Haem hard to recall Flashcards
What is the most important cell in the initiation of normal haemostasis?
Endothelial cell
What is the main component involved in stabilising the primary haemostatic plug?
fibrin
Which protease assists in the break down of blood clots by binding to the clot and localising agents which break it down?
Tissue plasminogen-activator (t-PA)
Name a potent inhibitor of plasmin in the blood
a2 macroglobulin
Which product of the cyclic endoperoxides induces platelet aggregation?
Thromboxane A2
What does an increase in both APTT and PT suggest?
Vit K deficiency
Which inherited disease has a raised APTT and bleeding time but a normal PT?
Von Willebrand deficiency
What would you expect to find on blood film of ITP?
Megakaryocytes
Which protein, important in haemostasis, is vitamin K dependent but is not a serine protease?
Protein S
What synthesises tissue factor, vWF, prostacyclin, plasminogen activator, antithrombin III and thrombomodulin?
Vascular endothelium
Which key clotting factor activates both factors V and VIII, and also activates protein C?
Thrombin
Symptoms of osler-webu-rendu syndrome? AKA hereditary haemorrhagic telangiectasia
Recurrent nose bleeds
Anaemia symptoms
Menorrhagia
What would a purple rash on bottom and lower legs be pathognomonic of?
What are some other symptoms associated with it
Henoch Schonlein Purpura
Abdo pain
Joint pain
What drug potentiates antithrombin III. Usually given subcutaneously. Can cause osteoporosis and hyperkalaemia?
Dalteparin (LMWH)
What type of heparin is reversible?
Unfractionated
What is used to monitor patients undergoing warfarin therapy?
PT
What is used to monitor patients undergoing unfractionated heparin therapy?
APTT
What Reflects the amount and activity of fibrinogen?
Thrombin time
In patients with cancer and acute venous thromboembolism, the most effective drug at reducing the risk of recurrent VTE is?
LMWH dalteparin
Which anticoagulant drug most likely to cause thrombocytopaenia with paradoxical thrombosis?
Unfractionated heparin
What drug is given as thrombotic prophylaxis in DIC?
LMWH dalteparin
BM features of Chronic Myelomonocytic Anaemia?
Ring sideroblasts
High monocyte count
Auer rods
What does Refractory Anaemia with excess Blasts in Transformation predispose you to?
AML
Bone marrow findings of 5q syndrome?
Hyperplasia of hypolobulated micromegakaryocytes
What treatment does 5q syndrome respond well to?
Lenalidomide
Blood film features of Refractory Anaemia with excess Blasts?
Pelger-Huet neutrophils
Blast cells
What would you expect to see on blood film of someone with myelofibrosis?
Tear drop poikilocytes
What is imatinib used to treat?
BCR-ABL positive CML
What do auer rods indicate?
AML
Describe Richter’s transformation
CLL converts to diffuse large B cell lymphoma
What cancer is bcr-abl involved in?
CML
Patients with which inherited disorder have an increased risk of developing acute leukaemia?
Down’s
What is the commonest adult leukaemia?
CLL
Why might CML present with a painful toe?
Increased mass of turning-over cells generates urate
Dykeratosis congenita triad?
Nail dystrophy
Leukoplakia
Cutaneous manifestations e.g. areas of hyperpigmentation
What test is used for paroxysmal nocturnal haemoglobinuria?
Ham’s test (positive)
A 19 year old Jewish male presenting with multiple pathological fractures and hypersplenism. What condition do they have?
Gaucher’s disease
In which condition might a Donath-Landsteiner be positive?
Paroxysmal cold haemoglobinuria
Dapsone is used to treat dermatitis herpetiformis. Dapsone is known to cause haemolysis in which patients?
G6PD deficient patients
What characterises delayed haemolytic transfusion reaction (DHTR)?
Manifests days to weeks after transfusion
Falling Hb
Jaundice
Haemoglobinuria
Infection with which virus may result in a false positive Paul Bunnell test?
parvovirus B19
What infection are patients with sickle cell disease at risk of causing aplastic crisis in?
Bone marrow findings?
parvovirus B19
Red cell aplasia
What is a cause of anaphylaxis to blood transfusion?
IgA deficiency
What is Found in patients with Myeloma with low serum albumin and oedema?
Amyloid
Which monoclonal immunoglobulin appears as a dense narrow band (M band) on electrophoresis?
Paraprotein
What is stimulated by IL-6 secreted by cells in the vicinity of the myeloma cells. Produces radiological changes resembling those of generalized osteoporosis?
Osteoclasts
Misfolded protein deposited in myeloma-associated amyloidosis?
AL amyloid
A condition involving a low level of paraprotein in the blood in usually asymptomatic elderly patients?
MGUS
Induction chemotherapy regimen with the best evidence pre autologous stem cell transplant in multiple myeloma
Lenalidomide + low dose dexamethasone
Monoclonal immunoglobulin raised in macroglobinaemia?
IgM
Factor(s) associated with a very poor prognosis in multiple myeloma?
Anaemia & renal failure
Proteasome inhibitor drug which is active in myeloma?
Bortezomib
Tx regimen with the best evidence in multiple myeloma if not suitable for autologous SCT
Melphalan + Pred + Thalidomide = first line
3 diagnostic criteria for MM?
- Presence of a serum or urine monoclonal protein. No minimum value.
- Presence of clonal plasma cells in bone marrow >10%
- Presence of end organ damage due to the plasma cell dyscrasia: CRAB
Note - second it’s symptomatic it’s myeloma
2 diagnostic criteria for smouldering myeloma?
- Serum monoclonal protein >=30g/L and/or marrow plasma cells >=10%
- AND No CRAB
3 diagnostic criteria for MGUS?
- Serum monoclonal protein present but <30g/L
- Marrow plasma cells <10%
- AND No CRAB
Which paraprotein is most commonly raised in patients with multiple myeloma?
IgG
Which cytokine is an important growth factor in the development of myeloma?
IL-6
Elevated levels of what imply a poor prognosis in myeloma patients?
Beta2 microglobulin
What is Waldenstrom’s macroglobulinaemia?
What do the cells secrete and what is their appearance?
Who is it seen in?
Median survival?
Low grade non-Hodgkin’s lymphoma
Lymphoplasmacytoid in appearance and secrete IgM
Elderly men
3-5 years
A 43 year old man presents to his GP with concerns over a tender lump on the left side of his abdomen. Investigation showed a normal haemoglobin but a mildly raised white-cell count (13.2 x 109/l). On the blood film these cells were mainly small mononuclear cells resembling lymphocytes. These cells stained positively for tartrate-resistant acid phosphatase.
Hairy Cell Leukaemia
Best Tx for corticosteroid refractory haemolytic anaemia?
Splenectomy
Best Tx for CLL?
Cyclophosphamide, fludarabine and rituximab
Best Tx for CML?
Imatinib
What operation are DiGeorge children ineligible for?
Thymectomy
What drug is used in solid organ transplantation, multiple sclerosis, NHL. Depletes lymphocytes? Side effects include ITP and Graves disease.
Campath (anti CD52, alemtuzumab)
Anti-proliferative used in polycythaemia rubra vera and essential thrombocythaemia?
Hydroxyurea
What drug Reduces platelet count in thrombocythaemia but causes palpitations, tachycardia, fluid retention?
Anagrelide
Which Rescue drug after high dose methotrexate is used for gestational trophoblastic disease or intrathecal ALL?
Leucovorin (Folinic acid, Formyl tetrahydrofolate)
Advanced Hodgkins disease should be treated with?
Combination chemo
Pathological stage IA or IIA Hodgkins disease may be treated with?
ABVD combination chemotherapy + radiotherapy if required
A classical but rare constitutional symptom of Hodgkin lymphoma?
Pel-Ebstein fever
An 81 year old man with known prostatic carcinoma presents to his GP with severe bone pain. Blood tests reveal a mild anaemia and peripheral blood film shows nucleated red blood cells and immature myeloid cells. What are these haematological features collectively known as?
Leucoerythroblastic anaemia
A 21 year old student recently returning from India complains to his GP of cough, headache and diarrhoea. He is febrile and rose spots are present on his chest. Blood culture reveals salmonella typhi. Which immune cells out of the list are most likely to be raised?
Monocytes
Anaemia and neutropenia are more common in which of the above autoimmune disease?
SLE
What infection could cause a Monocytosis but with a normal neutrophil count?
Brucella infection