Proliferative Disorders Flashcards
Polycythaemia
Causes?
Primary - polycythaemia rubra Vera, familial Polycythaemia
Secondary - disease states (renal Ca), high altitude, chronic hypoxia
Pseudo polycythaemia
Essential thrombocytopenia
Treatment?
Aspirin (prevent thrombosis)
Anagrelide (inhibits plt formation, SE palpitations, flushing)
Hydroxycarbamide (antimetabolic)
Myeloproliferative disorders
Define.
Name.
Group of conditions characterised by coronal proliferation of one or more myeloid components.
polycythaemia, essential thrombocythaemia, idiopathic myelofibrosis, idiopathic erythrocytosis, chronic granulocytic leukaemia,
Diamond Blackman syndrome
What?
Pure red cell aplasia
Normal WCC and plts
Presents at 1 year
Bone marrow failure
Drug causes?
Chemicals - benzene
Drugs - cytotoxic, gold salts, sulphonamides, thiazides, carbimazole, phenylbutazone
Dyskeratosis congenita.
Pancytopenia
X linked
DKC1 gene (telomerase function) - short telomeres
Skin pigmentation, nail dystrophy, leukoplakia
Most common form of inherited BM failure?
Fanconis Anaemia
BM Failure.
Management?
1) Find cause
2) conservative- blood/plts transfusion, Fe chelation therapy, Abx
3) medical - oxymetholone (growth factors)
4) SCT
Myelodysplasia.
Define?
Proliferation of abnormally maturing stem cells.
Numerical reduction and defective function.
MM treatment?
Treat CRAB Symptoms : bisphosphonates, IV fluids, steroids,
Chemo: melphalan , 2nd line bortezomib, 3rd line lenalidomide
SCT
> 10% plasma cells in the BM
No CRAB symptoms
30g/L monoclonal protein in the serum
Smouldering / asymptomatic Myeloma
R-CHOP?
Rituximab Cyclophosphamide Adriamycin Vinblastine Prednisolone
Mycosis fungoides.
Ax with ?
Cutaneous T cell lymphoma
Longstanding coeliac disease.
Ax with?
Enteropathy- associated T Cell lymphoma
HTLV1 infection.
Ax with …?
Adult T Cell leukaemia/lymphoma
