12. Extrinsic Defects Flashcards

1
Q

Extravascular Lab Findings

A
  • Increased serum bilirubin
  • Increased urine and fecal urobilinogen
  • Spherocytes may be seen (due to macrophages plucking abnormalities from RBC)
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2
Q

Extrinisc Hemolytic Anemia can be caused by:

A
  • Immune anemias

- non-immune (infections, mechanical, chemical, physical)

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3
Q

Immune Hemolytic Anemias

and Lab findings

A
  • Alloimmune
  • Autoimmune
  • Drug-induced

Lab findings:

  • spherocytes (HgB normal)
  • polychromasia
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4
Q

Alloimmune

A
  • antibodies react to foreign RBC antigens
  • Usually associated with transfusions, pregnancy, or transplant
  • Acute hemolytic transfusion reactions - usually IgM (ABO incompatibility)
  • Delayed hemolytic transfusion reactions - usually IgG (Patient is sensitized by previous exposure, Anamnestic response)
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5
Q

IgM

A
  • large antibodies that activate complement in the plasma

- typically cause intravascular hemolysis

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6
Q

IgG

A
  • small antibodies capable of crossing the placenta

- tags RBC to be hemolyzed in the spleen (Extravascular hemolysis)

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7
Q

Hemolytic Disease of the Newborn

A
  • Alloimmune response of mother against antigens on fetal RBC
  • Usually IgG, RH blood group system, anti-D
  • Erythroblastosis fetalis
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8
Q

Erythroblastosis fetalis

A
  • hemolytic anemia in newborn infant
  • numerous nucleated RBCs
  • hyperbilirubinemia
  • leads to neural cell death.
  • Putting baby under UV light or even sunlight can help bilirubin levels lower.
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9
Q

Autoimmune Hemolytic Anemia

  • types
  • diagnosis
A

Warm:

  • Secondary to infection, immunologic disorders, pregnancy.
  • 70% of AIHA
  • Usually IgG

Cold:

  • Cold agglutinin
  • Paroxysmal cold hemoglobinuria

Diagnosis: Variable anemia, positive DAT, spherocytes, increased poly, increased retic, increased bilirubin.

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10
Q

Cold Agglutinin Syndrome

A
  • Antibody has wide thermal range (optimum 4 degrees C)
  • Usually IgM, anti-I specificity
  • May be secondary to infections (mycoplasma)
  • IgM anti-i secondary to infectious mononucleosis
  • RBC clumping at room temperature may cause technical problems in CBC
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11
Q

Paroxysmal Cold Hemoglobinuria

A
  • Very rare
  • Associated with viral disorders: measles, mumps, flu, etc.
  • Anti-P specificity, biphasic hemolysin (binds at cold temperature but destroys red blood cell at warm temperature)
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12
Q

Drug-Induced Immune Hemolytic Anemia

A
  • Autoimmune
  • Drug Adsorption
  • Immune Complex
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13
Q

Drug-Induced: Autoimmune

A
  • 70% of all cases of drug-induced hemolytic anemia
  • drugs cause patient to make antibodies against self
  • IgG antibodies, RBC destroyed in spleen
  • Patients may demonstrate antibody long after discontinuing drug
  • Seen in hypertensive drugs (Aldomet, l-dopa)
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14
Q

Drug-Induced: Drug Adsorption

A
  • Hapten
  • Penicillins, cephalosporins
  • Drug is adsorbed to RBC surface
  • Drug-RBC complex illicits antibody response
  • IgG, extravascular hemolysis
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15
Q

Drug-induced: Immune complex

A
  • Quinidine, phenacetin
  • Antibody is formed against drug and forms complex with drug
  • Complex adsorbs onto patients RBC and activates complement
  • Activated complement = typically associated with intravascular hemolysis
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16
Q

Non-Immune Hemolytic Anemia: Infections

A

Intracellular - the organism becomes a part of the RBC. (ex: Malaria, Babesiosis)

Extracellular - create toxins that have an effect on the RBC causing them to burst. (Bartonella, Clostridium)

17
Q

Non-Immune Hemolytic Anemia: Mechanical

A

March hemoglobinuria- pounding of the feet of marchers caused the red blood cells to be destroyed as they passed through their feet.

Cardiac Prosthesis- heart valve that is foreign to the body

Microangiopathic- destruction in the small vessels. Fibrin strands that cross the vessel act like a chain linked fence.

18
Q

Microangiopathic Hemolytic Anemia

  • TTP
  • HUS
A

Thrombotic Thrombocytopenic Purpura (TTP)

  • RBC fragmentation
  • Thrombocytopenia
  • Neurologic dysfunction
  • Fever
  • Renal failure

Endothelial damage leads to deposition of platelet thrombi and fibrin

Hemolytic Uremic Syndrome (HUS)

  • MAHA
  • Thrombocytopenia
  • Acute renal failure
  • Most commonly found in children (as opposed to TTP which is most common in 30-40 yo)
19
Q

Non-Immune Hemolytic Anemia:

Chemical

A

Oxidative - denatures hemoglobin = heinz bodies (usally prevented by Hexose monophosphate shunt)
- Naphthalene (mothballs)
- Individuals deficient in G6PD are particularly susceptible
Examples: heavy metals = Arsenic, lead, copper

20
Q

Non-Immune Hemolytic Anemia: Venoms

A

Direct hemolysins

  • Brown recluse spider
  • Bees and wasps
  • Snakes
21
Q

Non-Immune Hemolytic Anemia: Physical

A
  • Burns - thermal damage to RBCs circulating in the area of burn
  • Characteristic budding and microspherocytes
  • body quickly clears them