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Haematological Malignancies in General Practice. Judith Hanslip Consultant Haematologist Lister Hospital, Stevenage. Jon Lambert Consultant Haematologist UCLH & Mt Vernon Cancer Centre. Epidemiology Lymphoma Myeloma Referral of patients with suspected haematological malignancies.
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Haematological Malignanciesin General Practice Judith Hanslip Consultant Haematologist Lister Hospital, Stevenage Jon Lambert Consultant Haematologist UCLH & Mt Vernon Cancer Centre
Epidemiology • Lymphoma • Myeloma • Referral of patients with suspected haematological malignancies
UK Cancer Registrations 2007 HMRN data http://www.hmrn.org/Statistics/Incidence.aspx
Subtypes of haematological malignancies Annual crude rates per 100,000 (2004–2009). Myeloid Lymphoid Smith et al, BJC (2011) 105, 1684–1692
Lymphoma • Different lymphomas – why the subtype matters • When to suspect lymphoma – are any tests useful? • Referral to hospital, when and how quickly?
Many different subtypes of lymphoma… HMRN data 2012
mostly curable mostly incurable …with very different clinical behaviour Aggressive Indolent Diffuse large B-cell Follicular Hodgkin Mantle cell T-cell Burkitt
Survival in non-Hodgkin lymphoma depends on subtype HMRN 2004-2009
But also on extent of spread Hodgkin lymphoma SEER data, NCI, 2008
An isolated node, or the tip of the iceberg? Patient presenting with inguinal lymphadenopathy…
Lymphoma – when to suspect • Can affect any organ, and symptoms vary accordingly • Typically present with an enlarging cervical, axillary or inguinal lump • B symptoms are rare and indicate high disease burden • Main question should be: is there an obvious reactive cause for LN?
Lymphoma – are any tests helpful? • In most cases of lymphoma, the FBC, biochem and LDH are normal • Only whole-body imaging +/- biopsy are likely to be diagnostic (FNA is no use) • The best guide is from the history and examination
Referral to hospital – when and how quickly? • Rapidly enlarging nodes with systemic or neurological symptoms need urgent referral - discuss same day • Otherwise follow 2-week wait procedure
Myeloma – epidemiology • Annual UK Incidence: 40 per x 106 (2500 new cases per year) • Median age at diagnosis 60-65 yrs • Higher incidence in Afro-Caribbean people
Myeloma – epidemiology • 2% under 40 yrs • 35% under 65 yrs • 37% older than 75 yrs
Myeloma – improvements in outcome over 30 years Kumar S K et al. Blood 2008;111:2516-2520
Myeloma – survival varies with age at diagnosis Brenner et al, Haematologica, Feb 2009
82% 51% 81% 78% 79% n = 11,000 L Ellis-Brookes et al, Brit J Cancer, Sept 2012 Myeloma – route to secondary care… …and its effect on outcome 1-year overall survival
Backache particularly if persistent, unexplained or associated with loss of height and osteoporosis (esp in males and pre-menopausal females) Bone marrow suppressionesp normochromic or macrocytic anaemia, but also neutropenia or thrombocytopenia Renal impairment When to suspect myeloma? • Recurrent infectiondue to ↓immunoglobulins or neutropenia • Hypercalcaemia • Persistent ↑ESR(or plasma viscosity) esp if no obvious infective or autoimmune cause • Spinal cord/nerve root compression
ESR or plasma viscosity FBC U & E, Calcium Protein electrophoresis Immunoglobulin profile Urine for BJP X-rays of painful sites + Skeletal Survey, BM, BJP quantitation Investigations in Suspected Myeloma
Monoclonal protein (M-protein or paraprotein) • Monoclonal immunglobulin secreted by abnormal plasma cell clone – detectable in serum and/or urine • Can either whole (heavy and light chain) Ig or just free Ig light chain
Myeloma = M-protein + one of… Bone marrow plasma cells >10% Lytic lesions on skeletal survey Anaemia Hypercalcaemia or impaired renal function MGUS = M-protein < 3g/L and none of the above M-protein doesn’t necessarily indicate myeloma
Kyle et al, NEJM, March 2006 MGUS Uncommon below age of 50 Prevalence increases with advancing age: Cumulative risk of progression c.1% per year
Guidelines for referring patients with suspected haematological malignancies
2010 Patient Experience Survey • 51% of myeloma patients had visited their GP at least 3 times before referralhighest probability of delay out of 24 cancers captured in survey • The overall probability of people with suspected cancer visiting their GPs > 3 times was increased in: • Younger pats • Women • Ethnic minorites