jslum.

com | Medicine
Approach to Diagnosi s of Haemolytic Anaemias

Definition Classification of Haemolytic Anaemias

Anemias which result from ↑ Rate of Red Cell Destruction Site of Haemolysis (Many cases – combination of Both )
Any condition that lead to ↓ in Mean Lifespan of Red Cell Extravascular Intravascular
Compensated Haemolytic Anaemia Macrophages of RES Within the circulatory system
Erythropoietin Hyperplasia Red cell destruction in RES Red cell destruction occur inside blood
Anatomical Extension of BM vessels
Red Cell destruction ↑ several fold before patient become anaemic (release of contents into plasma)
After Full Expansion, Normal adult marrow can
produce red cells 6-8 times normal rate

History Taking
Family History – Hereditary conditions
Ethnic Origin – G6PD common in Mediterranean & Chinese populations
Past History – NNJ indicative of Congenital conditions (eg. HS, G6PD Def.)
(NNJ – NeoNatal Jaundice)(HS – Hereditary Spherocytosis)
Triggering Events – History of Drugs, Infections

Clinical
Pallor of mucous membranes
Mild fluctuating Jaundice
Splenomegaly in Chronic Haemolysis
Dark Urine
Pigment Gall Stones
Ulcers around ankle
Aplastic Crisis – complicate viral infections
Growth Retardation
Hypertrophic skeletal changes
Lymphadenopathy/ Hepatosplenomegaly – lymphoproliferative disorder,
malignancy

Pathophysiol ogy of Haemolytic Anaemias (HA)

Haemolytic state Lab Features (Intravascular) Free Hb released from Damaged RBC
Presence of ↓ Red Cell Lifespan Haemoglobinaemia Hb Saturates Plasma Haptoglobins
Compensatory ↑ in Rate of Erythropoiesis Haemoglobinuria (Complex removed by Liver)
Anaemia Haemosiderinuria Excess free Hb filtered in Glomerulus
Frequently Absent – Compensatory ↑ Erythropoiesis (Iron storage protein in Urine) (Enter urine, Iron released)(Renal
Severe Decompensated Haemolysis – can lead to Severe Anaemia Methaemalbumin Tubules loaded with Haemosiderin)
Jaundice Methaemalbumin, Haemopexin
Haemolysis accompanied by ↑ breakdown of liberated Heme (Found in process of IV Haemolysis)
Bilirubin (1° breakdown product) – responsible for discoloration of Jaundice Haemoglobinuria Causes
Pigment Gallstones Dark Colour Urine Mismatched Blood Transfu sion (ABO)
Precipitated Bilirubin crystals (Chronic Congenital Haemolysis) Sign of G6PD Deficiency with Oxidant Stress
Splenomegaly Intravascular Red cell Fragmentation Syndrome
Extramedullary Haemopoiesis Haemolysis Some AIHA
Spleen is site of Destruction of Red Cells Some Drug, Infection Induce d HA
Haemoglobinuria (HbUria) PNH
Dark Red Urine/ Black March Haemoglobinuria
Intravascular Haemolysis Unstable Haemoglobin
Leg Ulcers Leg Ulcers
Sickle Cell Disease Severe Congenital
Due to Ischaemia caused by Sickle Cells Haemolytic Disorder
(Sickle Cell Anaemia)

Aplastic Crisis
Acute Arrest of Erythropoiesis
Following Parvovirus Infection
Chronic Haemolytic Disorders (common )
Growth Retardation & Delayed Puberty
Severe Congenital Haemolytic Disorders (Homozygous β Thalassaemia) Site of Defect
Hypertrophic Skeletal Changes Skeletal Changes Intrinsic (Intracorpus cular)
Expansion of Erythropoietic Marrow β Thalassaemia Structural, Functional Defect within
Congenital Haemolytic Disorders Only the Red Cell
Prominent Inherited/ Acquired
Frontal Bone Inherited (Hereditary)
Maxillary Bone Caused by Intrinsic Defect
Membrane Defects
(eg. Hereditary Spherocytosis)
Metabolic Defect
(eg. G6PD Deficiency)
Haemoglobin Defects
(Qualitative – Sickle Cell)
(Quantitative – Thalassaemia)
Exception
Severe Hereditary G6PD Deficiency
(Presence of E xtrinsic Trigger –
AntiMalarial Drug for Intrinsic
Defect to manifest)
Extrinsic (Extracorpuscular)
Abnormality in Red Cell
Environment
Acquired
Caused by Extrinsic Defect
Immune
(Autoimmune – AIHA)
(Alloimmune – HDA, HTR, Drugs)
Red cell Fragmentation Syndromes
March Haemoglobinuria
Infections
Chemical, Physical Agents
PNH
Exception
Paroxysmal Nocturnal
Haemoglobinuria (PNH)
(Acquired Intrinsic Defect)
 jslum.com | Medicine

Laboratory Findings
↑ Red Cell Breakdown ↑ Red Cell Production Damaged Red Cell
↑ Serum Bilirubin (Unconjugated) Reticulocytosis Morphology
↑ Urine Urobilinogen BM Erythroid Hyperplasia Microspherocytes, Elliptocytes, Fragments
↑ Faecal Stercobilinogen Special Tests
↑ LDH In absence of concomitant BM Disease, a brisk Osmotic Fragility, Autohaemolysis
Serum Haptoglobin Absent reticulocytosis should be observed within 3-5 days Red Cell Survival Shortened
after a decline in Hb 51Cr Labelling with study of sites of destruction

Polychromatic cell
(Red cell with Blue staining) Haemolytic Anaemia
Reticulocyte (appears in the usual stain) Fragmented cells, Bitten Cells
Signs of Damaged cells occurring in Haemolysis

Cold AIHA
Red cell clumping (agglutination)

(In Warm AIHA – Spherocytes)

Reticulocytosis Sickle Cell Disease
Feature of ↑ Red Cell Production Sickle Cells
Methylene Blue is used to stain the Reticulocytes