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8. CP - Core Haematology > (41) Lymphoma and myeloma > Flashcards

(41) Lymphoma and myeloma Flashcards

1
Q

What stages of B cell differentiation occur in the bone marrow?

A

Pro-B cells become pre-B cells which become immature B cells

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2
Q

What is the pathway of plasma cell differentiation called?

A

NF-kB pathway

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3
Q

What does an error in the NF-kB pathway lead to?

A

Plasma cell dysplasia

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4
Q

What are immunoglobulins?

A

Glycoprotein molecules produced by plasma cells in response to an immunogen

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5
Q

Describe the structure of an immunoglobulin

A

Composed of 4 polypeptide chains - 2 light chains and 2 heavy chains which are held together by covalent disulphide bridges

Each chain has one variable and one constant region

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6
Q

How are immunoglobulins classified?

A

According to the amino acid sequences in the constant region of the:

  • heavy chains (IgG, IgM, IgA, IgD, IgE)
  • light chains (kappa or lambda)
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7
Q

What is protein electrophoresis?

A

Lab technique whereby serum is placed in a gel and exposed to an electric current

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8
Q

In protein electrophoresis, 5 major fractions are normally identified. What are they?

A
  • serum albumin
  • alpha-1 globulins
  • alpha-2 globulins
  • beta globulins
  • gamma globulins
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9
Q

What is immunofixation?

A

Enables the detection and identification of monoclonal immunoglobulins - performed when “M-spike” seen on electrophoresis

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10
Q

How is immunofixation done?

A
  • serum or urine is placed on a gel and electric current is applied to separate the proteins
  • anti-immunoglobulin antisera is added to each migration lane
  • if the immunoglobulin is present, a complex is precipitated
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11
Q

What is myeloma?

A

An incurable malignant disorder of clonal plasma cells

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12
Q

What is the incidence of myeloma?

A

Annual incidence = 60-70 millions in the UK

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13
Q

What is the median age at presentation of myeloma?

A

70 years

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14
Q

Which ethnicity has a higher incidence of myeloma?

A

Afro-Carribean ethnic groups have a higher incidence compared with Caucasians

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15
Q

Myeloma is preceded by what?

A

Asymptomatic MGUS

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16
Q

Myeloma is one of a spectrum of plasma cell dyscrasias. What is at the bottom and top end?

A

Bottom end = MGUS

Top end = myeloma and then plasma cell leukaemia (PCL)

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17
Q

What else is on the spectrum of plasma cells dyscrasias?

A
  • MGRS
  • high-risk MGUS (almost certainly will progress to myeloma)
  • asymptomatic myeloma
  • amyloidosis
  • solitary plasmacytoma (with or without minimal bone marrow involvement)
  • systemic AL amyloidosis
  • POEMS syndrome
  • myeloma with adverse cellular features
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18
Q

How is myeloma diagnosed? (using IMWG diagnostic criteria)

A

Clonal bone marrow plasma cells more than 10% or biopsy-proven bony or extra medullary plasmacytoma AND any one of the - CRAB features - MDEs

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19
Q

In myeloma, more than 10% of the plasma cells in the bone marrow are what?

A

Noeplastic / clonal

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20
Q

What is a plasmacytoma?

A

Discrete, solitary mass of neoplastic monoclonal plasma cells in either bone or soft tissue (extra medullary) eg. lump of plasma cells on femur

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21
Q

What are the CRAB features? (used in diagnosis of myeloma)

A

C = hypercalcaemia (more than 2.75mmol/L)

R = renal insufficiency (creat clearance less than 40ml/min or serum creat more than 177mmol/L)

A = anaemia (Hb less than 100g/L)

B = bone lesions (one or more osteolytic lesions on skeletal radiography, CT, or PET/CT)

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22
Q

What is the ‘C’ of the myeloma CRAB features?

A

Hypercalcaemia (more than 2.75mmol/L)

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23
Q

What is the ‘R’ of the myeloma CRAB features?

A

Renal insufficiency (creatinine clearance of less than 40ml/min OR serum creatinine of more than 177mmol/L)

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24
Q

What is the ‘A’ of the myeloma CRAB features?

A

Anaemia (Hb of less than 100g/L)

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25
What is the 'B' of the myeloma CRAB features?
Bone lesions (one or more osteolytic lesions on skeletal radiography, CT, or PET/CT)
26
What are the myeloma-defining events (MDEs) using in diagnosing myeloma?
- over 60% clonal plasma cells on bone marrow biopsy - SFLC ratio of more than 100mg/L provided the absolute level of the involved LC is more than 100mg/L - more than 1 focal lesion on MRI measuring more than 5mm
27
What does SFLC stand for?
Serum free light chains
28
How does myeloma affect the kidney?
- 20-25% will have renal insufficiency at diagnosis - 50% will have renal sufficiency at some point during disease course - 50% will have persistent renal impairment despite therapy - 2-12% will require RRT
29
Name 9 external factors that can affect the kidney, other than myeloma
- renal vein thrombosis - bisphosphonates - hypercalcaemia - ACE inhibitors - dehydration - NSAIDs - CT contrast - hyperviscosity - type 1 cryoglobulinaemia
30
Acute kidney injury with suspected myeloma is a medical injury. What drug should you give before test results even come back?
Steroids
31
Acute kidney injury with suspected myeloma is a medical emergency. What tests should be done? (within 24 hrs)
- blood film - electrophoreses - immunofixation - bone marrow biopsy with flow cytometry
32
Describe the process of intensive therapy in myeloma
- chemotherapy is given (VCD) - G-CSF is given and stem cells are harvested - chemotherapy (melphalan if given) - stem cells are given back This allows bone marrow to recover
33
What is G-CSF?
Granulocyte-colony stimulating factor
34
What are some newer agents in myeloma intensive therapy?
- daratumumab (antiCD138) - carfilzomib (proteosome inhibitor) - ixazomib (proteosome inhibitor) - various combinations of the above
35
Allogenic hematopoietic stem cell transplantation (HSCT) is sometimes used in second remission but it is controversial, why?
- high transplant-related mortality | - high relapse rate
36
What does the future involve for myeloma treatment?
- individualised therapy (particularly for those with high risk disease) - monoclonal antibodies - targeted therapies - maintenance therapy
37
What are the basics in management of myeloma?
- early diagnosis and intervention - STEROIDS! | - simple measures like hydration, avoid nephrotoxics, and appropriate chemotherapy (attenuated dosing)
38
What is the MGUS diagnostic criteria?
- serum M-protein (paraprotein) less than 30g/L - less than 10% clonal plasma cells in bone marrow - absence of end-organ damage (CRAB)
39
What is MGUS?
Monoclonal gammopathy of undetermined significance (MGUS) is a condition in which an abnormal protein (monoclonal protein, or M protein) is in the blood. M protein is produced by plasma cells, a type of white blood cell. Monoclonal gammopathy of undetermined significance usually causes no problems
40
Who gets MGUS?
3. 2% = less than 50 years 5. 3% = less than 70 years 8. 9% = less than 80 years affects more men than women
41
What is the risk of progression of MGUS?
Approx 1% per year
42
What does MGUS progress to?
Majority progress to myeloma (27%) Others progress to: - Waldenstrom's macroglobulinaemia - primary AL amyloidosis - lymphoproliferative disorders
43
What makes MGUS more likely to progress?
If there is high M-protein (more than 15g/L), IgA/IgM rather than IgG paraprotein, abnormal SFLC ratio
44
Congo red stain preparates is indicative for what?
The presence of amyloid fibrils
45
What is AL amyloidosis?
Amyloid light chain (AL) amyloidosis | - light chain fragments misfold and self-aggregate to form beta-pleated fibrils
46
How common is AL amyloidosis?
600 new cases in the UK per year
47
Describe the proteinuria in AL amyloidosis?
Nephrotic-range proteinuria: - mainly albumin - small monoclonal light chain component
48
What are the other systemic components of AL amyloidosis?
- cardiac and liver involvement in 30% - peripheral neuropathy in 10% - end stage renal failure in 40%
49
What are the possible malignant causes of a neck mass?
- lymphoma - chronic lymphocytic leukaemia - metastatic cancer of the lung/breast/cervix
50
What are the possible non-metastatic causes of a neck mass?
- infective (bacterial, viral, mycobacterial) - inflammatory (sarcoidosis) - lipoma - fibroma - haemangioma
51
What would you see on a lymph node biopsy in a follicular lymphoma?
Lots of large pale follicles in the lymph nodes (follicles normal but increase in number) Lymphoma - B cells form giant follicles
52
What is follicular lymphoma?
Neoplastic disorder of lymphoid tissue - type of non-Hodgkin lymphoma characterised by slowly enlarging lymph nodes
53
Follicular lymphoma is a type of what?
Non-Hodgkin lymphoma - accounts for 15% of all non-Hodgkin lymphoma diagnoses
54
Incidence of follicular lymphoma rises with what?
With age M=F
55
What is the chromosomal abnormality in follicular lymphoma?
t(14;18) Translocation between chromosomes 14 and 18 - over expression of bcl-2 protein = gives survival advantage to neoplastic lymphoid cells by inhibiting apoptosis
56
What is the prognosis for follicular lymphoma?
Median survival = 8-10 years Five year overall survival = 72-77%
57
What is used to prognosticate in follicular lymphoma?
The Follicular Lymphoma International Prognostic Index (FLIPI)
58
What factors does the FLIPI include?
- age over 60 years - Ann Arbor stage III or IV - LDH above limit of normal at diagnosis - Hb less than 120g/L - presence of more than 4 nodal sites of disease 4 or more prognostic factor, 10 year survival = 36% rather than 71%
59
What is the incidence of Hodgkin lymphoma?
2.7 per 100,000 per year
60
Hodgkin lymphoma is characterised by...
- presence of Hodgkin Reed-Sternberg (HRS) cells within a cellular infiltrate of non-malignant inflammatory cells eg. eosinophils
61
How do HRS cells (in Hodgkin lymphoma) evade apoptosis?
HRS fails to express surface immunoglobin and evade apoptosis through several mechanisms eg. - activation of NFkB - incorporation of EBV and latent membrane proteins (LMP1 and LMP2)
62
What are different classifications of Hodgkin lymphoma?
- nodular lymphocyte predominant - classical: - nodular sclerosis - mixed cellularity - mixed lymphocyte-rich - lymphocyte depleted
63
How is Hodgkin lymphoma treated?
- chemotherapy eg. ABVD | - radiotherapy
64
What signs are favourable in Hodgkin lymphoma?
- ESR less than 50 and no B syx - ESR less than 30 and B six - no large mediastinal LN - age les than 50 - 1-3 LN sites involved
65
What is the prognosis for Hodgkin lymphoma?
- high proportion are cured (86% 5 year survival) - long term effects of therapy are important: - increased mortality still see over 20 years post-therapy - pulmonary toxicity - cardiovascular disease - secondary malignancies

8. CP - Core Haematology (11 decks)

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