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S3B4 Pathology > Anemia Due to Decreased RBC Survival: Hemolytic Anemias > Flashcards

Anemia Due to Decreased RBC Survival: Hemolytic Anemias Flashcards

1
Q

What are the general features of Hemolytic Anemias?

A
  • increased rate RBC destruction
  • Shortened RBC lifespan
  • Increased erythropoietin secretion
  • bone marrow erythroid hyperplasia
  • peripheral blood reticulocytosis
  • accumulation of RBC breakdown products (vary based on intravascular or extravascular)
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2
Q

What type of hemolysis is always pathologic?

A

intravascular

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3
Q

Describe the pathogenesis of intravascular hemolysis

A

Red blood cells lyse within the blood vessels

Free hemoglobin is released - then binds to haptogloblin

haptoglobin is cleared by the reticuloendothelial cells

if all haptoglobin is used up, then you will have free heme that will head toward the kidneys or will become oxidized to methemoglobin

in the kidneys you will see hemoglobinuria & hemasidenuria

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4
Q

What are the clinical findings associated with extra-vascular anemia?

A

anemia

jaundice

splenomegaly

normal / mildly decreased plasma haptoglobin

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5
Q

What are the clinical & lab findings associated with intra-vascular hemolysis?

A

anemia

jaundice

hemoglobinemia

hemoglobinuria

hemosiderinuria

decreased / absent plasma haptoglobin

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6
Q

What are the common causes of extravascular hemolytic anemia?

A

cytoskeletal abnormalities

enzyme deficiencies

globin chain abnormalities

antibody-mediated

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7
Q

What are the common causes of intravascular hemolytic anemia?

A

mechanical injury

complement fixation

intracellular parasites

exogenous toxins

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8
Q

What is seen in the bone marrow in hemolytic anemia?

A

erythroid hyperplasia - erythroid islands

  • when the bone marrow is capable of responding
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9
Q

What finding is shown in the provided image?

A

erythroid island

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10
Q

What findings in the peripheral blood are consistent with hemolytic anemia?

A

polychromasia & reticulocytosis

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11
Q

What findings in bone marrow / other organs are consistent with hemolytic anemia?

A

hemosiderosis

extramedullary hematopoiesis

pigment gallstones

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12
Q

If you suspect hemolytic anemia, what test should you order?

A
  • serum total bilirubin
  • serum indirect (unconjugated) bilirubin
  • lactate dehydrogenase
  • plasma haptoglobin
  • urine hemoglobin
  • plasma hemoglobin
  • serum / plasma methemoglobin
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13
Q

If spherocytes are seen in a patient suspected to have hemolytic anemia – what are the possible causes?

A

hereditary membrane abnormalities

autoimmune

delayed hemolytic transfusion reaction

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14
Q

If schistocytes are seen in a patient suspected to have hemolytic anemia – what are the possible causes?

A

DIC

TTP / HUS

vasculitis

valve disorders

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15
Q

If sickle cells are seen in a patient suspected to have hemolytic anemia – what are the possible causes?

A

sickling disorders

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16
Q

If target cells are seen in a patient suspected to have hemolytic anemia – what are the possible causes?

A

hemoglobinopathies

liver disease

post-splenectomy

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17
Q

Why do we see an overlap of areas in the world with the highest concentration of alpha & beta-thalassemia with malaria?

A

thalassemia offers a protective avantage in malarial infections

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17
Q

What causes thalassemia?

A

quantitative deficiency in the alpha (alpha-thalassemia) or beta (beta-thalassemia) chain in hemoglobin

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18
Q

The alpha chain for hemoglobin is encoded for by what chromosome?

A

chromosome 16

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19
Q

The beta chain for hemoglobin is encoded for by what chromosome?

A

chromosome 11

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20
Q

What are the pathologic consequences associated with thalassemia?

A

chronic hemolytic anemia

toxic effects of excess globin chains → insoluble inclusions causing membrane damage & decreased survival of RBC precursors

iron overload

expansion of hematopoietic compartment

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21
Q

What are the risks & benefits associated with treating beta-thalassemia major with blood transfusion?

A

treat symptoms

adds to systemic iron overload

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22
Q

The provided image is suggestive of what condition?

A

beta-thalassemia major

marked expansion of the hematopoiesis in severe thalassemia causes new bone formation in the outer table of the skull, causing perpendicular radiations

23
Q

What is the general difference in symptoms between beta-thalassemia major & minor?

A

major: severe & requires frequent blood transfusions (probably shortened lifespan)
minor: asymptomatic or mild

24
What are the 4 different genotype possibilities of alpha-thalassemia & how are their clinical presentations different?
25
What are the clinical features seen in beta-thalassemia?
severe anemia HbF or HbA2 is elevated shortened lifespan, unless transfused untreated children- growth retardation & death hepatosplenomegaly, lymphadenopathy, bony abnormalities iron overload
26
What feature of the provided peripheral blood smear are characteristic of beta-thalassemia?
* severe microcytic hypochromic anemia * marked anisocytosis & poikilocytosis * many nucleated RBC (black arrow) * polychromasia (red arrow)
27
What features would you expect to seen in a peripheral blood smear in a patient with beta-thalassemia minor?
morphology may be normal or microcytic hypochromic basophilic stippling & target cells may be seen degree of anemia is variable usually asymptomatic
28
How do we diagnose hemoglobin abnormalities?
high-performance liquid chromatography different hemoglobins elute at different times & concentrations are read automatically
29
Fill out the provided table
30
What is the mutation associated with sickle cell anemia?
single point mutation in beta-globin gene Valine for Glutamate substitution position 6 beta chain
31
What is the pathogenesis of sickle cell anemia?
sickling with hypoxia or dehydration if resolve in a timely manner, cells will go back to the correct shape-- if not, they become irreversible sickled
32
Why is HbS so prevalant?
because heterozygosity confers protection against malaria
33
What features would you expect to see in a peripheral blood smear of a patient with sickle cell disease?
normochromic normocytic anemia sickle cells during crises target cells (dehydration) anisoporikilocytosis polychromasia/reticulocytosis
34
What are the clinical symptoms that are common in persons with sickle cell diseas?
splenic infarction (b/c sludge in blood) skin ulcers bone infarcts osteomyelitis streptococcus pneumonia infections (esp if asplenic)
35
What is the abnormality associated with hereditary spherocytosis? Describe the pathogenesis associated with this disease.
cytoskeletal abnormality normal when first produced, but as the RBC ages, it is notable to make more cytoskeletal proteins, so the cytoskeleton will degenerate make blebs, lose part of their membrane & become spherocytic (much less deformable & destroyed in the spleen)
35
What is the abnormality associated with hereditary spherocytosis? Describe the pathogenesis associated with this disease.
cytoskeletal abnormality normal when first produced, but as the RBC ages, it is notable to make more cytoskeletal proteins, so the cytoskeleton will degenerate make blebs, lose part of their membrane & become spherocytic (much less deformable & destroyed in the spleen)
36
What features would you expect to see in a peripheral blood smear of a patient with hereditary spherocytosis?
cytoskeletal defects spherocytes, loss of membrane
37
How do you diagnose hereditary spherocytosis?
osmotic fragility test & flow cytometry
38
What is the function of G6PD?
protect red cells from oxidative damage catalyzes the first step in the pentose phosphate pathway & reduces NADP to NADPH - keeps glutathione in the reduced state- protects cell form oxidation damage
39
What can happen to patients with a G6PD deficiency?
when red blood cells experience oxidative stress (certain medications/food/infections) transient intravascular hemolysis form Heinz bodies that attach to RBC membranes that are plucked out from the spleen -creating “bite cells”
40
What are the genetics of G6PD deficiency?
X-linked (many variants)
41
What are the genetics of G6PD deficiency?
X-linked (many variants)
42
What features would you expect to see in a peripheral blood smear of a patient with G6PD deficiency?
anemia ONLY after exposure to oxidizing agents bite cells (center) Heinz bodies (insert)
43
When should you test for G6PD deficiency and how do you do it?
Do NOT test at time of hemolytic episode – wait until after they have cleared it Rapid: Fluorescent Spot Test Quantitative: Spectrophotometry
44
What are the 3 groups of antibodies that are involved in immune hemolytic anemia?
autoantibodies alloantibodies drug-related
45
Immune hemolytic anemia can be cause by autoantibodies due to what cause?
infections lymphoproliferative disorders autoimmune disorders
46
Immune hemolytic anemia can be cause by alloantibodies due to what cause?
transfusion-related hemolytic disease of fetus & newbord
47
Drug-related immune hemolytic anemia can be due to what causes?
drug adsorption to RBC membrane autoantibody induction neoantigen formation
48
What features would you expect to see in a peripheral blood smear of a patient with warm autoimmune hemolytic anemia?
* spherocytes/microspherocytes * polychromasia (larger blue erythrocytes) * nucleated red blood cells (in severe cases)
49
What process is being depicted in the provided image of a peripheral blood smear?
cold agglutination (IgM) exception in old people- Donath Landsteiner hemolytic anemia (IgG)
50
What is the hemolytic anemia due to acquired intrinsic RBC defect?
paroxysmal nocturnal hemoglobinuria
51
What is the mutation that leads to acquired hemolytic anemia? Describe this pathogenesis.
Acquired stem cell defect: **GPI deficiency -** anchors proteins to the surface of RBC lose anchor CD59 & CD55, which regulate complement in the blood stream → leads to inappropriate coagulation cascade **chronic intravascular hemolytic anemia**
52
What are the clinical features of PNH?
chronic intravascular hemolytic anemia impaired bone marrow function susceptibility to thrombosis varying degrees of hemolysis at different times of day
53
What feature of PNH is depicted in the provided slide?
hemosiderin deposition in the renal tubules
54
What is the test of choice to diagnose PNH?
Flow Cytometry

S3B4 Pathology (6 decks)

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