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Disease What is it? Types Pathology Aetiology Symptoms Diagnosis Prognosis Treatment Lymphoma Proliferation of lymphoid cells – more commonly B, but can be T Hodgkin’s Reed-Sternbery cells –giant multi-nucleate malignant B cells present on histology. They express no antibodies Slightly more common in men. Peak incidence in 3rd,and 6th-8th decades. EBV, CMV infection. Genetic factors. Incidence is stable Enlarged rubbery lymph nodes – particularly in neck Alcohol induced pain in lymph nodes Itch, fatigue, anorexia B-symptoms – fever, night sweats, weight loss Hepatosplenomegaly, Lymphocytonpaenia, Eosinophililia, Raised LDH, Lymph node biopsy for histology – fine needle aspiration is useless as it only give cytology Worse with B symptoms and raised LDH. Treatment is often curative. Median survival is longer than 5 years even in those with severe disease. Spread is contiguous (only to adjacent structures) Irradiation with/without chemo. Chemo for more severe cases. Disease is classified using the Ann Arbor scale. Survival rates: Stage I – 95% @ 5yrs Stage IV – 40% @ 5yrs Non-Hodgkin’s Malignant tumour of lymphoid cells without RS cells present 7th decade onwards Incidence is rising. High incidence in developing countries due to Burkitt’s lymphoma – a T cell lymphoma caused by EBV T cell virus. Lymphadenopathy, B symptoms, compression symptoms (extranodal symptoms), Pancytopaenia often present All the investigations of HL, as well as bone marrow aspiration, immunophenotyping. Spread in non-contiguous (multicentric) Not generally curable. Mild disease will relapse/remit. Sever disease has a worse prognosis, but also a higher chance of cure. Worse if B symptoms and raised LDH Varies between patients. If no symptoms; monitor. Radiotherapy can cure localised disease. Chemo used in widespread disease. Survival rates: Low grade: 50% @ 5yrs High: 30% @ 5 yrs Myeloma Monoclonal proliferation of B cells Infiltration of bone marrow by lots of abnormal B cells. leads to pancytopaenia. Lytic lesions in bone. Renal impairment, hypercalcaemia, spinal cord compression Over 60’s. Slight male predominance. More common in black Africans. Genetic factors Hyperviscosity, recurrent infections due to reduced level of normal Ig, usually in resp. tract. Bone pain. Often asymptomatic – disease discovered on routine bloods FBC, ESR and CRP, blood film, LDH, calcium (normal or raised), total protein, urine immunofixation (Bence-Jones proteins), DEXA scan Bone marrow aspiration shows loads of plasma cells. Incurable – treatments just induce remission. Death from infection, renal failure, haemorrhage. Median survival is 5 years, but 1/3 of patients will die within 3 months of diagnosis. Can be divided into supportive (i.e. treating symptoms and complications) and specific (i.e. treating the disease, with bisphosphonates). True remission is never maintained, and patients will relapse when treatment is stopped. Leukaemia ALL – acute lymphoblastic leukaemia Basically an acquired genetic defect that means blast cells do not mature, and they also avoid apoptosis, thus proliferate wildly. Unknown – many factors involved, possibly radiation, benzene, genetic susceptibility, infection by certain viruses (HLTV-1) Children! Anaemia, bleeding, infection (fever), bone pain, petichae, lymphadenopathy, >20% blast cells in bone marrow, raised WCC, low Hb, low platelets Hepatosplenomegaly, testicular enlargement Very poor without treatment. Younger patients more likely to be cured. For ALL in childhood, 80% will have CR after 5 years. For ALL, all under 60’s normally given curative treatment, and 2/3’s will not relapse. If cure appears possible: Remission induced by chemo, then bone marrow transplantation (if suitable HLA match) , then maintenance therapy. This will usually give ‘complete remission’, which in many is curative, but some still relapse. Last 6-12 months in total. Risk of death. Iif no cure possible, then supportive (i.e palliative) care is available. AML – acute myeloid leukaemia Adults, 2/3 are >60 Anaemia, bleeding, infection, bone pain CLL – chronic lymphocytic leukaemia Usually from B lymphocytes. The most common leukaemia. WCC 20-40, hepatosplenomegaly Media survival is 10 years. This is related to the severity of the symptoms at presentation CML – chronic myeloid leukaemia Philadelphia chromosome – this is the abnormality in 97% ofcases. Slow progression Peak age 40-60. Slightly higher incidence in men. SOB (anaemia), abdominal discomfort (splenomegaly), weight loss, fever and sweats (B symptoms), lymphadenopathy, gout, retinal haemorrhage, cerebral disorders. WCC > 100, hepatosplenomegaly, high neutrophils, blood film shows cells at all stages of development, high uric acid production (renal failure, gout) due to high cell turnover rate Disease progresses from crhonic stage ≫ aggressive phase ≫ blast crisis. The blast crisis is essentially terminal. Imitinab – prevents the action of the abnormal protein produced by the Ph gene. Stem cell transplantation is curative in 70% of cases – but is not suitable for everyone

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