Haematology Flashcards
What is aplastic anaemia?
Characterised by diminished haematopoietic precursors in bone marrow and a deficiency of all blood cell elements (Pancytopenia)
What are the causes of aplastic anaemia?
Idiopathic (40%)
Drugs:
GCS ma nigga
Gold, Chloramphenicol, Sulphonamides, Methotrexate, Nifedipine
Radiation
Viruses:
Parvovirus, HIV, EBV, Hep B and C
What are the symptoms and signs of aplastic anaemia?
Anaemia:
Lethargy, Tiredness, Dyspnoea
Pallor
Thrombocytopenia:
Easy bruising, Epistaxis, Bleeding gums
Petechiae
Leukopenia:
Recurrent Infections (severe)
Signs of infection
What are the investigations for aplastic anaemia?
Bloods:
FBC - Normocytic anaemia, thrombocytopenia, leukopenia, low or absent reticulocytes
Bone marrow biopsy:
Hypocellular marrow
Increased fat/adipose cells
What is the management of aplastic anaemia?
Stop any causative drugs
Supportive:
Blood/platelet transfusions
Antibiotics for infections
Haematopoietic stem cell transplantation
What are the differential diagnoses for aplastic anaemia?
Myeolodysplastic syndrome
Paroxysmal nocturnal haemoglobinuria
ALL/AML
(Other causes of pancytopenia eg sepsis and infection)
What is haemolytic anaemia?
Characterised by premature destruction of red blood cells causing a shortened life span
(Less than 120 days) and anaemia.
Haemolytic anaemia in general occurs due to a problem with the RBCs, which causes them to haemolyse and be removed from the circulation quicker than normal. This problem with the RBCs can be:
- Intrinsic (Defect within RBCs)
- Extrinsic (Something effects RBCs from outside)
What are the intrinsic causes of haemolytic anaemia?
3 main intrinsic causes - all end in opathies
- Haemoglobinopathies
• Sickle cell anaemia
• Thalassemia
Misformed haemoglobin causes premature destruction - Membranopathies
• Hereditary spherocytosis (spherical RBCs)
• Hereditary eliptocytosis
Membrane of RBC is malformed which prevents the normal biconcave shape to form - Resultant RBCs more prone to haemolysis - Enzymopathies
• G6PD deficiency
• Pyruvate kinase deficiencies
What are the extrinsic causes of haemolytic anaemia?
With extrinsic causes, the RBCs are normal and fully functional, but something extrinsic causes damage to them and hence their premature destruction.
- Autoimmune haemolytic anaemia
• Immune system responds to antigens on the surface of the red blood cells —> Premature destruction.
Can be warm or cold
Warm = Usually associated with lymphoma/CLL or autoimmune conditions (SLE, RA, IBD)
Cold = Can be caused by infection - Mycoplasma, EBV, syphilis
• Precipitated by reduced body temperature.
- Both are Coombs test positive
- Microangiopathic haemolytic anaemia
- Abnormal clotting occurs in small blood vessels of body. As red blood cells come through, they hit along these clots and they shear and break up. This forms fragments of cells called schistocytes (Triangular)
• Clotting can be caused by TTP or DIC - Infection
• Malaria, Sepsis. - Isoimmune
• Blood transfusion
• Haemolytic Disease of newborn (Rhesus) - Paroxysmal nocturnal haemoglobinuria
- Drugs eg penicillin, quinine
What are the symptoms and signs of haemolytic anaemia?
Fatigue, Tiredness, Dyspnoea
Jaundice, Icterus, Pruritus
Constitutional symptoms (Fever, Malaise)
Splenomegaly
Pallor
Gallstones
What are the investigations for haemolytic anaemia?
Bloods
FBC:
- Low Hb
- Normal MCV
- Decreased haptoglobin
- Increased LDH
- Increased reticulocytes
- Increased bilirubin
Urinalysis - May show haemoglobinuria
Direct Coombs test - identifies RBCs coated with antibodies using antihuman globulin.
• Cold and warm autoimmune haemolytic anaemia
• Drug induced haemolytic anaemia
Osmotic fragility test - To identify membrane abnormalities
Acidified serum lysis (Ham test) - Lysis of RBCs in acidified serum in PNH
Bone marrow biopsy - Erythroid hyperplasia
How do you manage autoimmune haemolytic anaemia?
Warm AHA
Folate supplementation
Steroids - Prednisolone
Splenectomy
Cold AHA
Avoid cold exposure
Folate supplementation
Rituximab
How do you manage Hereditary spherocytosis?
Folate replacement
Splenectomy
What is a macrocytic anaemia?
Anaemia associated with a high MCV of RBCs (>100fl)
What are the causes of macrocytic anaemia?
Megaloblastic:
Vitamin B12 deficiency
• Decreased absorption (Gastrectomy, pernicious anaemia, terminal ileal resection)
• Decreased intake (Vegans)
Folate deficiency
• Alcoholics, malnourished
Non megaloblastic:
- Alcohol
- Liver Disease
- Hypothyroidism
- Pregnancy
- Myelodysplasia
- Reticulocytosis