RBC and Bleeding, Lung and Pleura, Head and Neck Flashcards
Differentials for microcytic anemia (MCV <80 fL)
TAILS
*Thalassemia
*Anemia of chronic inflammation
*Iron deficiency anemia
*Lead poisoning
*Sideroblastic anemia
Splenomegaly in the case of hemolytic anemia can be seen in what type of hemolysis?
*Extravascular hemolysis
*Chronic hemolysis
For intravascular and acute, usually hemoglobinemia, hemoglobinuria, hemosiderinuria due to hemoglobin being liberated inside the blood vessel (soon be cleared by kidney)
Defect in hereditary spherocytosis
Intrinsic - defect in membrane skeleton (spectrin, ankyrin, Band 3, Band 4.2) making them less deformable and easily lysed in spleen due to entrapment there
Etiology of G6PD
Intrinsic - G6PD deficiency –> ↓ NADPH –> ↓ reduced glutathione –> ↑ RBC susceptibility to oxidative stress
Triggers: infections, drugs, foods
Cell findings in G6PD
Heinz bodies (precipitated denatured Hb) - asplenic
Bite cells - intact spleen
Spherocytes
Etiology of Sickle cell disease
Autosomal recessive (SA is asymptomatic, SS is symptomatic)
Intrinsic: 6th codon of B-globin chain has point mutation (glutamate –> valine) –> hemoglobin S (HbS)
Situations favoring sickling of RBCs in Sickle cell disease
↓ intracellular pH
↓ O2 tension
Lab findings in Sickle Cell Anemia
Microcytic anemia
Target cell
Sickle cell
↓ HbA
↑ HbS
Thalassemia general etiology and pathogenesis
Etiology
↓ Globin chains –> ↓ Hb synthesis –> anemia
Pathogenesis
↓ in one globin chain –> excess of other globin chain –>precipitate inclusions –> membrane damage –> apoptosis within marrow –> anemia
Thalassemia morphology and lab:
CBC:
PBS:
Skull X-ray:
Others:
CBC: Microcytic hypochromic
PBS:Poikilocytosis, anisocytosis, Target cells
Skull xray: Crew cut appearance
Secondary hemochromatosis (liver, pancreas, heart)
Etiology:
Beta Thalassemia
Alpha Thalassemia
Beta thalassemia:
B-globin mutations (2 copies in Chromosome 11)
Alpha thalassemia:
A-globin gene deletions (4 copies in Chromosome 16)
Beta thalassemia that is transfusion dependent
Beta thalassemia major (2 mutated)
*Beta thalassemia intermedia (variable mutated), transfusion in special situations
*Beta thalassemia minor (1 mutated), asymptomatic or mild
Alpha thalassemia trait, how many alpha globin gene deletions
2
HbH, how many alpha globin gene deletions
3
(B4)
Hydrops fetalis, how many alpha globin gene deletions
4
(γ4)
When does anemia occur in those with Beta thalassemia? How about Alpha thalassemia
Beta thalassemia: 6-9 months after birth (shift from HbF –> HbA)
Alpha thalassemia: At birth (need of alpha globin chains even for HbF)
Why is Alpha thalassemia less severe than Beta thalassemia?
Because it only misses B-globin chains (and γ-globin chains) which are more soluble than a chains –> lesser inclusions –> ↓ membrane damage –> ↓ apoptosis in marrow –> less severe anemia
Defect in Paroxysmal Nocturnal Hemoglobinuria
PIGA mutations –> ↓ GPI anchor –> ↓ CD55, CD59, C8 binding protein –> ↑ susceptibility to complement mediated lysis
Antibody in Warm-Antibody Type Autoimmune Hemolytic Anemia
IgG active at 37C
*Associated with SLE, drugs, CLL
Antibody in Cold Agglutinin Disease
IgM active at <37C
*Associated with infections of Mycoplasma pneumoniae, CMV, EBV, HIV, and CLL
Antibody in Paroxysmal Cold Hemoglobinuria
IgG Anti P (Donath-Landsteiner antibody)
Coomb’s test that detects antibodies present in patient’s serum
Indirect Coombs’ Test
Used for compatibility testing, bloodbank etc.
*Direct Coombs’ Test detects antibodies attached to RBCs
ex: Hemolytic disease of fetus and newborn
Type of hemolysis in Cell finding in Hemolytic Anemia 2ndary to red cell trauma
Intravascular due to sheer stress
Cell finding in Hemolytic Anemia 2ndary to red cell trauma
Schistocytes (fragmented red cell)
Echinocytes (Burr cell)