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31 Cards in this Set

  • Front
  • Back
Extravascular hemolysis
takes place within macrophages outside vascular stream
Physiologically normal conditions
Low-grade chronic hemolytic states
Intravascular hemolysis
major red cell lysis occurs within the circulation
15% of Hb catabolism follows this pathway
Transfusion reactions
Role of The Spleen
Primary site of RBC destruction
especially for less adaptable (spherocytes), less flexible membranes (older cells), inclusions (heinz bodies and howell-jolly bodies)
What takes the place of the spleen in splenectomized patients?
the macrophages of the Liver and Bone marrow
What is the size of the spleen vasculature?
3uM
Heinz bodies
denatured Hb
Howell-Jolly bodies
nuclear remnants
Haptoglobin (Hp)
-a Hemoglobin (Hb) binding glycoprotein made in the liver
-an acute phase reactant
-absent in the newborn
What happens with haptoglobin during hemolysis?
-Rapid saturation of Hp and rapid clerance (into hepatocyte) of HB/Hp complex
-Low or absent plasma Hp is an indicator of recent or ongoing intravascular hemolysis
What binds free hemoglobin in the blood?
haptoglobin
hemopexin
methamalbumin
* all of these form complexes and are taken up into hepatocytes
hemosiderine
-storage of hemoglobin
-visualized with prussian blue staining
-occurs with low chronic intravascular hemolysis
Hemolytic Anemia
- Shorter life-span of erythrocytes
- Compensatory increase in erythrocyte production (reticulocytosis)
Hemolytic Anemia - Etiology
Intrinsic red cell abnormalities
Extrinsic causes of hemolysis
Immune mediated hemolysis
Non-immune mediated hemolysis
Intrinsic red cell abnormalities
Hemoglobin Disorders
Red Cell Enzyme Disorders
Red Cell Membrane Disorders
Hemoglobin Disorders
Unstable Hemoglobins
Methemoglobinemia
Thalassemia
Sickle Cell Disease
Red Cell Enzyme Disorders
G6PD Deficiency
Pyruvate Kinase Deficiency
Red Cell Membrane Disorders
Hereditary Spherocytosis
Hereditary Elliptocytosis
Extrinsic Causes of Hemolysis
Immune mediated hemolysis
Schistocytic hemolytic anemia
Immune mediated hemolysis
Warm Reactive Autoimmune Hemolytic Anemia (AIHA)
Cold Agglutinin Disease
Paroxysmal Cold Hemoglobinuria (PCH)
Schistocytic hemolytic anemia
Mechanical destruction of RBC as they travel
Hemangiomas (Kasabach-Merritt syndrome)
Prosthetic heart valves
Microangiopathic hemolytic anemia (MAHA)
Microangiopathic hemolytic anemia (MAHA)
TTP
HUS
Clinical Presentation of Hemolytic Anemia
Pallor
Icterus, jaundice
Fatigue
Splenomegaly
Gallstones
Cholecystitis
Dark urine
May present with parvovirus associated aplasia
Family history
Increased Nutritional Requirements of Chronic Hemolysis
- Greatest requirement is for folic acid and lowest body stores (10 day storage)
- Amino acid to make globin chains (normal nutritional status)
- B12 (10 years storage)
- No increase Fe requirement since it is “recycled” and stored in bone marrow
Direct Coombs
Direct Antiglobulin Test
- to identify the presence of immunoglobulins or complement on the surface of the red cells
- Sample from the patient is mixed with reagent that has antibodies against human immunoglobulins and complement.
- If agglutination forms, the test is +.
If the direct coombs test is positive, further tests are done
IgG only on surface of RBC
IgG and C3complement
C3 only on surface cells, antibody on cells may be IgM
Indirect Coombs Testing
take the serum of a patients blood and test the antibodies that are present
how long does it take babies to develp Antibodies?
6 months
Warm Reactive Autoimmune Hemolytic Anemia (AIHA)
IgG mediated
Extravascular clearance primarily via the reticuloendothelial system (spleen)
May be idiopathic or associated with SLE, lymphoid malignancies, immunodeficiency
Cold Agglutinin Disease
IgM mediated
Can be associated with Mycoplasma, EBV
Intravascular lysis
Paroxysmal Cold Hemoglobinuria (PCH)
-Acute illness, often after viral URI
>Inciting infections include measles, mumps, varicella, syphilis, Mycoplasma
-Caused by cold reactive IgG (Donath Landsteiner Antibody)
-Intravascular hemolytic anemia
Management of Hemolytic Anemia
1. Observe growth, development
2. Determine baseline hemoglobin
3. Follow for splenomegaly
4. Educate family regarding risks for gallstones, parvovirus B19 aplastic crisis
5. Folate supplementation
6. Erythrocyte transfusions, intermittent vs chronic
7. Splenectomy
8. Cholecystectomy if symptomatic gallstones