Flashcards in Hemolytic Anemias Deck (50):
What results from hemolytic anemia?
RBC destruction leading to loss of RBC mass and release of cellular contents
Inherited/intrinsic hemolytic anemias
RBC membrane disorders
RBC enzyme deficiencies
Acquired/extrainsic hemolytic anemias
autoimmune (ab mediated)
What is extravascular hemolysis?
spleen, liver, and bone marrow degradation of RBCs
What is intravascular hemolysis?
destruction of RBCs w/in the vessels
RBC membrane d/o
RBC enzyme d/o
sickle cell anemia
Lab findings w/hemolysis
increased LDH, AST, K+
hemoglobinemia (free Hb in plasma)--> decreased haptoglobin
Causes of hemoglobinuria
it's Hb not scavenged by haptoglobin
what is hemosiderinuria?
iron deposits in renal epithelium then shed in urine
Common symptoms of all hemolytic anemias
Symptoms of acute decrease in Hb
restlessness, anxiety, DOE, syncope, confusion (by increasing age)
Clinical features of hyperbilirubinemia
Rare complications of hemolytic anemia
pigment induced kidney injury
folate deficiency due to increased utilization
increased risk of venous and arterial thrombosis (esp atypical sites)
What disease do you get chipmunk facies with?
beta thal major
What disease do you see asplenia with?
When do you see splenomegaly, hepatomegaly related to exrtra medullary hematopoiesis?
thal, PK deficiency, hereditary spherocytosis
When do you see microangiopathic hemolytic anemia? (7 diseases)
Causes of spherocytic hemolytic anemia (2)
autoimmune hemolytic anemia (AIHA)
What is the most common inherited hemolytic anemia in the US?
Mutations in hereditary spherocytosis?
ankyrin (most common)
Complications of HS?
aplastic crisis (parvo b19)
AIHAs qualifying feature
host antibodies reactive with autologous RBCs
shortened in vivo RBC survival
Warm AIHA features
secondary-lymphoprolypherative disoders, AI disorders, non hematopoietic neoplasms, drugs
Cold AIHA features
secondary-post mono or mycoplasma pneumo, lymphoproliferative disorders
mycoplasma, cold AIHA
mono, cold AIHA
When are cold aggluinins seen
Blood smear findings for warm/cold AIHA
What does warm AIHA extravascular hemolysis include?
Ab mediated or complement mediate hemolysis
How do cold agglutinins occur in cold AIHA?
IgM binds in cold, then mediates agglutination, blocks superfiscial distal vessels
IgM can also mediate complement mediated RBC injury, leads to extravascular hemolysis
Symptoms of Warm AIHA?
Symptoms of cold AIHA?
follows cold exposure
symptoms of anemia
post infection 2-3 weeks
What does a positive DAT/Coombs test indicate?
an autoimmune hemolytic anemia or transfusion reaction
What are the 3 congenital RBC enzyme disorders?
Pyruvate Kinase deficiency
What does the mutation in GDPDH deficiency cause?
defect in the pathway resonsible for reducing oxidants
decreased glutathione production
buildup of G6P
enzyme stability decreases with age of the RBC
Examples of oxidant stressors
drugs-dapsone, sulfonamides, primaquine, nitrofurantoin, quinolones
G6PDH deficiency inheritance?
common in malarial endemic areas
G6PD A- predominant abnormal varient, 11% all AAs
G6PD A- ceaveat
only old RBCs hemolyze
reticulocytes and new RBCs do not
G6PDH screen findings?
NADPH fluorescence (low to intermediate)
Quantitative assay to confirm
Examples of mechanical trauma to RBCs
defective cardiac valves
Antiphospholipid antibody syndrome
What is Paroxysmal nocturnal hemoglobinuria?
acquried mutation in PIGA gene (X) resulting in decreased GPI linked proteins
lack of GPI linked anti-complement proteins makes cells susceptible to complement lysis
stem cell disorder, RBCs most sensitive to lysis
What is it?
chronic low level hemolysis
acute episodic hemolysis events