Haematology Flashcards
What causes sickle cell anaemia?
Point mutation in beta globin chain
How does multiple myeloma present?
hyperCalcaemia Renal Anaemia Bone pain - lytic lesions Hyperviscosity syndrome - spontaneous bleeding, neurological symptoms, visual disturbance
What proportion of BM must be plasma cells for MM diagnosis?
> 10%
What causes hereditary spherocytosis? How is it diagnosed? When do flares occur?
Mutation in structural red cell membrane protein
Spherocytes on blood film, increased osmotic fragility
Flares precipitated by acute illness
In lymph nodes what causes a response from
- B cells
- Phagocytes
- T cells
B cells - infection or autoimmune
Phagocytes - metastases
T cells - viral, drugs eg phenytoin
What is the triad of hypersplenism
Splenomegaly
Decrease in 2 or more blood compartments
Correction of cytopenia by splenic removal
What infections does splenectomy increase the risk of?
S pneumonia, H influenza, Neisseria meningitidis
Malaria
What is myelodysplastic syndrome? What can it progress to?
Acquired DNA mutation in progenitor cells –> stem cell malignancy resulting in ineffective haematopoiesis
Can progress to AML
Which mutations are found in PRV?
JAK2
Which mutations are found in essential thrombocytosis? How is it managed?
JAK2 (50%), CALR (10%)
1st line hydroxyurea
What is the precursor of a neutrophil?
Myelocyte
What is the normal BM myeloid:erythroid ratio? When can this change?
1.5:1
Reverses in haemolytic anaemia - need more RBCs
How is acute intermittent porphyria caused? What can it be precipitated by? How does it present?
Porphobilinogen deaminase enzyme deficiency
Alcohol
Intermittent abdo pain, nausea, vomiting
What is the Kleihauer test?
Detects HbF in maternal blood in feto-maternal haemorrhage
What are effects of alcohol on the blood?
Macrocytosis
Thrombocytopenia
Low clotting factors
What can B12 deficiency cause?
Subacute degeneration of the spinal cord
How long does reticulocytosis take to occur after haemorrhage?
1-2 days
What is the management of autoimmune haemolytic anaemia?
Steroids and folic acid
What is the management of neutropenic sepsis?
Piperacillin/tazobactam
Add Gent if NEWS>/5
How to Protein C and S work?
Bind to Va and VIIa to switch off clotting
What does prostacyclin do?
Inhibits platelet activation
What does plasmin do?
Breaks down fibrin