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Heme 2 > Hemolytic Anemia > Flashcards

Hemolytic Anemia Flashcards

1
Q

What is hemolytic anemia?

A

RBC destruction resulting in loss of RBC mass and release of cellular contents that can be inherited or aquired

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2
Q

What are some inherited forms of hemolytic anemia?

A

– RBC membrane disorders
– RBC enzyme deficiencies
– Hemoglobinopathies

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3
Q

What are some acquired hemolytic anemias?

A 
– Autoimmune (antibody mediated) hemolysis
– Mechanical trauma
– Infections
– Chemical
– Splenic sequestration
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4
Q

Hemolysis can occur extravascular or intravascular which is more common?

A

Extravascular: Spleen&raquo_space;»> liver, bone marrow

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5
Q

What labs are used to identify hemolytic anemia?

A
  • Normocytic anemia
  • Reticulocytosis
  • ↑ LDH (lactate dehydrogenase), AST (aspartate aminotransferase), potassium from release of cellular contents
  • Bilirubinemia: Unconjugated bilirubinemia (indirect)
  • Hemoglobinemia (free hgb in plasma): More pronounced in intravascular hemolysis and Scavenged by haptoglobin (↓ hapto)
  • DAT (direct antiglobulin test)
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6
Q

What types of urine screens could you do for hemolytic anemia?

A

Hemoglobinuria and Hemosiderinuria (most often seen with intravascular hemolysis)

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7
Q

What can cause a “false positive” for hemolytic anemia?

A

“phlebotomy induced”

hemolysis from hyperkalemia

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8
Q

Clinical features of hemolysis?

A

anemia and Hyperbilirubinemia

sometimes other symptoms related to cause

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9
Q

It is not uncommon for patients with hemolytic anemia to have baseline hemoglobin of 6-7 g/dL without symptoms why?

A

chronic hemolytic conditions (eg sickle
cell anemia, thalassemia, pyruvate kinase deficiency) can often tolerate extreme anemia with minimal symptoms

Acute changes in red blood cell volume are poorly tolerated

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10
Q

What are some clinical features of Hyperbilirubinemia?

A
  • Jaundice is detectable in most fair-skinned individuals when bilirubin exceeds 2.5 mg/dL
  • Scleral icterus can be detected by experienced physicians when bilirubin exceeds 1.7 mg/dL
  • Increased risk of bilirubin gallstone (pigmented)
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11
Q

What are some rare complications of hemolytic anemia?

A
  • Pigment induced kidney injury from heme
  • Folate deficiency due to increased utilization
  • Increased risk of venous and arterial thrombosis related, including venous thrombosis of atypical sites (portal vein, cerebral venous sinus)
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12
Q

Splenomegaly, hepatomegaly related to extra-medullary hematopoiesis is seen with which types of hemolytic anemia?

A

Thalassemia, PK deficiency, Hereditary spherocytosis

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13
Q

Skeletal changes related to expansion of marrow ‘chipmunk facies’, convex bones is seen with which hemolytic anemia?

A

Beta thalassemia major

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14
Q

What hemolytic anemia has asplenia?

A

sickle cell anemia

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15
Q

Microangiopathic hemolytic anemia is seen with what causes of hemolytic anemia?

A

Seen in TTP, HUS, DIC, malignant hypertension, CREST syndrome, vasculitis, HELLP syndrome

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16
Q

What does Microangiopathic hemolytic anemia cause?

A

Microvascular infarction causing acute kidney injury, liver disease, abdominal pain, fever, mental status changes, thrombocytopenia, rash, hemorrhagic diarrhea

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17
Q

35 yo female with mild jaundice, splenomegaly, low Hb (9.4) and spherocytes on blood smear

What would happen to her MCHC?

A

Increase due to spherocytes becoming dehydrated as they move through the vessels causing increased concentration of Hb

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18
Q

What are spherocytes?

A

• Normal-sized RBCs with increased hemoglobin concentration that are less flexible, trapped in
spleen causing shortened life span

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19
Q

What are the Spherocytic hemolytic anemias?

A
  • Hereditary spherocytosis

* Autoimmune hemolytic anemia (AIHA) - warm and cold

20
Q

What are the Congenital RBC membrane disorders?

A

• Hereditary spherocytosis and Hereditary elliptocytosis

both are Characterized by defects in structural cytoskelton proteins

21
Q

What test is MOST important for differentiating between the causes of spherocytosis?

A

Direct antiglobulin test which tells you whether the RBCs have Ab or complement attacking them

22
Q

Most common inherited hemolytic anemia in US

1/5000

A

Hereditary spherocytosis

23
Q

What goes on genetically with Hereditary spherocytosis?

A

75% autosomal dominant and 25% sporatic: recessive and new mutations

Primary defects described in: ankyrin (most common), α/β-spectrin, band 3, band 4.2 and most patients have deficiencies in both ankyrin AND spectrin due to a protein imbalance

24
Q

Clinical hereditary spherocytosis presentation?

A

Wide variability in severity of hemolysis

Splenomegaly

Aplastic crisis: Parvovirus infects/kills erythroid precursors

25
Q

What tests can be used to diagnose hereditary spherocytosis?

A
  • Family history
  • Peripheral blood review (spherocytes) with negative direct antiglobulin test
  • Osmotic fragility not used much anymore
26
Q

Treatment for hereditary spherocytosis?

A
  • Depends on clinical severity
  • Managing chronic hemolysis, Gallstones and Jaundice
  • Aplastic crisis
  • Splenectomy: complete (in adults) and partial (in children < age 6)
27
Q

If a DAT is positive with spherocytes on the blood smear, jaundice, fatigue, splenomegaly what does the pt probably have?

A

Autoimmune hemolytic anemias (AIHAs)
– Warm autoantibodies → 37°C
– Cold autoantibodies → 0-5°C

Host antibodies reactive with autologous RBCs

28
Q

Which is more common warm or cold AIHA?

A 
Warm-autoantibody type
(~80%)
– Idiopathic/Primary (60%)
– Secondary: Lymphoproliferative disorders, Autoimmune disorders, Non-hematopoietic neoplasms ie. Ovarian cancer and Drugs, ie. α-methyldopa,
cephalosporins
29
Q

What causes cold AIHA?

A
  • Idiopathic

* Secondary: Postinfectious, ie. Infectious mononucleosis, Mycoplasma) and Lymphoproliferative disorders

30
Q

What is the pathogenesis of warm AIHA?

A

Extravascular hemolysis
• Antibody mediated (IgG) OR
• Complement mediated (classical pathway)
• Spherocyte production

31
Q

What is the pathogenesis of cold AIHA?

A

– IgM binds in cold and IgM-mediated agglutination impeding blood flow and RBC injury

32
Q

How does warm look on blood smear?

A
  • Spherocytes

* Polychromasia

33
Q

How does cold look on blood smear?

A

Cold Agglutinins: Usually do not cause anemia but may cause obstructive symptoms

34
Q

– Symptoms of anemia • Mild → Severe (with massive hemolysis)
– Organomegaly

Which AIHA?

A

Warm

35
Q

– Symptoms of anemia • Mild → Severe (with massive hemolysis)
– Acrocyanosis
– Raynaud phenomenon

A

Cold

36
Q

Cold comes in chronic and acute forms what is the difference?

A
  • Acute form occurs 2-3 weeks into infectious illness (EBV/Mycoplasma)
  • Chronic form is associated with lymphoproliferative disorders
37
Q

Congenital Red cell enzyme disorder?

A

Glucose 6-phosphate dehydrogenase deficiency (Pentose Phosphate pathway)

38
Q

G6PDH deficiency causes what to happen in the biochem pathway?

A

Defect in the pathway responsible for reducing oxidants
– Build up in G-6-P
– Decreased glutathione production
– Inability to reduce oxidant stressors

39
Q

How are RBCs affected by Increased susceptibility to oxidant stress?

A

RBCs affected; resulting in a hemolytic anemia of variable clinical severity
• Denatured hemoglobin (Heinz bodies) directly damage the RBC → intravascular hemolysis
• Heinz bodies are removed in the spleen → extravascular hemolysis and BITE cells

40
Q

What are some causes of oxidant stress?

A
  • Infection
  • Fava beans
  • Drugs: dapsone, sulfonamides, primaquine, nitrofuantoin, quinolones
41
Q
  • 400 million people affected worldwide
  • Common in malarial endemic areas
  • X-linked disorder
A

G6PDH deficiency

42
Q

What should you do to diagnose G6PDH deficiency?

A

peripheral blood smear
NADPH fluorescence
quantitative assay

43
Q

What can cause fragmented RBCs?

A

Mechanical trauma to RBCs: heat, defective cardiac valves, microangiopathic hemolytic anemias, malignant hypertension, thrombotic thrombocytopenic purpura

44
Q

What is Paroxysmal nocturnal hemoglobinuria (PNH)?

A

Rare Acquired mutation in PIGA gene (X chromosome) resulting in decreased GPI-linked proteins

45
Q

Pathogenesis of Paroxysmal nocturnal hemoglobinuria?

A

Lack of GPI-linked anti-complement proteins makes cells suspectible to complement lysis

Stem cell disorder so all myeloid lineages are affected, though RBCs are most sensitive to the C’ lysis

46
Q

Clinical presentation of Paroxysmal nocturnal hemoglobinuria?

A

– Chronic low level hemolysis
– Acute, episodic hemolysis events
– Hemoglobinuria
– Venous thromboses

47
Q

What tests should be used to diagnose Paroxysmal nocturnal hemoglobinuria?

A

– Flow cytometry for GPI-linked proteins: CD55 (DAF), CD59 examined on WBCs AND RBCs

Heme 2 (14 decks)

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