ANEMIA DUE TO ACCELERATED RED CELL DESTRUCTION Flashcards
HEMOLYTIC ANEMIA MAY BE DUE TO: (2)
Intrinsic Hemolytic Anemias
Extrinsic Hemolytic Anemia
defect or red cell Itself, usually hereditary & grouped as membrane, metabolic, or hemoglobin defects
Intrinsic Hemolytic Anemias
Factor outside the red cell & acting upon it. Almost always ACQUIRED
Extrinsic Hemolytic Anemia
Inherited a a non-sex-linked dominant trait
Hereditary Spherocytosis/Congenital Hemolytic Jaundice Anemia
Most common in North Europeans
Hereditary Spherocytosis/Congenital Hemolytic Jaundice Anemia
in Hereditary Spherocytosis/Congenital Hemolytic Jaundice Anemia, spherocytes die ____
Spherocytes die prematurely
SPLENOMEGALY
Hereditary Spherocytosis/Congenital Hemolytic Jaundice Anemia
● Very rare chronic and acquired defect
● Chronic intravascular hemolysis
● Nocturnal hemoglobinuria occurs during sleep or after awakening
Paroxysmal Nocturnal Hemoglobinuria (PNH)/ Machiava-Micheli Syndrome
Paroxysmal Nocturnal Hemoglobinuria (PNH)/ Machiava-Micheli Syndrome pH of plasma
pH of plasma is low due to red cells
Paroxysmal Nocturnal Hemoglobinuria (PNH)/ Machiava-Micheli laboratory findings:
(+) in
Low in
● (+) Sucrose hemolysis test or Ham’s Acidified Serum Test or Sugar Water Test
● LOW: WBC & Platelet Count
Hemosiderinuria is a feature
Paroxysmal Nocturnal Hemoglobinuria (PNH)/ Machiava-Micheli
Paroxysmal Nocturnal Hemoglobinuria (PNH)/ Machiava-Micheli Peripheral smears:
ELEVATED
______/______ anemia
● ELEVATED: Reticulocyte Count
● Normocytic, normochromic anemia is present
● Inherited dominant trait
● Associated with severe hemolytic anemia in infants
● Defect involves the impaired association of spectrin dimers resulting in FREE, UNCONNECTED dimers
Hereditary Elliptocytosis/ Ovalocytosis
Hereditary Elliptocytosis/ Ovalocytosis is increased in
Osmotic Fragility Test
Autohemolysis of red cells is present
Hereditary Elliptocytosis/ Ovalocytosis
Hereditary Elliptocytosis/ Ovalocytosis peripheral smear
Non-hypochromic elliptocytes are abundant on blood films Count
● Rare, moderately severe congenital hemolytic anemia
● Inherited as recessive autosomal traits
● Occurs in blacks
Hereditary Pyropoikilocytosis
Hereditary Pyropoikilocytosis peripheral smear
Microcytosis, Striking Micro-poikilocytosis & Fragmentation
● Rare congenital anemia
● Inherited as recessive autosomal trait
Hereditary stomatocytosis (Hydrocytosis)
Hereditary stomatocytosis (Hydrocytosis) is inherited as recessive autosomal trait caused by ______ Na and ______ K due to increased permeability of membrane
INCREASED; DECREASED
In Hereditary stomatocytosis (Hydrocytosis) 10-30% red cells appear as
10-30% red cells appear as mouth like
● Caused by absence of beta-lipoprotein
● Associated w/ plasma lipid abnormalities
Hereditary Acanthocytosis (Abeta-lipoproteinemia)
Hereditary Acanthocytosis (Abeta-lipoproteinemia) is low in
LOW: total lipid, cholesterol & phospholipids
● Sample: EDTA
● Autohemolysis occurs
● Reticulocyte count ranges from normal to increased
● Presence of MILD ANEMIA
Hereditary Acanthocytosis (Abeta-lipoproteinemia)
