Lymphoma suspected NS MOM

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Macmillan 2WW guidance

Scope − Hodgkin lymphoma (HL):

• this pathway covers the clinical presentation, investigation, diagnosis, and management of HL, including:
  • classification of lymphoma
  • investigations, such as:
    • immunophenotyping/immunohistochemistry
    • molecular studies in selected research patients
    • cytological studies such as bone marrow aspiration

Scope − non-Hodgkin lymphoma (NHL):

• this pathway covers the clinical presentation, investigation, diagnosis, and management of different subtypes of NHL, including:
  • low grade lymphoma:
    • follicular lymphoma (FL)
    • cutaneous T-cell lymphoma (CTCL)
    • chronic lymphocytic leukaemia (CLL)/small lymphocytic lymphoma (SLL)
  • high grade lymphoma:
    • diffuse large B-cell lymphoma (DLBCL)
    • Burkitt's lymphoma (BL)
    • primary mediastinal large B cell lymphoma (PMBL)
    • mantle cell lymphoma (MCL)
  • immunodeficiency associated lymphoma:
    • AIDS-related non-Hodgkin lymphoma
    • AIDS related primary central nervous system (CNS) lymphoma
    • post-transplant lymphoproliferative disorder (PTLD)
• this pathway does not cover:
  • extranodal lymphoproliferative disease
  • anaplastic large cell lymphoma
  • Waldenstrom’s macroglobulinaemia
  • splenic marginal zone lymphoma
  • nodal marginal zone B cell lymphoma
  • gastric lymphoma
  • lymphoblastic lymphoma

Scope − general:

• this pathway contains general information on:
  • chemotherapy regimes
  • radiotherapy regimes
  • CNS prophylaxis
  • complications of treatment, including tumour lysis syndrome, Hepatitis B reactivation, and progressive multifocal leukoencephalopathy (PML)
  • follow up, late effects, and protection of reproductive function
  • palliative care
• both primary and secondary care settings, in adults
• this pathway does not cover the care of lymphoma during pregnancy

Definition:

• HL is a cancer of lymphocytes involving lymph nodes and the lymphatic system that is diagnosed based on the recognition of Reed-Sternberg cells and/or Hodgkin cells in an appropriate cellular background [7,21]
• NHL are a heterogeneous group of cancers of lymphocytes originating in B-lymphocytes, T-lymphocytes, or natural killer (NK) cells [12]

Incidence and prevalence:

• HL:
  • annual incidence is about 3 per 100,000 in the UK [7]
  • bimodal distribution with peaks between ages 15-30 years, and over age 55 years [1]
  • early stage nodular sclerosis HL is the most common form in children in developed countries [7]
  • in Europe and the USA the incidence in adult men is double that of women, but in adolescents there is equal distribution between gender [7]
• NHL [12]:
  • DLBCL is the most common lymphoid neoplasm in adults:
    • annual incidence is 3-4 per 100,000
    • incidence increases with age
  • in the USA B-cell lymphomas represent 80-85% with 15-20% being T-cell lymphomas
  • NK-cell lymphomas are very rare

References:

[1] National Comprehensive Cancer Network (NCCN). Hodgkin Lymphomas 1.2013. NCCN; 2013.

[7] Rahemtulla A, Terpos E. Hodgkin's lymphoma. Clin Evid (online) 2009; pii: 2404.

[12] National Comprehensive Cancer Network (NCCN). Non-Hodgkin's Lymphoma 1.2013. NCCN; 2012.

[21] Practice informed recommendation. London; 2013.

Map of Medicine is not responsible for the correctness or accuracy of any content uploaded, referred to or linked to from the system.

Efforts are made to ensure the accuracy and agreement of these guidelines. However, we cannot guarantee this. This guidance does not override the individual responsibility of healthcare professionals to make decisions appropriate to the circumstances of the individual patient, in consultation with the patient and/or guardian or carer, in accordance with the mental capacity act, and informed by the summary of product characteristics of any drugs they are considering. Practitioners are required to perform their duties in accordance with the law and their regulators and nothing in this guidance should be interpreted in a way that would be inconsistent with compliance with those duties

 At time of referral please issue the patient information leaflet below:

BNSSG  2WW Patient Information Leaflet. - COVID-19 Edition

Link to leaflet on CCG website: https://bnssgccg.nhs.uk/library/understanding-two-week-wait-urgent-referrals-and-covid-19/

• 'Hodgkin's lymphoma' (URL) from Cancer Research UK at http://www.cancerresearchuk.org
• 'Hodgkin's lymphoma' (URL) from Macmillan Cancer Support at http://www.macmillan.org.uk
• 'Hodgkin's lymphoma' (PDF) from Patient UK at http://www.patient.info

 NSCCG Sign off form Jan 2018

Updated in line with NICE Guideline NG12: Suspected cancer: recognition and referral to coincide with updated  BNSSG 2WW Referral forms

Lymphoma Sign Off Form Sept 2016

Date of publication: 31-Oct-2013

This care map represents best clinical practice according to the highest quality evidence available, including:

• [1] National Comprehensive Cancer Network (NCCN). Hodgkin Lymphomas 1.2013. NCCN; 2013.
• [2] Engert A, Eichenauer DA, Dreyling M. Hodgkin's lymphoma: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol 2010; 21 Suppl 5: v168-v171.
• [3] The Royal College of Physicians (RCP), The Royal College of Radiologists (RCR). Evidence-based indications for the use of PET-CT in the UK 2012. London: The Royal College of Physicians (RCP) and The Royal College of Radiologists (RCR); 2012.
• [4] Clinical Knowledge Summaries (CKS). Neck lump. February 2010. New castle upon Tyne: CKS; 2010.
• [5] Clinical Knowledge Summaries (CKS). Bruising. March 2010. New Castle upon Tyne: CKS; 2010.
• [6] National Institute for Health and Care Excellence (NICE).Clinical guideline 156.  Fertility: Assessment and treatment for people with fertility problems. London: NICE; 2013.
• [7] Rahemtulla A, Terpos E. Hodgkin's lymphoma. Clin Evid (online) 2009; pii: 2404.
• [11] British Committee for Standards in Haematology (BCSH). Best practice in lymphoma diagnosis and reporting. London: BSCH; 2010.
• [12] National Comprehensive Cancer Network (NCCN). Non-Hodgkin's Lymphoma 1.2013. NCCN; 2012.
• [13] British Committee for Standards in Haematology (BCSH). Guidelines on the investigation and management of Chronic Lymphocytic Leukaemia. London: BCSH; 2013.
• [14] British Committee for Standards in Haematology (BCSH). Guidelines on the investigation and management of follicular lymphoma. London: BCSH; 2011.
• [15] Willemze R, Dreyling M. Primary cutaneous lymphomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol 2010; 21 Suppl 5: v177-v180.
• [16] National Institute for Health and Care Excellence (NICE). Rituximab for the first-line treatment of stage III-IV follicular lymphoma. NICE technology appraisal guidance 243. London: NICE; 2012.
• [17] Dreyling M, Ghielmini M, Marcus R et al. Newly diagnosed and relapsed follicular lymphoma: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol 2011; 22 Suppl 6: vi59-vi63.
• [18] British Committee for Standards in Haematology (BCSH). Guidelines for the investigation and management of mantle cell lymphoma. London: BCSH; 2012.
• [19] Tilly H, Vitolo U, Walewski J et al. Diffuse large B-cell lymphoma (DLBCL): ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol 2012; 23 Suppl 7: vii78-vii82.
• [24] British Committee for Standards in Haematology (BCSH). Guideline on the Management of Primary Resistant and Relapsed Classical Hodgkin Lymphoma. London: BCSH; 2013.
• [25] British National Formulary (BNF). BNF September 2013. London: BMJ Group and RPS Publishing; 2013.

Practice-based recommendations and literature endorsed by the contributors include:

• [20] Byrd JC, Furman RR, Coutre SE et al. Targeting BTK with ibrutinib in relapsed chronic lymphocytic leukemia. N Engl J Med 2013; 369: 32-42.
• [21] Practice informed recommendation. London; 2013.
• [22] Lopez-Olivo MA, Pratt G, Palla SL et al. Rasburicase in Tumor Lysis Syndrome of the Adult: A Systematic Review and Meta-analysis. Am J Kidney Dis 2013.
• [23] Wang ML, Rule S, Martin P et al. Targeting BTK with ibrutinib in relapsed or refractory mantle-cell lymphoma. N Engl J Med 2013; 369: 507-16.

 Clinical presentation [9]:

• Hodgkin lymphoma (HL):
  • typically presents with non-tender cervical and/or supraclavicular lymphadenopathy
  • natural history is long (months)
  • only a minority will experience constitutional or systemic symptoms, such as [21]:
    • B-symptoms – night sweats, weight loss, fever
    • itching
    • alcohol-induced lymph node pain
• Non-Hodgkin lymphoma (NHL):
  • typically more rapid progression of symptoms
  • may present with:
    • lymphadenopathy
    • breathlessness
    • superior vena cava (SVC) obstruction
    • abdominal distension

References:

[9] National Institute for Health and Care Excellence (NICE). Referral guidelines for suspected cancer. London: NICE; 2005.

[21] Practice informed recommendation. London; 2013.

 History:

• B-symptoms [1]:
  • night sweats – drenching
  • fever
  • weight loss – greater than 10% in 6 months
• fatigue [1,4]
• pruritis [1]
• bruising [4]
• bleeding [4]
• recurrent infections [4]
• bone pain [4]
• alcohol-induced pain [1,4]
• new lumps [4]
• risk factors:
  • Epstein-Barr virus (EBV) [7]:
    • association with Hodgkin lymphoma (HL) varies with age
    • is most common in children and older people
  • age – bimodal distribution with first peak age 15-30 years, and second peak greater than age 55 years [1]
  • HIV-positive [12]
  • immunosuppression
  • cigarette smoking – weak association with follicular lymphoma [21]

Examination:

• neck [4]:
  • anterior triangle
  • posterior triangle
  • midline
  • thyroid examination:
    • if a lump is present determine if it is nodular or diffuse
    • ask patient to swallow and note if lump moves – thyroid lumps and thyroglossal cysts will move up on swallowing
    • ask patient to protrude their tongue – thyroglossal cysts move superiorly
  • engorged neck veins and fullness of face in superior vena cava (SVC) obstruction [21]
• skin [5]:
  • pallor
  • jaundice
  • rashes – purpura, petechial haemorrhages
• chest [21]:
  • auscultation and percussion for pleural effusions
  • visible collateral veins on chest in SVC obstruction
  • pneumonia
• abdomen [5]:
  • splenomegaly
  • hepatomegaly
  • ascites
  • palpable masses [21]
• lymphadenopathy [4]:
  • location:
    • axillae
    • groin
    • cervical
    • supraclavicular
    • submandibular [21]
  • size
  • mobility
  • characteristics, eg compressible or pulsatile
• ear, nose, throat exam [4]:
  • should be performed if cervical lymphadenopathy is present, and/or if considering differential diagnoses, eg squamous cell carcinoma of the head and neck [21] 
  • ears – for infection, eg otitis externa
  • nose
  • oral mucosa and tongue – examine the lateral borders of the tongue and look for occult malignancy in oral cavity
  • tonsils and pharynx – enlarged tonsils [21], infection 

References:

[1] National Comprehensive Cancer Network (NCCN). Hodgkin Lymphomas 1.2013. NCCN; 2013.

[4] Clinical Knowledge Summaries (CKS). Neck lump. February 2010. New castle upon Tyne: CKS; 2010.

[5] Clinical Knowledge Summaries (CKS). Bruising. March 2010. New Castle upon Tyne: CKS; 2010.

[7] Rahemtulla A, Terpos E. Hodgkin's lymphoma. Clin Evid (online) 2009; pii: 2404.

[12] National Comprehensive Cancer Network (NCCN). Non-Hodgkin's Lymphoma 1.2013. NCCN; 2012.

[21] Practice informed recommendation. London; 2013.

 Investigation of patients with unexplained lymphadenopathy should include [4]:

• full blood count (FBC)
• C+Es
• LFTs
• LDH (Lactate Dehydrogenase)

Other investigations to consider:

• chest X-ray  – mediastinal or hilar mass warrant immediate referral [9]

References:

[4] Clinical Knowledge Summaries (CKS). Neck lump. February 2010. New castle upon Tyne: CKS; 2010.

[9] National Institute for Health and Care Excellence (NICE). Referral guidelines for suspected cancer. London: NICE; 2005.

 Immediate/emergency referral (within a few hours) is indicated in the following:

• children or young people with [9]:
  • hepatosplenomegaly
  • mediastinal or hilar mass
  • unexplained petechiae, particularly if accompanied by pallor, unexplained bruising, lymphadenopathy, persistent fatigue, fever, unexplained persistent infection - see Childhood Cancer - suspected pathway
• blood count/film reported as acute leukaemia [21]

References:

[9] National Institute for Health and Care Excellence (NICE). Referral guidelines for suspected cancer. London: NICE; 2005.

[21] Practice informed recommendation. London; 2013.

 
Arrange emergency admission

 Differential diagnoses [4]:

• inflammatory causes:
  • bacterial:
    • beta-haemolytic streptococci
    • Staphylococcus aureus
    • tuberculosis (TB)
  • viral:
    • Epstein-Barr virus (EBV)
    • herpes simplex virus (HSV)
    • cytomegalovirus (CMV)
    • HIV
  • parasitic, eg toxoplasmosis
  • fungal infections 
  • non-infective:
    • sarcoid
    • connective tissue diseases
• thyroid lumps
• malignant solid tumour [21], eg salivary, thyroid, parathyroid, teratomas [21], sarcomas, chondrosarcomas

References:

[4] Clinical Knowledge Summaries (CKS). Neck lump. February 2010. New castle upon Tyne: CKS; 2010.

[21] Practice informed recommendation. London; 2013.

 2 Week Wait referral is recommended if any of the following occur, or are present [8,9]:

• lymphadenopathy greater than 1cm, persisting for six weeks [8]
• lymph nodes are greater than 2cm in size [9]
• lymph nodes are non-tender, firm, or hard [9]
• lymph nodes are progressively enlarging [9]
• axillary lymph nodes are involved in the absence of local infection or dermatitis [9]
• supraclavicular nodes are involved [9]
• hepatosplenomegaly [8]

When considering referral, take into account any associated symptoms, particularly:

• unexplained high fever
• drenching night sweats (with or without weight loss)
• weight loss
• pruritus
• breathlessness
• alcohol-induced lymph node pain

2WW Haematology Services are available at Weston (WAHT), Bristol Haematology & Oncology Centre (UHB) and Southmead (NBT)

References:

[8] National Institute for Health and Care Excellence (NICE). Guidance on cancer services: improving outcomes in haemato-oncology cancer - the manual. London: NICE; 2003.

[9] National Institute for Health and Care Excellence (NICE). Referral guidelines for suspected cancer. London: NICE; 2005.

 While awaiting referral [9]:

• information should be given to patients and/or carers on issues such as:
  • where the patient is being referred to and what to expect from the service they will be attending
  • how long they will have to wait for an appointment
  • how to obtain information on the type of cancer suspected
  • who they will be seen by
  • the types of tests that will be performed
  • how long it will take to get a diagnosis
  • whether they can take someone with them to the appointment
  • other sources of support, including those for minority groups
• assess the need for continuing support
• invite the patient to contact you again
• follow current advice on communicating with patients and carer
• inform the specialist of any additional support needs, with the patient's agreement
• take into account personal circumstances, such as:
  • age
  • family and work responsibilities
  • isolation
  • other health issues
  • social issues

References:

[9] National Institute for Heal and Care Excellence (NICE). Referral guidelines for suspected cancer. London: NICE; 2005.

  
2WW Referral forms are available as EMIS templates