Haem: Lymphomas 2 - CLL and quiz Flashcards
Why do we need to check LDH and B2 microglobulin in lymphomas?
Give an indication about how aggressive the lymphoma is and cell turnover (LDH is a marker of cell turnover
Why do you need a HIV serology in lymphomas?
as loss of T cell function can increase the incidence of B cell lymphomas
Why do you need a Hep B serology in lymphomas?
If B cell depleting therapy is carried out and the patient is a carrier of Hepatitis B, they may run risk of fatal liver failure due to reactivation of Hepatitis B
How will women normally present with HL?
Nodular sclerosing subtype
Cervical and mediastinal lymphadenopathy
How can HL present in general?
Patients present with PAINLESS enlargement of lymph node/nodes
This may cause obstructive symptoms/ signs
Great vessels (IVC, SVC, etc.)
Tracheal obstruction
Oesophageal obstruction
Bile duct obstruction
Constitutional symptoms (B symptoms):
Fever
Night sweats (drenching)
Unexplained weight loss (10% in 6 months)
+ pruritis may be present
What are the 4 subtypes of classical HL (cHL)?
Nodular sclerosing
Mixed cellularity
Lymphocyte rich
Lymphocyte depleted
How is cHL staged?
FDG-PET/CT scan leading to Ann-Arbor staging
How does cHL spread?
Contiguously
What are the different stages in cHL?
1 - One group of nodes
2 - >1 group of nodes same side of diaphragm
3 - Nodes above and below the diaphragm
4 - Extra nodal spread
A - If no systemic symptoms
B - If you have FLAWS
How is HL treated?
Chemotherapy - ABVD
Follow up with PET CT to see how effective treatment has been
What are the long term side effects of cHL?
Pulmonary fibrosis
Cardiomyopathy
Why is radio AND chemo not advised in terms of treatment?
Combined modality treatment (radiotherapy AND chemotherapy) leads to a VERY HIGH risk of secondary malignancy
Is the risk of relapse a bigger risk than secondary malignancy after treatment?
Until 10 years post treatment, risk of relapse is bigger, then secondary malignancy is the bigger risk
What are the 3 subtypes of NHL in terms of prognosis?
Very aggressive - Easier to treat lol
Aggressive
Indolent - Harder to treat lmao
What is the main difference in the way HL and NHL present?
Hodgkin’s tends to be contiguous and patients present with mediastinal masses. NHL is more widespread.
What are some very aggressive NHL?
Burkitt’s
B or T cell lymphoblastic leukaemia/lymphoma
What are some aggressive NHL?
Diffuse large B cell (DLBCL)
Mantle cell
What are some indolent NHL?
Follicular
CLL
MALT
How is DLBCL treated?
6-8 cycle of R-CHOP
If they relapse consider autologous stem cell transplant
What is follicular NHL associated with?
t 14:18 translocation resulting in over espression of bcl-2
How is follicular NHL treated?
Watch and wait
Only treat if clinically indicated like:
- compression due to nodes e.g. bowel, ureter, vena cava
- massive painful nodes, recurrent infections
What is Extranodal Marginal Zone Lymphoma?
A marginal zone NHL involving extranodal lymphoid tissue e.g. MALT
What are some conditions associated with Extranodal Marginal Zone Lymphomas?
Sjogren’s syndrome- parotid lymphoma
Hashimoto’s thyroiditis- thyroid MZL
Psittaci infection- lacrimal gland
H. pylori- gastric MALToma
Where do Hy Pylori gastric MALTs usually arise and how don they present?
In the stomach
Usually present with dyspepsia, epigastric pain, ulceration, or bleeding
What is Enteropathy Associated T cell Lymphoma (EATL) and how does it present?
T cell NHL associated with Coeliac disease
What cells does EATL involve and where does it occur?
Mature T cells
Small intestine
How does EATL present?
Abdominal pain, obstruction, perforation, GI bleeding
Malabsorption
Systemic symptoms
Responds poorly to chemotherapy
What cells does CLL involve and what kind of mutation is it associated with?
Mature B cells
Acquired sporadic mutation
What are the lab findings in CLL?
LYMPHOCYTOSIS between 5-300 x 109/L
Smear cells present- Artefactual problems as the cells break down (damaged by smearing)
Normocytic normochromic anaemia
Thrombocytopaenia
Bone marrow lymphocytic replacement of normal marrow elements- due to lots of infiltration
What cell markers do normal mature B cells express?
CD19
What cell markers do normal mature T cells express?
CD 3
CD 5
Cd 4 or 8 depending on whether its a cytotoxic cell or helper cell
What cell markers do CLL B cells express?
CD 5 - Should never be expressed by B cells
CD 19
What indicated poor prognosis in CLL?
CD 38 expression
IgH unmutated
Deleted 17p (TP53)
What causes increased risk of infection in CLL?
Malignant and non functional mature B cells cannot secrete polyclonal immunoglobulins, leading to hypogammaglobulinaemia
What causes bone marrow failure in CLL?
Proliferation of B cells in bone marrow causing effacement of bone marrow
What causes lymphadenopathy and splenomegaly in CLL?
B cells circulating to nodes, spleen and blood
What is Richter transformation and what causes it in CLL?
Transformation into a higher grade lymphoma
Further acquired mutations cause this
What other diseases can CLL cause?
Autoimmune diseases such as haemolytic anaemia
Which vaccines are not given to CLL patients?
Live vaccines such as VZV vaccine
What supportive treatments can be given to CLL patients?
Vaccines
Acyclovir
Pneumocystis jiroveci pneumonia prophylaxis with pentamidine for those who are immunosuppressed
IVIg recommended for those with hypogammaglobulinaemia and recurrent bacterial infections
What are the treatment options for CLL
Chemo if possible otherwise watch and wait
What is the aim of treatment?
Establish remission
What are the indications for treatment?
Progressive lymphocytosis (count doubling < 6 months)
Progressive bone marrow failure
Hb < 100
Platelets < 100
Neutrophils < 1
Massive or progressive lymphadenopathy/ splenomegaly
Systemic symptoms (B symptoms)
Autoimmune cytopaenias (treat with steroids)
How does venetoclax cure CLL?
BCL 2 protein inhibitor
How does Rituximab cure CLL?
Targets CD20 Ag on B cells
How does Ibrutinib cure CLL?
BCR kinase inhibitor
