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Hemolytic Anemia - Pathophysiology and Diagnostic Approach: Debdatta Basu Department of Pathology Jipmer Pondicherry

Hemolytic anemia can result from extravascular or intravascular red blood cell destruction. Extravascular hemolysis is caused by inherent red blood cell defects like spherocytosis, while intravascular hemolysis results from extrinsic factors like infections. A hemolytic anemia workup involves establishing evidence of hemolysis and its cause through history, examination, blood tests, and specific diagnostic evaluations for conditions like thalassemia, sickle cell disease, autoimmune hemolytic anemia, and paroxysmal nocturnal hemoglobinuria.

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119 views55 pages

Hemolytic Anemia - Pathophysiology and Diagnostic Approach: Debdatta Basu Department of Pathology Jipmer Pondicherry

Hemolytic anemia can result from extravascular or intravascular red blood cell destruction. Extravascular hemolysis is caused by inherent red blood cell defects like spherocytosis, while intravascular hemolysis results from extrinsic factors like infections. A hemolytic anemia workup involves establishing evidence of hemolysis and its cause through history, examination, blood tests, and specific diagnostic evaluations for conditions like thalassemia, sickle cell disease, autoimmune hemolytic anemia, and paroxysmal nocturnal hemoglobinuria.

Uploaded by

Mike Ross
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
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Download as PPT, PDF, TXT or read online on Scribd
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Hemolytic anemia –

Pathophysiology and diagnostic


approach

Debdatta Basu
Department of Pathology
JIPMER
Pondicherry
Hemoglobin structure

Hb A = 2 2 - 95-97%
Hb F = 2 2 - < 1%
Hb A2 = 2 2 - < 3%
Senescent Red Cells Broken down to Amino
 Acids

RE Cells Haemoglobin releasd Haem + Globin


In RE Cells and
of Spleen broken down

In Plasma Iron + Protoporphyrin

Carried to iron stores Biliverdin


like Bone Marrow

Bilirubin (unconjugated)
Iron deposited in
Conjugated in Liver
Marrrow

Bilirubin excreted in bile

Bilirubin acted upon by
Bacterial enzymes in intestine

Enterohepatic  absorbed  Urobilinogen /
stercobilinogen
Circulation 
 Fecal stercobilinogen
Kidney

Urobilinogen in urine
(2-4 mg / day)
Hemolytic anemia

• Hemolysis

• Compensated hemolytic state

• Hemolytic anemia
Sites of destruction
• Extravascular
– RE cells in spleen and BM
– Life span less than 120 days
– Inherent defect of RBC

• Intravascular
– Circulating blood
– Extrinsic factors
– RBC essentially normal
Extravascular hemolysis
• RE cells
•  unconjugated Bilirubin
•  stercobilinogen
•  urinary urobilinogen - dark urine
•  iron stores
• Diseases like thalassemia, hereditary
spherocytosis
Intravascular hemolysis
• In circulating blood
• Hemoglobinemia
•  serum haptoglobin
• Hemoglobinuria
• Hemosiderinuria
•  LDH
• Malaria, fragmentation syndromes,
G6PD deficiency
Compensatory mechanisms

• Vigorous blood regeneration

• Erythroid hyperplasia

• Reticulocytosis
Clinical features
• Anemia

• Jaundice

• Splenomegaly
Clinical features (contd)
• Gall stones
– pigment stones

• Leg ulcers
– Bilateral
– medial or lateral
malleolus
Skeletal abnormalities
Acquired hemolytic anemia

• Anemia
• Jaundice
• Fever
• Chills and rigors
• Abdominal pain
Classification of HA

• Intrinsic defect
– Inherited
– Morphological abnormality, target cell

• Extrinsic defect
– Acquired
– Morphologically normal, fragmentation
Hereditary spherocytosis

• Deficiency of
spectrin

• Membrane instability

• Spherocytes
Disorders of globin chain

• Thalassemia
– Quantitaive deficiency
– Alpha or Beta
• Hemoglobinopathy
– Qualitative defect
– Hb S, Hb E, Hb D Punjab, Hb C
Thalassemia
Beta thalassemia
• Deficiency/Absence of beta chains
• Decreased Hb A - anemia
• Excess alpha chains precipitate -
erythroid cells destroyed in marrow -
anemia
• Hb F increase
• Iron overload
Electrophoresis at pH 8.6
Thalassemia trait
• Heterozygotes, asymptomatic
• Genetic counselling
• Near normal Hb with micro hypo RBC
• Increased Hb A2
• Column chromatography
• HPLC
Hemoglobinopathy
• Sickle cell disease
• Africa, Mediterranean, Central India
• Valine for Glutamine at 6th position of
Beta chain
• Sickling phenomenon
• Autosplenectomy
• Crises
Extracorpuscular defects
• Immune mediated
– Alloimmune hemolytic anemia
• Mismatched transfusion
• Hemolytic disease of newborn
– Autoimmune hemolytic anemia
• Infections - falciparum malaria
• Fragmentation syndrome - Mechanical
– Cardiac
– MAHA - DIC, TTP, HUS
Infections causing HA
• Malaria
– Plasmodium falciparum
– Black water fever

• Babesiosis
• Clostridial sepsis
Hemolytic disease of newborn

• Rh incompatibilty

• Rarely ABO
incompatibilty
Autoimmune haemolytic
anaemia AIHA
Warm antibody type: antibodies react
with RBC at 37oc
• Idiopathic - 35-45 % of cases
• Secondary - 55- 65% of cases are
associated with
–autoimmune disease like SLE,rheumatoid
arthritis, ulcerative colitis
–CLL and Non-Hodgkin’s lymphoma
–drugs like M-DOPA, Procainamide,
Levodopa
Autoimmune haemolytic
anaemia AIHA (contd.)
Cold antibody type: Antibodies react with
RBCs at lower temperature < 370 c
• Idiopathic-Cold agglutinin disease
• Secondary
– Paroxysmal cold haemoglobinuria PCH
– Infectious mononucleosis
– M.Pneumonia infection
Causes of Red Cell Fragmentation

A. Cardiac Haemolytic Anaemia


– Valvular prosthesis
– Aortic Stenosis
– Mitral valve disease
– Coarctation aorta
B. Microangiopathic Haemolytic anaemia-MAHA
– Haemolytic uremic syndrome(HUS)
– Thrombotic thrombocytopenic purpura(TTP)
– DIC
c. March Haemoglobinuria
Drug induced HA
• Hapten type mechanism
– Penicillins and cephalosporins
• Imune complex ( Innocent bystander)
– Quinine, Quinidine, Isoniazid
• Auto immune type
– Alpha methyl dopa, Procainamide,
Ibuprofen
Paroxysmal Nocturnal
Hemoglobinuria
• Acquired intrinsic defect
• Stem cell disorder
• Susceptibility to complement mediated
lysis
• Mutations in PIG-A gene leads to
defective anchorage of certain proteins
like DAF
• Intravascular hemolysis
Approach to Hemolytic Anemia
• Establish hemolysis
– Signs of increased RBC destruction
– Signs of accelerated erythropoiesis
• Establish cause of hemolysis
– History and examination
– Peripheral blood examination
– Specific tests

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