Hemolytic Anemia Flashcards
CBC – Usually N/N anemia, reticulocytosis
Blood - Target cells, sickle cells, NRBC’s, Howell-Jolly bodies, Pappenheimer bodies
Lab - Sickledex screening test (turbid)
90% HbS, 8% HbF, 2% HbA2, 0% HbA
HbF protective-inc with hydroxyurea
Sickle Cell Disease
CBC - usually normal
Blood – few targets, no sickles
Lab – Sickledex screening test +
52% HbA, 45% HbS, 2% HbA2, 1% HbF
Sickle Cell Trait
CBC - Usually N/N anemia, reticulocytosis
Blood – Targets, Hb C crystals
Lab – 95% HbC, 2% HbA2, 3% HbF, 0% HbA
Hemoglobin C Disease
CBC - Usually normal
Blood – Targets
Lab – 60% HbA, 37% Hb C, 2% HbA2, 1% HbF
Hemoglobin C Trait
CBC - Usually N/N
Blood – Targets, HbC crystals, sickles, hybrids
Lab – Sickledex screening test +
HBC = HbS, 2% HbF, 1% HbA2, 0% HbA
Hemoglobin SC Disease
CBC - M/H anemia MVC 50-70, nl to high RBC count
Blood – Targets, stippling
Lab – 93% HbA, 5% HbA2, 2% HbF
Minor Beta Thalassemia
CBC - M/H anemia, MCV 50-60
Blood – Targets, NRBCs, stippling, anisopoikilocytosis
Intermedia Beta Thalassemia
CBC - Marked M/H anemia
Blood –Extreme aniscopoikilocytosis, targets, NRBCs, HJ-Bodies, pappenheimer bodies, stippling
Lab – 90% HbF, 10% HbA2
Major Beta Thalassemia
CBC - Microcytic anemia (MCV-70)
Blood – poikilo, targets, stippling, polychromasia
Lab – Sickledex screening test +
80% HbS, 1-20% HbF, >3% HbA2, 0-30% HbA
Sickle Cell Beta Thalassemia
What’s seen clinically in Sickle Cell Disease?
Clinical - Hand-foot synd, acute splenic seq, S. pnuemoniae, H. influenza, Mycoplasma, Salmonella (osteomyelitis), Pap necrosis, CRF, neph synd, UTIs, Aplastic crisis (parvo B19), Acute chest synd, leg ulcers, liver pain, Ca bilirub stones, marrow expansion
What’s seen clinically in Sickle Cell Trait?
usually none
What’s seen clinically in Hemoglobin C Disease?
usu asymptomatic, splenomegaly, jaundice, salmonella
What’s seen in hemoglobin C trait?
asymptomatic
What’s seen clinically in hemoglobin SC disease?
Asymptomatic to intermediate between HbSS and HbAS
What’s seen clinically in aa/-a (silent carrier) alpha thalassemia?
asymptomatic, no anemia
What’s seen clinically in –/-a (HbH Dz) alpha thalassemia?
MCV 50, mod/severe anemia, splenomegaly, HbH (beta tetramers)
What’s seen in –/– (hydrops fetalis) alpha thalassemia?
Hb Bart’s (gamma tetramers), Not viable
What’s seen clinically in minor beta thalassemia?
Clinical – Usually asymptomatic, do not confuse with Fe deficiency
What’s seen clinically in intermedia beta thalassemia?
Clinical – Symptomatic anemia (transfusions)
What’s seen clinically in major beta thalassemia?
Clinical – Transfusions, Fe overload, skeletal changes, cardiomyopathy, HSM, delayed growth
What’s seen clinically in sickle cell beta thalassemia?
Clinical – Resembles HbSS but milder, splenomegaly
AD, Ankyrin defect (Na/K ATPase)
CBC: Inc MCHC/MCH, numerous spherocytes
Labs: Inc LDH/Ind bili, dec hapto, DAT-, osmotic fragility+, flow band
Hererditary spherocytosis
AD, Abnormal spectrin
CBC: Usually non-anemic, 4+ elliptos
Labs: Dx by history, morphology
Hereditary Elliptocytosis
AR, spectrin deficiency + defect
CBC: 4+ poikilocytosis
Labs: Inc LDH/Ind bili, dec hapto
Hereditary Pyropoikilocytosis
AD, abn stomatin (Na/K, swelling)
CBC: High MCV 150, stomatos 4+
Labs: Inc LDH/Ind bili, dec hapto
Hereditary Stomatocytosis
Stem cell disorder, dec GPI –> dec CD55/59, complement lysis
CBC: N/N anemia, pancytopenia, retics < expected
Labs: Inc LDH, dec hapto, hemosiderinuria, flow for CD55/59
PNH
Xlink, oxidative denaturation Hb
CBC: N/N anemia, bites cells
Labs: high retics, Inc LDH/Ind bili, dec hapto, Heinz body stain, fluor screen test, enzyme assay
G6PD Deficiency
AR, dec ATP, membrane damage/dehydration
CBC: N/N anemia, aniscopoikilo, echinos, NRBCs, polychromasia
Labs: high retics, Inc LDH/Ind bili, dec hapto, enzyme assay
Pyruvate Kinase Deficiency
P. Falcip - rings, Babesia - tetrads
Parasites
CBC frags, polychromasia, dec plts
MAHA
dec ADAMTS13, inc Lg MW vWF, renal/CNS microthrombi, pentad young females, IgG autoAb,
TTP (MAHA)
Renal failure, fever, previous bloody diar, E. coli H7
HUS (MAHA)
Many underlying causes, endothelial damage, clots, dec factors/bleeding, low fibrinogen, high d-Dimers
DIC (MAHA)
OB, RUQ pain, inc LFTs, delivery
HEELP (MAHA)
IgG Ab, CVDs (lupus), lymphoma, drugs
CBC: Microspherocytes, polychromasia
Labs: high retics, Inc LDH/Ind bili, dec hapto, DAT+
Warm AIHA
IgM Ab, Viral/mycoplasma
CBC: RBC agglut, inaccurate values, polychromasia
Labs: high retics, Inc LDH/Ind bili, dec hapto, DAT+, thermal ampl test+
Cold AIHA (not PCH)
IgG anti-P Ab, children viral infections
CBC: micropspherocytes, polychromasia
Labs: Inc LDH/Ind bili, dec hapto, DAT+, Donath-Landsteiner test+
Cold AIHA (PCH)
What’s seen clinically in hereditary spherocytosis?
Variable, jaundice, splenomegaly, gallstones
What’s seen clinically in hereditary elliptocytosis?
Usually assymptomatic, splenomegaly
What’s seen clinically in hereditary pyropoikilocytosis?
Moderate/severe anemia
What’s seen clinically in hereditary stomatocytosis?
Variable anemia, thrombosis with splenectomy
What’s seen clinically in PNH?
young adult, back/abd/esophageal pain, HA, dark urine (night/am), venous thromboses
What’s seen clinically in G6PD Deficiency?
Rapid onset with exposure, Med > AA type due to retics involved, infection, drugs, fava beans (med type)
What’s seen clinically in pyruvate kinase deficiency?
Mild to moderately severe anemia, splenomegaly
What’s seen clinically in warm AIHA?
splenomegaly, jaundice
What’s seen clinically in cold AIHA (not PCH)?
CAD, Reynaud
What’s seen clinically in cold AIHA (PCH)?
sudden fever, chills, abd/back pain, jaundice with cold exposure