Cell
106
Aggressive B Cell Lymphomas
Diffuse B Cell Lymphoma
Mantle Cell
Burkitt
107
T Cell Lymphomas - Cutaneous
Myocosis fungoides
| Searzy Syndrome
108
NLHs, cell type
B > T Cell
109
T Cell Lymphomas - Peripheral
Anaplastic Large Cell Lymphoma
| Angioimmunoblastic T Cell Lymphoma
110
Hodgkin's Lymphoma - General
Bimodal, common in 2nd & 3rd decade
| Biopsy shows Reed-Sternburg/Owls eye nodes
111
ABVD
doxorubicin
bleomycin
vincristine
dacarbazine
112
Hodgkins Lymphoma Treatment
ABVD
113
Follicular Lymphoma
B cell, t14:18
Treat with ritubixmab +/- CHOP
transform to large B cell lymphoma
114
CLL/SLL clinical pearls
associated with hypogammaglobulinemias, AIHA, ITP
| can transform to large cell/Richter transformation
115
Hairy Cell Lymphoma Rx
Purine Nucleoside Agents (pentostatin + cladribine)
116
Diffuse Large Cell Lymphoma Rx
R-CHOP
117
Burkitt Lymphoma Types
Endemic/Africa/EBV
Sporadic/US w/ abd or pelvic involvement
HIV associatd
118
Burkitt Lymphoma Rx
Rituximab + CVAD
| R-hyper CVAD
119
Lymphoblastic Lymphoma
T or B Cell; T more prevalent in peds
| usually anterior mediastinal mass
120
Lymphoblastic Lymphoma Rx
CHOP +/- Rituximab
121
Use of bevacizumab
colon cancer + renal cell carcinoma
122
Mantle Cell Lymphoma
Cyclin D1, t(11:14), CD 19,20,5 positive
123
Treatment of Aplastic Anemia
Stem Cell Transplant
| If not possible, immunosuppression
124
Causes (3) of Pure Red Cell Aplasia
- Parvovirus B19
- Thymoma
- large granular lymphocyte leukemia
125
MDS Treatment
cure: HPSCT
otherwise treatment is based on symptomatic cytopenias + reducing transformation to AML
transfusions, EPO stim, hypomethalating agents
126
MDS Agents for preventing progression to AML
hypometh: azacytidine and decitabine
| 5q - lenalidomide
127
Things that progress to AML
MDS, CMP, PV
128
CML associated chromosome
t9:22, Philadelphia, so can treat with imatinib
129
Acute Porphyria symptoms
- abd pain
- sensory & peripheral neuropathies w/ weakness
- ANS dysregulation
130
Acute Porphyria testing
urine porphilinogen
131
Colorectal genetic mutations associated with EGFR inhibitor (cetuximab and panitumumab) resistance
KRAS and NRAS
132
Most common indications for autologous HSCT
MM, relapsed NHL
133
Resected Pancreatitis Adjuvant Treatment
Chemotherapy (gemcitabine, capecitabine)
134
Tumor marker that is negative in pure seminoma
AFP
135
Common Causes of Vitamin K deficiency
Poor oral intake, antibiotics, malabsorption
136
Cold agglutinin hemolytic anemia treatment
Rituximab
137
CLL diagnostic test
peripheral blood flow cytometry
138
Androgen deprivation therapy equivalent treatments
Orchiectomy, GnRH agonist, GnRH antagonist
139
Treatment of factor VIII-inhibitor–related bleeding
factor VII
140
Rx AL Amyloidosis
Autologous HSCT
141
Transfusion-related infectious cause of hemolytic anemia
Babeiosa
142
Febrile Nonhemolytic Transfusion Rcxn Rx
leukoreduction
143
Atypical HUS Rx
Eculizumab
144
Chemotherapy & ATN
Cisplatin
145
CUP - isolated cervical lymphadenopathy
Head & Neck Cancer
146
ITP infectious causes
HIV
Hepatitis C
H pylori
147
Rx breast cancer + bone metases
chemo + bisphosphonates
148
Crizotinib
ALK and ROS1 + NSCLC
149
Types of SCD
Hb SS - SCD
Hb S - trait
Hb SC - hemoglobin C
Hb SB+ thal
150
Protein C associations
Vitamin K dependent
- warfarin induced skin necrosis
- thrombisis
- neonatal purpura fulminans
151
Sickle Cell Disease New Treatments
L-glutamine
| Crizanlizumab
152
Paroxysmal Noctural Hemoglobinuria
unexplained hemolysis, thrombosis, pancytopenia
| CD55 and CD59 def
