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Hematology Exam 1 > Hemoglobinopathies > Flashcards

Hemoglobinopathies Flashcards

1
Q

Usually with beta thal trait, one allele is normal and the other is ___

A

B+

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2
Q

How would you treat a stroke in someone with sickle cell disease?

A

Exchange transfusion; Transfuse children in order to PREVENT the stroke

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3
Q

Should you supplement iron in a patient with beta thal major?

A

NEVER

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4
Q

How do you treat acute chest syndrome?

A

Antibiotics, oxygen, simple or exchange transfusion to lower the HbS concentration

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5
Q

Target cells and what other unique type of findings are on a blood smear of someone with Hb C disease?

A

Hb C crystal

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6
Q

1/3 of patients with sickle cell will have had a ____ ____ by the time they are 30

A

Venous clot

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7
Q

a-/–; 3 alpha deletion

A

Hemoglobin H disease

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8
Q

Hydrops fetalis alpha globin genotype

A

–/–

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9
Q

Sickle cells are sticky and tend to attached to _____ cells

A

Endothelial

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10
Q

With an infant with Cooley’s Anemia, will it be symptomatic at birth?

A

No, because has not switched from the gamma to beta gene yet; symptoms emerge after ~ 6 mo

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11
Q

HbSB+ is milder/worse than HbSS

A

Milder

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12
Q

What is the most common cause of death in patients with sickle cell disease?

A

Acute chest syndrome

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13
Q

People with iron overload due to hyper absorption in the gut are more susceptible to infection with ____ spp., ____ and should avoid eating raw _____

A

Vibrio, mucor, shellfish

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14
Q

Iron overload may cause ____, ____, and endocrine failure

A

heart, liver

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15
Q

HbC substitution switches glu to __

A

lys at 6th aa

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16
Q

HbS substitution switches glu to __

A

val at 6th aa

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17
Q

Hb Bart’s

A

gamma4 tetramers in newborns; seen in alpha thal

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18
Q

In an adult, one unit of blood raises the Hb level by __

A

1

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19
Q

In a patient with sickle cell anemia with an acute drop in baseline Hb, should look for infection of what virus?

A

Parvovirus B19

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20
Q

Adult sickle cell patients do or do not have a spleen

A

Do not; it has infarcted

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21
Q

SE Asians have which alpha thalassemia?

A

aa/–

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22
Q

Splenic sequestration crisis

A

When a child has sickle cells sequestered in the outflow of the spleen that causes splenomegaly, increased drop in Hb and very low BP

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23
Q

Patients with sickle cell anemia and sickle cell trait are at an increased risk for ____ clots

A

Venous

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24
Q

HbC leads to increased

A

Cellular dehydration

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25
In a newborn who has alpha thal, what will the gel electrophoresis be like?
It will be abnormal due to the production of gamma4 tetramers (Hb Bart's)
26
The _____ ion channel is activated abnormally in sickle cell disease and that leads to ____ _______
Gardos; cellular dehydration
27
What are some respiratory complications in sickle cell disease?
Acute chest syndrome and pulmonary hypertension
28
How do you treat iron overload?
Iron chelation therapy
29
In patients with sickle cell anemia, when the Hb falls below the baseline, should look for what causes?
- Infection with parvovirus B19 - Splenic or hepatic sequestration - Acute chest syndrome
30
If someone has beta thal trait, what are some lab findings?
- very microcytic, so low MCV (~70) - high RBC - normal RDW
31
As soon as you find out someone has beta thal major, what are the steps to take?
Transfusion and iron chelation therapy
32
What are the effects of hydroxyurea?
- Increases baseline Hb values - Reduces the number of sickle crises - Reduces episodes of acute chest syndrome - Prevents pulmonary hypertension - Saves lives
33
What is the most common crisis that occurs in sickle cell patients?
Painful (vaso-occlusive) crisis
34
Aplastic crisis
Parvovirus B19 leads to bone marrow suppression that leads to rapid development of anemia
35
In B-thalassemia major, alpha globin tetramers are formed and they produce _____ bodies and cause destruction of red cells in periphery and in the marrow
Heinz
36
Give 2 units of blood to someone with Hb of 6 means they should then have Hb of __
8
37
What is the cause of stroke in children with sickle cell disease?
NOT atherosclerosis; Due to disordered blood vessels
38
In sickle cell patients, sepsis may occur from _____ organisms and if undergoing iron chelation therapy are at increased risk of infection with ____ or Vibrio organisms
encapsulated; Yersinia
39
Other complications in sickle cell disease
- Renal - Leg ulcers - Avascular necrosis (AVN) - Priapism (can lead to permanent erectile dysfunction)
40
How does hydroxyurea work?
Increases amount of HbF and that decreases the concentration of HbS
41
In thalassemias, you generally have full deletions of ___ genes or point mutations in ____ genes
alpha; beta
42
On gel electrophoresis, which Hb is the most positive and which is the most negative
Pos: HbF Neg: HbA
43
Beta thalassemias are mainly seen in what part of the world?
Mediterraneans
44
aa/a-; one alpha deletion is typically _____
asymptomatic
45
Who can never take hydroxyurea?
Pregnant women
46
Children with beta thal major either die from iron-overload causing organ failure or _____ ____
overwhelming anemia
47
B-thalassemias are most common in southern _____
Europe; Greece and Italy
48
T/F; you can only give a patient with acute chest syndrome an exchange transfusion
False
49
Pulmonary hypertension occurs in about ___ of sickle cell diseased adults
1/3
50
What is an effective oral treatment for sickle cell?
Hydroxyurea
51
Beta thal major and Hemoglobin H disease create what kind of tetramers, respectively?
Alpha4 and Beta4
52
A patient presents with new infiltrates on CXR and an O2 sat of 82%. How would you treat them?
With antibiotics, oxygen, simple or exchange transfusion
53
How could you diagnose Beta thalassemias major?
Gel with alpha tetramers present and absence or marked deficiency in HbA
54
Triggers of painful crisis
- Exercise - Dehydration - Infection - Cold - Stress - Menstruation - Surgery/trauma - Pregnancy EDICSMSP; "ED is Complaining of Some Major Sickle Pain"
55
HbSC= sickle cell _____
disease
56
Side effect of hydroxyurea
Bone marrow suppression
57
Children with sickle cell anemia need to be on ______ prophylaxis bc their ____ is dysfunctional
Penicillin; spleen
58
What is an exchange transfusion?
Put in a catheter and take out old red cells and then replace with new red cells over and over again and titrate to a certain percentage of HbS
59
What is the predominant chain formed in place of the alpha chain in hydrops fetalis?
Hb Bart's; gamma4 tetramer
60
The degree of ____ chain excess determines the severity of a thalassemia
Alpha
61
What is the only current cure for sickle cell disease?
Bone marrow transplant
62
What are the 2 complications in transfusing someone with sickle cell disease?
- Iron overload | - Allo-immunization
63
Main etiologies of acute chest syndrome
- Infection- especially with atypical organisms such as chlamydia or mycoplasma - Fat embolism from necrotic bone marrow
64
What blood groups should be avoided in transfusions for African Americans?
C,E, Kell
65
Autosplenectomy from splenic infarct occurs in sickle cell patients and that leads to increased susceptibility to _______ organsims
encapsulated
66
In children, rapid and extensive trapping of RBCs in the ____ cause massive enlargement, profound anemia (steep drop in Hb) and hypovolemic shock
spleen
67
African Americans tend to have which alpha thal?
a-/a-
68
Sickle cell disease is one of the most frequent causes of pulmonary ________
Hypertension; blood cannot get from the heart to the lungs
69
aa/-- and a-/a-; two gene deletions have ____ anemia and are microcytic with a ___ of around 70
mild; MCV
70
Alpha tetramers are unstable and lead to intramedullary hemolysis and what else?
Destruction of produced RBCs
71
What is a simple transfusion?
Giving red cells
72
HbSS = sickle cell _____
anemia
73
How would you treat hydrops fetalis?
Intrauterine exchange transfusion and bone marrow transplant
74
What is the median age for stroke in children with sickle cell disease?
5 years old
75
Where is the pain during a painful crisis in sickle cell anemia?
Bones and large joints
76
Sickle cell anemia is a problem of production/destruction
Destruction --> hemolysis
77
Other possible etiologies of acute chest syndrome
- Sequestration of sickle cells - Pulmonary infarction - Hypoventilation from rib infarct, narcotic administration - Pulmonary edema
78
Cooley's Anemia has what arrangement of B-globin genes?
B0/B0; most severe
79
How do you treat a painful crisis?
Treat the underlying infection if necessary, treat the pain, supplement O2, folate supplement, fluids is dehydrated (watch for pulmonary edema)
80
Symptoms of Beta thalassemia major may include:
- Anemia - Splenomegaly - Bone deformities - Iron overload associated symptoms (bronze skin, liver failure, endocrine failure)
81
Hemoglobin H=
B4 tetramers; these form Heinz bodies and cause hemolysis
82
Patients with this condition are functionally apslenic and more susceptible to infections with encapsulated organisms
Sickle cell anemia
83
What are 3 common sickle cell crises
1. Splenic sequestration crisis 2. Aplastic crisis 3. Painful (vaso-occlusive) crisis **SAP
84
In sickle cell anemia, the cells eventually may become irreversibly _____ and obstruct small blood vessels
sickled
85
Definition of acute chest syndrome
- Hypoxemia - New infiltrates on CXR - New fever, chest pain, dyspnea, or cough
86
With a stroke, what type of transfusion must be used?
Exchange
87
______ can worsen acute chest syndrome
Pneumonia
88
When transfusing, the Hb value should not get about __
10
89
Hemoglobin H disease will have what kind of cells present on the smear that is produced from Heinz bodies?
Bite cells
90
In an adult who has alpha thal, what will the gel electrophoresis be like?
It will be normal
91
HbSC genotype of sickle cell disease causes more intracellular _______ and worsening sickling
dehydration
92
What is the order of gene switching at the Beta-like globin gene locus?
Gamma --> Delta --> Beta
93
Salmonella ______ is unique to what disease?
Osteomyelitis; Sickle cell
94
In a gel electrophoresis of a patient with beta thal trait, there will be an increase in which band?
HbA2 bc there is an upregulation of the delta chain in the absence of the beta chain

Hematology Exam 1 (16 decks)

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