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SALMAN HARRISON'S > 96 - Hemolytic Anemias > Flashcards

96 - Hemolytic Anemias Flashcards

1
Q

What are the main signs of HA (Hemolytic Anemia) (4)?

A
  1. Jaundice
  2. Discoloration of urine
  3. Spleno/hepatic- megaly
  4. Skeletal changes
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2
Q

What do you find in HA lab workup?

A
  1. Unconjugated bilirubin and AST increase
  2. MCV + MCH increase
  3. LDH increase
  4. Haptoglobin reduce
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3
Q

What is the definition of HA?

A

When the rate of RBC destruction exceed the capacity of the BM to produce more RBC

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4
Q

What are the main clinical findings in HS (Hereditary Spherocytosis)?

A
  1. Jaundice
  2. Splenomegaly
  3. Gallstone
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5
Q

What is the CBC finding that suggests most strongly that the patient might have HS?

A

MCHC (Mean Corpuscular Hemoglobin Concentration)>34

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6
Q

What is the treatment for HS?

A
  1. Mild- no treatment

2. Moderate- postpone splenectomy until after puberty

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7
Q

What does PKD (pyruvate Kinase Deficiency) treatment include?

A
  1. Supportive
  2. Folate
  3. Iron chelation
  4. Blood transfusion
  5. Splenectomy
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8
Q

What is the HA triad for diagnosis?

A
  1. Normomacrocytic anemia
  2. Reticulocytosis
  3. Hyperbilirubinemia
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9
Q

How can we differentiate between enzymatic and membranous disorders?

A

Enzymopathies should be considered when coombs negative HA

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10
Q

What is the role of G6PD?

A

Redox metabolism of all aerobic cells- In RBC its the only source of NADPH

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11
Q

What is the genetic location of G6PD?

A

X-linked

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12
Q

When challenged by an oxidative agent what do the majority of people with G6PD may present with?

A
  1. Neonatal jaundice (NNJ)

2. AHA (Acute HA)

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13
Q

What is the result of inadequately managed NNJ+G6PD deficiency?

A

Kernicterus and permanent neurological damage

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14
Q

What can cause AHA in people with G6PD deficiency? (3)

A
  1. Fava beans
  2. Infections
  3. Drugs (antimalarials, sulpho, antibacterials, antipyretic)
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15
Q

What is the prodrome of AHA in people with G6PD deficiency?

A

Malaise, weakness, abdominal/lumber pain.

After several hours-3 days jaundice and dark urine develop

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16
Q

What do we see in the lab of AHA in people with G6PD deficiency? (5)

A
  1. Hemoglobinemia
  2. Hemoglobinuria
  3. High LDH
  4. Low/absent plasma haptoglobin
  5. High unconjugated bilirubin
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17
Q

What are the typical features of G6PD deficiency blood cells

A
  1. Hemighosts (unevenly distributed hemoglobin
  2. Bite cells
  3. Heinz bodies
18
Q

What is the most serious threat from AHA in adults with G6PD deficiency?

A

Acute renal failureq

19
Q

Who usually gets affected by aHUS? what will we see?

A
  1. Children

2. microangiopathic HA, thrombocytopenia, ARF

20
Q

What is the mortality rate of aHUS in the acute phase and in the case of progressing to ESRD?

A
  1. 15%

2. 50%

21
Q

What is the treatment of aHUS?

A
  1. Eculizumab (anti C5 complement inhibitor) indefinitely to prevent ERSD
  2. Traditionally plasma exchange was given, but now less.
22
Q

What are the two situations in which mechanical destruction of RBC occur?

A
  1. March hemoglobinuria (marathon runners)

2. Chronic and iatrogenic- Microangiopathic hemolytic anemia due to prosthetic heart valve

23
Q

What are the most frequent infectious causes of HA?

A
  1. Malaria- in endemic areas

2. Shiga toxin-producing E.coli O157:H7- in the rest of the world

24
Q

How can open wounds/septic abortion lead to HA?

A

Clostridium perfringens sepsis-> toxin with lecithinase activity

25
Q

What is the mortality rate due to AIHA (Autoimmune HA) when treated?

A

5-10%

26
Q

What is clinical triad of AIHA?

A
  1. Abrupt drop in hemoglobin level (up to 4 g/dl)
  2. Jaundice
  3. Splenomegaly
27
Q

How do you call the direct antiglobulin test?

A

Coombs test

28
Q

Which lab test is diagnostic for AIHA?

A

Coombs test. You can use to to determine if its C3 or Ig implicated.

29
Q

Which disease might have AIHA as its first manifestation?

A

SLE

30
Q

Where does the RBC destruction occur in AIHA?

A

Extravascular hemolysis (spleen, liver, BM)

31
Q

What is the treatment for severe acute AIHA?

A

RBC transfusion

32
Q

What is the treatment for non-life-threatening AIHA?

A

Prednisone (1 mg/kg per day) + rituximab (anti DC20) (100 mg wk * 4)

33
Q

How will you treat patients with relapsing AIHA?

A

Splenectomy

34
Q

What is CAD (Cold Agglutinin Disease)?

A
  1. A form of AIHA which occur in areas exposed to cold temperature.
  2. It is a chronic condition.
  3. Usually IgM produced by expanded B lymphocyte clone
  4. regarded as Waldenstorm macroglobulinemia
35
Q

How do you treat CAD?

A
  1. Mild- avoiding cold temperature
  2. Rituximab (60% of patients respond to this treatment)
  3. Blood transfusion
36
Q

What proved to be ineffective in CAD treatment?

A
  1. Splenectomy

2. Prednisone

37
Q

What is PNH (Paroxysmal Nocturnal Hemoglobinuria) ?

A

Acquired chronic HA characterized by persistent intravascular hemolysis with occasional or frequent exacerbations.

38
Q

What is the PNH triad?

A
  1. Hemolysis
  2. Pancytopenia
  3. Venous thrombosis (may lead to Budd-Chiari syndrome)
39
Q

Describe a typical complaint of a patient with PNH?

A

Blood like urine in the first pass of urine in the morning

40
Q

What kind of anemia do we usually see in PNH?

A

Normo/macrocytic with reticulocytosis

41
Q

What is the gold standard for diagnosing PNH?

A

Flow cytometry for RBC and granulocytes- presenting CD59 and CD55 negative

42
Q

How do you treat PNH?

A

Eculizumab + folic acid
or
Blood transfusion

SALMAN HARRISON'S (163 decks)

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