Haematology Flashcards
Hypersensitivity Reactions
Type 1( Allergic Reactions)
-Hay Fever
-Eczema
-Contact Dermatitis
-Asthma
-Anaphylaxis
Type 2( Antibody-dependent cytotoxic reactions)
-Drug-induced hemolytic anemia
- Acute transfusion reactions
- Goodpasture syndrome
Hypersensitivity Reactions
Type 3( Immune complex-mediated reactions)
-SLE
- Poststreptococcal glomerulonephritis
Type 4 ( Cell mediated delayed reactions)
-Contact Dermatitis
-BCG immunization
Von Willebrand Disease
- Deficiency of Von Willebrand factor.
-3 types.
-vWF is a protein made in endothelial cells and megakaryocytes.
-Nose bleeding, Menorrhagia, Joint bleeding, Recurrent GI bleeding.
-Rx is VWF, Factor 8 and Desmopressin (DDAVP).
Aplastic crisis in Heredatry spherocytisis or sickle cell Anemia
Parvovirus B19
CLL
Administering intravenous immunoglobulin (IVIg) therapy is recommended for patients with chronic lymphocytic leukaemia (CLL) who have severe hypogammaglobulinemia and recurrent bacterial infections despite prophylactic antibiotics.
Primary polycythemia Ix
Molecular studies for JAK2, MPL and CALR mutation.
Aplastic Crisis
Aplastic crisis in sickle cell anaemia (SSA) is caused by infection with the parvovirus B19.
Coagulopathy
To correct a coagulopathy you need to aim for:
Fibrinogen >1.0 g/L
Platelets >50 ×109/L
PT and APTT <1.5 upper range of normal
CLL
Immunophenotyping of white Cells
Hemochromatosis
- Autosomal Recessive
- It is caused by the inheritance of mutations in the HFE gene on both copies of chromosome 6.
SCREENING
- Transferrin saturation and Ferritin
- Genetic testing for HFE mutation
- transferrin saturation > 55% in men or > 50% in women
- raised ferritin (e.g. > 500 ug/l) and iron
- low TIBC
Haptoglobin
Low haptoglobin levels are found in haemolytic anaemias
Intravascular and Extravascular Haemolysis
Intravascular haemolysis: causes
- mismatched blood transfusion
- G6PD deficiency*
- red cell fragmentation: heart valves, TTP, DIC, HUS
- Paroxysmal nocturnal haemoglobinuria
- Cold autoimmune haemolytic anaemia
Extravascular haemolysis: causes
- haemoglobinopathies: sickle cell, thalassaemia
- hereditary spherocytosis
- Haemolytic disease of newborn
- Warm autoimmune haemolytic anaemia
Difference between Leukaemoid reaction and CML
Leukaemoid reaction
- high leucocyte alkaline phosphatase score
- toxic granulation (Dohle bodies) in the white cells
- ‘left shift’ of neutrophils i.e. three or fewer segments of the nucleus
Chronic myeloid leukaemia
low leucocyte alkaline phosphatase score
TTP
in TTP there is a deficiency of ADAMTS13 (a metalloprotease enzyme) which breakdowns (‘cleaves’) large multimers of von Willebrand’s factor.
TTP presents with a pentad of fever, neuro signs, thrombocytopenia, haemolytic anaemia and renal failure
Polycythemia Rubra Vera
Polycythaemia rubra vera - JAK2 mutation
