Anemia II Flashcards
How would warm autoimmune hemolytic anemia look under scope?
Spherocytes and not much else
What is the antibody in question in warm autoimmune hemolytic anemia?
IgG
What should you look to as the cause for WAHA?
Primary causes: no idea
Secondary causes: Leukemia/Lymphoma, other malignancies, Autoimmune disorders, infection, drugs
After IgG coats RBC’s, what is their fate?
Entire phagocytosis or take a bite
What clinical physical exam finding is indicative of WAHA?
Splenomegaly
How do you diagnose WAHA? Treatment?
Diagnosis: DAT
Treatment: Steroids/Splenectomy (severe)
What immune components are in question in Cold autoimmune hemolytic anemia?
IgM, Complement
What are the primary and secondary causes?
Primary: Unknown
Secondary: Infections and lymphoproliferative diseases
What is the biochemical process behind pathogenesis?
IgM and complement coat RBC’s, but fall off in warm parts of the body
So in cold parts, IgM cause agglutination and complement cause lysis
How do you diagnose CAHA?
Treat?
Diagnose: DAT for IgM and complement
Treat: Keep patient warm, treat underlying cause
What is the best lab to find hemoglobinopathy?
Hemoglobin electrophoresis
Which chain is at fault in sickle cell anemia? What is the substitution?
Beta chain, Valine for glutamate
What happens at the cellular level for pathogenesis?
Once cell loses oxygen, the cells becomes like a sickle,
they aggregate and clog up vessels
What can you expect to find on a blood smear of a patient with sickle cell, post-splenectomy?
Sickle cells Nucleated RBC's Howell-jolly bodies Pappenheimer bodies Targets Increased platelets
What is the underlying cause of thalassemia?
Quantitative defect in production of alpha or beta chains of hemoglobin
What will a blood smear look like for a patient with thalassemia?
Microcytic, hypochromic with increased RBC and target cell count
What is different about alpha and beta chain deficiencies in thalassemia?
Alpha chain has 4 genes, the problem is a deletion of a gene
Beta chain has 2 genes, the problem is defective gene
Describe the severity of the following annotations:
beta gene
beta0 gene
beta+ gene
Beta gene: normal
beta0 gene: no beta chains produced
beta+ gene: some beta produced
The most severe beta thalassemia symptoms are those with what genotypes?
Beta0 and Beta+
Beta 0 and Beta0
Describe the severity of the genotypes of alpha thalassemia.
a - / a a: silent
a - / a - or aa/–: alpha thal trait
–/-a: HbH disease
–/–: Hydrops fetalis
What type of hemoglobin do you see in infants with alpha thalassemia? Adults?
Infants: gamma4 tetramers (Hb Barts)
Adults: beta4 tetramers (HbH)
What is the morphology of thalassemia?
hypochromic Microcytic Poikilocytosis anisocytosis target cells Basophilic stippling
What demographic is alpha thalassemia most common for? beta thal?
Alpha: Asians/blacks
Beta: mediterranian/asian/black
What is the underlying cause of microangiopathic hemolytic anemia?
trauma to RBC’s
Causes of MAHA?
Artificial heart valve
DIC, TTP, HUS
What type of cell is most indicative of MAHA?
Triangulocyte
What does anemia of chronic disease look like?
normocytic, normochromic
What is overproduced in AOCD?
Hepcidin
What is the root cause of anemia in kidney failure?
Lack of erythropoietin production
What do you start to see in renal failure anemia?
Echinocytes
What is anemia of the liver characterized by on a blood smear?
Poikilocytes, acanthocytes
What is indicative of aplastic anemia? What is the cause?
Just decreased cells and decreased marrow
Idiopathic