HAEMATOLOGY FORMATIVE ASSESSMENT

Question 1

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Answer 1

1. Polycythaemia rubra vera - uncontrolled proliferation is usually a malignancy (the Myeloproliferative disorders are technically haematological malignancies) - as the cells are normally functioning this is B - PRV 2. Acute myeloid leukaemia is a bone marrow based malignancy resulting in the production of immature cells in the blood-increased proliferation with block in differentation 3. CLL - excessive proliferation of dysfunctional B cells (in this the cells circulate, in Hodgkins-confined to nodes mostly

Question 2

What may be seen on blood film in acute myeloid leukaemia and in chronic lymphocytic leukaemia? What are the different age groups affected?

Answer 2

* In acute myeloid leukaemia -it usually occurs in patients >60 years old but can affect younger patients * Can see the presence of Auer rods on blood film * Abnormalities of granulation in blasts can be visualised in the form of Auer rods in AML * Chronic lymphocytic leukaemia mostly affects people over 60 and rare is patients under 40 years * Can often see smear cells on blood film

Question 3

What age groups does acute lymphoblastic leukaemia and chronic myeloid leukaemia affect?

Answer 3

Acute lymphoblastic leukaemia - occurs in children - commonest childhood leukaemia Chronic myeloid leukaemia - occurs usually in patients aged 40-60 - can occur in all ages technically

Question 4

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Answer 4

1. A - Sickle cell anaemia The detection of HbS indicates that the patient has sickle cell anaemia 2. G - Beta Thal Trait - Disproportionate reduction in MCV relative to the Hb reflects a haemoglobinopathy trait which accompanied by an increase in HbA2 (α2δ2) 3. C - Red cells that have lost central pallor are called spherocytes, and an excess of these in a patient with family history of haemolytic anaemia makes hereditary spherocytosis the likely diagnosis.

Question 5

1. A 28 year old woman attending the antenatal clinic has a blood count performed with the results as follows: Haemoglobin 82g/L, MCV 87 fl, white cell count 6.4 x 10 /l, neutrophil count 3.8 x 10 /l and platelet count 270 x 10 /l. Haemoglobin analysis identifies a high level of Hb S without detectable HbA. Why was it likely that this person had sickle cell anaemia over sickle cell trait?

Answer 5

Likely because there was no identifiable HbA (HbA is when there is 2 alpha and 2 beta chains, it is present in some quantity if there is one B mutation from one parent but if both copies of B chain have the mutation then there is no HbA) Also it is unliekly that a person with sickle cell trait would be anaemic

Question 6

What chromsomes do the B globulin and A globulin genes lie on?

Answer 6

B globulin lies on chromosome 11 7 and alpha globulin lies on chromosome 16

Question 7

What causes sickle cell disease?

Answer 7

Sickle cell disease is caused by a point mutation in codon 6 of the B-globulin gene resulting in the conversion of glutamine to valine Results in abnormal B globulin resutling in HbS instead of HbA If only one parent has the abnormal B globulin passed then heteroygous and sickle cell trait If homozygous (both parenets passed the mutation) - Sickle cell disease

Question 8

2. A 28 year old man has a blood count performed with the results as follows: Haemoglobin 152g/L, MCV 65 fl, white cell count 6.4 x 10 /l, neutrophil count 3.8 x 10 /l and platelet count 270 x 10 /l. Haemoglobin analysis identifies the presence of raised Hb A2. Why is the haemoglobin count so high in beta thalassemia trait and the MCV so low? What would be seen in beta thal major?

Answer 8

Hameoglobin count is high because the patient is trying to produce more red cells to carry more oxygen - patient is still usually asymptomatic MCV is low as haemoglobin formed is not normal HbA2 is increased to try and provide more oxygen In beta thal major - usually shows in first 6-24 months as HbF falls off - extramedullary haemaopoeisis - frontal bossing and hepatosplenomegaly

Question 9

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Answer 9

1. Febrile non-haemolytic transfusion reaction - It is most commonly caused by antibodies directed against donor leukocytes and HLA antigens 2. Transfusion associated circulatory overload - TACO can occur when patients with chronic anaemia and a compensatory high cardiac output are given a large volume load. The result is often pulmonary 3. Transfusion related acute lung injury - TRALI

Question 10

What usually causes TRALI? What people are the donors who cause TRALI usually?

Answer 10

Usually when plasma cells are being transfused - these contain anti-leucocyte antibodies present in the donors plasma which attack the patients white blood cells and there is degranulation of the neutrophils in the lungs shown as pulmonary infiltrates on CXR Usually the donor is a multiparous women who has developed lots of antibodies during pregnancy

Question 11

Which are causes of hereditary thrombophilia * a. Factor V Leiden, antiphospholipid syndrome, (APS) antithrombin deficiency, protein C deficiency * b. Factor V Leiden, APS, antithrombin deficiency, protein S deficiency * c. Factor V Leiden, APS, protein C deficiency, protein S deficiency * d. Factor V Leiden, antithrombin deficiency, protein C deficiency, protein S deficiency * e. APS, antithrombin deficiency, protein C deficiency, protein S deficiency

Answer 11

d) - Factor V Leiden, antithrombin deficiency, protein C deficiency and protein S deficiency The only other hereditary thombophilia we test for is Prothrombin 20210 mutation

Question 12

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Answer 12

1. B - Ritxuimab is used in both R-CHOP (rituximab + 4chemo) therapy for Non-hodgkins lymphoma and FCR (2chemo+rituximab) therapy for CLL Monocloncal antibody directed against CD20, expressed on Bcellls and B cell lymphomas 2. C - Imatinib is a tyrosine kinase inhibitor that is inhibits BCR-ABL-1 protein unique to chronic myeloid leukaemia. 3. I - Aspirin inhibits COX1 - necessary for TXA2 synthesis for platelet aggregation

Question 13

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Answer 13

1. D - Pernicious anaemia - B12 deficiency - causes a pancytopenia and abnormal red cells known as macroovalocytes are present and the neutrophilas are hypersegmeneted 2. A - AML causes a pancytopenia - low Hb, neutrophil and platelets - blasts on film also but Auer rods are diagnostic 3. F - Essential thrombocythaemia is a chronic MPD characterised by excess production of platelets in the bone marrow and an increased risk of thrombosis.

Question 14

Even though Auer rods are diagnostic of AML What must be carried out to differentiate from ALL as only blasts seen on film?

Answer 14

Need to diagnose via Immunophenotyping by flow cytometry on peripheral blood (or marrow?)

Question 15

Peter Smith, a sixty-five year old man is admitted to hospital with community acquired pneumonia. He is found to have a high neutrophil count but his platelet count is low. 1. Which term best describes the platelet abnormality? 2. Which term best describes the neutrophil abnormality? * A Thrombocytosis * B Neutropenia * C Thrombocytopenia * D Neutrophilia * E Thrombophilia

Answer 15

1. Low platelet - thrombocytopenia C 2. High neutrophil - neutrophilia - D

Question 16

Which type of cell is there an excession where you can use philia to denote an excess?

Answer 16

Platelets The exception to this rule is thrombophilia does not mean high platelet count (the correct term is thrombocytosis). Thrombophilia refers to an increased thrombotic tendency.

Question 17

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Answer 17

1. A - This is the only film that shows a neutrophil 2. Can see the presence of an eosinophil here - often described as spectacle shaped as it is bilobed and has the typical cytoplasmic granulations that stain red 3. E - Monocytes - have the typical horseshoe nucleus and vacuolation in the cytoplasm and is very large * Picture B shows a small (mature) lymphocytes * Picture D shows a basophil i believe - bilobed nucleus with purplish granules over nucleus

Question 18

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Answer 18

1. Asymptomatic microcytic hypochromic anaemia due to iron deficiency anaemia - A - oral iron supplement 2. D - The results suggest an autoimmune haemolytic anaemia, front-line treatment of which consists of steroid (1mg/kg of prednisolone per day) and folic acid 3. B - This lady has pancytopenia due to druginduced marrow failure (as part of her therapy). Therefore, in the absence of intrinsic haemopoietic cell activity, she requires a red cell transfusion, in the presence of symptoms.

Question 19

Why are steroids and folic acid given for autoimmune haemolytic anaemias?

Answer 19

Steroids are given to suppress the autoimmune process and folic acid is given to prevent deficiency which can occur due to the high red cell turnover in this disease - evident by the reticulocytosis

Question 20

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Answer 20

E - VitK is fat soluble, requires bile salts for asorption, carboxylates specific preformed clotting factors II,VII, IX and X (and Protein C and S)

Question 21

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Answer 21

1. H - Iron deficiency anaemia is most likely - measure serum ferritin 2. C - Chronic lymphocytic leukaemia - This is a clonal (neoplastic) disorder of B cells. Many patients will have asymptomatic disease. 3. E - Mutliple myeloma - back pain due to spread causing osteolytic bone lesions Bence Jones are monoclonal immunoglobulin light chains found in the urine (kappa or lambda chains only)

Question 22

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Answer 22

1. Clopidogrel 2. Aspirin 3. Rivoroxaban

Question 23

What does dabigatran inhibit? What does abcixmab inhibit? What other drug is an ADP receptor antagonist? What is the ADP receptor? What is another direct factor Xa inhibitor?

Answer 23

* Dabigatran directly inhibits thrombin (Factor IIa) * Abciximab inhibits GPIIbIIIA which prevents platelet aggregation * Prasgruel is another ADP receptor antagonist (clopidogrel is another) * ADP receptor antagonsitis are also known as P2Y12 inhibitors as this is the receptor Another direct Factor Xa inhibitor as well as rivoroxaban is apixaban

Question 24

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Answer 24

1. H - Sickle cell disease is due to a point mutation in codon 6 of the B globulin gene resulting in glutamine becoming valine producing Bs (abnormal beta chains) resulting in HbS forming 2. D - Myelodysplaia occurs due to acquired DNA mutations in haematopoietic stem cells 3. Hereditary spherocytosis is due to a congenita mutation in structural red cell proteins

Question 25

What does having the abnormal Bglobulin chains in sickle cell disease result in? What can myelodysplasia result in? (Occurs in 30%) Hereditary spherocytosis is due to a mutation in one of the structural red cell membrane proteins resulting in reduced red cell deformability. What does this cause?

Answer 25

Sickle cell disease is caused by a point mutation in the beta chain and this predisposes the haemoglobin to polymerisation resulting in sickled cells during hypoxia and reduced red cell survival. Myeldysplsia transforms to acute myeloid leukaemia in 30% The red cell deformity in HS results in the membrane being removed by the spleen forming spherocytes - seen on film

Question 26

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Answer 26

1. Pernicious anaemia - causes B12 deficiency - pancytopenia 2. I -Patients with sickle cell disease can have uncomplicated vaso-occlusive crisis in their musculoskeletal system. Sickle cell crisis in the pulomonary vasculature is a life threatening emergency that required prompt therapy with exchange transfusion 3. - Von willebrand’s disease is a disorder of primary haemostasis due to deficiency of von Willebrand factor which bridges platelets to sub-endothelial collagen following endothelial injury

Question 27

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Answer 27

1. Normal PT, Prolonged APTT with normal platelet and fibrinogen - single clotting factor deficiency in the intrinsic pathway (could also be VWD but not an option) - this is G 2. Prolonged PT, Normal APTT, normal platelet and fibrinogen - single clotting factor deficiency in the extrinsic pathway - this is A - only option 3. Disseminated intravascular coagulation - inappropriate activation of primary and secondary haemostasis and fibrinolysis

Question 28

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Answer 28

1. G: The mild anaemia in the presence of a normal serum ferritin reflects the dilutional (physiological) anaemia of pregnancy. The normal serum ferritin makes iron deficiency unlikely - likely to be that the woman has a beta thal trait 2. A - A high haemoglobin in someone without a history to suggest a secondary polycythaemia should be investigated by the analysis of the JAK2 gene, which has a somatic mutation in up to 95-97% of patients with polycthaemia 3. D - The cytopenias and excess of blasts are highly suggestive of an acute leukaemia. Immunophenotyping will clarify the lineage involved (ie myeloid or lymphoid)