Lymphoma
Oncology Dx
3rd most common cancer (15 per 1,000,000)
lymphoreticular system
Lymphoma
HL
Presentation
CXR prior to Bx to assess for mediastinal mass (for any unexplained lymphadenopathy)
Excisional biopsy (not needle)
If confirmed
FBE, ESR, Ferritin, LFT`
CT chest / abdo / pelvis as part of staging
gallium scan or positron emission tomography (PET) scan to look for increased uptake (PET better)
BMA and biopsy to rule out metastasis
Bone scan if bone pain ro ALP elevation
Ann Arbor Staging Classification for Hodgkin Lymphoma
Rx
Chemotherapy +/- radiotherapy
Choice bases on stage + presents of B symptoms + mediastinal bulk
Radiation alone in low stage disease was effective but had significant long term side effects
Complete response = at least 70% reduction in disease on imaging and change to negativity on PET
Relapse
Low stage
Event free survival at 5y = 85%
Overall survival at 5y = 95%
NHL
Usually high grade and aggressive
Associated with
Presentation
Ix
FBE, EUC, CMP, LFTs, uric acid
BMA and biopsy
LP cytology, cell count and protein
CXR
CT neck, chest, abdominal, and pelvic (head CT if CNS disease)
PET scan
Tumor tissue (i.e., biopsy, bone marrow, CSF, or pleurocentesis/paracentesis fluid)
Flow cytometry: define cell type (T,B,null)
Cytogenetics: Karyotype
FISH, PCR for translocations, genes
Molecular profiling
Rx
TLS prevention
Multiagent systemic chemo + IT chemo
Radiotherapy only in some cercumstances
Surgery occasionally
Relapse
Reinduction
Stem cell transplant
Prognosis
3rd most common cancer (15 per 1,000,000)
lymphoreticular system
Lymphoma
- Hodgkin lymphoma (HL) (40%) (from the germinal center B cells)
- Nodular lymphocyte predominance
- Classical Hodgkin lymphoma
- Lymphocyte rich
- Mixed cellularity
- Nodular sclerosis
- Lymphocyte depletion
- non-Hodgkin lymphoma (NHL) (60%)
- Almost always B-cell origin
- Burkitt lymphoma (BL) (38%)
- diffuse large B-cell lymphoma (DLBCL) (20%)
- germinal center B-cell like (GCB) (the majority if DLBCL: favorable prognosis
- activated B cell–like (ABC) (poor prog)
- primary mediastinal B-cell (PMB) (poor prog)
- 80% T- cell origin, 20% B-cell (some ALCL are null cell origin)
- lymphoblastic lymphoma (LL) (29%)
- anaplastic large cell lymphoma (ALCL) (10%)
- Almost always B-cell origin
HL
- rare <10
- Most common cancer 15-19yo
- EBV confers 4 x increase in risk (?HHV6/CMV)
- Reed-Sternberg (RS) cell is pathognomonic (multiple or multilobulated nuclei) originate in germinal center B cells
- HL arises in lymphoid tissue and spreads to adjacent lymph node areas in a relatively orderly fashion
- Hematogenous spread also occurs, leading to involvement of the liver, spleen, bone, bone marrow, or brain, and is usually associated with systemic symptoms
Presentation
- painless, nontender, firm, rubbery, cervical or supraclavicular lymphadenopathy
- Usually some degree of mediastinal involvement
- hepatosplenomegaly is rarely
- airway obstruction (dyspnea, hypoxia, cough)
- pleural or pericardial effusion
- hepatocellular dysfunction
- bone marrow infiltration (anemia, neutropenia, or thrombocytopenia)
- B symptoms that are considered important in staging, are
- drenching night sweats
- weight loss >10% over 3 mo
- unexplained fever >39°C
- pruritus, lethargy, anorexia, or pain that worsens after ingestion of alcohol
CXR prior to Bx to assess for mediastinal mass (for any unexplained lymphadenopathy)
Excisional biopsy (not needle)
If confirmed
FBE, ESR, Ferritin, LFT`
CT chest / abdo / pelvis as part of staging
gallium scan or positron emission tomography (PET) scan to look for increased uptake (PET better)
BMA and biopsy to rule out metastasis
Bone scan if bone pain ro ALP elevation
Ann Arbor Staging Classification for Hodgkin Lymphoma
- I) Involvement of a single lymph node (I) or of a single extralymphatic organ or site (Ie)
- II) Involvement of two or more lymph node regions on the same side of the diaphragm (II) or localized involvement of an extralymphatic organ or site and one or more lymph node regions on the same side of the diaphragm (IIe)
- III) Involvement of lymph node regions on both sides of the diaphragm (III), which may be accompanied by involvement of the spleen (IIIs) or by localized involvement of an extralymphatic organ or site (IIIe) or both (IIIse)
- IV) Diffuse or disseminated involvement of one or more extralymphatic organs or tissues with or without associated lymph node involvement
Rx
Chemotherapy +/- radiotherapy
Choice bases on stage + presents of B symptoms + mediastinal bulk
Radiation alone in low stage disease was effective but had significant long term side effects
- (growth retardation, thyroid dysfunction, and cardiac and pulmonary toxicity)
- cyclophosphamide, procarbazine, vincristine or vinblastine, prednisone or dexamethasone, doxorubicin, bleomycin, dacarbazine, etoposide, methotrexate, and cytosine arabinoside
Complete response = at least 70% reduction in disease on imaging and change to negativity on PET
- (the 30% allows for some fibrosis)
- 80% complete response
- 50% cure
Relapse
- most within 3y
- reported up to 10y
Low stage
Event free survival at 5y = 85%
Overall survival at 5y = 95%
NHL
Usually high grade and aggressive
Associated with
- inherited or acquired immune deficiencies (e.g. severe combined immunodeficiency syndrome, Wiskott-Aldrich syndrome)
- viruses (e.g. HIV, EBV)
- genetic syndromes (e.g. ataxia-telangiectasia, Bloom syndrome)
Presentation
- Fever wt loss
- painless, rapid lymph node enlargement
- cough, superior mediastinal syndrome (SMS), dyspnea with thoracic involvement
- abdominal (massive and rapidly enlarging) mass, intestinal obstruction, intussusception-like symptoms, ascites with abdominal involvement;
- nasal stuffiness, earache, hearing loss, tonsil enlargement with Waldeyer ring involvement
- localized bone pain (primary or metastatic
- acute paraplegia from cord compression
- Tumour lysis syndrome
Ix
FBE, EUC, CMP, LFTs, uric acid
BMA and biopsy
LP cytology, cell count and protein
CXR
CT neck, chest, abdominal, and pelvic (head CT if CNS disease)
PET scan
Tumor tissue (i.e., biopsy, bone marrow, CSF, or pleurocentesis/paracentesis fluid)
Flow cytometry: define cell type (T,B,null)
Cytogenetics: Karyotype
FISH, PCR for translocations, genes
Molecular profiling
Rx
TLS prevention
Multiagent systemic chemo + IT chemo
Radiotherapy only in some cercumstances
Surgery occasionally
Relapse
Reinduction
Stem cell transplant
Prognosis
- Localised ~95% survival
- Advanced ~75%