Lymphoid Malignancy Flashcards by Chloe Mackle

Features of lymphoma

Cancers of lymphoid origin
Can present with enlarged lymph nodes (lymphadenopathy) or with extra-nodal involvement or with bone marrow involvement
Systemic symptoms
> 50 different disease entities

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How can lymphoma present?

Lymphadenopathy
Extra-nodal involvement
Bone marrow involvement
Systemic symptoms

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What are the systemic symptoms associated with lymphoid malignancy?

Weight loss - > 10% in 6 months
Fever
Night sweats
Pruritis
Fatigue

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What is the disease entity in lymphoma defined by?

Malignant cell characteristics

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What will determine the type of lymphoma?

Biopsy - lymph node or bone marrow

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What does clinical examination and imaging of lymphoma tell us?

Where it is - not what it is

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What are the lymphoproliferative disorders?

Acute lymphoblastic leukaemia
Chronic lymphocytic leukaemia
Hodgkin lymphoma

Non-Hodgkin lymphoma

high grade (diffuse large B cell lymphoma)
low grade (follicular, marginal zone)

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Acute lymphoblastic leukaemia is a neoplastic disorder of

lymphoblasts

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How is ALL diagnosed?

By > 20% lymphoblasts present in bone marrow

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What is the incidence of acute lymphoblastic leukaemia?

1-2 per 100,000 population per year

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What percentage of cases of ALL occur in children < 6 years?

75%

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What percentage of cases of ALL are of B cell lineage?

75-90%

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What is the typical presentation of ALL?

Present with 2-3 week history of bone marrow failure or bone/joint pain

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What are the investigations of ALL?

FBC - Hb (low), WCC (high), platelets (high)
Bone marrow biopsy
Lymph node biopsy
Imaging

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What are the treatment options of ALL?

Chemotherapy 
Consolidation therapy 
CNS directed treatment 
Maintenance treatment 
Stem cell transplantation if high risk

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What are poor risk factors for ALL?

Increasing age
Increase WCC
Immunophenotype
Cytogenetics/molecular genetics - t(9;22), t(4;11) 
Slow/poor response to treatment

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What is the outcome for adults with ALL?

remission rate and leukaemia-free survival

Complete remission rate 78-91%

Leukaemia-free survival at 5 years 30-35%

What is the outcome for children with ALL?

5 year overall survival

5 year overall survival around 90%

Poor risk patients 5 year overall survival 45%

What is the typical treatment of ALL?

Multi-agent intensive chemotherapy +/- allogeneic stem cell transplant

How is CLL diagnosed?

Blood > 5 x 10^9/L lymphocytes
Bone marrow > 30% lymphocytes
Characteristic immunophenotyping - B cell markers (CD 19, 20, 23) and CD5 positive

What is the incidence of CLL?

> 1700 new cases per year in UK
Commonest leukaemia worldwide

Rare in far-East
Occasionally familial

What is the male:female incidence of CLL?

2 males:1 female

What is the clinical presentation of CLL?

Often asymptomatic at presentation

Frequent findings

bone marrow failure (anaemia, thrombocytopenia)
lymphadenopathy
splenomegaly (30%)
fever and sweats (< 25%)

Less common findings

hepatomegaly
infections
weight loss

What are the associated findings of CLL?

Immune paresis

Haemolytic anaemia

20% have positive direct anti-globulin test
8% have clinical evidence of haemolytic anaemia

What is the Binet staging of CLL?

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

What is the median survival of Binet stage A, B and C CLL?

A - same as age-matched controls B - around 8 years C - around 6 years

What are the indications for treatment of CLL?

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

What is the treatment of CLL?

Often nothing - watch and wait Cytotoxic chemotherapy e.g. fludarabine, bendamusine Monoclonal antibodies e.g. rituximab, obinutuzamab Novel agents - bruton tyrosine kinase inhibitor e.g. ibrutinib - PI3K inhibitor e.g. idelalisib - BCL-2 inhibitor e.g. venetoclax

What are the poor prognostic markers of CLL?

``` Advanced disease - Binet stage B or C Atypical lymphocyte morphology Rapid lymphocyte doubling time (< 12 months) CD38+ expression Loss/mutation of p53; del 11q23 Un-mutated IgVH gene status ```

``` 27 y/o male 1 month impaired vision in both eyes 1/2 stone weight loss Breathless on minimal exertion Hb 38 g/L WCC 370 x 10^9/L Platelets 68 x 10^9/L Bone marrow 90% B lymphoblasts ``` What is the likely diagnosis?

Acute lymphoblastic leukaemia

What is the presentation of lymphoma?

``` Lymphadenopathy Hepatosplenomegaly Extranodal disease B symptoms Bone marrow involvement ```

What are the B symptoms?

``` Fever Night sweats (drenching) Weight loss (> 10% in 6 months) ```

How is lymphoma assessed/staged?

Lymph node biopsy CT Bone marrow aspirate and trephine biopsy

What is the epidemiology of Hodgkin lymphoma?

30% of all lymphomas Bimodal age curve - 1st peak at 15-35 years - 2nd peak later in life

What is the male:female incidence of Hodgkin lymphoma?

1.9 males:1 female

What virus is Hodgkin lymphoma associated with?

Epstein Barr Virus

What is the treatment of Hodgkin lymphoma?

Combination chemotherapy +/- radiotherapy | Use of PET scan to assess response to treatment and limit use of radiotherapy

What is non-Hodgkin lymphoma classified according to?

Lineage - B cell or T cell, majority are B cell in origin Grade of disease - high or low Histological features of disease

What are the grades of non-Hodgkin lymphoma and their features?

Low Grade Lymphoma - indolent, often asymptomatic - responds to chemotherapy but incurable - median survival varies by subtype High Grade Lymphoma - aggressive, fast growing - requires combination chemotherapy - can be cured but varies widely

What are the commonest specific disease entities of lymphoma and their features?

Diffuse large B cell lymphoma - commonest subtype of lymphoma - high grade Follicular lymphoma - 2nd commonest subtype of lymphoma - low grade

What is the treatment of Non-Hodgkin lymphoma?

Combination chemotherapy - typically anti-CD20 monoclonal antibody and chemotherapy