Hemolytic anemias Flashcards
warm agglutinin AIHA (autoimmune hemolytic anemia) etiology
drugs (penicillin),
viral infections
autoimmune (SLE)
immunodeficiency
lymphoproliferative like CLL
warm agglutinin AIHA clinical presentation
asymptomatic to life threatening anemia direct coomb’s positive with anti IgG anti-C3 or both
Treatment of warm agglutinin AIHA (autoimmune hemolytic anemia)
steroids
splenectomy in refractory cases
complications of warm agglutinin AIHA
VTE, lymphoproliferative disorders
etiology of cold agglutinin AIHA
infections (mycoplasma pneumoniae infection, infectious mononucleosis)
lymphoproliferative diseases
clinical presentation of cold agglutinin AIHA
symptoms of anemia
livedo reticularis
acral cyanosis with cold exposure that disappear with warming
direct Coombs positive with anti C3 OR anti IgM but not IgG
treatment of cold agglutinin AIHA
avoidance of cold temperatures even in warm weather
rituximab +/- fludarabine
not steroid responsive, don’t give antibiotics
complications of cold agglutinin AIHA
ischemia,
peripheral gangrene
lymphoproliferative disorders
findings of autoimmune hemolytic anemia (AIHA)
autoantibodies (can be either warm agglutinins IgG or cold agglutinins or IgM) destroying red blood cells
what are lab findings of AIHA?
increased haptoglobulin,
decreased Hgb,
increased indirect bilirubin
increased LDH.
See spherocytosis on peripheral smear due to partial phagocytosis of IgG coated RBCs
see increased MCV due to reticulocytosis.
diagnosis is via positive direct antiglobulin (Coomb’s test)
what causes pure red cell aplasia?
drugs, thymoma and parvo B19 infection. NOT AIHA.
chart for AIHA
G6PD deficiency is seen in
X linked disorder seen in Mediterranean and AA populations.
Occurs with TMP-SMX and due to a enzymatic disorder of RBCs and see oxidative stress that is caused by medications, favism, infections and metabolic abnormalities.
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what causes G6PD deficiency
Occurs with TMP-SMX and due to a enzymatic disorder of RBCs
and see oxidative stress that is caused by
medications (dapsone, antimalarials and sulfonamide abx)
favism (eating fava beans)
infections -
and metabolic abnormalities. - DKA
When do G6PD pts present after stressful event?
2-4 days after a oxidatively stressful event and see evidence of hemolysis or a 3-4 g/dl drop in Hgb with rise in reticulocyte count
Pathophysiology of G6PD and why we don’t see deficiency in G6PD during acute hemolysis
See drop in Hgb and rise in reticulocyte counts.
older deficient RBCs are replaced by younger cells which have sufficient G6PD enzyme activity to sustain further oxidative damage without hemolysis after the hemolysis event when older RBCs have hemolyzed due to stressful oxidative event.
lab findings of G6PD
drop in Hgb. See elevated LDH, low haptoglobin, indirect bilirubin increased.
See bite cells on peripheral smear
Reticulocyte count is high and normal G6PD levels during acute episode
Negative DAT or direct and indirect Coomb’s test.
when to check G6PD levels if one is suspected to have G6PD?
never check during acute hemolysis event. Check 2-3 months after hemolytic episode.
pyruvate kinase deficiency
Second most common cause of enzyme deficient hemolytic anemia after G6PD.
PK patients don’t have hemolysis after oxidative stress. No characteristic morphological RBCs on peripheral smear.
Bactrim can cause
hemolytic anemia in G6PD deficiency pts
Treatment of cold hemolytic anemia and cold agglutinin’s dx
Treatment of hemolytic anemia and cold agluttin dx related to this is avoidance of cold (even in warm weather) and heavy socks and coats to prevent agluttination.
seen with mycoplasma pneumoniae or have an underlying EBV with 1 antibodies.
severe dx: need rituximab.
transfusion related reactions chart
unusual cause of hemolytic anemia
Wilson’s dx
check serum ceruloplasm
elevated LFTs and hemolytic anemia in a young person
Wilson’s dx
