Haematology

Question 1

What is the specific treatment for DIC in APML?

Answer 1

All-transretinoic acid

Question 2

What does giving FFP help replace?

Answer 2

Clotting factors

Question 3

What does giving cryoprecipitate help replace?

Answer 3

Fibrinogen

Question 4

What does a d-dimer test measure?

Answer 4

Fibrinogen degradation products

Question 5

Which organs are enlarged in myelofibrosis?

Answer 5

Liver and spleen - extramedullary haemopoesis.

Question 6

What is the treatment for myelofibrosis?

Answer 6

Hydroxycarbamide
Thalidomide +/- presnisolone
BMT

Question 7

What do iron studies show in haemochromotosis?

Answer 7

Increased iron and ferritin. Increased transferrin saturation.
Low total iron binding capacity.

Question 8

What is the treatment for haemochromotosis?

Answer 8

Genetic counselling, low iron diet
Venesection or desferrioxamone
Manage diabetes and other complications

Question 9

What is the medical treatment for sickle cell disease?

Answer 9

Prophylactic PenV, vaccination, Folate.
Hydroxyurea if recurrent painful crises in community, >3 episodes of acute pain in community, >2 acute chest syndrome
SCT if <17 and brain disease / severe complications.

Question 10

What is the treatment for acute priapism?

Answer 10

Analgesia
Alpha-adrenergic agonist (phenylephrine, etilefrine)
Penile aspiration and irrigation with dilute adrenaline

Question 11

What is the inheritance of Von willebrand disease?

Answer 11

Autosomal dominant, apart from type 3 (total quantitative deficiency) which is autosomal recessive.

Question 12

Why does Von willebrand disease leads to a reduced factor 8 assay?

Answer 12

Because vWF stabilises fVIII

Question 13

What is the treatment for vW disease?

Answer 13

Desmopressin nasal spray for types 1 and 2 (increases release of vWF from endothelium).
vWF factor concentrates in type 3.

Question 14

What coagulation test is prolonged in haemophilia?

Answer 14

The APTT - measure if the intrinsic pathway.

PT and bleeding time are normal.

Question 15

What is HbH disease?

Answer 15

Alpha thalassaemia with 3 out of the 4 alpha chain genes being mutated.

Question 16

What is the medial treatment for beta thalassaemia major?

Answer 16

Hydroxyurea, repeat blood transfusions every 2-4w, iron chelation therapy (desferrioxamine SC and Deferiprone PO), high dose ascarobic acid.

Question 17

What causes petechiae?

Answer 17

Extravasion if bleed from capillaries into skin and mucous membranes.

Question 18

What auto-antibodies are associated with ITP?

Answer 18

Anti-Glp2b-3a

Question 19

What’s the difference between TTP and ITP?

Answer 19

ITP is low platelets resulting from autoimmune destruction, whereas TTP is a low platelet count resulting from small vessel thromboses.

Question 20

What is the Pentad presentation in TTP?

Answer 20

MAHA, thrombocytopenic purpura, AKI, fever and CNS features.

Question 21

What is the first-line treatment for TTP?

Answer 21

Plasma exchange. Can give rituximab is anti-body mediated disease.
Very poor prognosis without treatment.

Question 22

What happens to haptoglobins in intravascular haemolysis?

Answer 22

Reduced or absent (they bind free Hb)

Question 23

What are schistocytes indicative of?

Answer 23

MAHA

Question 24

Three causes of MAHA?

Answer 24

TTP, DIC, malignancy

Question 25

What test is use to diagnose Paroxysmal Nocturnal Hameturia?

Answer 25

Hams test

Question 26

What does a raised MCV and polychromasia suggest?

Answer 26

Reticulocytosis.

Question 27

Which Ig mediated warm AIHA?

Answer 27

IgG

Question 28

What are the symptoms of hyperviscosoty?

Answer 28

Slowed thinking, headaches, visual disturbances, dyspnoea, fatigue.

Question 29

What are some causes of secondary increased haematocrit?

Answer 29

Appropriate EPO secretion - chronic lung disease, smoking, OSA, cyanotic heart disease Inappropriate EPO secretion - renal tumours, renal disease, HCC

Question 30

Polycythaemia and aquagenic priorities?

Answer 30

PCV. Causes by hyper-mast cell degranulation.

Question 31

What is the management of PCV?

Answer 31

Venesection, aspirin, manage CV risk factors, Cytoreductive therapy - <40 first line is alpha interferon - >40 first line is hyfroxycarbamide.

Question 32

What are the phases of chemotherapy used in the treatment of ALL?

Answer 32

Remission, intensification, consolidation, maintenance.

Question 33

What do smudge or smear cells suggest?

Answer 33

CLL

Question 34

What is richters transformation?

Answer 34

CLL transforming to high grade lymphoma (DLBCL)

Question 35

How is CLL staged?

Answer 35

Rai / Binet staging

Question 36

What at the phases of CML?

Answer 36

Chronic phases, accelerated phase, blast crisis

Question 37

Which leukaemia commonly has the Philadelphia chromosome mutation?

Answer 37

CML

Question 38

What is the first line treatment in CML?

Answer 38

Imatinib (tyrosine kinase Inhibitor) Allogenic SCT

Question 39

What lymphoma is associated HTLV1?

Answer 39

Adult T cell lymphoma

Question 40

How aggressive is Burkitt's lymphoma?

Answer 40

Very aggressive

Question 41

How aggressive is T or B cell lymphoblastic leukaemia / lymphoma

Answer 41

Very aggressive

Question 42

How aggressive are mantle zone and DLCB lymphoma?

Answer 42

Aggressive

Question 43

How aggressive is follicular lymphoma?

Answer 43

Indolent.

Question 44

What is the pattern of spread in Hodgkin's lymphoma?

Answer 44

Single nodule with contiguous spread

Question 45

What is a Pal-Ebstein fever?

Answer 45

A monthly fever classically associated with Hodgkin's lymphoma.

Question 46

What chemotherapy cycle is used to treat Hodgkin's lymphoma?

Answer 46

ABVD

Question 47

What is non-classical Hodgkin's lymphoma called?

Answer 47

Nodular lymphocyte predominant

Question 48

What are the three types of Burkitt's lymphoma?

Answer 48

Endemic, sporadic and immunocompromised. EBV is associated with endemic and sporadic forms, but not with the immunocompromised form.

Question 49

What is the microscopic appearance of Burkitt's lymphoma?

Answer 49

Starry sky appearance

Question 50

What kind of Burkitt's lymphoma is a jaw mass associated with?

Answer 50

Endemic.

Question 51

How does adult T cell lymphoma present?

Answer 51

Heoatosplenomegaky, skin symptoms, lymphadenopathy, hypercalcaemia. Generally in the Caribbean or Japanese populations.

Question 52

What is microscopic appearance of adult T cell lymphoma?

Answer 52

Clover leaf nuclei

Question 53

What lymphoma is associated with long-standing coeliac disease?

Answer 53

Enteropathy associated T cell lymphoma

Question 54

What are the causes of B12 deficiency?

Answer 54

Dietary (vegans) Malabsorption - pernicious anaemia, crohns, coeliac disease, tapeworm, bacterial overgrowth, pancreatic insuffiency Congenital metabolic errors

Question 55

What is the classic triad of reflexes in b12 deficiency associated subacute combined degeneration of the spinal cord?

Answer 55

Extensor plantars, brisk knee jerks, absent ankle jerks.

Question 56

What would you see on the blood film in b12 deficiency?

Answer 56

Hypersegmented neutrophils and macrocytes

Question 57

What the highest risk surgeries for DVT development?

Answer 57

Orthopaedic surgery - TKR, hip fracture, elective hip replacement Pelvic abdominal surgery - total abdominal prostatectomy

Question 58

What are flowtrons?

Answer 58

Active compression stockings used for DVT prophylaxis. It does the job of the soleus muscle (slow twitch) in pumping blood.

Question 59

What is the active ingredient in Clexane?

Answer 59

Enoxaparin

Question 60

What needle is used to do a trephine biopsy?

Answer 60

Jamshidi needle

Question 61

Classically, which bone is used for a bone marrow aspiration?

Answer 61

Posterior Superior Iliac Spine

Question 62

What blood group of FFP should be given in an emergency?

Answer 62

AB-

Question 63

What are the symptoms of TRALI?

Answer 63

Dyspnoea, dry cough, hypoxia, chills, fever. CXR shoes new bilateral pulmonary infiltrates.

Question 64

What are the symptoms of transfusion associated graft versus host disease?

Answer 64

Severe diarrhoea, liver failure, red mqculopapular rash, bone marrow failure, skin desquamation. Usually affects patients who are very immunosuppressed (Hodgkin's, SCT) and can be avoided by irradiation.