Haematology Flashcards

1
Q

What proportion of lymphomas are Hodgkin lymphomas?

A

15%

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2
Q

What proportion of lymphomas are non-Hodgkin lymphomas?

A

85%

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3
Q

What percentage of non-hodgkin lymphomas B cell?

A

> 90%

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4
Q

What is the peak incidence of Hodgkin’s lymphoma?

A

20-24

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5
Q

How do patients with lymphoma generally present?

A

Painless lymphadenopathy
Splenomegaly
Anaemia
B symptoms (pyrexia of unknown origin, night sweats, weight loss)

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6
Q

What are the investigations for a patient with suspected lymphoma?

A

History - symptoms, duration, B symptoms

Examination - lymph nodes, splenomegaly

Bloods - FBCs, U&Es, LFT, Ca
ESR

Imaging - CT scan, PET-CT

Bone marrow biopsy - aspirate and trephine

Additional tests: pulmonary function, ECHO

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7
Q

What staging system is used in Lymphoma?

A

Ann-Arbour Classification System

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8
Q

Define the Ann-Arbour Classification System for Lymphoma

A

Stage 1 = single lymph node group

Stage 2 = >1 lymph node group same side of diaphragm

Stage 3 = Lymph node groups both sides of diaphragm (includes spleen)

Stage 4 = Extranodal involvement

A/B to signify absence/presence of B symptoms

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9
Q

At what stage are most HL diagnosed?

A

Early stage

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10
Q

At what stage are most NHL diagnosed?

A

Advanced stage

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11
Q

Describe Follicular Lymphoma

A
Low grade lymphoma
Median age of diagnosis 65 yrs
Often present at Stage IV 
B symptoms are less common 
Indolent clinical cause 
Usually incurable
Slow growth but reduced apoptosis
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12
Q

What cells do follicular lymphoma resemble?

A

Normal germinal centre cells

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13
Q

What translocation is associated with follicular lymphomas?

A

BCL2 gene (t14:18)

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14
Q

Describe the RRx for follicular lymphomas

A

Aim: relieving symptoms

If early stage - localised radiotherapy (may be curable)

Advanced stage:
Asymptomatic + no end organ compromise - watch and wait

Symptomatic/end organ compromise –> immunochemotherapy

Ritixumab (anti-CD20) + chemotherapy

Maintenance Ritixumab every 2/12 for 2 years

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15
Q

What is the survival of follicular lymphoma like?

A

Survival >15 yrs

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16
Q

Describe Diffuse Large B-cell Lymphoma

A

High grade lymphoma
Associated with various translocations and genetic abnormalities
High proliferation fraction, variable rate of cell death

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17
Q

What do the neoplastic cells in Diffuse Large B Cell Lymphoma resemble?

A

Activated B cells

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18
Q

Describe the presentation of Diffuse Large B cell Lymphoma

A

Lymphadenopathy - rapidly enlarging LN mass

Extra-nodal presentation is common (Waldeyer’s ring, GIT, Skin, Bones, CNS)

B symptoms

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19
Q

Describe the treatment in Diffuse Large B Cell Lymphoma

A

Aggressive chemotherapy with intention to cure

1A: R-CHOP x 3 + radiotherapy

Other stages: R-CHOP x 6

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20
Q

Describe R-CHOP chemotherapy

A
Rituximab
Cyclophosphamide
Adriamycin 
Vincristine
Prednisolone
21
Q

Describe Burkitt Lymphoma

A

High grade lymphoma

Very high rate of proliferation and high rate of apoptosis (tumour lysis syndrome –> release of K –> cardiac arrest)

22
Q

What gene is involved in Burkitt Lymphomas?

A

MYC gene

23
Q

Describe the presentation of Burkitt lymphoma

A

Short history
Marked B symptoms
Rapidly growing tumour with massive tumour bulk

Presents with extra nodal disease 
Jaw and facial bones
Illeocaecal region of GIT
Ovaries
Kidneys
Breasts
24
Q

Describe the treatment for Burkitt Lymphoma

A

Requires intensive chemotherapy managed in ITU/HDU

25
Q

Describe Classic Hodgkin Lymphoma

A

High grade lymphoma with prominent component of reactive cells

Approximately 40% of cases are associated with EBV infections

26
Q

What do the neoplastic cells in Hodgkin lymphoma resemble?

A

Atypical activated B cells as seen in some viral infections

27
Q

What is the characteristic pathology cell type seen in Hodgkin lymphoma?

A

Reed-Steinburg cells

28
Q

What is strongly expressed in Hodgkin Lymphoma?

A

CD30

29
Q

Describe the presentation of Hodgkin Lymphoma

A

Peak in 20s and elderly patients

Painless lymphadenopathy (may be sore with alcohol consumption)

Cough, SOB - mediastinal disease

Itch - may precede diagnosis by many months

30
Q

How does Hodgkin lymphoma spread

A

Initially to immediately adjacent lymph nodes

Later, haematogenous spread to liver, lungs, bone marrow

31
Q

How is early stage Hodgkin lymphoma treated?

A

Chemo + Radio

32
Q

How is late stage Hodgkin Lymphoma treated?

A

Chemo

33
Q

What Chemo regimen in used to treat Hodgkin lymphoma?

A

ABVD

Adriamycin
Bleomycin
Vanblastine
Dacarbazine

34
Q

When should a person being treated for Hodgkin lymphoma have a PET-CT scan?

A

After 2nd cycle of chemo

Negative = scar tissue not active disease

Positive = change regimen

35
Q

Describe plasma cell myeloma

A

Neoplasm of mature plasma cells with varied clinical cause

36
Q

What do neoplastic cells in plasma cell myeloma resemble?

A

Normal plasma cells

37
Q

Describe the presentation of plasma cell myeloma

A

Non-specific symptoms

Backache/rib pain
Fatigue
Symptoms associated with hypercalcaemia
Recurrent infections
Renal impairment
38
Q

Describe paraprotein

A

In myeloma, abnormal plasma cells produce an abnormal monoclonal protein called paraprotein or M protein

5 different types (IgA etc)

39
Q

What is IgM myeloma associated with?

A

Waldenstroms macroglobulinaemia

40
Q

What is light chain myeloma?

A

Only part of the immunoglobulin is produced

41
Q

What is non-secretory myeloma

A

No Ig is produced

42
Q

What is the classical myeloma triad?

A
  1. Increased plasma cells in bone marrow
  2. Clonal immunoglobulin or paraprotein
  3. Lytic bone lesions
43
Q

What blood tests should be arranged in suspected myeloma?

A

FBCs
ESR (>100)
U&E
Ca

Serum protein electrophoresis - rouleaux

SFLC quantity (serum free light chain)

44
Q

What proteins may be present in the urine of a patient with myeloma?

A

Bence-Jones proteins (immunoglobulin light chains)

45
Q

What is the diagnostic requirement for myeloma?

A

Neoplastic plasma cells in bone marrow >10% of total cells

AND >1 of:

HyperCalcaemia
Renal insufficiency
Anaemia
Bone lesions

46
Q

What biomarkers are associated with myeloma?

A

Clonal plasm cell % >40%
Serum light free chain ratio >100
>1 focal lesion on MRI

47
Q

Describe the treatment of myeloma

A

Asymptomatic = watch and wait

Symptomatic (CRAB criteria)

Chemotherapy + steroid + thalidomide

Radiotherapy - severe bone pain

Bisphosphonates

Blood transfusions/EPO

48
Q

Why are bisphosphonates useful in patients with myeloma?

A

Reduces pain
Reduces pathological fractures
Reduces hypercalcaemia
Reduces need for radiotherapy