Extracorpuscular Hemolytic Anemias Flashcards

1
Q

Classifications of hemolysis

A
  • acute vs chronic
  • inherited vs acquired
  • intravascular vs extravascular
  • intracorpuscular vs extracorpuscular (intrinsic vs extrinsic)
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2
Q

acute

A

rapid onset, shorter time course

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3
Q

chronic hemolysis

A

slow, with compensation from bone marrow

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4
Q

premature destruction of structurally and functionally normal RBCs due to mechanisms outside of the RBCs

A

extracorpuscular hemolytic anemia

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5
Q

Causes of Intracorpuscular Hemolysis

A
  • membrane defects
  • enzyme defects
  • hemoglobinopathies
  • PNH
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6
Q

Causes of Extracorpuscular Hemolysis

A
  • non-immune causes: microangiopathic hemolytic anemias, infectious agents, mechanical forces
  • immune causes: alloimmune hemolytic anemias, autoimmune hemolytic anemias
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7
Q

caused by a condition that leads to physical or mechanical RBC damage

A

non-immune hemolytic anemias

- microangiopathic, macroangiopathic

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8
Q

abnormalities in the microvasculature

A

microangiopathic

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9
Q

abnormalities in the heart or large blood vessels

A

macroangiopathic

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10
Q

two other causes on non-immune hemolytic anemias

A
  • infectious agents

- chemical and physical agents: chemicals, drugs, venom, thermal injury

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11
Q

microangiopathic hemolytic anemia

A
  • intravascular hemolysis = RBC fragmentation

- thrombocytopenia

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12
Q

thrombotic microangiopathies (TMAs)

A
  • damage occurs when RBCs quickly pass through turbulent areas of small blood vessels partially blocked by microthrombi
  • combination of MAHA and thrombocytopenia in the appropriate clinical setting
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13
Q

PBS Morphologies of MAHAs and TMAs

A
  • keratocytes (horn cells)
  • helmet cells
  • triangulocytes
  • microspherocytes
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14
Q

Top 4 scary microangiopathic hemolytic anemias

A
  • thrombotic thrombocytopenic purpura
  • hemolytic uremic syndrome
  • disseminated intravascular coagulation (DIC)
  • HELLP syndrome
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15
Q

What causes thrombotic thrombocytopenic purpura?

A

a deficiency of ADAMTS13

- protease responsible for cleaving ‘ultra-large’ multimers of von Willebrand Factor, which are involved in coagulation

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16
Q

ULVWF can trigger this

A

inappropriate clotting which leads to ischemia

17
Q

this causes RBC fragmentation and trapping of platelets

A

TTP = results in hemolysis, severe thrombocytopenia

- neurologic dysfunction, fever, and renal failure may also occur

18
Q

Peripheral smear findings of TTP

A

schistos and polychromasia

19
Q

urinalysis of TTP

A

protein, RBCs, casts if extensive renal damage

- Tx: plasma exchange

20
Q

AKA ‘hamburger disease’

A

hemolytic uremic syndrome

21
Q

Hemolytic Uremic Syndrome

A
  • caused by bacterial toxins (Shiga) - stimulates microvascular thrombi
  • presents w acute gastroenteritis and bloody diarrhea
  • damage to glomerular endothelial cells => acute renal failure
  • Tx: fluid replacement, renal support (why not antibiotics?)
22
Q

~10% are atypical HUS that have other causes

A

inappropriate complement activation

23
Q

HELLP Syndrome

A
  • hemolysis, elevated liver enzymes (transaminases), and low platelet count
  • complication in some pregnancies, correlation w pre-eclampsia, and eclampsia
24
Q

the inappropriate activation of the coagulation system results in clots throughout the microvasculature

A

DIC

  • organ damage and bleeding
  • many causes: malignancies, infections, acute leukemias, obstetric complications, tissue damage, transfusion rxns, venoms, etc.
25
Q

Mechanical causes of MAHAs

A
  • prosthetic cardiac valves
  • circuits (ECMO)
  • vascular malformation
  • malignant hypertension
  • exercise (or hobby-induced hemoglobinuria)
26
Q

immune hemolytic anemia

A
  • shortened red cell survival due to an Ab-mediated mechanism
    > autoimmune: Ab specificity against one’s pwn RBC antigens
    > alloimmune: Ab specificity against RBC antigens of another person
    > drug-induced: multiple mechanisms
27
Q

autoimmune hemolytic anemia

A
  • can be some complement activation
  • results from immune system dysregulation + loss of immune tolerance, exposure to antigen similar to self, etc
  • if all of complement cascade is fixed to RBC => intravascular cell lysis
  • if complement is only partially fixed = macs recognize Fc receptor of Ig and/or C3b complement - phagocytose RBC = extravascular RBC destruction
28
Q

if all of complement cascade is fixed to RBC vs if complement is only partially fixed

A
  • if all of complement cascade is fixed to RBC => intravascular cell lysis
  • if complement is only partially fixed = macs recognize Fc receptor of Ig and/or C3b complement - phagocytose RBC = extravascular RBC destruction
29
Q

most common type of autoimmune hemolytic anemia

A

warm autoimmune hemolytic anemia

30
Q

antibody characteristics of warm autoimmune hemolytic anemia

A
  • most are IgG
  • react best at 37 degrees C
  • may fix complement (but only up to C3)
  • idiopathic cause (unknown)
  • secondary WAIHA (~70%)
  • Tx: steoids, splenectomy
31
Q

cold autoimmune hemolytic anemia

A
  • Ab characteristics = IgM, optimal reactivity at lower than 30 degrees C (~4C), and can bind complement
  • fix entire complement cascade
  • leads to the formation of membrane attack complex => RBC lysis in the vasculature
  • typically only complement found on cells
  • primary CAIHA = cold agglutinin disease
  • secondary ‘’ (90%) = mycoplasma pneumonia infection, viral infections (mono)
32
Q

lab findings for autoimmune hemolytic anemia

A
  • decreased Hb
  • increased retics
  • increased serum bilirubin, lactate dehydrogenase
  • decrease haptoglobin
  • PBS: poly, spherocytes +/- agglutination
33
Q

direct Ab test for autoimmune hemolytic anemia

A
  • determines if RBCs are coated with Abs or complement

- can help to establish immune cause for hemolysis

34
Q

Alloimmune hemolytic anemia

A
  • hemolytic transfusion rxns

- hemolytic disease of the fetus and newborn

35
Q

hematologic emergencies

A
  • microangiopathic hemolytic anemia (> or equal to 1+ schisto)
  • suspected hemolytic anemia with > 1+ sphero