Aani Haem: Lymphomas Flashcards
3 commonest sites for lymphomas?
Lymph nodes
BM
Blood
From which cell do lymphomas arise?
Lymphocytes
other Lymphoid tissue associated with lymphomas?
MALT
Spleen
Can occur anywhere e.g. breast lymphoma, CNA lymphoma etc
Why are lymphomas so common?
Because lymphocytes have genetic instability
3 Risk Factors for lymphomas?
- Antigenic stimulation (e.g. infections - H.Pylori etc)
- Direct Viral Integration e.g. HTLV1 (Caribbean/Japan) causes Adult T Cell Lymphoma/leukaemia
- Immunosuppression
Why are HIV patients more at risk of B cell lymphomas after EBV?
Immunosuppressed pts have lost T cell regulatory function
What usually prevents B cell lymphomas when you get EBV?
T cells normally attack the infected B cells so they cannot proliferate too much
What is the role of T cells?
They regulate B cells via cytokines
Direct cytotoxic function
What percentage of all lymphomas are Hodgkins?
20%
Which virus is associated with Hodgkins Lymphoma?
EBV
What is the peak incidence of Hodgkins Lymphoma?
Bimodal: 20-29 years (most common) and 60+ years
What are the symptoms of Hodgkins?
- Asymmetrical painless lymphadenopathy
- Pain after alcohol in affected nodes
- Obstructive symptoms e.g. compression of foodpipe
- Can get B symptoms (low grade fever/eight loss/sweats)
- Pel Ebstein Fever in 20% (cyclical)
Which cells are commonly seen in Hodgkin’s?
Reed-steinberg Cells (binucleate owl-eyed cells) - seen on a background of lymphocytes & reactive cells
Most common subtype of Hodgkins?
Nodular Sclerosing
Subtypes of Hodgkins?
Nodular Sclerosing
Mixed cellularity
Lymphocyte depleted
Lymphocyte Rich
How is the staging done for Hodgkins?
1 - ONE LN region affected
2 - 2+ LNs on same side of diaphragm
3 - 2+ LNs on opposite side of diagram
4 - Extranodal sites e.g. Live/BM
Add B if they have B constitutional symptoms
What is the management for Hodgkin’s Lymphoma?
ABVD chemo Adriamycin Bleomycin Vinblastine Decarbazine
How many cycles of chemo needed for stage 1-2 Hodgkin’s?
2-4 cycles
How many cycles of chemo needed for stage 3-4 Hodgkin’s?
6-8 cycles
What is the name for SC transplant that comes from patient’s own body?
Autologous
What is the name for SC transplant that comes from HLA-matched donor?
Allogenic
How do you treat someone with relapsing Hodgkins?
Aggressive Chemo + Autologous SCT
Which lymphoma gives you pain after alcohol?
Hodgkin’s Lymphoma (NOT NHL!)
Symptoms of Non-Hodgkin’s Lymphoma?
Varies between subtypes. Generic: Painless lymphadenopathy Asymmetrical No pain after alcohol Constitutional symptoms
What are the types of B-Cell NH Lymphomas?
Follicular MALT Diffuse Mantle Burkitt's
What is a mnemonic to remember the B-Cell NH Lymphomas in order of indolent to aggressive?
Fairies Make Dust on the Mantle place Burkitt's (I know that's the most aggressive)
Which is the least responsive to therapy of the B cell NH Lymphomas?
Follicular (fairies are innocent/indolent but MIGHT resistant)
Which oncogene is expressed in Burkitt’s lymphoma?
C-myc
(MIKE (Myc) BURKITT) - the burkitt monster
Physical symptoms of Burkitt’s?
Jaw involvement and abdo mass
Describe the histology appearance of Burkitt’s?
Starry Sky (Burkitt’s monster comes out at night when the stars are out)
What is the translocation in Burkitt’s lymphoma?
t(8;14) - B looks like 8.
What is the translocation in Mantle Cell lymphoma?
t(11;14) (Mantlle ll looks like 11. 11 and 14 cups on the mantle place)
What is the translocation in Diffuse Large B Cell lymphoma?
t(14;18) (you look DIFF between aged 14 and 18)
What is the translocation in MALT/marginal zone lymphoma?
t(11;18) - drink MALT at secondary school (aged 11-18)
What is the translocation in Follicular lymphoma?
t(14;18) (Follicular starts with F). Same as Diff. Cos you get follicles on your face
What histology do you see in Mantle Cell lymphoma?
Angular Nuclei
Why do people in Africa get Burkitt’s lymphoma?
Because in places where Malaria is high, P falcuparum wears down EBV resistance
What histology do you see in Diffuse Large B cell lymphoma?
Sheets of large lymphoid cells
What histology do you see in Follicular lymphoma?
Nodular/follicular appearance
What are 2 causes of MALT/Marginal Zone Lymphoma?
Causes by chronic antigen stimulation commonly by:
- H.Pylori –> gastic MALT lymphoma
- Sjogren’s Syndrome –> Parotid lymphoma
(TIP: Drink MALT at school t(11;18). It was tasty so you drooled- parotid. But you got a tummy bug sadly - H.Pylori).
Which gene is affected in Follicular B Cell NH Lymphoma?
BCL 6+ gene (t;14-18)
Treatment of Follicular B Cell NH Lymphoma?
- Wait + Watch
2. Rituximab
Treatment of Marginal Zone/MALT NH Lymphoma?
- Remove antigenic stimulation
- Triple therapy for H.Pylori: amoxicillin, clarithromycin, and a proton pump inhibitor such as omeprazole, lansoprazole
Treatment of Diffuse Large B-Cell NH Lymphoma?
- Rituximab - CHOP
(So diffuse you need to chop it) - Auto-SCT for relapse
Treatment of Mantle Cell NH Lymphoma?
- Rituximab - CHOP
(So diffuse you need to chop it) - Auto-SCT for relapse
Treatment of Burkitt’s NH Lymphoma?
- Rituximab
2. Auto-SCT
Which lymphomas are associated with EBV
Hodgkin’s
AND Burkitt’s B-Cell Non-Hodgkins
Which cellular protein is increased in Mantle Cell NH Lymphoma?
Cyclin D1
Increased Cyclin D1 is associated with which lymphoma?
Mantle Cell NH Lymphoma
Which demographic are most affected by Mantle Cell NH lymphomas?
Middle Aged Men (man = mantle cell)
What is Rituximab?
Anti CD20 Monoclonal Ab. CD 20 is found in B-Cells so Rituximab is used in B Cell NH Lymphomas
What percentage of Lymphomas are Non-Hodgkins?
80%
What are the categories of NH Lymphomas?
B-Cell derived or T-Cell derived
What will B cells begin to express in NH lymphomas?
CD5 and CD23 (which they shouldn’t normally express)
What is Alemtuzumab? When is it used?
Anti CD52 - used in CLL and T-Cell NH Lymphoma
What investigations do you do for Hodgkin’s Lymphoma?
CT/PET scan and LN/BM biopsy - especially useful for staging
Mnemonic to remember T-Cell NH Lymphomas?
Ana’s CAPE:
Anaplastic Large Cell Lymphoma Cutaneous T Cell Lymphoma Adult T Cell Lymphoma Peripheral T Cell Lymphoma Enteropathy Assoicated T Cell Lymphoma (EATL)
Which infection is ass. with Adult T Cell NH Lymphoma?
HTLV-1 viral infection
Which age group are affected by Anaplastic Large Cell lymphomas?
Children & young adults (like Ana)
Which protein is seen in 30% of Adult-T Cell NH Lymphomas?
Philadelphia Chromosome
Which cells are seen in Anaplastic Large T Cell lymphomas?
Large Epitheloid cells
Which ethnic groups are prone to getting Adult T-Cell Lymphomas?
Japanese and Caribbean because HTLV-1 is found there
What skin infection is ass. with cutaneous T Cell lymphoma?
MYCOSIS FUNGOIDES
What condition is ass. with Enteropathy Associated T Cell Lymphoma (EATL)
long standing Coeliac Disease
Which Hodgkins Lymphoma has the best prognosis?
Lymphocyte predominant
Which Hodgkins Lymphoma has the worst prognosis?
Lymphocyte depleted
What are the 2 types of Burkitt’s lymphoma?
- endemic (African) form: typically involves maxilla or mandible
- sporadic form: abdominal (e.g. ileo-caecal) tumours are the most common form. More common in patients with HIV
Explain the Starry Sky appearance in Burkitt’s lymphoma?
lymphocyte sheets interspersed with macrophages containing dead apoptotic tumour cells
What is a side effect of treating Burkitt’s lymphoma with chemo?
Tumour lysis syndrome
What are complications of tumour lysis syndrome?
hyperkalaemia hyperphosphataemia hypocalcaemia hyperuricaemia acute renal failure