Hemolytic Anemia Flashcards
What is are Heinz Bodies?
Where do you find them?
Denatured Hb (supravital)
G6PD
What are Howe-Jolly Bodies?
Where do you find them?
Nuclear Remnants
Splenectomy, Ineffective hematopeiesis
What is Basophillic Stipling?
Where do you find them?
Residual RNA/Polysomes
Thalassemia, Lead, Enzymopathies, MDS
What are Pappenheimer Bodies?
Where do you find them?
Iron inclusions
Sideroblastic anemia, Asplenia
What are HbH inclusions?
Where do you find them?
Beta Tetramers (supravital)
Alpha Thalassemia (3 gene del)
What are the AD mutations for Hereditary Spherocytosis
Vertical Mutations
Ankyrin - ANK1
Band3 - SLC4A1
Beta Spectrin - SPTB
What are the AR mutations for Hereditary Spherocytosis
Vertical Mutations
Alpha Spectrin - SPTA1
Protein 4.2 - EPB42
What are the mutationsin Hereditary Eliptocytosis
Horizontal Mutations
Spectrin - SPTA,SPTB
Band 3
Protein 4.2
What is the mutation in Hereditary Pyropoikilocytosis (HPP)
AR
Spectrin - SPTA1
Lab Findings for
Hydrocytosis
Increased MCV
Decreased MCHC
Na+ gain
RhaG gene
Lab findings for Xerocytosis
Increased MCV & MCHC
Decreased Osmotic Fragility (opp HS)
K+ Loss
AD: PIEZO1, KCCN4
Very high thrombosis risk after splenectomy (avoid)
Left Shifted Oxygen
Dissociation Curve
Oxygen “Latches on”
Everything “Lower”
CO2, Acid, 2,3DPG, Temp
Right Shifted Oxygen
Dissociation Curve
Oxygen is Relinquished”
Everything “Rises”
CO2, Acid, 2,3DPG, Temp
Unstable Hb
Koln - Most freq, left shift curve
Zurich - Incr CO affinity, hemolytic
Poole - unstablegamma chain
Indianapolis - too unstableto find in PB, acts like Thal Major, AD inheritance
Most common type of
G6PD
Class 3
Activity 10-60%
t1/2 13 days
G6PD A - (African american)
Kaiping
Which class of G6PD can have decreased WBC fuction
Class 1
Pyruvate Kinase Mutation
PKLR
PKD - O2 Dissociation Curve
Right Shift - Incr 2,3 DPG
Glucose Phosphate Isomerase Deficiency
AR
Neuro Symptoms
Bland Smear
Pyrimidine 5’ Nucleaotidase Deficiency
Neuro Symptoms
Basophilic Stipling
Dx - Incr pyrimidine in RBC, Molec Testing
RBC Enzymopathies that cause
Myopathy
Phosphofructokinase
Aldolase
Phosphoglycerate Kinase (X linked)
RBC Enzymopathies that cause
Neuro Symptoms
Glucose Phosphate Isomerase Def
Pyrimidine 5’ Nucleotidase Def
Triosephosphate Isomerase Def
Phosphoglycerate Kinase Def (X linked)
Adenylate Kinase Def
Pathophys of PNH
Loss of CD55/59
PIGA mutations
Toxins that target phospholipases
Clostridium
Brown Recluse/Snake Venom
Where do you find DonathLandsteiner ab
Paroxysmal Cold Hemaglobinuria
