Medicine (8)

Question 1

What’s Primary True Polycythaemia?

Answer 1

Primary True Polycythaemia = Rubra Vera

• One of the myeloproliferative disorders
• Uncontrolled production of red cells by the bone marrow, even though EPO is switched off

Question 2

Clinical features Primary True Polycythaemia (Rubra Vera)

Answer 2

Features:

• Hypertension
• splenomegaly
• arterial and venous thromboses
• pruritus (typically after a hot bath) → due to the release of histamine and prostaglandins from neoplasm in bone marrow

Question 3

Lab findings in Polycythaemia Rubra Vera

Answer 3

• High RBCs count, Hb, haematocrit
• Neutrophil and platelet count are often raised and helps distinguish true from relative
• Of primary polycythaemia patients, 90% have a gene mutation, JAK-2, detectable by PCR on blood cells

Question 4

Treatment of polycythaemia vera

Answer 4

• aspirin
• venesection - first line treatment
• hydroxyurea -slight increased risk of secondary leukaemia
• phosphorus-32 therapy

Question 5

What’s Essential Thrombocythaemia?

Answer 5

Essential Thrombocythaemia

• Clonal myeloproliferative disorder that chiefly involves the megakaryocyte cell line
• Associated with a markedly high platelet count

Question 6

What’s myelofibrosis?

Answer 6

• clonal disorder of the haematopoietic stem cell

The proliferation of the abnormal clone of the stem cells in the bone marrow → fibrosis of the bone marrow and other sites

Characterised by:

• massive splenomegaly
• immature circulating cells in the blood
• distorted (tear-drop) red cell

Question 7

What’s aplastic anaemia?

Answer 7

Aplastic anaemia

• bone marrow and stem cells do not produce enough blood cells
• autoimmune condition

Question 8

Features of aplastic anemia

Answer 8

• normochromic, normocytic anaemia
• leukopenia, with lymphocytes relatively spared
• thrombocytopenia
• may be the presenting feature acute lymphoblastic or myeloid leukaemia
• a minority of patients later develop paroxysmal nocturnal haemoglobinuria or myelodysplasia

Question 9

Management of aplastic anaemia

Answer 9

• Blood transfusion
• Immunosuppression (ciclosporin + anti-thymocyte globulin (ATG)) → theory that T cells attack bone marrow – ATG is anti-T cell)
• Bone marrow transplant in severe

Question 10

What’s myelodysplastic syndrome?

Answer 10

• also known as myelodysplasia
• acquired neoplastic disorder of hematopoietic stem cells
• pre-leukaemia, may progress to AML

Features

• more common with age
• presents with bone marrow failure (anaemia, neutropaenia, thrombocytopenia)

Question 11

Management of myelodysplastic syndrome

Answer 11

• Growth Factors
• antibiotics
• blood transfusions
• Chemotherapy
• Immunosuppression + Bone marrow transplant

Question 12

Hodkin’s vs non-Hodkin’s lymphoma

Answer 12

Question 13

Staging of Hodkin’s lymphoma

Answer 13

Question 14

Clinical staging of Hodkin’s lymphoma

Answer 14

Clinical stage may be given the letter A or B to reflect

symptoms:

A → no ‘B’ symptoms

B:

• Significant fever (>38)
• Night sweats
• Weight loss >10% in the last 6 months

‘B’ symptoms also imply a poor prognosis

Question 15

Lymphoma vs leukaemia

Answer 15

Question 16

What’s multiple myeloma?

Answer 16

Multiple myeloma:

• neoplasm of the bone marrow plasma cells
• peak incidence is patients aged 60-70 years

Question 17

Clinical features of Multiple Myeloma

Answer 17

• bone disease: bone pain, osteoporosis + pathological fractures (typically vertebral), osteolytic lesions
• Back Pain
• lethargy
• Bacterial infection (due to acquired hypogammaglobulinaemia)
• hypercalcaemia
• renal failure
• other features: amyloidosis e.g. macroglossia, carpal tunnel syndrome; neuropathy; hyperviscosity, nephrotic syndrome
• Raised EsR

Question 18

Diagnosis of multiple myeloma

Answer 18

• monoclonal paraprotein (usually IgG or IgA) in the serum
• monoclonal paraprotein in the urine (Bence Jones proteins)
• increased plasma cells in the bone marrow
• bone lesions on the skeletal survey

Question 19

Management of Multiple Myeloma

Answer 19

• Adequate hydration
• s/c EPO
• bisphosphonates for high calcium
• Radiotherapy +Chemotherapy

Question 20

Solve this

Answer 20

Question 22

Typical features of rheumatoid arthritis

Answer 22

• swollen, painful joints in hands and feet
• stiffness worse in the morning
• gradually gets worse with larger joints becoming involved
• presentation usually insidiously develops over a few months
• positive ‘squeeze test’ - discomfort on squeezing across the metacarpal or metatarsal joints
• Swan neck and boutonnière deformities are late features of rheumatoid arthritis and unlikely to be present in a recently diagnosed patient

Question 23

How to examine patient with RA?

Answer 23

• Hands
• Elbows → Rheumatoid nodules
• Ears → Psoriatic plaques

-Assess their function by seeing how
they hold a pen/coin

Question 24

Signs of RA on examination

Answer 24

Question 25

Extra-articular manifestations of RA

Answer 25

Question 26

Investigations for RA

Answer 26

• Urinalysis (?haematuria/proteinuria)
• Rheumatoid Factor → positive in 70%
• Anti-CCP (highly specific)
• ESR/CRP → reflect disease activity
• FBC → anaemia
• x- ray

Question 27

Characteristic X-ray changes in RA

Answer 27

LESS

Loss of Joint Space

Erosions (periarticular osteopenia)

Soft tissue swelling

Subluxation

Question 28

What score is used to monitor disease activity in RA?

Answer 28

DAS-28

Question 29

What is the best single treatment of RA?

Answer 29

Methotrexate + (bridging) Prednisolone

• Specialists will usually offer a conventional disease modifying anti-rheumatic drug (cDMARD) as monotherapy, ideally within 3 months of the onset of symptoms —e.g. oral methotrexate, leflunomide, or sulfasalazine
• Short-term bridging treatment with glucocorticoids (oral, intramuscular or intraarticular) may be used when starting a new cDMARD to improve symptoms while waiting for the new DMARD to take effect (which can take 2–3 months)

Question 30

Management of RA

Answer 30