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Pediatrics > Hemolytic Anemia > Flashcards

Hemolytic Anemia Flashcards

1
Q

What’s the mutation\Ab in TTP?

A

ADMAST 13

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2
Q

What’s the role of ADMAST 13?

A

Cleaving VwF

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3
Q

Name types of hemolytic anemia from most extrinsic to most intrinsic:

A
  • AHA, MAHA, mechanical shearing, medications
  • Spherocytosis, eliptocytosis
  • PKD, G6PD
  • Quantitative\Qualitative Hb-pathies
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4
Q

How to differentiate between HUS & TTP:

A

Both (low Hb & platelet)

- HUS: post E-coli + diarrhea + renal failure.

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5
Q

Differentiate between dark urine and tea colored urine in Hb-pathies:

A
  • Dark: Hb urea (intravascular)

- Tea: Urobilinogen (extravascular in spleen)

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6
Q

Clinical presentation in hemolytic anemia:

A
  • jaundice & pallor
  • Fatigue & tachycardia
  • Splenomegaly & hepatomegaly
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7
Q

What is the state of reticulocyte count in hemolytic anemia?

A

High

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8
Q

What is the basic screening blood work-up for Hb-pathies:

A
  • CBC (w\picture + Reticulocyte)
  • LDH
  • Urinalysis
  • LFT (Total\ direct bilirubin)
  • DCT
  • EF
  • osmotic fragility test
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9
Q

What to do if you see target cell or sickle cell in blood smear?

A

Electrophoresis

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10
Q

What test would be most supportive of your diagnosis if you suspect spherocytosis?

A

Osmotic fragility

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11
Q

What are the types of AHA?

A

Warm, cold and paroxysmal cold

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12
Q

Differentiate between warm, cold and paroxysmal cold AHA:

  • AB
  • temp of agglutination
  • site of hemolysis
  • presence of hemoglobinuria
A
  • Warm: IgG, >36 extravascular, no.
  • Cold: igM, intravascular, yes.
  • PCH: IgG against P-antigen, extravascular
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13
Q

Which of the following is a life-threatening condition

Warm, cold, paroxysmal cold AHA

A

Warm

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14
Q

What is the clinical feature of warm AHA?

A

Sudden (pallor - jaundice - dark urine) + spleenomegaly

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15
Q

What are the findings in warm AHA?

  • CBC, blood film, Reticulocyte
  • LDH & heptaglobin
  • DCT
  • Urinalysis
  • bilirubin
A
  • low Hb, normal indices, high reticulocyte, agglutinations & go to spleen for destruction
  • high & low “in all hemolytic anemias”
  • positive
  • normal
  • high indirect
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16
Q

Main treatment of

  • warm
  • cold
  • paroxysmal cold AHA
  • hereditary spherocytosis
A
  • Steroid
  • supportive & plasmapheresis
  • supportive + steroid
  • folic acid
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17
Q

What are other option to treat warm AHA:

A

IVIG, immunosuppression, anti-CD20 “retixumab”, splenectomy.

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18
Q

What are the findings in warm AHA?

  • CBC, blood film, Reticulocyte
  • LDH & heptaglobin
  • DCT
  • Urinalysis
A
  • low Hb, normal indices, high retic.
  • High & low
  • positive
  • hemoglobinurea (tea colored)
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19
Q

What do we do as a supportive care for cold AHA?

A

Warm patients

20
Q

In which group of population do we see PCH more often?

A

In children median age of 5 years.

21
Q

Name 2ndary causes of MAHA:

A

1- DIC
2- Drug induced
3- Autoimmune vasculopathy

22
Q

How does hemolysis occur in MAHA

A

RBCs are damaged when passing through obstructed\narrowed lumen

23
Q

Most severe type of defective cell membrane hemolytic anemia

A

Pyropoiklocytosis

24
Q

The following key words can give you hint about the diagnosis:

symptoms of hemolysis with viral infections & has history of neonatal jaundice.

A

Heriditary spherocytosis

25
Q

What is the CBC and film findings in hereditary spherocytosis?

A

High MCHC and spherocytes

26
Q

Is heriditary spherocytosis extravascular or intravascular hemolysis

A

Extra; thus splenomegaly

27
Q

What are the genes\their corresponding proteins affected in hereditary spherocytosis

A
  • Ankyrin (band3)

- Spectrin (4.2)

28
Q

What is the shape of RBC in heriditary spherocytosis?

A

Round - small - less deformable.

29
Q

Differentiate between the inheritance of heriditary spherocytosis & elliptocytosis:

A

Both Autosomal dominant.

  • spherocytosis: more severe if recessive
  • elliptocytosis: more severe if dominant
30
Q

Other differentials for spherocytes in blood film:

A

Lead poisioning and ABO incompatiability (few cells)

31
Q

What is the screening test and the confirmatory test used to diagnose heriditary spherocytosis?

A

Screening: osmotic fragility test
Confirmatory: Eosin-5-malemide “dye staining by flow cytometery”

32
Q

LFT findings in heriditary spherocytosis:

A

Increased total bilirubin - if gallstone: increased direct billirubin.

33
Q

Horizontal vs vertical defects will cause which type of cell membrane hemolytic anemias:

A
  • horizontal: Elliptocytosis

- vertical: Spherocytosis

34
Q

Most commonly Heriditary eliptocytosis is inherited as:

A

Autosomal dominant

35
Q

If you were given a case of elliptocytosis, and you want to determine the severity, what CBC indices will reflect that

A

MCV

36
Q

Mutations in pyropoiklocytosis is in which gene

A

Spectrin

37
Q

Usually patients with pyropoiklocytosis have family members with:

A

heridetray eliptocytosis

38
Q

What is the mainstay treatment of hereditary pyropoiklocytosis during infancy?

A

Intermittent transfusion to avoid splenic crisis

39
Q

What is the role of splenectomy in heriditary pyropoiklocytosis?

A

Very significant, increase in Hb, however not totally eliminated

40
Q

What determines the severity of G6PD?

A

1-Quantity

2- type of G6PD

41
Q

What is the mode of inheritance in G6pd

A

X-linked

42
Q

Color of urine in G6pd hemolysis is expected to be

A

Tea colored urine

43
Q

Name agents that can induce hemolysis in g6pd

A

Fava beans, sulfa medications, bacterim

44
Q

The only intravascular hemolysis mentioned in this lecture is

A

Cold AHA

45
Q

How to treat pyruvate kinase deficency?

A

Splenictomy, folic acid, transfusion

Pediatrics (41 decks)

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