Haematology Flashcards
Which anticoagulants do the following reverse:
- Idarucizumab
- Andexanet alfa
- Protamine sulphate
- Vitamin K
- Idarucizumab - dabigatran
- Andexanet alfa - rivaroxaban, apixaban
- Protamine sulphate - heparin, enoxaparin or dalteparin
- Vitamin K - warfarin
What is the treatment of acute intermittent porphyria?
IV haematin/haem arginate
IV glucose should be used if haematin/haem arginate is not immediately available
What is the presentation of acute intermittent porphyria?
Combination of abdominal, neurological and psychiatric symptoms:
* abdominal: abdominal pain, vomiting
* neurological: motor neuropathy
* psychiatric: e.g. depression
* hypertension and tachycardia common
Which sickle cell crises exist?
Thrombotic crises
Acute chest syndrome
Aplastic crises
Sequestration crises
Infection
What are features of thrombotic crisis in sickle cell disease?
- also known as painful crises or vaso-occlusive crises
- precipitated by infection, dehydration, deoxygenation (e.g. high altitude)
- painful vaso-occlusive crises should be diagnosed clinically - there isn’t one test that can confirm them although tests may be done to exclude other complications
- infarcts occur in various organs including the bones (e.g. avascular necrosis of hip, hand-foot syndrome in children, lungs, spleen and brain
What are features of acute chest syndrome in sickle cell crisis?
- vaso-occlusion within the pulmonary microvasculature → infarction in the lung parenchyma
- dyspnoea, chest pain, pulmonary infiltrates on chest x-ray, low pO2
- management: pain relief, respiratory support e.g. oxygen therapy, abx
What are features of aplastic crisis in sickle cell crisis?
- caused by infection with parvovirus
- sudden fall in haemoglobin
- bone marrow suppression causes a reduced reticulocyte count
What are features of sequestration crisis in sickle cell crisis?
- sickling within organs such as the spleen or lungs causes pooling of blood with worsening of the anaemia
- associated with an increased reticulocyte count
What is the empirical abx therapy preferred in neutropenic sepsis?
Piperacillin with tazobactam (Tazocin)
What leukaemia can myelodysplastic syndromes progress into?
Acute myeloid leukaemia
What are the different types of von Willebrand’s disease?
Type 1: low levels of VWF
Type 2: deficiency in function of VWF compared to level (mutations causing poor function)
Type 3: Absent VWF - can present like haemophilia
What can CLL transform to?
CLL can undergo Ritcher’s transformation into diffuse large B-cell lymphoma (DLBCL) which is a subtype of non-Hodgkin’s lymphoma
What is the mechanism of thrombotic events in factor V Leiden?
Activated factor V is inactivated 10 times more slowly by activated protein C than normal
What are haptoglobins and how are its levels affected in haemolysis?
Haptoglobin is produced in the liver, and its primary function is to bind to free haemoglobin (from damaged or lysed cells)
In haemolysis, you would see low haptoglobin since since the bound ones would then be excreted
What is the Hb threshold for blood transfusions?
<80g/L for patients with acute coronary syndrome
<70g/L for any other patient
