Leukaemia's and lymphomas brief Dr Amin Islam MB, MRCP UK, FRCPath UK Consultant Haematologist. SUHFT GP Refresher course 3 rd October 2016 Education Centre Southend University Hospital NHS FT Trust.

Leukaemia's and lymphomas An overview of Common symptoms Presentations When to refer Common types Diagnosis Treatments prognosis

Common symptoms lymphomas  Significant weight loss and night sweats  Loss of 10% body weight over 6 months  Drenching night sweats  Asymptomatic lumps  Commonly neck, axillae, groin  Mass can be over soft tissues  Confusion with soft tissue sarcomas/tumours

presentations  Can be rapid or  Gradual onset Occasional  SVC obstruction  Breathlessness due to pleural effusion  Or collapse due to tracheal compression external  Dysphagia due to oesophageal compressions  Bowel obstruction  Jaundiced due to porta hepatis nodes or liver involvements

cont.  Bilateral leg oedema due to pelvic mass Occasional presentation  Headache and posterior fossa signs symptoms  Back pain  Cord compressions  Urinary obstructions  Autoimmune haemolytic anaemia's

When to refer  Difficult some times to  Which specialty to refer?  If symptoms are suggestive or suspicious of lymphomas  Please refer or discuss with us via switch  Urgent ED referral in case of suspected SVCO or respiratory compromise or acute obstructive pictures as above

Common types of lymphomas Broadly speaking  Hodgkin's lymphomas  Non Hodgkin's lymphomas  Small lymphocytic lymphomas (SLL)

Hodgkin's  4 subtypes but not graded  All treated as same  Outcome varies slightly according to subtypes  Commonest: nodular sclerosis  Good prognosis: NS  >90% are cured now a days

Non Hodgkin's  High grades and  Low grades  Several subtypes For treatment purposes  Only high grades and low grades are important  Also whether T cell or b cell subtypes are important

Commonest types of NHL High grades  Diffuse large B cell subtypes are the commonest  Angio immunoblastic T cell lymphomas  Anaplastic large cell lymphomas  Burkitts Lymphomas

Cntd. Low grades  Follicular NHL commonest  Mantle cell lymphomas  Lymphoplasmocytic lymphomas (WM)  Marzinal zone lymphomas  MALT lymphomas

Other two types  Small lymphocytic lymphoma SLL  This is a type of CLL but Lymphocytes are <5.0  Diagnosis is base on LN tissue biopsy not from liquid blood flow cytometry  Treatment is same as CLL

Cntd.  Nodular lymphocytes predominant hodgkins lymphomas  Previously treated as HL  But now a new entity  Treatment and prognosis are different  Treatment as B cell HNL

Diagnosis  All needs tissue from the involved filed  CT or increasingly PET CT scan for staging  Blood test as part of staging  LDH and B2 microglobulins prognostics factors  Cytogenetic and molecular  Bone marrow test for staging but not needed for Hodgkin's as PET is sufficient.

Cntd.  Viral serology mandatory  Hep B, C, HIV  Pulmonary function test for ABVD HL  MRI and CSF if neurological symptoms  ECHO as baseline over 40 or cardiac history

Treatments  Hodgkin's  All receives ABVD chemotherapy  Limited disease and stage 1-2 A treated with  Courses of chemotherapy then IF radiotherapy  Advanced stage and stage 1-4 B  All receives at least 6 ABVD chemotherapy  No role of HSCT upfront at 1 st remission

Non Hodgkin's  High grade B cell  R-CHOP  CHOP for T cell lymphomas  3-4 courses for stage A 1-2 disease the IF RT  Advanced stage 3-4 or B at least 6 courses  Role of HSCT at 1 st remission in case of T cell NHL

Low grade  For B cell  Bendamustine and Rituxmab B-R standard  Watch and wait or R chemo for low grade if treatment indicated  Idelalisib or clinical trials options

Relapse setting Depends on  Age, PS and stage, types of lymphomas  Intensive salvage chemo  Leading to ASCT if in remission  Or clinical trials options

prognosis  Excellent in high grade NHL DLBCL % cures if treated with intensive chemo R-CHOP  T cell: poor only in remission but relapse is invariable  Consolidate with ASCT at 1 st remission  Low grade lymphomas  Overall response 50-70% or palliative approach if frail

GK DLBCL feb 2015

GK may 2016

KR april 2016

AA july 2016

leukaemia's An overview of Common symptoms Presentations When to refer Common types Diagnosis Treatments prognosis

Common symptoms  Sudden or  Gradual onset, non specific abdominal discomfort due to splenomegally  Symptoms of anaemia's  Petechial rash due to thrombocytopepnia  Jaundiced due to ineffective erythropoiesis  Infections such as pneumonia  Chest symptoms due to either infection or  life threatening pulmonary leucostasis and AML  Pyrexia and occasional neurological symptoms

presentation  Majority acute leukaemia's are picked up by the laboratory  Confirmed by consultant haematologist  Usually have blood test for being unwell or hospital admissions etc  Occasionally dramatic with multi organ failure and DIC  Chest and neurological events  CML usually non specific and abdominal symptoms  CLL asymptomatic mainly blood test for others

When to refer  Majority acute leukaemias are picked up in the lab and patient/GP contacted urgently Chronic  High WCC counts with film comments suggestive  Children with non specific any cytopenia, bone pains and blood film comments to refer  Lymphocytes >5 and persistent CLL common for adults usually >60 yrs  Any blood film suggest to contact please discuss

Common types Acute  Commonest AML denov  MDS-AML  Rare ALL in children rare in adults Chronic CLL very common >70 yrs up to 10%  CML 3-4 cases in southend per year

Rare types  T and B PLL  A TLL associated with HTLV1 virus

Diagnosis  Blood film usually specific  Immunophenotyping to confirm the types  Molecular and cytogenetic to risk stratify  cytogenetic has prognostic values  Bone marrow test  CSF and MRI if neurological involvement rarely needed

treatments AML Intensive chemotherapy at Barts DA 3+7 We are trying to get AML intensive back to southend collaboration with Barts  Non intensive  MAC locally in southend or palliative best supportive care majority elderly

 ALL rare in adult Intensive at Barts with UK ALL protocols Children at GOS ALL protocol for children's Elderly Best supportive care

CLL  FCR is standard  BR for elderly or RP Relapse setting  Ibrutinib and idelalisib options  Clinical trials option available

T cell  Campath  Ideally treated at RMH or Barts  Poor prognosis

Prognosis  Excellent in childhood ALL>97 cured  AML according to molecular and cytogenetics  Overall 50% long term survival  Elderly  Outcome poor overall  Some countries 10% elderly survive at 5 years

aml

ALL

CLL

CML