Lymphoma Flashcards
What are the types of lymphoma?
Hodgkin - characterised by presence of Hodgkin/Reed Sternberg cells
Non-Hodgkin - more than 60 subtypes, B cell lymphomas most common
What are the types of Hodgkin lymphoma?
Classical - nodular sclerosis, mixed cellularity, lymphocyte rich and lymphocyte depleted
Nodular lymphocyte predominant
What are Reed-Sternberg cells?
HRS cells
Large multinucleate malignant cells
Described as having an owl like appearance
Hodgkin cells - mononuclear variant of these
What are the clinical features of HL?
Lymphadenopathy - painless, firm, enlarged nodes
Most commonly around neck
B symptoms - fever, night sweats, weight loss
Mediastinal mass - may be incidental finding on CXR, or present with SOB, cough, pain, SVCO
Pruritus
Hepatosplenomegaly
Malaise
Fatigue
What investigations are required for a diagnosis of HL?
Excision biopsy - FNA or core biopsy, of affected lymph nodes
Bloods - FBC, UE, LFT
Bone profile, LDH, uric acid
ESR, BBV, HTLV-1
Imaging PET/CT for staging CXR, CT, MRI if suspected CNS involvement LP, CSF analysis ECHO PFTs Bone marrow biopsy
What is Lugano staging?
System for classifying HL
Limited - Stage I and II
I - one node or group of adjacent nodes
II - two or more nodal groups on same side of diaphragm
Stage II BULKY
II with bulky disease
Advanced - III and IV
III - on both side of diaphragm, nodes above diaphragm with spleen involvement
IV - additional non-contiguous extra lymphatic involvement
What is ‘bulky disease’?
Typically refers to disease >10cm
What is the management of cHL?
Chemo and radio
ABVD common chemo regimen: Doxorubicin (A) Bleomycin (B) Vinblastine (V) Dacarbazine (D)
What is the function of doxorubicin?
Inhibits topoisomerase II
leads to inhibition of DNA and RNA synthesis
What are some of the side effects of the ABVD regimen?
A - cardiomyopathy, myelosuppression, skin reactions
B - pulmonary fibrosis, idiosyncratic reaction - hypotension, confusion, fever, wheeze
V - peripheral neuropathy, bladder atony
D - bone marrow suppression, hepatic necrosis
What treatment is given in early stage of HL?
Stage I/II - ABVD chemo
3-4 cycles
Advanced stage III/IV
ABVD
BEACOPP
Stanford V regimen
Radiotherapy may be used as an adjunct
For relapse -
Salvage chemotherapy
Radiotherapy
Autologous haematopoietic cell transplantation
Must receive irradiated blood for life for transfusion - reduce risk of graft versus host disease
What is the presentation of NHL?
Lymphadenopathy
Fever, WL, night sweats
Pruritus
Splenomegaly, hepatomegaly
High grade lots of features
Low grade more indolent, gradual lymphadenopathy
Each subtype has own pattern of clinical features
Dermatological, SVCO etc
Primary CNS lymphoma - neurological features, headache, confusion etc
Primary cutaneous lymphoma - rashes, plaques, ulcers
Primary GI tract lymphoma - abdo pain, nausea, obstruction
ONCOLOGICAL EMERGENCIES
SVCO Cord compression Hypercalcaemia TLS Neutropenic sepsis
What are the investigations for NHL?
Biopsy - excision preferred, FNA not adequate
Bloods
FBC, UE, LFT, bone profile
Uric acid, LDH, ESR, BBV
Imaging
CXR, CT chest abdo pelvis
PETCT, MRI, testicular USS
Bone scan
Bone marrow aspirate
Lumbar puncture
FISH
What is the staging of NHL?
Lugano staging
What is R-CHOP?
Regimen of chemo for NHL
Rituximab Cyclophosphamide Doxorubicin Vincristine Prednisolone
What are the side effects of rituximab?
Monoclonal antibody Activity against CD20 Infusion reactions Hep B reactivation Mucocutaneous reactions Leucoencephalopathy
What are the side effects of cyclophosphamide?
Alkylating agent Carcinogenic tendencies Linked to development of TCC of the bladder Bone marrow suppression Infertility
What is diffuse large B cell lymphoma?
Most common form of NHL
Slightly more common in women
Presents with rapidly enlarging mass
Commonly in the neck, abdomen or mediastinum
B symptoms, extranodal
Treatment is complex
Assessment for CNS
CNS prophylaxis
Limited stage - chemoimmunotherapy with R-CHOP
Advanced stage - R-CHOP
Autologous cell transplants
What is follicular lymphoma and its presentation?
B cell NHL
Second most common NHL
Insidious presentation
Gradual lymphadenopathy
Painless
85% have translocation on chromosomes 14-18
What is the treatment for follicular lymphoma?
Depends on Stage
Early can have radiotherapy
Cure not common in those with stage II, III and IV
Relapses frequently
Immunotherapy, chemo
What is Burkitt’s lymphoma?
High grade
Rapidly proliferating
B cell NHL
Commonly affects kids
Endemic - EBV, follows distribution of malaria
Sporadic - Europe, USA
Immunodeficiency - associated with AIDS and other conditions
What causes Burkitt’s lymphoma?
c-myc proto oncogene mutation
Due to a translocation between 8 and 14
What is the presentation of Burkitt’s?
Endemic - rapidly enlarging tumour in the jaw of a child
Enlarged nodes in neck, abdominal masses
Sporadic - abdominal symptoms, ileocaecal valve affected, bowel obstruction
Fever, weight loss, night sweats present in all
What is the management of Burkitt’s lymphoma?
Immunochemotherapy options
TLS prophylaxis is key
When does incidence of Hodgkins Lymphoma peak?
20-30
Smaller peak in old age
Describe the features of a Reed Sternberg Cell
Multi-nucleated Giant Malignant B-Cell Derivatives On a background of a variety of cells
What is a contiguous disease?
Only spreads to adjacent nodes and structures due to the flow of lymph
Describe Nodular Sclerosing Hodgkins Lymphoma
lacunar cells
young women
Describe mixed cellularity Hodgkins Lymphoma
+++Reed-Sternberg
lymphocytes, eosinophils, neutrophils, and histiocytes
no fibrotic bands
elderly
Describe lymphocyte rich Hodgkins Lymphoma
small lymphocytes
good prognosis
Describe lymphocyte depleted Hodgkins Lymphoma
associated with HIV
popcorn cells
worst prognosis
What are popcorn cells?
a variant of Reed Sternberg cells also seen in Nodular Lymphocyte Predominant HL
Of B cell lineage and express CD20
Also known as L&H hodgkin cells
What is Nodular Lymphocyte Predominant Hodgkins Lymphoma?
A non classical HL which has popcorn cells rather than Reed Sternberg cells
Has a different management
What is average 5 year survival for Hodgkins Lymphoma?
81%
What are poor prognostic indicators for Hodgkins Lymphoma?
Male >45 leucocytosis lymphocytopaenia low Hb low albumin
What are the sub-divisions of Non-Hodgkin’s lymphoma?
B Cell
T Cell
NK Cell
What are the subdivisions of B Cell Non-Hodgkin’s lymphomas?
High Grade
Low Grade
Give examples of high grade B Cell Non-Hodgkin’s lymphomas
Diffuse Large B Cell Lymphoma (DLBL)
Burkitt’s Lymphoma
Give examples of low grade B Cell Non-Hodgkin’s lymphoma
Follicular lymphoma
Chronic Lymphocytic Leukaemia
Mantle Cell Lymphoma
Identify risk factors for Non-Hodgkin’s lymphoma
Age - generally over 50 Prolonged immunosuppression: diabetes, steroids, HIV EBV H. Pylori Chlamydia autoimmune diseases: SLE, sjrogrens, RA
Apart from by cell type, how else can Non-Hodgkin’s lymphoma be classified?
By clinical behaviour:
Indolent - low grade
Aggressive - high grade
What is the prognosis of low grade non-hodgkins lymphoma?
incurable but compatible with a number of years survival
What is the prognosis of high grade non-hodgkins lymphoma?
rapidly fatal if untreated but modern treatment can cure in majority of cases
How do low grade non-hodgkins lymphomas present?
1 or more areas of painless lymphadenopathy
Hepatosplenomegaly
Bone marrow involvement (cytopaenia)
How are high grade non-hodgkins lymphomas treated?
What else is needed in the management of them?
R-CHOP
CNS prophylaxis - intrathecal methotrexate
Prevent tumour lysis syndrome: Rasburicase
Prevent herpes: aciclovir
Prevent pneumocystis: trimoxazole
Why must you be wary of low grade non-hodgkins lymphomas?
They can transform to high grade
