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FIP > 25. Plasma cell neoplasms and related disorders > Flashcards

25. Plasma cell neoplasms and related disorders Flashcards

1
Q

What are plasma cell dyscrasias

A

neoplastic proliferation of plasma cells which secrete monoclonal intact immunoglobulin or Ig fragments (free light chain)

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2
Q

Classification of plasma cell neoplasm (dyscrasias)

A

I. multiple myeloma
II. smoldering multiple myeloma
III. solitary myeloma (plasmocytoma)
IV. monoclonal gammaopathy of undetermined significance
V. lymphoplasmacytic lymphoma (Waldenstrom’s macroglobulinaaemia)
VI. other rare entities

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3
Q

What is multiple myeloma

A

multifocal bone marrow disease characterized by malignant proliferation of plasma cells with skeletal destruction

  • monoclonal B cells profuce a single type of Ig
  • usually adults > 50 years
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4
Q

Causes of multiple myeloma

A
  • previous exposure to irradiation
  • exposure to asbestos, petroleum products, rubber or plastic products
  • Human herpes virus 8
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5
Q

Pathogenesis of multiple myeloma

A

myeloma cells bind to bone marrow stromal cells via cell surface adhesion molecules -> myeloma cell growth -> survival -> drug resistance and migration in the bone marrow milieu

  • myeloma cells produce cytokines (e.g. IL6)
  • interaction with bone marrow stromal cells -> osteolast activation (RANK receptors) and osteoblast inhibition
  • plasma cells produce intact monoclonal Ig (M component) and light chain
  • intact monoclonal Ig has high molecular weight - not present in urine if no glomerular disease
  • IgG, IgA, light chain
  • light chain excreted in urine - can be filtered (Bence-Jones protein)
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6
Q

How is multiple myeloma diagnosed

A
  • radiology
  • bone marrow examination
  • serum and urine electrophoresis and immunoglobulin levels
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7
Q

What can you see on bone marrow examination for someone who has multiple myeloma

A
  • plasma cell increased
  • perinuclear clearing and eccentric nucleus
  • atypical cells with bi-, tri- or multinucleated forms
  • immature blasts
  • intracytoplasmic inclusion (Russell bodies)
  • Mott cells
  • intranuclear inclusions (Dutcher bodies)
  • immunohistochemistry: single type of Ig and single type of light chain (kappa/lambda)
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8
Q

Serum and urine analysis of multiple myeloma

A
  1. serum
    - monoclonal globulin spike on serum electrophoresis
    - M spike most commonly IgG or IgA
  2. urine
    - proteinuria (light chain)
    - kappa/lambda
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9
Q

Clinical features of multiple myeloma

A 
CRAB
C- calcium (elevated)
R - renal failure
A - anaemia
B - bone lesion
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10
Q

What is bone lesion

A

proliferation of tumour cells which infiltrate the bone

-production of IL6/osteoclast activating factor

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11
Q

How is bone lesion presented

A
  • bone pain
  • lytic lesions
  • pathological fractures
  • diffuse osteoporosis
  • hypercalcaemia (confusion, weakness, lethargy, abdominal pain, constipation, polyuria, depression, renal stones)
  • spinal cord compression (lumbar vertebrae)
  • tumour cell replace bone marrow -> anaemia, thrombocytopenia, leucopenia
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12
Q

Type of anaemia in multiple myeloma

A

normocytic or normochromic

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13
Q

What causes anaemia in multiple myeloma

A

-replacement of normal bone marrow by tumour cells
-inhibition of normal RBC production by cytokines
-decrease in erythropoietin production
decrease charge of RBC -> Rouleaux formation on blood smear

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14
Q

Why does bleeding occur in multiple myeloma

A
  • monoclonal immune proteins interfere with normal coagulation
  • infiltration of the bone marrow -> thrombocytopenia
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15
Q

Why does hyperviscosity occur in multiple myeloma

A

high volume of monoclonal protein -> blood viscosity increases -> complications: stroke, MI, myocardial ischaemia
-blood becomes sticky and thick

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16
Q

Why does sepsis occur in multiple myeloma

A
  • decreased productin of normal Ig -> recurrent bacterial infection
  • leucopenia
17
Q

Clinal features of renal failure in multiple myeloma

A
  1. myeloma kidney = myeloma cast nephropathy
    - Bence-Jones toxic to renal tubular epithelial cells
  2. amyloidosis
    - amyloid light chain type
  3. light chain nephropathy
  4. hypercalcaemia and hyperuricaemia
  5. pyelonephritis
  6. drug induced
18
Q

How to determine amyloidosis in multiple myeloma

A

systemic deposition of amyloid light chain type in:
kidneys - nephrotic syndrome
heart - restrictive cardiomyopathy
tongue enlargement
-require tissue biopsy from rectum
-congo red staining with apple green birefringence under polarised light

19
Q

How does neurological symptoms occur in multiple myeloma

A

hyperviscosity, hypercalcaemia, nerve compression

20
Q

Prognosis of multiple myeloma

A

-6 to 12 months if untreated
-medial survival 4 to 7 years
Cause of death:
-infection
-renal failure

21
Q

Treatment of multiple myeloma

A
  • currently not curable
  • immunomodulatory, proteasome inhibitors
  • bisphosphonates
  • bone marrow transplantation: autologous, allogenic
22
Q

What is smoldering multiple myeloma

A
  • asymptomatic

- highly likely to progress to multiple myeloma

23
Q

What is solitary plasmacytoma of bone

A
  • involve spine, pelvis and femur
  • single symptomatic area of bone destruction
  • progression to multiple myeloma in 10-20 years
24
Q

What is extramedullary plasmacytoma

A
  • involve lung, oropharynx and nasal sinuses
  • extra-ossesous lesions can be cured by local resection or radiotherapy
  • progression to multiple myeloma is rare
25
Q

What is monoclonal gammopathy of undetermined significance

A
  • starts out as a relatively benign condition
  • most common cause of monclonal gammopathy
  • asymptomatic
  • increased serum protein with M spike on spectrum electrophoresis
  • plasma cells less than 10%
  • no Bence-Jones protein, bone lesions, hypercalcaemia or amyloid
  • early stage of myeloma
26
Q

What is lymphoplasmacytic lymphoma

A

low-grade B cell lymphoproliferative neoplasm characterized by small lymphocytes and monoclonal IgM monoclonal gammopathy
-elderly
-males more than female
-neoplastic cells produce monoclonal IgM
(macroglobulin = Waldenstrom’s macroglobulinaemia)
-mean survival is 4-5 years

27
Q

Clinical features of lymphoplasmacytic lymphoma

A
  • no bone lesions
  • weakness, weight loss
  • lymphadenopathy, hepatosplenomegaly
  • increased serum protein with M spike (IgM)
  • autoimmune haemolysis (IgM binds to RBC)
  • hyperviscosity syndrome
28
Q

Clinical features of hyperviscosity syndrome

A
  1. visual impairment
    - distension of retinal veins and haemorrhage
  2. neurological symptoms
    - headaches, dizziness due to sluggish blood flow
  3. bleeding
    - macroglobulins bind to clotting factors and interfere with platelet function
  4. cryoglobulinaemia
    - precipitation of macroglobulins at low temp -> Raynaud’s phenomenon
29
Q

Treatment of hyperviscosity syndrome

A

Plasmapheresis

-removal, treatment, and return or exchange of blood plasma or components thereof from and to the blood circulation

FIP (24 decks)

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