Haematology Flashcards
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
What are the key histopathological features of hereditary spherocytosis?
Presence of spherocytes on blood smear in association with negative direct anti-globulin test and an elevated reticulocyte count
What is hereditary spherocytosis?
An inherited abnormality of the red blood cells caused by defects in structural membrane proteins. The normal biconcave disc shape is replaced by a sphere-shaped RBC
What are the key diagnostic factors for hereditary spherocytosis?
- Pallor
- Jaundice
- Splenomegaly
- Fatigue
What are the key differences between hereditary spherocytosis and glucose-6-phosphate dehydrogenase deficiency?
G6PD deficiency = male, African and Mediterranean ethnicities, Heinz bodies + blister cells on film
Hereditary spherocytosis = male and female, Northern European ethnicities, splenomegaly, spherocytes on film
What is the cause of sickle cell anaemia?
An autosomal recessive single gene defect in the beta chain of haemoglobin
What is used as prophylaxis of sickle cell? How does it work?
Hydroxyurea
Increases HbF levels
What is thalassemia?
An inherited microcytic anaemia caused by mutation(s) or the alpha- or beta-globulin gene
What is a typical carboxyhaemoglobin level for smokers?
5-10%
What carboxyhaemoglobin level indicates carbon monoxide poisoning?
Elevated above patient baseline
> 50% = acute
What does a positive anticardiolipin antibody test indicate?
Antiphospholipid syndrome
