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PGY-4 Board Prep > Hematology > Flashcards

Hematology Flashcards

1
Q

Fanconi Anemia

A
  • usually pancytopenias
  • absent/abnormal thumbs
  • other congenital anomalies
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2
Q

Diamond-Blackfan Anemia

A

macrocytic anemia usually in first year of life

lots of congenital anomalies

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3
Q

Transient Erythroblastopenia of Childhood (TEC)

A

self limited normocytic anemia between age 1-3

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4
Q

MDS can transform to

A

AML

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5
Q

Treatment of CML

A

Tyrosine kinase inhibitors (TKIs)

imatinib, dasatinib, and nilotinib

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6
Q

Polycythemia Vera

A

JAK 2 + low EPO
transform to AML
Rx phlebotomy, ASA, hydroxurea

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7
Q

Essential Thrombocytosis

A

1/2 of ppl will have JAK2 mutation

asymptomatic or vasomotor symptoms (HA, parathesias)

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8
Q

Common cause of reactive thrombocytosis

A

IDA

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9
Q

Essential Thrombocytosis Complications

A

thrombosis
hemorrhage
acquired VWD and increased bleeding risk

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10
Q

Treatment of Essential Thrombocytosis

A

hydroxyurea if >1,000,000

ASA for vasomotor/high risk

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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
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12
Q

Primary Myelofibrosis Treatment

A

BMT

if JAK2 mutation, can use Ruxolitinib

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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
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14
Q

Medications Associated with Eosinophilia

A

carbamazepine, sulfonamides

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15
Q

Hypereosinophilic Syndrome

A

eosinophils >1500
end-organ damage attributed to eosinophilia, typically affecting the skin, lungs, heart, gastrointestinal tract, and brain

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16
Q

Hypereosinophilic Syndrome Treatment

A

steroids

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17
Q

Definition of Acute Leukemias

A

more than 20% blasts

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18
Q

Acute promyelocytic leukemia

A

chromosomal translocation t(15;17)

treatment with all-trans retinoic acid

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19
Q

AML treatment

A

induction therapy with an anthracycline (such as daunorubicin) and infusional cytarabine.

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20
Q

ALL in adults

A

tends to be B cells
can have Philadelphia chromosome
can have CNS involvement

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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

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22
Q

Screenings for adult survivors of leukemia

A

lipid profile
a1c/glucose
ECHO if anthracycline received

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23
Q

Elevated RBC association

A

thalassema

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24
Q

Corrected retic count

A

Retic % x hgb/normal hgb

normal hgb: 40 for women, 45 for men

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25
Q

MGUS

A

M protein less than 3
or less than 10% plasma cells on bone marrow
no end organ damage!

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26
Q

Multiple Myeloma - CRAB

A

Hypercalcemia
Renal Disease
Anemia
Bone Disease

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27
Q

Lenalidomide and pomalidomide adverse effects

A

VTE

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28
Q

Bortezomib AE

A

peripheral neuropathy

herpes zoster reactivation

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29
Q

Light chain amyloidosis

A

cardiac involvement is common

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30
Q

Waldenström macroglobulinemia (WM) i

A

indolent B cell lymphoma
secrete IgM
can develop hyperviscosity syndrome

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31
Q

Type I cryoglobulinemia

A

monoclonal immunoglobulin- usually IgM, associated with plasma cell dyscrasia
asymptomatic or hyperviscosity, thrombosis

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32
Q

Type II or III cryoglobulinemias

A

mixed

associated with hep C, connective tissue diseases

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33
Q

Treatment of skeletal lesions in multiple myeloma

A

Zoledronic acid and pamidronate

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34
Q

Treatment of acquired hypogammaglobulinemia associated with multiple myeloma

A

IVIG

will present with recurrent infections

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35
Q

Evaluating for bone lesions in smoldering multiple myeloma

A

Whole body MRI

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36
Q

Diagnosis of amyloidosis

A

if whole body symptoms- consider fat bad biopsy (less invasive)
otherwise, consideration of renal biopsy

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37
Q

5 Causes of Microcytic Hypoproliferative Anemia

A 
F- IDA
L - Lead poisoning
A - ACD
T - thalassemia
S - sideroblastic anemia
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38
Q

IDA Symptoms (board question style)

A

PICA
Restless Leg Syndrome
Dysphagia due to esophageal web or stricture/Plummer-Vinson Syndrome

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39
Q

IDA Lab Tests

A

decreased Iron
Increased TIBC and transferrin
decreased transferrin sat
decreased ferritin

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40
Q

IDA- Oral Fe Treatment will work in

A

6 weeks; therapy for 3-6 months

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41
Q

Beta Thalassemia’s

A

Minor: 1 normal allele, microcytic anemia
Intermedia: ?
Major: deletion of both alelles; severe hemolytic microcytic anemia

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42
Q

Alpha Thalassemia’s

A

Minima: no abnormalities
Minor/Trait: microcytosis and/or anemia
Hemoglobin H: microcytic anemia w/ hemolysis
Hydrops Fatalis

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43
Q

Causes of Sideroblastic Anemia

A

Lead, Alcohol, Linezolid

MDS

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44
Q

Symptoms of Lead Poisoning

A

abdominal pain, motor neuropathy

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45
Q

Lead Poisoning Treatment

A

EDTA chelation

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46
Q

soluble transferrin receptor

A

elevated in IDA, normal in ACD

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47
Q

ACD Labs, differences from IDA

A

normal or elevated ferritin

normal or decreased TIBC

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48
Q

Causes of Normocytic Anemia

A

ACD, Renal Failure
Thyroid Disease
Aplastic Anemias, Pure Red Cell Aplasias
MDS, MM, etc

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49
Q

Teardrop cells

A

MDS

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50
Q

Methylmalonic acid and homocysteine

A

Both elevated in B12

Only homocysteine elevated in folate

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51
Q

Differential for Macrocytic Anemia

A

B12, folate, alcohol
MDS
Liver disease, hypothyroidism

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52
Q

Extravascular Hemolysis

A
  • red cells removed by liver an spleen
  • spherocytes on smear
  • urine negative for Hgb and hemosiderin
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53
Q

Intravascular Hemolysis

A
  • red cells lyse while in circulation

- urine positive for Hgb and hemosiderin

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54
Q

What is elevated reticulocyte count?

A

Greater than 2%

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55
Q

Spherocytes differential

A

Dat + AIHA

Dat - hereditary spherocytosis

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56
Q

Bite Cells

A

G6PD

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57
Q

Goal Ferritin level in Anemia of Renal Disease

A

> 100

transferrin saturation >20%

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58
Q

Pernicious Anemia Antibodies

A

parietal cells > intrinsic factor Ab

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59
Q

What can be determined on electrophoresis

A

alpha thal- Hemoglobin H
Sickle cell disease
all Beta thals

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60
Q

Treatment of Thalassemias

A

folate supplementation

avoid iron as easily overloaded

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61
Q

Congenital Hemolysis Syndromes

A

Sickle cells
hereditary spherocytosis
G6PD def
thals

62
Q

G6PD Def Inheritance

A

X-linked; primarily in males

63
Q

Drugs Use associated with TTP

A

cocacine, ectasy, oxomorphone

64
Q

Primary Hemostasis

A

interaction between platelets, von Willebrand factor (vWF), and the vessel wall

65
Q

Secondary Hemostasis

A

activation of coagulation factors that eventually lead to fibrin clot formation

66
Q

PT evaluates

A

more sensitive to the effects of the vitamin K–dependent factors (II, VII, and X)

67
Q

PTT evaluates

A

factors VIII, IX, XI, and XII.

68
Q

Differential for prolonged PT

A

Factor VII deficiency
Vitamin K deficiency & Warfarin
Liver disease, DIC

69
Q

Differential for prolonged PTT

A

Von Willebrand Disease
Def of VIII, IX, XI, XII
Heparin Exposure

70
Q

Von Willebrand Factor Function

A

promotes platelet adhesion

functions as a protective carrier protein for factor VIII

71
Q

VWF Evaluation

A

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
Q

Treatment of VWD

A

Type 1 - desmopression

Type 2b, 3 - factor concentrates

73
Q

Active Bleeding in Liver Disease- treatment

A

treat to fibrinogen >100 and platelets >75

74
Q

Acquired von Willebrand Disease Causes

A

high circulatory shear stress (valvular heart disease, hypertrophic cardiomyopathy, circulatory assist devices, and extracorporeal membrane-oxygenation system

75
Q

Acquired Hemophilia

A

autoantibody directed against factor VII

often associated with pregnancy, autoimmune diseases, malignancy

76
Q

DIC Lab Findings

A

prolonged PT, PTT
low fibrinogen, low platelets
elevated D-dimer

77
Q

How to test for Thrombophilia

A

Stop anticoagulation for at least two weeks

78
Q

What to consider for heparin resistance

A

antithrombin defiency

79
Q

Most Common Causes of Thrombophilia

A

Factor V Leiden
Prothrombin G20210A Gene Mutation
Antithrombin Defiency

80
Q

Protein C and Protein S act as

A

natural anticoagulants

81
Q

Warfarin Induced Skin Necrosis, what commonly causes this…

A

Protein C deficiency

82
Q

Antiphospholipid Syndrome Lab Abnormality

A

elevated PTT

83
Q

Additional Evaluation of Budd-Chiari

A

usually associated with myeloproliferative neoplasm

consider screening including JAK2 mutation

84
Q

Dabigatran MOA

A

direct thrombin inhibitor

85
Q

NOAC Mechanisms

A

factor Xa inhibitors

86
Q

Monitoring unfractionated heparin

87
Q

Monitoring LMWH

A

Factor Xa if needed

88
Q

Bridging/Overlapping Warfarin when initiating for acute DVT/PE

A

overlap for at least 5 days and until INR >2 for 24 hours

89
Q

Heparin Antidote

A

protamine sulfate

90
Q

Supratherapeutic INR while on warfarin - asymptomatic

A

4.5 - 10: Withhold

>10: Vitamin K 2.5mg

91
Q

Supratherapeutic INR while on warfarin - symptomatic

A

vitamin k + four factor PCC

92
Q

Idarucizumab

A

monoclonal antibody to reverse dabigatran

93
Q

Therapetuic Level of Heparin, way to estimate…

A

1.5-2.5 x PTT

94
Q

Chronic ITP Treatment During Pregnancy

A

Monitory unless platelets <30K

95
Q

ALL Risk Factor

A

EBV, HIV/T-CL

96
Q

CLL Risk Factors

A

Agent orange, first degree relatives

97
Q

AML Symptoms

A

gingivial hyperplasia but no LAD or splenomegaly

98
Q

AML Treatment

A

cytarabine + idarubicin

99
Q

Vincristine side effects

A

neurotoxicity

100
Q

Vinblastine side effects

A

blasts the bone marrow –> pancytopenic

101
Q

Bleomycin side effects

A

lung toxicity/IPF

102
Q

ALL treatment

A

1) BCR/ABL (t9:22) with imatinib
2) CD 20+ rituximab
3) CVAD (cyclophos, vincristine, Adriamycin/doxorubicin, dexameth)

103
Q

ALL treatment for pediatrics includes

A

asaparaginase

104
Q

Hairy Cell Leukemia specifics

A

older pts,
CDIIc+, dry tap
treat w/ Cladribine (2-Cda)

105
Q

Indolent B Cell Lymphomas

A

Follicular
MALT
CLL
Hairy Cell

106
Q

Aggressive B Cell Lymphomas

A

Diffuse B Cell Lymphoma
Mantle Cell
Burkitt

107
Q

T Cell Lymphomas - Cutaneous

A

Myocosis fungoides

Searzy Syndrome

108
Q

NLHs, cell type

A

B > T Cell

109
Q

T Cell Lymphomas - Peripheral

A

Anaplastic Large Cell Lymphoma

Angioimmunoblastic T Cell Lymphoma

110
Q

Hodgkin’s Lymphoma - General

A

Bimodal, common in 2nd & 3rd decade

Biopsy shows Reed-Sternburg/Owls eye nodes

111
Q

ABVD

A

doxorubicin
bleomycin
vincristine
dacarbazine

112
Q

Hodgkins Lymphoma Treatment

113
Q

Follicular Lymphoma

A

B cell, t14:18
Treat with ritubixmab +/- CHOP
transform to large B cell lymphoma

114
Q

CLL/SLL clinical pearls

A

associated with hypogammaglobulinemias, AIHA, ITP

can transform to large cell/Richter transformation

115
Q

Hairy Cell Lymphoma Rx

A

Purine Nucleoside Agents (pentostatin + cladribine)

116
Q

Diffuse Large Cell Lymphoma Rx

117
Q

Burkitt Lymphoma Types

A

Endemic/Africa/EBV
Sporadic/US w/ abd or pelvic involvement
HIV associatd

118
Q

Burkitt Lymphoma Rx

A

Rituximab + CVAD

R-hyper CVAD

119
Q

Lymphoblastic Lymphoma

A

T or B Cell; T more prevalent in peds

usually anterior mediastinal mass

120
Q

Lymphoblastic Lymphoma Rx

A

CHOP +/- Rituximab

121
Q

Use of bevacizumab

A

colon cancer + renal cell carcinoma

122
Q

Mantle Cell Lymphoma

A

Cyclin D1, t(11:14), CD 19,20,5 positive

123
Q

Treatment of Aplastic Anemia

A

Stem Cell Transplant

If not possible, immunosuppression

124
Q

Causes (3) of Pure Red Cell Aplasia

A
  • Parvovirus B19
  • Thymoma
  • large granular lymphocyte leukemia
125
Q

MDS Treatment

A

cure: HPSCT
otherwise treatment is based on symptomatic cytopenias + reducing transformation to AML
transfusions, EPO stim, hypomethalating agents

126
Q

MDS Agents for preventing progression to AML

A

hypometh: azacytidine and decitabine

5q - lenalidomide

127
Q

Things that progress to AML

A

MDS, CMP, PV

128
Q

CML associated chromosome

A

t9:22, Philadelphia, so can treat with imatinib

129
Q

Acute Porphyria symptoms

A
  • abd pain
  • sensory & peripheral neuropathies w/ weakness
  • ANS dysregulation
130
Q

Acute Porphyria testing

A

urine porphilinogen

131
Q

Colorectal genetic mutations associated with EGFR inhibitor (cetuximab and panitumumab) resistance

A

KRAS and NRAS

132
Q

Most common indications for autologous HSCT

A

MM, relapsed NHL

133
Q

Resected Pancreatitis Adjuvant Treatment

A

Chemotherapy (gemcitabine, capecitabine)

134
Q

Tumor marker that is negative in pure seminoma

135
Q

Common Causes of Vitamin K deficiency

A

Poor oral intake, antibiotics, malabsorption

136
Q

Cold agglutinin hemolytic anemia treatment

137
Q

CLL diagnostic test

A

peripheral blood flow cytometry

138
Q

Androgen deprivation therapy equivalent treatments

A

Orchiectomy, GnRH agonist, GnRH antagonist

139
Q

Treatment of factor VIII-inhibitor–related bleeding

A

factor VII

140
Q

Rx AL Amyloidosis

A

Autologous HSCT

141
Q

Transfusion-related infectious cause of hemolytic anemia

142
Q

Febrile Nonhemolytic Transfusion Rcxn Rx

A

leukoreduction

143
Q

Atypical HUS Rx

A

Eculizumab

144
Q

Chemotherapy & ATN

145
Q

CUP - isolated cervical lymphadenopathy

A

Head & Neck Cancer

146
Q

ITP infectious causes

A

HIV
Hepatitis C
H pylori

147
Q

Rx breast cancer + bone metases

A

chemo + bisphosphonates

148
Q

Crizotinib

A

ALK and ROS1 + NSCLC

149
Q

Types of SCD

A

Hb SS - SCD
Hb S - trait
Hb SC - hemoglobin C
Hb SB+ thal

150
Q

Protein C associations

A

Vitamin K dependent

  • warfarin induced skin necrosis
  • thrombisis
  • neonatal purpura fulminans
151
Q

Sickle Cell Disease New Treatments

A

L-glutamine

Crizanlizumab

152
Q

Paroxysmal Noctural Hemoglobinuria

A

unexplained hemolysis, thrombosis, pancytopenia

CD55 and CD59 def

PGY-4 Board Prep (18 decks)

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