Disorders Of Hemolysis Flashcards
What are the primary etiologies of thrombotic microangiopathy (TMA)?
(1) TTP
(2) Atypical HUS (complement mediated)
(3) HUS (Shiga toxin)
(4) Drug-mediated TMA
What are the secondary causes of TMA (7)?
(1) Pregnancy (Pre-eclampsia/HELLP)
(2) Malignancy
(3) Infection
(4) HTN Emergency
(5) Autoimmune: Scleroderma renal crisis, APLA, SLE, Vasculitis.
(6) Transplant Associated
(7) DIC
How do you treat TTP (6)?
(1) PLEX (within 4-8hrs)
(2) FFP to bridge to PLEX
(3) Steroids: Pred 1 mg/kg or Solumedrol 1g/d
(4) Folic Acid 5 mg OD
(5) ASA if elevated troponin
(6) DVTp once platelets > 50
What are 3 common etiologies of spherocytes?
(1) Warm Autoimmune Hemolytic Anemia (IgG)
(2) Hereditary Spherocytosis
(3) Delayed Hemolytic Tranfusion Rxn
What are 2 causes of agglutination on blood smear?
(1) Cold autoimmune hemolytic anemia (IgM)
(2) Paroxysmal Cold Hemoglobinuria
What is the first line treatment for warm autoimmune hemolytic anemia?
Prednisone 1-1.5 mg/kg/day, tapered once hemoglobin levels have stabilized or markers of hemolysis are normal or clearly improving.
What investigations should you order if you suspect warm autoimmune hemolytic anemia based on spherocytes on the blood smear?
(1) Hemolysis Labs
(2) DAT
(3) D-dimer - if + get bilateral lower extremity Dopplers
What are the secondary etiologies of warm autoimmune hemolytic anemia?
(1) Lymphoproliferative Disorders (CLL)
(2) Autoimmune Disorders: SLE, RA, scleroderma
(3) Drugs (Methyldopa, NSAIDs)
What are the secondary causes of cold autoimmune hemolytic anemia?
(1) Infection: Mycoplasma pneumonia, EBC
2) Lymphoproliferative Disorders (MGUS, Waldenstroms, lymphoma
What is the treatment for cold autoimmune hemolytic anemia?
(1) Warm patient!
(2) Plasmaphersis to remove IgM ONLY if severe.
(3) Rituximab for refractory cases.
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