Hemolytic Anemias
Hemolytic Anemias
Hemolytic Anemias
DEFINITION
Those anemias which result from an increase in RBC
destruction coupled with increased erythropoiesis
Morphological abnormalities
•Spherocytes, sickle cell, target cell, schistocytes,
acanthocytes
Polychromatophilia Nucleated RBC
Sickle cell Thalassemia
Bone marrow findings
• Erythroid hyperplasia
• Reticulocytosis
5 % infancy
1 % after 1 year
Normal bone marrow Erythroid hyperplasia
Biochemical tests
Other tests
• Sickling test - sodium metabisulphite
• Osmotic fragility test
• HB Electrophoresis
HB ELECTROPHORESIS
Classification of hemolytic Anemia
CLASSIFICATION OF HEMOLYTIC
ANEMIA Intracarpuscular defects
• Red cell enzyme deficiency- G6PD deficiency,
Pyruvate kinase deficiency
• Hereditary defects of red cell membrane -
Hereditary spherocytosis, Elliptocytosis
• Ineffective erythropoiesis - Thalassemia
• Haemoglobinopathies - Sickle cell and
Hb C,D or E Diseases
• Paroxysmal nocturnal haemoglobinuria
EXTRA CARPUSCULAR DEFECTS
Hb A [alpha 2, beta2]
Hb F [a2, gamma2]
Hb A2 [a2 , delta2 ]
VARIOUS TYPES OF
HAEMOGLOBINS
• Hb A1:Alfa 2 ,Beta2 -Adult
• Hb A2 :Alfa2 , Delta2-Adult
• Hb F: Alfa2, Gama2-Fetal
• Gower I: Zeta2,Epsilon2-Embryonic
• Gower II: Alfa2, Epsilon2-Embryonic
• Portland I: Zeta2,Gama2-Embryonic
• Portland II: Zeta2, Beta2-Embryonic
BETA THALASEMIA
• Chelation Therapy
TRANSFUSION THERAPY
• As soon as diagnosis firmly established
• If Hb drops < 7 gms%, regular transfusion regimen
• Maintain Hb level 10-12gm/dl to prevent ill effects of anemia (hyper
transfusion)
• May require 10-15ml/kg every 2-3 weeks
• Transfusion with Group, type specific, triple saline washed packed cells is
preferable.
PREVENTION OF TRANSFUSION TRANSMITTED
INFECTIONS
• Iron Overload
• Excess GI absorption
• Lack of excretory mechanism for excess iron in the body
- Chronic red cell transfusions
• Effects of iron overload(secondary haemochromatosis)
• Iron deposit in Liver, spleen, heart, endocrine glands and leads to their mal
function
• Specific to symptoms
• CXR if respiratory symptoms (may be nl in acute chest)
• Bone Xray if localized bone tenderness
• (Note: Bone scans can aid in early differentiation of bone infarction
and osteomyelitis)
• Abd US or CT if abd pain to r/o cholecystitis or acute
abdomen, also useful to assess liver and spleen size.
• Head CT/MRI in neurological symptoms
MANAEMENT
There is no known cure for sickle cell anemia.
These main treatment options for the painful crisis involves heavy
reliance on painkilling drugs and oral and intravenous fluids
whose main functions are to reduce pain and prevent
complications.
Indications for blood transfusion
• Symptomatic episodes of acute anemia.
• Severe symptomatic chronic anemia.
• Prevention of recurrent stroke.
• Acute chest syndrome with hypoxia.
• Surgery with general anesthesia, Eye surgery.
• Refractory leg ulcers.
• Refractory and protracted pain symptoms.
• Acute severe priopism
Hydroxyurea
• Selectively kills cells in BM --> Stimulate erythroblasts that make Hb
F s polymerization of HbS
• Use in severe complications
• Dosing:
• Start 500 mg/day and to 1,000mg/day after 6-8 weeks
• Maintenance: 1000-2000mg/day
• Monitoring:
• CBC q4-6 weeks (decreases WBC and PLTs)
• Monitor for a decline in granulocyte or platelet counts.
• MCV ~ marker of HbF levels
• Stop if a patient does not respond after several months of
hydroxyurea therapy
• Long term side-effects are unknown
Pain Crisis
• HYDRATION
• PAIN CONTROL
• Opiates
• Ketorolac
• R/O INFECTION
• Oxygen (only if hypoxic)
• Tranfusions
• Newer approaches:
• Anticoagulation
• Epidural anesthesia
• Steroids
• Poloxamer 188 : an artificial nonionic surfactant that reduces sickle
erythrocyte adherence to the endothelium
Chest Crisis
• Presence of the following signs and symptoms in a patient with
sickle cell disease:
• Chest pain
• Temperature >38.5ºC
• Tachypnea, wheezing, or cough
• Etiology: Unclear
• Infarction, infection, fat embolism
• Diagnosis: No lab/imaging study can differentiate from other
pulmonary causes
• Management: IVF, pain control with opiates,
• Blood transfusion if hypoxic
• Exchange transfusion if worsening infiltrates
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