Common Haematological Malignancies by HC120717191322

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									Common Haematological
Malignancies
  Clare Kettlewell GPST1.
Objectives

  Curriculum requirements
  Referral pathways
  Common haematological malignancies –
   the basics.
  Case discussion.
Curriculum Requirements
    Recognise cancer illness in its early stages.
    Knowledge of the epidemiology of major cancers
     along with risk factors and unhealthy behaviours.
    Knowledge of referral guidelines and protocols, both
     local and national.
    Knowledge of the appropriate investigations of
     patients with cancer and of how they fit in with national
     guidelines.
    The ability to communicate effectively with the patient
     and carer(s) regarding difficult information about the
     disease, its treatment or its prognosis.
The 20 most commonly diagnosed
cancers, UK, 2005.
Referral Guidelines for suspected
haematological cancers.
  Primary healthcare professionals should be
   aware that haematological cancers can
   present with a variety of symptoms that may
   have a number of different clinical
   explanations.
  A patient who presents with symptoms
   suggesting haematological cancer should be
   referred to a team specialising in the
   management of haematological cancer,
   depending on local arrangements.
Referral Guidelines for suspected
haematological cancers.
    Combinations of the following symptoms and signs may suggest haematological
     cancer and warrant full examination, further investigation (including a blood count and
     film) and possible referral:
    • fatigue
    • drenching night sweats
    • fever
    • weight loss
    • generalised itching
    • breathlessness
    • bruising
    • bleeding
    • recurrent infections
    • bone pain
    • alcohol-induced pain
    • abdominal pain
    • lymphadenopathy
    • splenomegaly.
    The urgency of referral depends on the severity of the symptoms and signs, and findings of
     investigations.
Referral Guidelines for suspected
haematological cancers.
  Investigation of patients with persistent
   unexplained fatigue should include a full blood
   count, blood film and ESR, plasma viscosity or
   CRP (according to local policy), and repeated
   at least once if the patient’s condition remains
   unexplained and does not improve.
  The same investigations should be performed
   in patients with unexplained lymphadenopathy.
Referral Guidelines for suspected
haematological cancers.
   Any of the following additional features of
    lymphadenopathy should trigger further
    investigation and/or referral:
 • persistence for 6 weeks or more
 • lymph nodes increasing in size
 • lymph nodes greater than 2 cm in size
 • widespread nature
 • associated splenomegaly, night sweats or
    weight loss.
Referral Guidelines for suspected
haematological cancers.
    Investigation of a patient with unexplained bruising,
     bleeding, and purpura or symptoms suggesting
     anaemia should include a full blood count, blood film,
     clotting screen and erythrocyte sedimentation rate,
     plasma viscosity or C-reactive protein (according to
     local policy).

    A patient with bone pain that is persistent and
     unexplained should be investigated with full blood
     count and X-ray, urea and electrolytes, liver and bone
     profile, PSA test (in males) and erythrocyte
     sedimentation rate, plasma viscosity or C-reactive
     protein (according to local policy).
Common Haematological Cancers

  Acute lymphoblastic leukaemia
  Acute myeloid leukaemia
  Chronic myeloid leukaemia
  Chronic lymphocytic leukaemia
  Hodgkins lymphoma
  Non Hodgkins lymphoma
  Myeloma
Acute lymphoblastic leukaemia
(ALL)
    Malignancy of lymphoid cells with uncontrolled proliferation of
     blast cells.
    Genetic susceptibility and environmental trigger.
    Associated with Downs Syndrome.
    Commonest cancer of childhood, rare in adults.
    Signs/symptoms – Marrow failure (pancytopenia) leading to
     anaemia, infection and bleeding. Hepato/splenomegaly. CNS
     infiltration (meningism, nerve palsies)
    Investigation – FBC, blood film, CXR/CT, LP.
    Treatment – Supportive (transfusion,antibiotics), chemotherapy
     (often intrathecal), allogenic marrow transplant.
    Prognosis – cure rates for children 70-90%, adults ~ 40%.
Acute myeloid leukaemia (AML)
  Neoplastic proliferation of blast cells derived from
   marrow myeloid elements.
  Commonest acute leukaemia of adults (1/10,000/year)
  Associated with radiation and Downs Syndrome.
  Symptoms – marrow failure, infiltration (gum
   hypertrophy, CNS involvement at presentation rare.)
  Investigation – FBC and film, bone marrow aspirate
   with immunophenotyping.
  Treatment – supportive (walking exercises can relieve
   fatigue), chemotherapy (intensive, long periods of
   immunosuppression) bone marrow transplant.
  Prognosis – Death in ~ 2 months if left untreated.
               - After treatment ~ 20% 3 yr survival.
Acute myeloid leukaemia (AML)
Chronic myeloid leukaemia (CML)
    Uncontrolled clonal proliferation of myeloid cells.
    Associated with Philadelphia chromosome ( present in >80%)
    Symptoms/Signs – chronic and insiduous – weight loss, fever,
     fatigue, sweats , gout, bleeding (platelet dysfunction), abdominal
     discomfort (splenomegaly >75%, often massive)
    Around 30% detected by chance on routine FBC.
    Investigations – WCC ↑, Hb low or normal. Bone marrow
     diagnosis.
    Treatment – Chemotherapy (imatinib- >90% response rate),
     allogenic transplant only hope of long term survival.
    Prognosis – Median survival 5-6 years.
Chronic lymphocytic leukaemia
(CLL)
    Accumulation of mature B cells.
    Commonest leukaemia (~4/100000/year)
    Symptoms/signs – often none, found on routine FBC.
     May have enlarged rubbery non tender
     lymphadenopathy, hepato/spleomegaly, systemic
     symptoms.
    Investigations – increased lymphocytes, later
     autoimmune haemolysis and pancytopenia.
    Treatment – monitoring, chemotherapy only indicated
     if symptomatic/specific genetic mutations, radiotherapy
     to enlarged nodes.
    Prognosis – 1/3 never progress, 1/3 progress in time
     and 1/3 are actively progressing.
Chronic lymphocytic leukaemia
(CLL)
Hodgkins Lymphoma
    Malignant proliferation of lymphocytes.
    Reed – Sternberg cells.
    Two peaks of incidence, young adults and elderly.
    Increased risk – affected sibling, EBV, SLE, post transplantation,
     westernisation, obesity.
    Symptoms/Signs – enlarged non tender lymphadenopathy. ‘B’
     symptoms (weight loss, fever, night sweats), other systemic
     symptoms (lethargy, pruritus)
    SVCO emergency presentation (mediastinal involvement)
    Investigation - FBC, ESR, LFTs, LDH, lymph node biopsy, CT
     for staging (Ann Arbor system)
    Treatment – chemotherapy, radiotherapy or both.
    Prognosis – depends on stage and grade:
                  1a - >95%
                   4b - <40%
Non Hodgkins Lymphoma
    All lymphomas without Reed Sternberg cells.
    Overall incidence doubled since 1970 – (1:10,000)
    Causes – immunodeficiency (congenital/acquired), infection
     (EBV, H.Pylori) environmental toxins.
    Signs and symptoms – nodal disease, extranodal disease
     (oropharynx, skin, bone, gut, CNS, lung), systemic upset,
     pancytopenia due to marrow involvement.
    Investigations – FBC, U&Es, LFTs, LDH (increased = worse
     prognosis), lymph node biopsy, CT for staging (Ann Arbor)
    Treatment – dependent on disease subtype (basically
     chemotherapy +/- steroids)
    Prognosis – dependent on histology. Worse if age>60, systemic
     symptoms, bulky disease, increased LDH.
Myeloma
   Malignant clonal proliferation of B lymphocyte derived plasma cells.
  Incidence 5/100,000, peak age 70 years.
  Symptoms /signs:
   - osteolytic bone lesions – backache, pathological fractures.
   - Hypercalcaemia
     - Anaemia, thrombocytopenia, neutopenia
     - Recurrent bacterial infections
      - Renal impairment (light chain deposition)
      - Can present acutely with SCC, hyperviscocity, ARF.
    Investigation – check serum electrophoresis and ESR in all
     patients over 50 with back pain, xrays (punched out lesions,
     pepperpot skull), bone marow biopsy.
    Treatment – supportive (analgesia, bisphosphonates,
     vertebroplasty, fluids, dialysis), chemotherapy.
    Prognosis – median survival 3-4 years, death usually due to
     infection or renal failure.
Myeloma
Case Discussion
References

  NICE: CG27 – Referral Guideline for
   suspected cancer, Full Guideline. 14 July
   2005.
  Oxford Handbook of Clinical Medicine 8th
   Edition.
  www.cancerresearch .org.uk

								
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