Introduction to Haemolytic Anaemia Flashcards
What is Haemolytic Anaemia
Anaemia due to an increase in red cell destruction with no impairment of BM function
True or false, HA is associated with the inability of the marrow to compensate for the increased red cell breakdown i.e. There is marrow failure.
False
By how much can normal marrow increase in production rate
6-8 N
What are the two sites of Haemolysis
Intravascular
Extravascular
True or false, Most HA are extravascular, i.e in the spleen
True
What is an exception of HA being acquired intrinsic
Paroxysmal nocturnal haemoglobinuria
True or false, most intrinsic HA are congenital and most extrinsic ones are acquired
True
What classification of HA is most useful clinically
Based on inheritance
What are the two types of Membranopathies
Hereditary Spherocytosis
Hereditary Elliptocytosis
What are the different types of Enzymopathies
Glycose 6- Phosphate dehydrogenase deficiency
Pyruvate kinase deficiency
What are the different classifications of inherited/congenital HA
Membranopathies
Enzymopathies
Haemoglobinopathies
What are he different types of Haemoglobinopathies
Sickle cell disease
Thalassemia
What are the different types of Acquired HA
Immune
Non-Immune
What are the different immune acquired HA
Autoimmune HA- idiopathic, secondary, drug induced
Incompatible blood transfusion
Haemolytic disease of the new born
What are the different Non immune Acquired HA
MAHA (Microangiopathic haemolytic Anaemia) Infections (malaria) Chemicals/ drugs/ venom Physical agents (thermal injury) PNH
What are the clinical features of congenital HA
Anaemia
Jaundice
Splenomegaly
Gall stones
What are the different crisis in congenital HA
Aplastic , Haemolytic
What disease is associated with Aplastic crisis
Parovirus B19 infection
What is the MOA of parovirus B19 infection
Faeco- oral, oral-oral, respiratory
Parovirus B19 infection decreases which two blood cells components
Haemoglobin, Reticulocyte
How long does Parovirus B19 infection last
6-8 days
What occurs in haemolytic crisis
Megaloblastic crisis Splenomegaly Cholelithiasis Leg ulcers Skeletal abnormalities
What deficiency causes megaloblastic crisis
Folate
In cholelithiasis, is can be due to supersaturation of the bile with
Calcium bilirubinate
What is stored in cholelithiasis
Black pigment
What is expanded in skeletal abnormalities of congenital HA haemolytic crisis
Erythroid bone marrow
What are some clinical features of acquired HA
Acute febrile illness
Pallor and other features of Anaemia
Jaundice
What laboratory feature of Haem catabolism is identified
Unconjugated hyperbilirubinaemia
Increased urobilinogen
What substance is increased as a laboratory feature of haemolytic Anaemia
Serum lactate dehydrogenase
What substance is absent in the laboratory feature of haemolytic Anaemia
Haptoglobin
What are signs of IV haemolysis in laboratory features of haemolytic Anaemia
Haemoglobinaemia Haemoglobinuria Haemosiderinuria Methaemalbuminaemia Reduced serum haemopexin
What are the features of accelerated erythropoiesis
Reticulocytosis Macrocytosis NRBC in blood (nucleated red blood cells) Leucocytosis and thrombocytosis BM erythroid Hyperplasia
What are the different tests to find the cause of HA
RBC morphology
Antiglobulin test (Coombs test)
Osmotic fragility
What is associated with the prescence of: Spherocytes Elliptocytes Echinocytes Sickle cells Target cells Schistocytes Autoagglutination Heinz bodies
HS, AIHA HE PK def SCD Thal, HBC Disease MAHA Cold AIHA G6PD deficiency
What are the symptoms of the differential diagnosis Anaemia and reticulocytosis
Bleeding
Recovery from deficiency of iron/folate/B12
Recovery after marrow failure
What are the symptoms of the differential diagnosis anaemia and acholuric jaundice
Ineffective erythropoiesis
Blood loss into body cavities/tissue
What is treatments are used as a supportive measure
transfuse where appropriate
splenectomy (sometimes)
steroids (sometimes)
folate supplementation
What are the three differential diagnosis of HA
Anaemia + reticulocytosis
Anaemia + Acohuric Jaundice
Marrow Invasion