Pics 2: hemoglobinopathy Flashcards

Hbopathy

1
Q

Thalassemia summary and map

A
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2
Q

Alpha thal slide
silent carrier
trait

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3
Q

HbH disease slide

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4
Q

Hydrops fetalis slide

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5
Q

HbE summary

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6
Q

Hb Lepore summary

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7
Q

Hb Constant spring summary

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8
Q

Thalassmias and Hbopathy summary slide overview

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9
Q

HbH disease
symptoms
genotypes

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10
Q

Hydrops fetalis
Pathophys of manifestations

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11
Q

Alpha thal
Genotype:phenotype associations

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12
Q

B thal major: pathophys and clinical features

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13
Q

B thal genotype:phenotype assocations

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14
Q

TDT vs NTDT full body complications

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15
Q

Iron chelator agents
Effects on iron overload/organs
side effects/monitoring

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16
Q

Schematic of thalassmias and their classifications

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17
Q

Thalassemia: newer therapies and their MOA

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18
Q

Gene therapies in Thalassemia

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19
Q

Summary table for thalassemias vs hemoglobinopathies

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19
Q

Hb electrophoresis and HPLC examples

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20
Q

Sickle cell trait and SCD phenotypes

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21
Q

SCD complications by spectrum of hemolysis/endothelial dysfunction to viscosity/vasoocclusion

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21
Q

Pathophys of RBC sickling

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22
Q

Management algorithm for suspected acute chest syndrome

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22
Q

Sickle cell disease full body complications
acute and chronic

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23
Q

Mgmt of TIA or ischemic stroke in sickle cell disease
by timeline of presentation

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24
Q

Peds: TCD and stroke prevention
primary vs secondary
trials

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25
Q

Acute sickle hepatopathies: mgmt

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26
Q

General overview of SCD treatments

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26
Q

Benefits of HU in SCD by physiology

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27
Q

HU therapy toxicities

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27
Q

Acute emergency transfusion indications
Simple vs RCE

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28
Q

Chronic transfusions
Targets
Indications for primary and secondary prevention

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29
Q

Transfusion around elective surgery
Low/Int/High risk surgery

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30
Q

Hemoglobin structure and globins, location of production over lifespan

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31
Q

Spectrum of thalassemias by transfusion dependence

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32
Q

Overview of SCD stuff to know

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33
Q

Thalassemia: Classification TDT or NTDT and management

A
34
Q

Complications TDT vs NTDT chart/table Ash SAP

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34
Q

Pathophys flowchart of beta thal

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35
Q

Iron chelators table ASH SAP

A
36
Q

Lab findings various Sickle Cell disease syndromes
severity, Hb fractions, Hb level and MCV

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37
Q

Important clinical trials in SCD

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37
Q

Acute exacerbation of anemia in SCD
Differential Dx

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38
Q

Immunizations for patients with SCD

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