Medicine (8) Flashcards
What’s Primary True Polycythaemia?
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
Clinical features Primary True Polycythaemia (Rubra Vera)
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
Lab findings in Polycythaemia Rubra Vera
- 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
Treatment of polycythaemia vera
- aspirin
- venesection - first line treatment
- hydroxyurea -slight increased risk of secondary leukaemia
- phosphorus-32 therapy
What’s Essential Thrombocythaemia?
Essential Thrombocythaemia
- Clonal myeloproliferative disorder that chiefly involves the megakaryocyte cell line
- Associated with a markedly high platelet count
What’s myelofibrosis?
- 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
What’s aplastic anaemia?
Aplastic anaemia
- bone marrow and stem cells do not produce enough blood cells
- autoimmune condition
Features of aplastic anemia
- 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
Management of aplastic anaemia
- 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
What’s myelodysplastic syndrome?
- 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)
Management of myelodysplastic syndrome
- Growth Factors
- antibiotics
- blood transfusions
- Chemotherapy
- Immunosuppression + Bone marrow transplant
Hodkin’s vs non-Hodkin’s lymphoma
Staging of Hodkin’s lymphoma
Clinical staging of Hodkin’s lymphoma
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
Lymphoma vs leukaemia
What’s multiple myeloma?
Multiple myeloma:
- neoplasm of the bone marrow plasma cells
- peak incidence is patients aged 60-70 years
Clinical features of Multiple Myeloma
- 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
Diagnosis of multiple myeloma
- 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
Management of Multiple Myeloma
- Adequate hydration
- s/c EPO
- bisphosphonates for high calcium
- Radiotherapy +Chemotherapy
Solve this
Typical features of rheumatoid arthritis
- 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
How to examine patient with RA?
- Hands
- Elbows → Rheumatoid nodules
- Ears → Psoriatic plaques
-Assess their function by seeing how
they hold a pen/coin
Signs of RA on examination
Extra-articular manifestations of RA
Investigations for RA
- Urinalysis (?haematuria/proteinuria)
- Rheumatoid Factor → positive in 70%
- Anti-CCP (highly specific)
- ESR/CRP → reflect disease activity
- FBC → anaemia
- x- ray
Characteristic X-ray changes in RA
LESS
Loss of Joint Space
Erosions (periarticular osteopenia)
Soft tissue swelling
Subluxation
What score is used to monitor disease activity in RA?
DAS-28
What is the best single treatment of RA?
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)
Management of RA
