Formative Flashcards
Does “ uncontrolled production of essentially normally functioning blood cells” describe secondary polycythaemia or polycythaemia rubra vera
Polycythaemia rubra vera
- in secondary this is controlled by high epo driven by the secondary cause eg hypoxia
What is a Clonal B cell disorder usually resulting in a large number of circulating malignant cells?
chronic lymphocytic leukaemia: excess of small mature lymphocytes in the blood
- acute leukaemia = block of differentiation
- chronic leukaemia = failure of cell death
Mechanism of sickle cell disease
point mutation in the beta chain (of globin gene) and this predisposes the haemoglobin to polymerisation resulting in sickled cells and reduced red cell survival.
Mechanism of myelodysplasia
Acquired DNA mutations in haematopoietic stem cells
Which drug
a) is an ADP agonist?
b) irreversibly inactivates cyclooxygenase 1?
c) is a highly selective direct inhibitor of activated factor X?
a) clopidogrel
b) aspirin
c) rivaroxaban
Haemoglobin analysis identifies the presence of raised Hb A2.
beta thalassaemia trait
Family history of haemolytic disorder, normocytic anaemia Blood film shows polychromasia and red cells with loss of central pallor.
hereditary spherocytosis
Causes of hereditary haemophilia
Factor V Leiden, antithrombin deficiency, protein C deficiency, protein S deficiency
What protein does rituximab target?
Rituximab is a humanised monoclonal antibody directed against CD20, expressed on B cells and B cell lymphomas.
What protein does imatinib target?
Imatinib is a tyrosine kinase inhibitor that is inhibits BCR-ABL-1 protein unique to chronic myeloid leukaemia.
48 year old man becoming breathlessness two hours after an infusion of fresh frozen plasma.
transfusion related acute lung injury: caused by anti- leucocyte antibodies present in the donation that bind to the patients white cells and cause acute lung injury by degranulation of the affected neutrophils in the lungs. Pulmonary infiltrates are seen on CXR.
Investigate
a) haemoglobinopathy
b) raised haemoglobin w/o history of secondary polycythaemia
c) acute leukaemia
a) Haemoglobin analysis by high performance liquid chromatography (HPLC)
b) JAK2 gene mutation analysis
c) immunophenotyping
Blood film showing macroovalocytes and hypersegmented neutrophils is indicative of what
b12/folate deficiency
pancytopenia + b12/folate deficiency
pernicious anaemia
The blood film shows an excess of platelets with some giant forms.
essential thrombocythaemia
What is vitamin K responsible for?
bile salt absorption and carboxylation of clotting factors
Is vitamin K water-soluble?
no
In which condition would you see prolonged prothrombin time, prolonged partial thromboplastin time, low platelet count, low fibrinogen.
DIC
Treat autoimmune haemolytic anaemia
steroid (1mg/kg of prednisolone per day) and folic acid. Steroids suppress the autoimmune process and folic acid supplements prevent deficiency which can occur when the red cell turnover is high (evidenced by the reticulocytosis).
A 21 year old man has 4 months of a gradually enlarging neck lump. His appetite is poor and he has lost 5 kg in weight recently. He has been experiencing night sweats.He has a single, non-tender, 3 cm mass in the left anterior triangle of the neck.
Which investigation is most likely to give a definitive diagnosis? A. Bone marrow aspirate B. CT scan of chest, abdomen and pelvis C. Epstein–Barr virus serology D. Excision biopsy E. Peripheral blood smear
D. excision biopsy
this is hodgkins lymphoma
A 65 year old woman has increasing lethargy and a sore, red tongue. She takes levothyroxine sodium 75 micrograms once daily but no other medication.She has vitiligo and looks pale. Her GP suspects that she has anaemia and requests a blood count and blood film.
Which is the most likely abnormality on the blood film? A. Blast cells B. Macrocytosis C. Pancytopenia D. Reticulocytosis E. Spherocytosis
B. macrocytosis
–> this woman has B12 deficiency as indicated by lethargy, anaemia, glossitis possiby due to pernicious anaemia which would have autoimmune association..
A child with parvovirus develop jaundice and anaemia, what is the diagnosis?
hereditary spherocytosis
