Lymphoid Malignancy Flashcards by Laura M

What are lymphomas?

Cancers of lymphoid origin

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How do lymphomas present?

Enlarged lymph nodes
(Extranodal involvement)
(Bone marrow involvement)
Systemic symptoms

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Systemic symptoms associated with lymphoma

Weight loss
Fever
Night sweats
Pruritis
Fatigue

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How is a lymphoma/leukaemia diagnosed?

Biopsy

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How is a lymphoma/leukaemia staged?

Clinical examination and imaging - where it is

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Which cancer is associated with lymphoid progenitor cells?

ALL

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Which cancer is associated with B cells and T cells?

Lymphomas/CLL

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Which cancer is associated with plasma cells?

Multiple myeloma

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What are the types of lymphoma?

Hodgkin lymphoma

Non hodgkin lymphoma

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What are the types of non Hodgkin lymphomas?

High grade

Low grade

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Lymphoproliferative disorders

Hodgkin lymphoma
Non-Hodgkin lymphoma
CLL
ALL

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What is ALL?

Cancerous disorder of lymphoid progenitor cells

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What normally happens with lymphoid progenitor cells?

Immature, rapidly differentiateinto lymphocytes

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What normally happens with lymphoid progenitor cells in leukaemia?

No differentiation - instead, rapid, uncontrolled growth and accumulation (usually in bone marrow but they can go anywhere)

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How does ALL present?

2-3 week history of bone marrow failure or bone/joint pain
Infection
Sweats
+/- raised white cell count

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Laboratory results in ALL

Decreased Hb
Decreased platelets
Increased white cell count

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What is the main cell type involved in ALL?

B cell lineage

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ALL cell characteristics

Large cells
Express CD 19 (contained by all B cells)
CD34, TDTD (markers of early, immature cells)

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ALL treatment

Chemo for remission
Consolidation therapy
CNS directed treatment
Maintenance treatment for 18 months

Stem cell transplantation if high risk

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Newer treatment ALL

Bi-specifc T-cell engagers

CAR (chimeric antigen receptor T-cells)

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T-cell immunotherapy side effects

Cytokine release syndrome

Neurotoxicity

Cytokine release syndrome

Fever
Hypotension
Dyspnoea

Neurotoxicity

Confusion with normal conscious level
Seizure
Headache
Focal neurology
Coma

ALL poor prognostic factors

Increasing age
Increased white cell count
Slow/poor response to treatment

What is the difference between cells in ALL and CLL?

Abnormal cells in CLL are very mature - usually resemble normal lymphocytes

What lymphocyte count is required for a diagnosis of CLL?

>5 (normal is <4)

CLL presentation

Often asymptomatic Bone marrow failure Lymphadenopathy Splenomegaly Fever and sweats

CLL associated findings

Immune paresis | Haemolytic anaemia

Immune paresis

Loss of normal immunoglobulin production

Binet CLL staging

A - <3 lymph node areas B - 3 or more lymph node areas C - stage B + anaemia or thrombocytopenia

CLL indications for treatment

``` Progressive bone marrow failure Massive lymphadenopathy Progressive splenomegaly Lymphocyte doubling time <6 months or >50% increase over 2 months Systemic symptoms Autoimmune cytopenias ```

CLL treatment

Often watch and wait Cytotoxic chemotherapy Monoclonal antibodies Novel agents

Cytotoxic chemotherapy agents

Fludarabine | Bendamustine

Monoclonal antibody agents

Rituximab | Obinatuzumab

Novel agents CLL

Bruton tyrosine kinase inhibitor

CLL poor prognostic factors

``` Advanced disease (Binet stage B or C) Atypical lymphocyte morphology Rapid lymphocyte doubling time (<12 mth) CD 38+ expression Loss/mutation p53; del 11q23 (ATM gene) ```

Presentation of lymphoma

Lymphadenopathy/ hepatosplenomegaly Extranodal disease “B symptoms” Bone marrow involvement

How is lymphoma staged?

Stage 1-4 => increasing number of sites A - absence of B symptoms B - Fever, night sweats, weight loss

How is non Hodgkin lymphoma classified?

Lineage - B cell or T cell (90% B cell) | Grade - high/low

Low grade lymphoma

Indolent, often asymptomatic | Responds to chemotherapy but incurable

High grade lymphoma

Aggressive, fast-growing Require combination chemotherapy Can be cured

What are the two main types of non Hodgkin lymphoma?

Diffuse large B-cell lymphoma | Follicular lymphoma

Diffuse large B-cell lymphoma

High grade | Commonest subtype of lymphoma (of any kind)

Follicular lymphoma

Low grade lymphoma

What is Hodgkin lymphoma associated with?

EBV | Familial and geographical clustering

Hodgkin lymphoma treatment

Combination chemotherapy +/- radiotherapy ``` Monoclonal antibodies (anti-CD30) Immunotherapy (checkpoint inhibitors) Stem cell transplant ```

Hodgkin lymphoma presentation

``` Systemic symptoms => Night sweats Unintentional weight loss Fever SOB/persistant cough ``` Swelling in neck, armpit or groin

High grade NHL

Diffuse large B-cell lymphoma

Low grade NHL

Follicular, marginal zone

Poor cancer prognosis factor

loss/mutation of p53

What is p53?

Tumour suppression gene

Lymphoma staging

A - absence of B symptoms B - fever, night sweats, weight loss Stage 1 - 1 node Stage 2 - more than one node but on the same side of the diaphragm Stage 3 - no extranodal involvement but both sides of the diaphragm Stage 4 - extranodal involvement