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Immune Haemolytic Anaemia
Definition
Group of disease – Haemolysis occurs be cause Antibody production by body
against its own Red cells
Characteristic
+ve DAT/ Coombs Test
Classification
Warm (70%) Cold (30%)
1° - Idiopathic 1° - Idiopathic
2° 2°
Malignancy – Lymphoma, CLL Infections – Mycoplasma pneu monia,
Alloallergic disease – SLE infectious mononu cleosis
Infections – Monon ucleosis syndrome Lymphoma
Drugs – Methyldopa, Fludarabine Paroxysmal Cold HbUria
Coombs Test
Clinical Manifestation of Autoimmune Haemolytic Anaemia (AIHA) Indirect Coombs Test / Indirect Antiglobuli n Test
Warm AIHA Paroxysmal Cold Cold Agglutinin Detects Antibodies against RBCs foun d in Patient’s Serum
Haemoglobunuria Syndrome Normal RBCs + Patient’s Serum → DAT
Onset 30-60 y/o Young adult 60-90 y/o
Sex F↑ M=F M↑
Severity Variable Acute, Intermittent, Mild, Moderate
Anaemia Massive
HbUria Rare Common Severe cases only
Spleneomegaly Common Intermittent Common
Symptoms Cold Urticaria, Cold Intolerance,
Paresthesis, Rigor, Raynaud’s
Anaphylactic phenomenon
Treatment Corticosteroids Treat 1° Disease Avoid Cold
Splenectomy (Syphilis, Viral Immunosuppressive
Immunosuppresive infection)
Avoid Cold
jslum.com | Medicine
Warm AutoImmune Haemolytic Anaemia Cold AutoImmune Haemolytic Anaemia
Definition
Acquired form of Haemolytic Anaemia caused by Interaction of drugs with
Immune System
Result is Production of Antibodies against RBC & Premature RBC destruction
= Immune Haemolytic Anaemia 2° to Drugs
= Anaemia – Immune Haemolytic - 2° to Drugs
Rare in Children
Etiology
Drugs
Cephalosproins (antibiotic)
Levodopa
Methyldopa (IgG mediated type II Hypersensitivity)
Penicillin & its derivatives (↑ dose)
Quinidine (IgM mediated activation of Classical Complement pathway & MAC)
NSAIDs
G6PD Deficiency – Breakdown of RBC d ue to stress in cell
(not immune system)(rare)
Mechanisms
Antibody directed Deposition of True autoimmune
against a drug-red cell complement via drug- haemolytic anaemia
membrane comples protein (antigen) (role of drug unclear)
Antibody complex onto
red cell surface
Penicillin Quinidine Methyldopa (+ve
Ampicillin Rifampicin Coombs w/o he molysis)
Clinical
Dark Urine
Fatigue
Pale Skin Colour
Tachycardia
Shortness of Breath
Yellow skin colour (Jaundice)
Splenomegaly
Investigations
DAT/ Coombs Test +ve (usually C3b)
Indirect Coombs +ve (if offen ding drug is added to test)
Indirect Bilirubin ↑
Serum Haptoglobin ↓
Hb present in Urine
Haemosiderin present in Urine
Urine Urobilinogen ↑
Absolute Reticulocyte count ↑
FBC shows Red Blood Cell Count & Hb ↓
Treatment
Cessation of Drug
Prednisone, Corticosteroids (↓ Immune respon se against RBC)
Folic acid supplementation (Treat Folic Acid Deficiency anaemia)
Blood Transfusions (Severe Symptomatic Anaemia)