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)
● Inherited due to gene suppression or presence of silent Rh gene (Xo)
● Membrane abnormalities due to absence of all Rh-Hr antigens on the red cells outer layer
Rhnull Disease
in Rhnull Disease, mother is Rh _____ while baby is Rh _____
Rh negative mother & Rh pos baby
Rhnull Disease lab findings
High
Inc
● HIGH: Reticulocyte Count
● INC: Autohemolysis & Osmotic Fragility
Rhnull Disease peripheral smears
● Mild, chronic normocytic normochromic hemolytic anemia
● Smear shows stomatocytes & spherocytes
Inherited; Represents an imbalance in the membrane phospholipids in the red cells
High Phosphatidyl Hemolytic Anemia
Anemia may increase due to infection or under condition of stress
High Phosphatidyl Hemolytic Anemia
High Phosphatidyl Hemolytic Anemia Peripheral Smears:
causes mild anemia w/ morphologically normal cells
