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51 Cards in this Set
- Front
- Back
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Which monoclonal proteins are most commonly overproduced in multiple myeloma? |
IgG (70%) and IgA (20%) |
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Which medication may be helpful for treating sideroblastic anaemia? |
Pyridoxine (vitamin B6) |
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What is sideroblastic anaemia? |
Anaemia secondary to impaired production of haem at a mitochondrial level, causing iron to deposit around the nucleus like a ring |
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Which conditions are most commonly associated with thymoma? |
Myasthenia gravis Red cell aplasia Dermatomyositis SLE |
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What causes paroxysmal nocturnal haemoglobinuria? |
Complement-mediated intravascular haemolysis due to lack of membrane surface proteins that stabilise complement |
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Is PNH acquired or congenital? |
Acquired |
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How does Factor V Leiden cause thrombophilia? |
Activated protein C resistance (activated factor V Leiden is inactivated 10 times more slowly than normal) |
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What are the expected flow cytometry findings in PNH? |
CD55 and CD59 negative cells |
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How does heparin work? |
Binds to and enhances the effects of antithrombin III, which inhibits the formation of thrombin and other clotting factors |
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What is the underlying pathology in hereditary spherocytosis? |
Erythrocyte cell membrane cytoskeleton defect with spherocyte destruction in the spleen. |
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What is the significance of reduced/absent TPMT activity? |
Haematological toxicity with thiopurine drugs (e.g. azathioprine) |
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Which test must be performed before commencing azathioprine? |
TPMT activity |
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When and where is hepcidin produced? |
Produced by the liver in response to elevated body iron levels |
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What is the action of hepcidin? |
Decreases serum iron levels by increasing iron storage in enterocytes |
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Hepcidin deficiency occurs in which condition? |
Haemochromotosis |
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What is tissue factor? |
Cell surface glycoprotein that activates Factor VII (Factor VIIa converts prothrombin to thrombin) |
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What is tissue factor? |
Cell surface glycoprotein that activates Factor VII (Factor VIIa converts prothrombin to thrombin) |
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Which cells produce tissue factor? |
Subendothelial fibroblasts, smooth muscle cells and leukocytes |
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What is tissue factor? |
Cell surface glycoprotein that activates Factor VII (Factor VIIa converts prothrombin to thrombin) |
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Which cells produce tissue factor? |
Subendothelial fibroblasts, smooth muscle cells and leukocytes |
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What is tissue factor also known as? |
Coagulation factor III |
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How does the composition of microthrombi differ between DIC and HUS/TTP? |
DIC clots contain fibrin and platelets, other TMAs have predominantly platelet-rich clots without coagulopathy |
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What is the most significant adverse effect of dasatinib? |
Pleural effusions / pulmonary toxicity |
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Which TKI used to treat CML carries an increased risk of vascular events? |
Nilotinib |
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What is the genetic mutation seen in CML? |
Philadelphia chromosome t(9;22) forming the BCR-ABL fusion gene |
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Antibodies to which protease are implicated in the pathogenesis of TTP? |
ADAMTS 13 (results in reduction of vWF cleavage) |
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Which test is elevated in intravascular haemolysis? |
Urinary haemosiderin |
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What is the universal red cell donor? |
O- |
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What is the universal plasma donor? |
AB |
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What is the relationship between iron deficiency and hepcidin? |
Inversely proportional (iron deficiency causes high hepcidin and vice versa) |
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How does hepcidin interact with ferroportin? |
Binds to ferroportin and causes its internalisation, resulting in less iron release into blood |
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What is the role of ferroportin? |
Located on the cell membrane of enterocytes to transport iron into the blood |
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Patients with bone marrow failure syndromes are at risk of which malignancy? |
AML |
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What is the key pathology in Fanconi anaemia? |
Defect in DNA repair causing chromosomal fragility |
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How is aplastic anaemia treated? |
Immunosuppression or bone marrow allograft |
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Which autosomal dominant gene mutation predisposes to haematological malignancy? |
GATA2 |
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What is a leukoerythroblastic blood picture? |
Left shift (immature leukocytes) with nucleated immature erythrocytes |
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What is the pathology of aplastic anaemia? |
Immune-mediated destruction of haematopoetic stem cells |
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What is the pathology of dyskeratosis congenita? |
Telomeropathy |
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Which diagnosis should be considered in a 30 year-old patient with anaemia and head/neck cancer? |
Fanconi anaemia |
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What is the clinical triad of dyskeratosis congenita? |
Abnormal skin pigmentation Nail dystrophy Oral leucoplakia |
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Name 3 inherited bone marrow failure syndromes. |
Fanconi anaemia Dyskeratosis congenita Diamond Blackfan anaemia Schwachnan Diamond anaemia Severe congenital neutropaenia |
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What can be used to rapidly reverse rivaroxaban and apixaban? |
Andexanet alpha (recombinant factor Xa that binds to and 'mops up' anti-Xa molecules) |
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What can be used to rapidly reverse dabigatran? |
Idarucizumab |
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What is phytomenadione also known as? |
Vitamin K |
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What can be used to rapidly reverse enoxaparin? |
Protamine reverses 65-70% of effect |
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What can be used to reverse unfractionated heparin? |
Protamine |
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What causes the O2 dissociation curve to shift to the right? |
High temperature Low pH / high CO2 / high H+ High 2,3 DPG (pregnancy, anaemia) |
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What causes the O2 dissociation curve to shift to the left? |
Low temperature High pH / low CO2 / low H+ Low 2,3, DPG Carbon monoxide Methaemoglobin HbF |
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What is the significance of a right shift of the O2 dissociation curve? |
Reduced Hb affinity for oxygen (causing increased offloading to tissues) |
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What is the significance of a left shift of the O2 association curve? |
Increased Hb affinity for oxygen (and less delivery to tissues, causing tissue hypoxia) |
