17 - Hemolytic anemias intro Flashcards

1
Q

What are the 3 classifications of anemia?

A
  1. blood loss
  2. accelerated destruction
  3. decreased production
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2
Q

What are 3 classes of inherited hemolytic anemias?

A

Also called intrinsic:

  1. RBC membrane disorders
  2. RBC enzyme deficiencies
  3. Hemoglobinopathies
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3
Q

What are 5 classes of acquired hemolytic anemias?

A

Also called extrinsic:

  1. autoimmune (Ab mediated) hemolysis
  2. mechanical trauma
  3. infections
  4. chemical/toxic injury
  5. splenic sequestration
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4
Q

What are the 2 types of pathology behind hemolysis?

A
  • Extravascular
    • most common
    • spleen >> liver, bone marrow
    • macrophage mediated (usually Ab mediated)
  • Intravascular
    • less common
    • within the vessels
    • usually complement mediated or from mechanical destruction
  • combination of the 2 also possible
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5
Q

What are some only extravascular disorders?

A
  • RBC membrane disorders
  • thalassemias
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6
Q

What are some strictly intravascular disorders?

A
  • PNH
  • mechanical
  • malaria, toxins
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7
Q

What disorders are both intravascular and extravascular?

A

RBC enzyme disorder

Sickle cell anemia

Autoimmune hemolysis

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8
Q

What are some consequences of hemolytic anemias?

A
  • red cell destruction (shortened RBC lifespan)
    • anemia
  • elevated EPO
    • compensatory increase in erythropoiesis
    • reticulocytosis
    • bone marrow hyperplasia
  • hemoglobin degradation products secondary to hemolysis
    • hemosiderosis (accumulation of iron in the spleen, liver, and bone marrow)
    • hyperbilirubinemia, pigmented gallstones
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9
Q

What are 4 blood tests you should get to evaluate hemolysis?

A
  • CBC
    • normocytic (to macrocytic) anemia
    • reticulocytosis
  • peripheral blood smear
  • biochemistry tests (look for LDH, AST, potassium, hyperbilirubinemia, hemoglobinemia)
  • DAT (direct antiglobulin test)
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10
Q

What cellular contents can be detected in the blood and indicate hemolysis?

A

LDH (lactate dehydrogenase), AST (aspartate aminotransferase), potassium

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11
Q

What is hemoglobinemia?

A

Free Hb in plasma

**more pronounced in intravascular hemolysis (detected by decreased haptoglobin in plasma)

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12
Q

What are these images showing?

A
  1. bite cells
  2. shistocytes
  3. sickle cells
  4. spherocytes
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13
Q

What are 3 urine tests you should get to evaluate hemolysis?

A
  • hemoglobinuria
    • usually from intravascular processes
      • blood by dipstick but absence of RBCs when you run a urinalysis
  • increased urobilinogen (dipstick)
    • breakdown product of biliverdin, formed in the gut
  • hemosiderinuria
    • usually from intravascular processes
    • iron storage deposits in renal epithelium
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14
Q

Describe erythroid precursors

A

You know they’re erythroid when they have perfectly round nuclei!

**she mentioned it would be good to know this for the exam!!

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15
Q

What are 2 common symptoms of all patients with hemolytic anemia despite differing underlying etiology?

A

Anemia and hyperbilirubinemia (other characteristic symptoms are needed to determine underlying pathology)

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16
Q

What is a normal hemoglobin level? What could affect how a patient reacts to decreased levels of Hb?

A
  • normal= 12-16 g/dL
  • young patients with chronic hemolytic conditions (e.g. sickle cell) can often tolerate extreme anemia with minimal symptoms because they are used to a lower baseline
17
Q

What are some symptoms of decreased Hb?

A
18
Q

What are some symptoms of hyperbilirubinemia?

A
19
Q

What are 2 rare complications of hemolytic anemia?

A
  • pigment induced kidney injury
    • heme pigment can cause tubular obstruction, direct cellular injury, and vasoconstriction
    • results in decreased medullary blood flow
    • urinalysis shows hemoglobinuria without RBCs
  • folate deficiency due to increased utilization (giving folate won’t correct this because it keeps being used up… need to address the underlying hemolysis)
20
Q

What are 4 clinical features of hemolytic anemia that point towards a specific underlying disease process?

A
  • splenomegaly/hepatomegaly
    • related to extra-medullary hematopoiesis
    • seen in thalassemias, PK deficiency, hereditary spherocytosis
  • skeletal changes (convex bones; abnormal facies)
    • related to expansion of marrow esp. in beta thalassemia major
  • asplenia
    • e.g. in sickle cell anemia
  • microangiopathic hemolytic anemia
    • TTP, HUS, DIC, malignant HTN, HELLP syndrome (idk what she means by this list but it’s the last slide of the lecture if you want to look it up…)