Microcytic Hypochromic Macrocytic Normochromic Normocytic: Anemia Hematocrit
Microcytic Hypochromic Macrocytic Normochromic Normocytic: Anemia Hematocrit
Microcytic Hypochromic Macrocytic Normochromic Normocytic: Anemia Hematocrit
Anemia – reduction of the total circulating red cell mass below normal limits
- Diagnosed by reduction in hematocrit (packed red cells/total blood volume)
Morphologic characteristics providing etiologic clues:
- red cell size (normocytic, microcytic, or macrocytic)
- degree of hemoglobinization, reflected in the color of red cells (normochromic or hypochromic)
- shape
Types of anemia:
- microcytic hypochromic anemias
o caused by disorders of hemoglobin synthesis (most often iron deficiency)
- macrocytic anemias
o abnormalities that impair the maturation of erythroid precursors in the bone marrow
- Normochromic, normocytic anemias have diverse etiologies;
o in some of these anemias, specific abnormalities of red cell shape (best appreciated through visual
inspection of peripheral smears) provide an important clue as to the cause.
o The other indices can also be assessed qualitatively in smears, but precise measurement is carried
out in clinical laboratories with special instrumentation.
The most useful red cell indices are as follows:
- MCV: Mean cell volume: the average volume of a red cell expressed in femtoliters (fL)
- MCH: Mean cell hemoglobin: the avg content (mass) of Hgb per red cell, expressed in picograms
- MCHC: Mean cell hemoglobin concentration: the average concentration of hemoglobin in a given
volume of packed red cells, expressed in grams per deciliter
- RDW: Red cell distribution width: the coefficient of variation of red cell volume
Extravascular Hemolysis
- reduced deformability of RBCs RBC sequestration and phagocytosis by macrophage
- symptoms: anemia, splenomegaly, jaundice
o decreased plasma haptoglobin (from binding extra hemoglobin)
- Tx: splenectomy
β Thalassemia
- β0 mutation no β-globulin; β+ mutation -> reduced β-globulin
- mutations deficit in HbA synthesis -> hypochromic, microcytic RBCs, reduced O2 capacity, decreased
RBC lifespan; membrane damage ineffective erythropoiesis (erythroid hyperplasia and
extramedullary hemotopoiesis and extravascular hemolysis cachexia), iron overload (from decreased
hepcidin)
- Pathogenesis of β-Thalassemia
o hallmark = aggregates of unpaired α-globulin chains (not seen on blood smears)
- β-Thalassemia syndromes
o β-Thalassemia Major (β0-β0, β+-β0, β+-β+) -
elevated HbF, severe transfusion-
dependent anemia 6-9 months after birth;
anisocytosis, poikilocytosis, microcytosis,
hypochromia, reticulocytosis, "crew cut"
x-ray, iron overload
o β-Thalassemia Minor (trait) (β0-β, β+-β) -
mild asymptomatic microcytic anemia
o β-Thalassemia Intermedia - severe non-
transfusion dependent anemia
- Clinical Features of β-Thalassemia
o growth retardation and early death in
untreated children
o Cardiac disease from iron overload and
secondary hemochromatosis (from
transfusions) -tx with iron chelators
o Tx: bone marrow transplant
α-Thalassemia
- inherited deletions that result in reduced or absent synthesis of α-globulin chains
- anema from lack of adequate hemoglobin and excess unpaired non-α-chains (β,γ,δ)-> Hemoglobin Barts
and HbH
- α-Thalassemia Syndromes
o severity depends on number of defective genes (there are 4 α-globulin genes)
o carrier=1: asymptomatic, no RBC abnormality (silent carrier state)
trait=2: asymptomatic, microcytic anemia
HbH disease=3: severe, non-transfusion-dependent anemia
hydrops fetalis=4: lethal in utero w/o transfusions; b/c hemoglobin Barts doesn't release O2
Immunohemolytic anemia
- caused by antibodies against RBCs premature destruction
- sometimes caused by a drug
- dx: direct and indirect Coombs test
- Warm antibody type, cold agglutinin type, cold hemolysin type
Warm antibody type (immunohemolytic anemia)
- most common - 50% idiopathic, rest is autoimmune, drug-induced (penicillins, cephalosporins, α-
methyldopa), or lymphomas
- IgG antibodies against Rh antigens coat RBCs which bind to Fc receptors on phagocytes partial
phagocytosis spherocytosis splenomegaly and extravascular hemolysis
Cold agglutinin type (immunohemolytic anemia)
- IgM antibodies bind to RBCs at low temperatures
- antibodies appear after infxns (mycoplasma pneumoniae, EBV, CMV influenza, HIV)
- agglutination occurs in "cold skin" areas (fingers, toes, nose, ears) -> pallor, cyanosis, Raynaud
Cold hemolysin type (immunohemolytic anemia)
- "paroxysmal cold hemoglobinuria" intravascular hemolysis and hemoglobinuria (sometimes fatal)
- autoantibodies (IgG) bind to P group RBC antigens in cool areas of body complement-mediated lysis
occurs when RBCs move to warm areas of body
- most cases follow viral infections and are transient
- treatment involves removing offending factors (drugs), or treating with immunosuppression and
splenectomy