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IHO Week 2 > Anemia - Krafts > Flashcards

Anemia - Krafts Flashcards

1
Q

Name two types of hemolytic anemias:

A

Hereditary Spherocytosis

Autoimmune Hemolytic Anemia

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2
Q

What anemias have weird shaped RBCs?

A

Hemolytic Anemias

Sickle Cell Anemias

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3
Q

Define Hemoglobinopathies

A

Change in the quality or structure of the hemoglobin

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4
Q

Define Poikiocytosis

A

Weird Shaped RBCs

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5
Q

What happens in Sickle Cells?

A

Hemolysis

Vaso-occlusion

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6
Q

What is the most important hemoglobinopathy?

A

Sickle Cell Anemia

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7
Q

What is the usual cause of hemoglobinopathies?

A

A single amino acid (Valine to Glutamate) point mutation in the B-Globin chain

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8
Q

What is the best way to test for hemoglobinopathies?

A

Hemoglobin Electrophoresis

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9
Q

What are the genes for Sickle-Cell Trait?

A

HbA - HbS

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10
Q

What are the genes for Sickle-Cell Anemia?

A

HbS - HbS

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11
Q

Sickle Cell Anemia Characteristics

A

Blacks (8% are heterozygous)
Variable severity
Increased infections due to autosplenectomy

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12
Q

What causes an autosplenectomy?

A

Spleen shrinks down due to infarctions healing and causing fibrosis. Spleen is needed to kill encapsulated organisms (pneumococcal and H. flu)

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13
Q

Morphology of Sickle Cell Anemia (Post-Splenectomy

A 
Nucleated RBCs
Target RBCs
Howell-Jolly Bodies
Pappenheimer Bodies
Increased Platelet Count
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14
Q

Target RBCs

A

Too much membrane or not enough volume

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15
Q

Pappenheimer Bodies

A

Aggregates of iron within the cell, usually removed by macrophages

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16
Q

What are the triggers for sickle cell anemia that need to be prevented?

A

Infection, Fever, Dehydration, Hypoxemia (keep patient from de-oxygenating)

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17
Q

What vaccinations should be given to Sickle Cell Anemia patients when the spleen is autosplenectomized?

A

Encapsulated bugs
Streptococcus Pneumoniae
Hemophilus Influenzae

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18
Q

What are the other treatments for Sickle Cell Anemia?

A

Blood Transfusions

Bone Marrow Transplantations (in children)

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19
Q

Thalassemia

A 
Hypochromic, Microcytic Anemia
Can't make enough alpha or beta hemoglobin chains
See Target Cells
Basophilic Stippling
Medullary Expansion
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20
Q

Normal Globin Genes

A

4 alpha-chain genes

2 beta-chain genes

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21
Q

Alpha Thalassemia Gene Defect

A

Deletion of one or more Alpha-chain gene(s)

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22
Q

Beta Thalassemia Gene Defect

A

Defective Beta-chain gene(s)

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23
Q

Cause of B-Thalassemia

A

Defective transcription, translation, or processing of mRNA of B-chain gene

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24
Q

Severity of B-Thalassemia

A

B gene = normal gene
B+ gene = produces some B-chains
B0 gene = produces no B-chains

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25
B-Thalassemia Minor
Asymoptomatic
26
B-Thalassemia Intermedia
Less Severe Thalassemia
27
B-Thalassemia Major
Severe Thalassemia
28
-a/aa Thalassemia
Silent
29
--/aa or -a/-a
Alpha-Thalassemia Trait: Asymptomatic
30
--/-a
Hemoglobin H Disease: Moderate/Severe Disease
31
--/-- (No alpha chain genes)
Hydrops Fetalis: Fatal in utero
32
What causes anemia in a-thalassemia?
Not enough a-chains Excess unpaired B, gamma, or delta chains Gamma tetramers in newborns Beta tetramers in adults
33
What causes anemia in B-thalassemia?
Not enough B-chains | Excess unpaired a-chains
34
Anisocytosis
Varying sizes of RBCs
35
Glucose-6-Phosphate Dehydrogenase Deficiency
Oxidant Exposure causes increased perioxides and premature cell lysis Bite Cells/Heinz Bodies
36
G6PD Pathway
G6PD keeps NADP in its reduced state of NADPH so that GSSG can be reduced to GSH in order to get rid of free radicals
37
G6PD RBCs Lyse because...
Heme breaks away from globin
38
Heinz Bodies
Denatured globin that sticks to the RBC membrane
39
Highest incidence of G6PD coincides with what disease?
Malaria
40
What are the triggers of G6PD deficiency?
Fava Beans, Drugs, Antibiotics, Aspirin, Peroxide
41
Microangiopathic Hemolytic Anemia
Physical Trauma to RBC Schistocytes Triangulocyte Secondary to other cause
42
Causes of Microangiopathic Hemolytic Anemia (MAHA)
Runner's Anemia Artificial Heart Valve Anything causing tiny blood clots (fibrin) all over the body in narrow blood vessels (DIC, TTP, HUS)
43
Normocytic Anemias are caused by...
Blood Loss Chronic Disease Kidney or Liver Disease Aplastic Anemia
44
Anemia of (acute) Blood Loss
Traumatic Cause Initially, Hemoglobin is NORMAL After 2-3 days, see Reticulocytes
45
Anemia of Chronic Disease
Infections, Inflammation, Malignancy | Iron Metabolism Disturbed
46
Why is there disturbed iron metabolism seen in Anemia of Chronic Disease?
Absorption is ok, but release of iron is disrupted | Can't get iron into Hemoglobin and Increased Hepcidin levels leads to short RBC life
47
Labs for Anemia of Chronic Disease
Low Iron, TIBC HIGH FERRITIN High Marrow Storage Iron
48
Labs for Iron Deficiency Anemia
Low Iron, Ferritin, Marrow Storage Iron | High TIBC
49
Cause of Anemia of Renal Disease (end stage)
Lack of Erythropoietin | See Echinocytes
50
Management of Severe Anemia of Renal Disease
Erythropoietin or Blood Transfusion
51
Anemia of Liver Disease
See acanthocytes and Target Cells | Due to multiple causes
52
Acanthocytes
Spur Cells - tiny spur-like projections
53
Echinocytes
Burr Cell, serrated edges over entire surface, sea urchin
54
Uncomplicated Anemia of Liver Disease (RARE!)
Decreased RBC survival and Impaired Marrow Response Mild Anemia Normocytic (sometimes macrocytic) Poikilocytosis
55
Complicated Anemia of Liver Disease
Megaloblastosis due to folate deficiency | Microcytosis due to iron deficiency
56
Aplastic Anemia
Normocystic Pancytopenia Marrow not responding Empty blood/bone marrow
57
Pancytopenia
Shortage of all blood cell types
58
Causes of Aplastic Anemia
``` Idiopathic Drugs Viruses Pregnancy Fanconi Anemia ```
59
Clinical picture of Aplastic Anemia
Pallor, Dizziness, Fatigue (Anemia) Recurrent Infection (Leukopenia) Bleeding/Bruising (Thrombocytopenia)
60
Treatment of Aplastic Anemia
``` From least to most invasive: Avoid further exposure Blood Products G-CSF, Prednisone, ATG Bone Marrow Transplant ```

IHO Week 2 (13 decks)

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