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
Q

B-Thalassemia Minor

A

Asymoptomatic

26
Q

B-Thalassemia Intermedia

A

Less Severe Thalassemia

27
Q

B-Thalassemia Major

A

Severe Thalassemia

28
Q

-a/aa Thalassemia

A

Silent

29
Q

–/aa or -a/-a

A

Alpha-Thalassemia Trait: Asymptomatic

30
Q

–/-a

A

Hemoglobin H Disease: Moderate/Severe Disease

31
Q

–/– (No alpha chain genes)

A

Hydrops Fetalis: Fatal in utero

32
Q

What causes anemia in a-thalassemia?

A

Not enough a-chains
Excess unpaired B, gamma, or delta chains
Gamma tetramers in newborns
Beta tetramers in adults

33
Q

What causes anemia in B-thalassemia?

A

Not enough B-chains

Excess unpaired a-chains

34
Q

Anisocytosis

A

Varying sizes of RBCs

35
Q

Glucose-6-Phosphate Dehydrogenase Deficiency

A

Oxidant Exposure causes increased perioxides and premature cell lysis
Bite Cells/Heinz Bodies

36
Q

G6PD Pathway

A

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
Q

G6PD RBCs Lyse because…

A

Heme breaks away from globin

38
Q

Heinz Bodies

A

Denatured globin that sticks to the RBC membrane

39
Q

Highest incidence of G6PD coincides with what disease?

A

Malaria

40
Q

What are the triggers of G6PD deficiency?

A

Fava Beans, Drugs, Antibiotics, Aspirin, Peroxide

41
Q

Microangiopathic Hemolytic Anemia

A

Physical Trauma to RBC
Schistocytes
Triangulocyte
Secondary to other cause

42
Q

Causes of Microangiopathic Hemolytic Anemia (MAHA)

A

Runner’s Anemia
Artificial Heart Valve
Anything causing tiny blood clots (fibrin) all over the body in narrow blood vessels (DIC, TTP, HUS)

43
Q

Normocytic Anemias are caused by…

A

Blood Loss
Chronic Disease
Kidney or Liver Disease
Aplastic Anemia

44
Q

Anemia of (acute) Blood Loss

A

Traumatic Cause
Initially, Hemoglobin is NORMAL
After 2-3 days, see Reticulocytes

45
Q

Anemia of Chronic Disease

A

Infections, Inflammation, Malignancy

Iron Metabolism Disturbed

46
Q

Why is there disturbed iron metabolism seen in Anemia of Chronic Disease?

A

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
Q

Labs for Anemia of Chronic Disease

A

Low Iron, TIBC
HIGH FERRITIN
High Marrow Storage Iron

48
Q

Labs for Iron Deficiency Anemia

A

Low Iron, Ferritin, Marrow Storage Iron

High TIBC

49
Q

Cause of Anemia of Renal Disease (end stage)

A

Lack of Erythropoietin

See Echinocytes

50
Q

Management of Severe Anemia of Renal Disease

A

Erythropoietin or Blood Transfusion

51
Q

Anemia of Liver Disease

A

See acanthocytes and Target Cells

Due to multiple causes

52
Q

Acanthocytes

A

Spur Cells - tiny spur-like projections

53
Q

Echinocytes

A

Burr Cell, serrated edges over entire surface, sea urchin

54
Q

Uncomplicated Anemia of Liver Disease (RARE!)

A

Decreased RBC survival and Impaired Marrow Response
Mild Anemia
Normocytic (sometimes macrocytic)
Poikilocytosis

55
Q

Complicated Anemia of Liver Disease

A

Megaloblastosis due to folate deficiency

Microcytosis due to iron deficiency

56
Q

Aplastic Anemia

A

Normocystic
Pancytopenia
Marrow not responding
Empty blood/bone marrow

57
Q

Pancytopenia

A

Shortage of all blood cell types

58
Q

Causes of Aplastic Anemia

A
Idiopathic
Drugs
Viruses
Pregnancy
Fanconi Anemia
59
Q

Clinical picture of Aplastic Anemia

A

Pallor, Dizziness, Fatigue (Anemia)
Recurrent Infection (Leukopenia)
Bleeding/Bruising (Thrombocytopenia)

60
Q

Treatment of Aplastic Anemia

A
From least to most invasive:
Avoid further exposure
Blood Products
G-CSF, Prednisone, ATG
Bone Marrow Transplant