Lymphoproliferative disorders Flashcards

1
Q

What can lymphoid cancers present with?

A

Enlarged lymph nodes OR extra nodal disease OR bone marrow involvement
May have systemic symptoms

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2
Q

What are the types of lymphoproliferative malignancies?

A

Hodgkin lymphoma
Non Hodgkin lymphoma
Acute lymphoblastic leukaemia
Chronic lymphocytic leukaemia

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3
Q

What is the diagnostic feature of ALL?

A

> 20% lymphoblasts present in bone marrow

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4
Q

Who are the majority of ALL cases seen in?

A

75% under 6

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5
Q

What is the presentation of ALL?

A

2-3 weeks history bone marrow failure or bone/joint pain

Infection, sweats

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6
Q

What investigations are done for ALL?

A

FBC
Bone marrow aspiration
LP if CNS involvement

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7
Q

What is the standard treatment of ALL?

A

Chemo- indiction, consolidate and maintenance

CNS directed treatment

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8
Q

What is the treatment for high risk ALL?

A

Standrad therapy + Stem cell transplant

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9
Q

What newer therapies are available for ALL?

A

Bispecific T cell engagers

CAR T cells

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10
Q

What are CAR T cells?

A

Genetically engineered T cells that have certain receptors to attack cancer

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11
Q

What are the possible side effects of CAR T cells?

A

Cytokine release syndrome

Neurotoxicity

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12
Q

What are the symptoms of cytokine release syndrome?

A

Fever
Hypotension
Dyspnoea

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13
Q

What symptoms can neurotoxicity in CAR T cell therapy cause?

A
Confusion
Seizure
HEadahe
Focal neurology
Coma
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14
Q

What are the indicators for a bad prognosis in ALL?

A
Increasing age
Increased WCC
Immunophenotype
Molecular genetics
Slow/poor response to treatment
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15
Q

What are the diagnostic features of CLL?

A

Bone marrow >30% lymphocytes

Blood >5x10^9 lymphocytes

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16
Q

Who is CLL mainly seen in?

A

Males

17
Q

What are the common presentations of CLL?

A
Asymptomatic
Bone marrow failure
Lymphadenopathy
Spelnomegaly
Fever and sweats
18
Q

What are the less common presentation of CLL?

A

Hepatomegaly
Infections
Weight loss

19
Q

What is the staging of CLL?

A
A= <3 lymph node areas
B= 3 or more lymph node areas
C= Stage B and anaemia or thrombocytopenia
20
Q

What is the prognosis for CLL?

A
A= good
B= ~8 years
C= ~6 years
21
Q

What are the indications for treatment of CLL?

A
Progressive bone marrow failure
Massive lymphadenopathy
Progressive splenomegaly
Lymphocyte doubling time <6 months, or >50% increase over 2 months
Systemic symptoms
Autoimmune cytopenias
22
Q

What is the treatment of CLL?

A

Watch and wait
Cytotoxic chemo
Monoclonal antibodies
Novel agents

23
Q

What gives CLL a bad prognosis?

A
Advanced disease
Atypical lymphocyte morphology
Rapid lymphocyte doubling time
CD38+ expression
Loss/mutation of p53
Unmutated IgVH gene status
24
Q

What is the presentation of lymphoma?

A
Lymphadenopathy
Hepatosplenomegaly
Extra nodal disease
Systemic disease
Bone marrow involvement
25
Q

How is lymphoma assessed?

A

Lymph node biopsy
CT
Bone marrow aspirate

26
Q

What is the numerical staging of lymphoma?

A

1- localised
disease
2- 2 or ore lymph node regions on same side of diaphragm
3- 2 or more lymph node regions on opposite side of diaphragm
4- Extranodeal disease

27
Q

What is the letter staging of lymphoma?

A

A- no systemic features

B- fever, night sweats, weight loss

28
Q

How can Non Hodgkin lymphoma be classed?

A

Lineage
Grade of disease
Histological features of disease

29
Q

How is non Hodgkin classed by lineage?

A

B cell or T cell- mainly B

30
Q

How is Non hodgkin classed by grade?

A

Low grade- indolent, often asymptomatic, responds to chemo but incurable
High grade- aggressive, require combination chemo, can be cured

31
Q

What are 2 specific disease entities within Non Hodgkin?

A

Diffuse large B cell lymphoma

Follicular lymphoma

32
Q

What is diffuse large B cell lymphoma?

A

Most common type of non Hodgkin
High grade
Treat with combination chemo

33
Q

What is follicular lymphoma?

A

Most common low grade non Hodgkin

Treat with combination chemo

34
Q

What is the epidemiology of Hodgkin lymphoma?

A

Males > females

35
Q

What is the treatment of Hodgkin lymphoma?

A

Combination chemo +/- radiotherapy
Monoclonal antibodies
Immunotherapy
Monitor with PET scans