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Haematology + Immunology > Lymphoproliferative Disorders > Flashcards

Lymphoproliferative Disorders Flashcards

1
Q

What is a lymphoma

A

Cancers of lymphoid origin ,

Can present with:

  • enlarged lymph nodes,
  • extranodal involvement
  • bone marrow involvement
  • systemic B symptoms
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2
Q

What are the systemic B symptoms of lymphoma

A 
Weight loss (>10% in 6 months),
Fever,
Night sweats, 
Pruritis,
Fatigue
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3
Q

How to diagnose a lymphoma/ leukaemia

A

Biopsy (diagnose)
Clinical examination
Imaging (eg CT) (staging)

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4
Q

Types of lymphoma

A

Hodgkin lymphoma

Non-Hodgkin Lymphoma (split into high-grade and low-grade)

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5
Q

Examples of lymphoproliferative disorders

A

Acute Lymphoblastic Leukaemia,
Chronic Lymphoblastic Leukaemia,
Hodgkin lymphoma,
Non-Hodgkin lymphoma
(High grade- diffuse large Bcell lymphoma
And Low grade- follicular, marginal zone)

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6
Q

What cells are affected in acute Lymphoblastic leukaemia

A

Lymphoid progenitor cells

Occurs in bone marrow but could spread anywhere

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7
Q

Most common age group for acute lymphoblastic leukaemua

A

75% occur in children <6 years

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8
Q

Presentation of acute lymphoblastic leukaemia

A

2-3 week history of bone marrow failure (low haemoglobin, low platelets, high WCC)

Bone/joint pain

Infiltration of bone marrow:
Hepatosplenomegaly,
Lymphadenopathy,
CNS involvement (CN palsies, menigism)

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9
Q

Typical ALL presentation

A

17year old
1 month impaired vision (retinal haemorrhage),
1/2 stone weight loss,
Breathless on minimal exertion

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10
Q

ALL cells characteristics

A

Large cells
Express CD19
CD34, TDT are markers of early immature cells

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11
Q

ALL treatment

A

Induction chemotherapy,
Maintenance treatment for 18 months,
CNS directed treatment (LP methotrexate)
Stem cell transplantation

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12
Q

ALL newer therapies

A

Bi-specific T cell engagers eg Blintumumab

CAR (Chimeric Antigen receptor Tcells)

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13
Q

ALL poor risk factors

A

Increasing age,
Increased WCC,
Cytogenetics/ molecular genetics,
Slow/ poor response to treatment

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14
Q

ALL summary

Presentation and treatment

A

Bone marrow failure,
Bone pain,
Infection,
Sweat

T:
multi-agent intensive chemotherapy
Allogenic stem cell transplant

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15
Q

Why is CLL different from ALL even though it is a leukaemia

A

The abnormal cells are mature

So they:
Grow slowly,
Resemble normal well behaved lymphocytes

Requires a lymphocyte count of >5

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16
Q

CLL presentation

A 
Often asymptomatic,
Bone marrow failure, 
Lymphadenopathy,
Splenomegaly, 
Fever and sweats

Hepatomegaly,
Infections,
Weight loss

17
Q

CLL associated findings

A

Immune paresis,

Haemolytic anaemia

18
Q

CLL Binet staging?

A

A < 3 lymph node areas
No affect

B 3 or more lymph node areas
8 year median survival

C Stage B + anaemia or thrombocytopenia
6 year median survival

19
Q

CLL indications for treatment

A 
Progressive bone marrow failure, 
Massive lymphadenopathy, 
Progressive splenomegaly, 
Lymphocyte doubling time <6 months or >50% increase over 2 months, 
Systemic symptoms,
Autoimmune cytopenia
20
Q

CLL treatment

A 
Watch and wait,
Cytotoxic chemotherapy (fludarabine, bendamustine)
Monoclonal antibodies (rituximab, obinatuzumab)
Novel agents ( Bruton tyrosine kinase inhibitor, P13K inhibitor, BCL-2 inhibitor)
21
Q

CLL poor prognostic markers

A 
Advanced disease (Binet stage B or C),
Atypical lymphocyte morphology, 
Rapid lymphocyte doubling time (<12 months),
CD 38+ expression, 
Loss/ mutation of p53,
Unmutated IgVH gene status
22
Q

Lymphoma presentation

A

Lymphadenopathy,
Hepatosplenomegaly,
B symptoms,
Bone marrow involvement

23
Q

Assessment of lymphoma

A

Lymph node biopsy,
CT scan,
Bone marrow aspirate and trephine

24
Q

Ann Arbor staging of Lymphoma

A

Stage 1- one lymph node affected
Stage 2- 2 lymph node groups on the same time
Stage 3- both sides affected
Stage 4- extranodal involvement

A- absence of B symptoms
B- B symptoms present

25
Q

How is Non-Hodgkin lymphoma classified

A

Lineage (B cell or T cell)
(Majority are B cell)
“Grade”
(High grade or low grade)

26
Q

Difference between low grade lymphoma and high grade

A

Low grade:

  • Indolent, often asymptomatic
  • responds to chemotherapy but incurable

High grade:

  • aggressive fast growing,
  • requires combination chemotherapy
  • can be cured
27
Q

Describe the specific disease entities of Non-Hodgkin lymphoma

A

Diffuse large B-cell lymphoma:

  • commonest subtype of lymphoma
  • high grade lymphoma

Follicular lymphoma:

  • low grade lymphoma
  • watch and wait treatment
28
Q

Treatement of Non-Hodgkin lymphoma

A

Combination chemotherapy

Anti-CD20 monoclonal antibody + chemotherapy

29
Q

Hodgkin lymphoma risk factors

A 
Peak 15-35 years
2nd peak later in life,
EBV,
Familial and 
Geographical clustering
30
Q

Hodgkin Lymphoma

A

Combination chemotherapy,
+/- radiotherapy,
Monoclonal antibodies (anti-CD30),
Immunotherapy

31
Q

ALL investigations

A 
Blood film,
Bone marrow (>20% lymphoblasts in bone marrow),
WCC,
CXR,
FBC (Hb),
LP

Haematology + Immunology (21 decks)

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