Haematology Flashcards
(34 cards)
In which diseases is serum or urine protein electrophoresis indicated?
- Multiple myeloma
- Plasma cell leukaemia
- B-cell lymphoma (Waldenström’s macroglobulinaemia)
- Plasmacytoma
- Amyloidosis
What is the mnemonic CRABBI and what disease is it used for?
Multiple myeloma
C = calcium: hypercalcaemia = constipation, nausea, anorexia, confusion
R = renal: light chain deposits within renal tubules = renal impairment = dehydration and increased thirst
A = anaemia
B = bleeding: thrombocytopaenia = increased risk of bleeding and bruising
B = bones: increased osteoclast activity creates bone lesions = bone pain (especially in the back) and increased risk of fractures
I = infection: reduced immune system due to reduction in production of normal immunoglobulins
What is the first-line treatment for transplant candidates in newly diagnosed multiple myeloma?
Induction therapy = chemo and steroids e.g. thalidomide/lenalidomide and dexamethasone
What is the first-line treatment for patients with newly diagnosed multiple myeloma that are not transplant candidates?
Chemotherapy and steroids e.g. melphalan and thalidomide and prednisolone
What is antiphospholipid syndrome?
The association of persistently elevated antiphospholipid antibodies with a variety of clinical features characterised by thromboses and pregnancy-related morbidity
What condition is strongly associated with antiphospholipid syndrome?
SLE
What is the most common inherited thrombophilia?
Factor V Leiden
What is the pathophysiology behind Factor V Leiden?
There is a gain-of-function mutation in the Factor V Leiden protein which causes it to be inactivated 10 times slower by activated protein C than normal increasing the risk of thrombosis
What is the cause of haemophilia A?
A deficiency of clotting factor VIII
What is the cause of haemophilia B?
A deficiency of clotting factor IX
Why is haemophilia almost exclusively male in epidemiology?
Because it is inherited with an X-linked recessive pattern
What is the hallmark sign of haemophilia?
Musculoskeletal bleeding
What does musculoskeletal bleeding present as?
- Pain
- Swelling
- Decreased range of motion
- Erythema
- Increased local warmth
What is immune thrombocytopaenia purpura?
An autoimmune haematological disorder characterised by isolated thrombocytopaenia in the absence of an identifiable cause
What investigation is used to confirm immune thrombocytopaenia purpura?
Peripheral blood smear
What platelet count is seen in patients with immune thrombocytopaenia purpura?
< 100 x 10^9 /L
What do you give a patient with immune thrombocytopaenia purpura with a platelet count <30 x 10^9 /L and/or bleeding symptoms?
- Corticosteroid
- IV human immunoglobulin
- Anti-D (Rh-+ve)
What is the most common inherited bleeding disorder?
Von Willebrand disease
What is haemolytic anaemia?
The umbrella term for a number of conditions that cause premature destruction of red blood cells.
What are the common signs and symptoms of haemolytic anaemia?
- Pallor
- Fatigue
- Jaundice
- Dyspnoea
- Splenomegaly
What is ‘warm’ autoimmune haemolytic anaemia?
Haemolysis of RBCs caused by IgG which happens best at room temperature and at extravascular sites such as the spleen
What is ‘cold’ autoimmune haemolytic anaemia?
Haemolysis of RBCs caused by IgM which happens best at 4 degrees C. Haemolysis is mediated by complement and happens intravascularly (Raynaud’s)
The presence of what in a peripheral blood smear indicates haemolytic anaemia?
- Blister or bite cells
- Heinz bodies (denatured haemoglobin)
Increased incidence of what condition is linked to glucose-6-phosphate dehydrogenase deficiency?
Cataracts
