ANEMIA DUE TO ACCELERATED RED CELL DESTRUCTION

Question 1

HEMOLYTIC ANEMIA MAY BE DUE TO: (2)

Answer 1

Intrinsic Hemolytic Anemias
Extrinsic Hemolytic Anemia

Question 2

defect or red cell Itself, usually hereditary & grouped as membrane, metabolic, or hemoglobin defects

Answer 2

Intrinsic Hemolytic Anemias

Question 3

Factor outside the red cell & acting upon it. Almost always ACQUIRED

Answer 3

Extrinsic Hemolytic Anemia

Question 4

Inherited a a non-sex-linked dominant trait

Answer 4

Hereditary Spherocytosis/Congenital Hemolytic Jaundice Anemia

Question 4

Most common in North Europeans

Answer 4

Hereditary Spherocytosis/Congenital Hemolytic Jaundice Anemia

Question 5

in Hereditary Spherocytosis/Congenital Hemolytic Jaundice Anemia, spherocytes die ____

Answer 5

Spherocytes die prematurely

Question 6

SPLENOMEGALY

Answer 6

Hereditary Spherocytosis/Congenital Hemolytic Jaundice Anemia

Question 7

● Very rare chronic and acquired defect
● Chronic intravascular hemolysis
● Nocturnal hemoglobinuria occurs during sleep or after awakening

Answer 7

Paroxysmal Nocturnal Hemoglobinuria (PNH)/ Machiava-Micheli Syndrome

Question 8

Paroxysmal Nocturnal Hemoglobinuria (PNH)/ Machiava-Micheli Syndrome pH of plasma

Answer 8

pH of plasma is low due to red cells

Question 9

Paroxysmal Nocturnal Hemoglobinuria (PNH)/ Machiava-Micheli laboratory findings:
(+) in
Low in

Answer 9

● (+) Sucrose hemolysis test or Ham’s Acidified Serum Test or Sugar Water Test
● LOW: WBC & Platelet Count

Question 10

Hemosiderinuria is a feature

Answer 10

Paroxysmal Nocturnal Hemoglobinuria (PNH)/ Machiava-Micheli

Question 11

Paroxysmal Nocturnal Hemoglobinuria (PNH)/ Machiava-Micheli Peripheral smears:
ELEVATED
______/______ anemia

Answer 11

● ELEVATED: Reticulocyte Count
● Normocytic, normochromic anemia is present

Question 12

● Inherited dominant trait
● Associated with severe hemolytic anemia in infants
● Defect involves the impaired association of spectrin dimers resulting in FREE, UNCONNECTED dimers

Answer 12

Hereditary Elliptocytosis/ Ovalocytosis

Question 13

Hereditary Elliptocytosis/ Ovalocytosis is increased in

Answer 13

Osmotic Fragility Test

Question 14

Autohemolysis of red cells is present

Answer 14

Hereditary Elliptocytosis/ Ovalocytosis

Question 15

Hereditary Elliptocytosis/ Ovalocytosis peripheral smear

Answer 15

Non-hypochromic elliptocytes are abundant on blood films Count

Question 16

● Rare, moderately severe congenital hemolytic anemia
● Inherited as recessive autosomal traits
● Occurs in blacks

Answer 16

Hereditary Pyropoikilocytosis

Question 17

Hereditary Pyropoikilocytosis peripheral smear

Answer 17

Microcytosis, Striking Micro-poikilocytosis & Fragmentation

Question 18

● Rare congenital anemia
● Inherited as recessive autosomal trait

Answer 18

Hereditary stomatocytosis (Hydrocytosis)

Question 19

Hereditary stomatocytosis (Hydrocytosis) is inherited as recessive autosomal trait caused by ______ Na and ______ K due to increased permeability of membrane

Answer 19

INCREASED; DECREASED

Question 20

In Hereditary stomatocytosis (Hydrocytosis) 10-30% red cells appear as

Answer 20

10-30% red cells appear as mouth like

Question 21

● Caused by absence of beta-lipoprotein
● Associated w/ plasma lipid abnormalities

Answer 21

Hereditary Acanthocytosis (Abeta-lipoproteinemia)

Question 22

Hereditary Acanthocytosis (Abeta-lipoproteinemia) is low in

Answer 22

LOW: total lipid, cholesterol & phospholipids

Question 23

● Sample: EDTA
● Autohemolysis occurs
● Reticulocyte count ranges from normal to increased
● Presence of MILD ANEMIA

Answer 23

Hereditary Acanthocytosis (Abeta-lipoproteinemia)

Question 24

● 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

Answer 24

Rhnull Disease

Question 25

in Rhnull Disease, mother is Rh _____ while baby is Rh _____

Answer 25

Rh negative mother & Rh pos baby

Question 25

Rhnull Disease lab findings
High
Inc

Answer 25

● HIGH: Reticulocyte Count
● INC: Autohemolysis & Osmotic Fragility

Question 26

Rhnull Disease peripheral smears

Answer 26

● Mild, chronic normocytic normochromic hemolytic anemia
● Smear shows stomatocytes & spherocytes

Question 27

Inherited; Represents an imbalance in the membrane phospholipids in the red cells

Answer 27

High Phosphatidyl Hemolytic Anemia

Question 28

Anemia may increase due to infection or under condition of stress

Answer 28

High Phosphatidyl Hemolytic Anemia

Question 29

High Phosphatidyl Hemolytic Anemia Peripheral Smears:

Answer 29

causes mild anemia w/ morphologically normal cells