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Clinical Pathology > Theme 7 Haematology: Myeloma, Lymphoma and Chronic Lymphocytic Leukaemia > Flashcards

Theme 7 Haematology: Myeloma, Lymphoma and Chronic Lymphocytic Leukaemia Flashcards

1
Q

What is myeloma?

A
  • an incurable malignant disorder of clonal plasma cells
  • mean age of presentation = 70
  • preceded by asymptomatic MGUS in all patients
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2
Q

Which laboratory technique is used when we suspect myeloma?

A

Protein electrophoresis:

  • serum is placed in a gel and exposed to an electric current
  • 5 fractions are usually identified
  • in myeloma, there is one gamma globulin produced by malignant plasma cells producing a spike
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3
Q

What is immunofixation?

A
  • performed when “M-spike” is seen on electrophosresis

- this test detects which immunoglobulin is being produced

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4
Q

What is meant by a spectrum of plasma cell dyscrasias?

A

“paraproteinaemias”

a spectrum of plasma cell disorders with MGUS having the lowest disease burden, and myeloma having the highest

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5
Q

What is the IMWG Diagnostic criteria for myeloma?

A
  1. clonal plasma cells > 10% AND
  2. CRAB features
  3. MDEs
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6
Q

What are CRAB features of myeloma?

A

C-Hypercalcaemia
R- Renal insufficiency
A-anaemia
B-bone lesions

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7
Q

What are MDEs?

A
  • myeloma defining events
  • > 60% clonal plasma cells on BM biopsy
  • SFLC ratio > 100mg/L provided the absolute level of the involved LC is > 100mg/L
  • > 1 focal lesion on MRI > 5mm
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8
Q

What is the relationship with myeloma and the kidney?

A

half will have persistent renal insufficiency/impairment during their disease course despite therapy

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9
Q

Which external factors in myeloma can contribute to renal disease?

A
  1. Renal Vein thrombosis (myeloma is a pro-thrombotic disease)
  2. Bisphosphonates (nephrotoxic)
  3. Hypercalcaemia
  4. ACEi
  5. Dehydration
  6. NSAIDs
  7. CT contrast
  8. Hyperviscosity
  9. Type 1 cryoglobunaemia
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10
Q

What are the clinical features of myeloma?

A 
confusion
bone pain
nausea
fractures
constipation
peripheral oedema
poor appetite
thirst
chest infections
breathlessness
polyuria or oligurua/anuria
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11
Q

What are the acute complications of myeloma?

A
  • hyperviscosity
  • hypercalcaemia
  • sepsis
  • spinal cord compression
  • acute kidney injury
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12
Q

What is rouleaux?

A

red cells on film stacking up like coins

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13
Q

How do we manage myeloma?

A
  1. steroids
  2. acute kidney injury with suspected myeloma is a medical emergency
    time = more destruction of nephrons
  3. blood film, electrophoresis, immunofixation, bone marrow biopsy
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14
Q

What do you investigate to confirm diagnosis of suspected myeloma?

A

Bloods:

  1. FBC: Hb, WB, platelets and blood film
    - anaemia
    - active infection
    - degree of bone marrow infiltration
    - rouleaux
    - plasma cell present
  2. U&Es: urea, creatinine, sodium, potassium
  3. Calcium
  4. C-reactive protein
  5. Immunoglobulin levels
  6. Protein electrophoresis looking for paraprotein
  7. Light chain analysis
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15
Q

What is MGUS?

A
  • monoclonal gammopathy of undetermined significance

- abnormal protein (‘M’ protein) found in your blood

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16
Q

What is the IMWG diagnostic criteria for MGUS?

A
  • benign disorder
  • serum M-protein > 30g/L
  • <10% clonal plasma cells in the bone marrow
  • absence of end organ damage (CRAB)
17
Q

What is the risk of progression from MGUS

A

1%

  • most progress to myeloma
  • others progress to waldenstrom’s macroglobinaemia, primary AL amyloidosis, lymphoproliferative disorders
18
Q

What is the pathogenesis of AL amyloidosis?

A

light chain (kappa or lamda) fragments misfold and self aggregate to form beta pleated fibrils then deposit in organs

19
Q

What are the clinical features of AL Amyloidosis?

A 
macroglossia
confusion
poor appetite
thirst
palpatations
breathlessness
peripheral neuropathy
oliguria/anuria
peripheral oedema
constipation
ascites
nausea
PND and orthopnoea
20
Q

What is lymphoma?

A

caused by malignant proliferations of lymphocytes (B or T cells)

21
Q

How are B cell lymphomas classified?

A

according to the presence or absence of Reed Sternberg Cells

22
Q

What is the name of the malignancy when Reed Sternberg cells are present?

A

Hodgkin Lymphoma

23
Q

What are the two types of Non-hodgkin lymphoma?

A

(this is when Reed Sternberg cells aren’t present)

  1. Aggressive e.g DLBCL, Burkitt lymphoma
    - rapid onset lymphadenopathy
    - night sweats
    - weight loss
    - curable
  2. Indolent e.g follicular lymphoma, marginal lymphoma
    - insidious onset
    - systemically well
    - usually not curable
24
Q

What are the features of Hogdkin Lymphoma?

A
  • bimodal age distribution (20s and 60s)
  • lymphadenopathy
  • night sweats
  • weight loss
  • fatigue
  • BM involvement uncommon
  • excellent cure rates
25
Q

What is follicular lymphoma/

A
  • neoplastic disorder of lymphoid tissue
  • type of non-hodkin lymphoma characterised by slowly enlarging lymph nodes
  • M=F, incidence rises with age, median survival 8-10 years
26
Q

How can we prognosticate follicular lymphoma

A

Using the Follicular international prognostic index (FLIPI):

  • age > 60 years
  • ann arbor stage III or IV
  • LDH above limit of normal
  • Hb < 120g/L
  • presence of more than four nodal sites of disease
27
Q

In the majority of cases of follicular lymphoma, what chromosomal translocation is usually present?

A

t (14;18) –> stops lymph nodes from apoptosing

28
Q

What is the pathogenesis of Hodgkin Lymphoma?

A
  • presence of Hodgkin Reed-Sternberg (HRS) cells within a cellular infiltrate of non-malignant inflammatory cells
  • HRSS fail to express surface immunoglobulin and evade apoptosis through several mechanisms
29
Q

What are the differences between acute and chronic leukaemias?

A

Acute:

  • rapid onset
  • dramatic presentation
  • severe sepsis
  • bleeding
  • life-threateing without urgent treatment

Chronic:

  • insidious onset
  • doesn’t always require treatment
30
Q

What are the differences between myeloid and lymphoid leukaemias?

A

Myeloid:
cells which develop into neuts, eosins, monos and basophils

Lymphoid:
cells which develop into lymphocytes

31
Q

What is chronic lymphocytic leukaemia (CLL)

A
  • a malignant disorder of mature B cells

- most common type of leukaemia in the UK

32
Q

How do patients with CLL present?

A

can range from an incidental finding of lymphocytosis to widespread lymphadenopathy, splenomegaly, bone marrow failure and systemic symptoms

33
Q

How do we stage CLL?

A

Binet system :
Stages A, B and C
looks at Hb, platelets and areas of lymphadenopathy

34
Q

What is DLBCL?

A
  • diffuse large B-cell lymphoma
  • high grade lymphoma
  • most common type of non-hodgkin lymphoma
35
Q

What are some possible complications of CLL?

A
  • recurrent infections secondary to reduced immunoglobulin production
  • ITP
  • Haemolytic anaemia
  • pure red cell aplasia
  • autoimmune neutropenia

Clinical Pathology (57 decks)

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