Haematology

Question 1

What is pernicious anaemia?

Answer 1

B12 deficiency

Question 2

What cells in the stomach produce intrinsic factor?

Answer 2

Parietal cells

Question 3

Where is B12 absorbed?

Answer 3

Ileum

Question 4

What is the pathophysiology of pernicious anaemia?

Answer 4

Autoimmune - antibodies against the parietal cells or intrinsic factor

Question 5

What are the symptoms of B12 deficiency?

Answer 5

Peripheral neuropathy
Loss of vibration sense or proprioception
Visual changes
Mood or cognitive changes

Question 6

What is the management for pernicious anaemia?

Answer 6

Oral cyanocobalamin
If severe: IM hydroxycobalamin

Question 7

If you have folate and B12 deficiency, which one should you treat first?

Answer 7

B12 first: otherwise it can cause subacute combined degeneration of the cord.

Question 8

What does the Direct Coombs test test for?

Answer 8

Autoimmune haemolytic anaemia

Question 9

What is the inheritance pattern in hereditary spherocytosis?

Answer 9

Autosomal dominant

Question 10

What virus can cause an aplastic crisis in hereditary spherocytosis?

Answer 10

Parvovirus

Question 11

What is the management of hereditary spherocytosis?

Answer 11

Folate supplementation
Splenectomy
Cholecystectomy if gallstones are a problem

Question 12

What is the inheritance pattern of hereditary elliptocytosis?

Answer 12

Autosomal dominant

Question 13

What is the inheritance pattern of G6PD deficiency?

Answer 13

X linked recessive. More common in mediterranean and African patients

Question 14

What can trigger a crisis in G6PD?

Answer 14

Fava beans, antimalarial primaquine, ciprofloxacin, sulfonylureas, sulfasalazine and other sulphonamide drugs, infections.

Question 15

What are the two types of autoimmune haemolytic anaemia?

Answer 15

Warm type and cold type (what temperature the antibodies attach themselves - agglutination)

Question 16

What conditions is cold type autoimmune haemolytic anaemia secondary to?

Answer 16

Lymphoma
Leukaemia
SLE
Infections such as: mycoplasma, EBV, CMV, HIV

Question 17

What is the management of autoimmune haemolytic anaemia?

Answer 17

Blood transfusion
Prednisolone
Rituximab (monoclonal antibody against B cells)
Splenectomy

Question 18

What is alloimmune haemolytic anaemia?

Answer 18

Foreign red blood cells causing an immune reaction (transfusion or newborn)

Question 19

What is paroxysmal nocturnal haemoglobinuria?

Answer 19

Genetic mutation in the stem cells
Realists in loss of proteins on the surface of red blood cells that inhibit the complement cascade -> complement cascade is activated and RBCs are destroyed

Question 20

What is the characteristic presentation of paroxysmal nocturnal haemoglobinuria?

Answer 20

Red urine in the morning
Also:
Anaemia
Predisposed to thrombosis and smooth muscle dystonia (e.g. oesophageal spasm and erectile dysfunction)

Question 21

What is microangiopathic haemolytic anaemia secondary to?

Answer 21

Haemolytic uraemia syndrome
DIC
Thrombotic thrombocytopenia purpura
SLE
Cancer

Question 22

Myeloma is a cancer of which cells?

Answer 22

Plasma cells (the B lymphocytes that produce antibodies)

Question 23

What are Bence Jones proteins

Answer 23

Light chains of antibodies found in the urine of patients with myeloma

Question 24

What causes renal impairment in patients with myeloma?

Answer 24

High levels of immunoglobulins can block flow
Hypercalcaemia
Dehydration
Bisphosphonates

Question 25

Raised plasma viscosity in myeloma can cause what?

Answer 25

Easy bruising Easy bleeding Reduced or loss of sight due to vascular disease in the eye Purple discolouration to the extremities heart failure

Question 26

What does CRAB stand for in myeloma?

Answer 26

Calcium (elevated Renal failure Anaemia Bone lesions/pain

Question 27

What are the risk factors for myeloma?

Answer 27

Older age Male Black African ethnicity Family history Obesity

Question 28

What investigations do you do for myeloma? (BLIP)

Answer 28

Bence Jones protein (request urine electrophoresis) L - Serum free light chain assay I - Serum Immunoglobulins P- Serum Protein electrophoresis Bone marrow biopsy

Question 29

What are the x-ray signs in myeloma?

Answer 29

Punched out lesions Lytic lesions 'Raindrop skull'

Question 30

What is the first line management for myeloma?

Answer 30

Chemo: Bortezomid, Thalidomide, Dexamethasone VTE prophylaxis if on thalidomide

Question 31

What is the management for myeloma bone disease?

Answer 31

Bisphosphonates (suppress osteoclasts activity) Radiotherapy Orthopaedic surgery Cement augmentation (injecting cement into vertebral fractures)

Question 32

What is myelofibrosis?

Answer 32

Fibrosis of the bone marrow, due to cytokines released from he proliferating cells

Question 33

Why does myelofibrosis cause organomegaly?

Answer 33

Extramedullary haematopoiesis has to happen in the liver and spleen. Can cause portal hypertension or spinal cord compression

Question 34

What are the signs of polycythaemia vera?

Answer 34

Conjunctival plethora (redness A ruddy complexion Splenomegaly

Question 35

What can a blood film look like in myelofibrosis?

Answer 35

Teardrop-shaped RBCs, varying sizes of RBCs (poikilocytosis) and immature red and white cells.

Question 36

How do you diagnose myelofibrosis

Answer 36

Bone marrow biopsy: dry bone marrow on aspiration

Question 37

What is the difference between myeloproliferative disorders and myelodysplastic syndrome?

Answer 37

Myeloproliferative: one stem cell is proliferating much more than it should Myelodysplastic: myeloid bone marrow cells not maturing properly and so low levels of the cells that should be being produced

Question 38

What can myelodysplastic and myeloproliferative syndromes transform into?

Answer 38

Acute myeloid leukaemia

Question 39

What does myelodysplastic syndrome present with?

Answer 39

Anaemia/neutropenia/thrombocytopenia. Often have a history of chemo or radiotherapy and are over 60. Confirmed on bone marrow aspiration and biopsy

Question 40

What is the most common inherited cause of abnormal bleeding?

Answer 40

Von Willebrand disease

Question 41

What is the inheritance pattern of VWD?

Answer 41

Autosomal dominant

Question 42

What is VWF?

Answer 42

A glycoprotein

Question 43

What is the management of VWD?

Answer 43

Desmopressin - can stimulate the release of VWF Infused VWF +/- factor VIII Women with heavy periods: tranexamic or mefanamic acid, norethisterone, COCP, Mirena,

Question 44

What can affect the production of platelets?

Answer 44

Sepsis B12 or folate def. Liver failure causing reduced thrombopoietin production Leukaemia Myelodysplastic syndrome

Question 45

What can cause the destruction of platelets?

Answer 45

Medications (sodium valproate, methotrexate, isotretinoin, antihistamines, proton pump inhibitors) Alcohol ITP Thrombotic thrombocytopenia purpura Heparin-induced thrombocytopenia HUS

Question 46

What platelet count is at high risk for spontaneous bleeding?

Answer 46

Below 10 x 10tothe9

Question 47

What is the pathophysiology of thrombotic thrombocytopaenic purpura?

Answer 47

Tiny blood clots form in the small vessels, using up platelets. A microangiopathy. These develop due to a shortage in the protein ADAMTS13 (either due to genetic mutation or autoimmune disease). This protein normally inactivates vWF and reduced platelet adhesion and clot formation.

Question 48

What is the management for thrombotic thrombocytopaenic purpura?

Answer 48

Plasma exchange Steroids Rituximab

Question 49

What is the pathophysiology of heparin induced thrombocytopenia?

Answer 49

Antibodies are formed against platelets in response to exposure to heparin. These antibodies activate clotting mechanisms and lead to thrombosis, as well as breaking down platelets. A surprising picture of a patient on heparin with low platelets forming unexpected blood clots.