Hemolytic Anemia Flashcards
What’s the mutation\Ab in TTP?
ADMAST 13
What’s the role of ADMAST 13?
Cleaving VwF
Name types of hemolytic anemia from most extrinsic to most intrinsic:
- AHA, MAHA, mechanical shearing, medications
- Spherocytosis, eliptocytosis
- PKD, G6PD
- Quantitative\Qualitative Hb-pathies
How to differentiate between HUS & TTP:
Both (low Hb & platelet)
- HUS: post E-coli + diarrhea + renal failure.
Differentiate between dark urine and tea colored urine in Hb-pathies:
- Dark: Hb urea (intravascular)
- Tea: Urobilinogen (extravascular in spleen)
Clinical presentation in hemolytic anemia:
- jaundice & pallor
- Fatigue & tachycardia
- Splenomegaly & hepatomegaly
What is the state of reticulocyte count in hemolytic anemia?
High
What is the basic screening blood work-up for Hb-pathies:
- CBC (w\picture + Reticulocyte)
- LDH
- Urinalysis
- LFT (Total\ direct bilirubin)
- DCT
- EF
- osmotic fragility test
What to do if you see target cell or sickle cell in blood smear?
Electrophoresis
What test would be most supportive of your diagnosis if you suspect spherocytosis?
Osmotic fragility
What are the types of AHA?
Warm, cold and paroxysmal cold
Differentiate between warm, cold and paroxysmal cold AHA:
- AB
- temp of agglutination
- site of hemolysis
- presence of hemoglobinuria
- Warm: IgG, >36 extravascular, no.
- Cold: igM, intravascular, yes.
- PCH: IgG against P-antigen, extravascular
Which of the following is a life-threatening condition
Warm, cold, paroxysmal cold AHA
Warm
What is the clinical feature of warm AHA?
Sudden (pallor - jaundice - dark urine) + spleenomegaly
What are the findings in warm AHA?
- CBC, blood film, Reticulocyte
- LDH & heptaglobin
- DCT
- Urinalysis
- bilirubin
- low Hb, normal indices, high reticulocyte, agglutinations & go to spleen for destruction
- high & low “in all hemolytic anemias”
- positive
- normal
- high indirect
Main treatment of
- warm
- cold
- paroxysmal cold AHA
- hereditary spherocytosis
- Steroid
- supportive & plasmapheresis
- supportive + steroid
- folic acid
What are other option to treat warm AHA:
IVIG, immunosuppression, anti-CD20 “retixumab”, splenectomy.
What are the findings in warm AHA?
- CBC, blood film, Reticulocyte
- LDH & heptaglobin
- DCT
- Urinalysis
- low Hb, normal indices, high retic.
- High & low
- positive
- hemoglobinurea (tea colored)
What do we do as a supportive care for cold AHA?
Warm patients
In which group of population do we see PCH more often?
In children median age of 5 years.
Name 2ndary causes of MAHA:
1- DIC
2- Drug induced
3- Autoimmune vasculopathy
How does hemolysis occur in MAHA
RBCs are damaged when passing through obstructed\narrowed lumen
Most severe type of defective cell membrane hemolytic anemia
Pyropoiklocytosis
The following key words can give you hint about the diagnosis:
symptoms of hemolysis with viral infections & has history of neonatal jaundice.
Heriditary spherocytosis
What is the CBC and film findings in hereditary spherocytosis?
High MCHC and spherocytes
Is heriditary spherocytosis extravascular or intravascular hemolysis
Extra; thus splenomegaly
What are the genes\their corresponding proteins affected in hereditary spherocytosis
- Ankyrin (band3)
- Spectrin (4.2)
What is the shape of RBC in heriditary spherocytosis?
Round - small - less deformable.
Differentiate between the inheritance of heriditary spherocytosis & elliptocytosis:
Both Autosomal dominant.
- spherocytosis: more severe if recessive
- elliptocytosis: more severe if dominant
Other differentials for spherocytes in blood film:
Lead poisioning and ABO incompatiability (few cells)
What is the screening test and the confirmatory test used to diagnose heriditary spherocytosis?
Screening: osmotic fragility test
Confirmatory: Eosin-5-malemide “dye staining by flow cytometery”
LFT findings in heriditary spherocytosis:
Increased total bilirubin - if gallstone: increased direct billirubin.
Horizontal vs vertical defects will cause which type of cell membrane hemolytic anemias:
- horizontal: Elliptocytosis
- vertical: Spherocytosis
Most commonly Heriditary eliptocytosis is inherited as:
Autosomal dominant
If you were given a case of elliptocytosis, and you want to determine the severity, what CBC indices will reflect that
MCV
Mutations in pyropoiklocytosis is in which gene
Spectrin
Usually patients with pyropoiklocytosis have family members with:
heridetray eliptocytosis
What is the mainstay treatment of hereditary pyropoiklocytosis during infancy?
Intermittent transfusion to avoid splenic crisis
What is the role of splenectomy in heriditary pyropoiklocytosis?
Very significant, increase in Hb, however not totally eliminated
What determines the severity of G6PD?
1-Quantity
2- type of G6PD
What is the mode of inheritance in G6pd
X-linked
Color of urine in G6pd hemolysis is expected to be
Tea colored urine
Name agents that can induce hemolysis in g6pd
Fava beans, sulfa medications, bacterim
The only intravascular hemolysis mentioned in this lecture is
Cold AHA
How to treat pyruvate kinase deficency?
Splenictomy, folic acid, transfusion
