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Plasma Cell Disorders Flashcards

1
Q

List 6 Plasma Cell Dyscrasias

A

Monoclonal Gammopathy of Uncertain Significance (MGUS)

Waldenstrom’s Macroglobulinaemia

Multiple Myeloma

Solitary Plasma Cytoma of Bone

Extramedullary Plasmacytoma (EMP)

Plasma Cell Leukemia

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2
Q

Define Paraproteinaemia

A

Term refers to the Presence of monoclonal immunoglobulin band in the serum.

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3
Q

Term refers to the Presence of monoclonal immunoglobulin band in the serum.

A

Paraproteinaemia

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4
Q

Note//

Normally serum immunoglobulins are polyclonal and represent the combined output from millions of different plasma cells

A

Note//

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5
Q

What is a monoclonal band/ paraprotein

A

Reflects the synthesis of immunoglobulin from a single clone of plasma cells

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6
Q

List 7 Malignant diseases associated with M- proteins

A 
Multiple Myeloma
Waldenström’s macroglobulinaemia
Malignant lymphoma
Chronic lymphocytic leukemia 
Primary amyloidosis
Plasma cell leukemia
Heavy chain disease
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7
Q

List 7 Benign diseases associated with M- proteins

A 
Benign monoclonal gammopathy
Solitary plasmacytoma
Chronic cold heamagglutinin disease
Acquired immune deficiency syndrome (AIDS)
Gaucher’s disease
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8
Q

Define Multiple Myeloma (myelomatosis)

A

Neoplastic proliferation characterized by:

  • plasma cell accumulation in the bone marrow
  • presence of monoclonal protein in the serum and / or urine

-related tissue damage

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9
Q

What is the incidence of Multiple Myeloma

A

98% of cases occur over the age of 40 yrs

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10
Q

When is the peak incidence of Multiple Myeloma

A

7th decade

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11
Q

Describe the myeloma cell

A

Post-germinal centre plasma cell
Underwent immunoglobulin class switching
Somatic hypermutation
Secretes paraprotein present in serum

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12
Q

What is the aetiology of Multiple Myeloma

A

Unknown

(But it is more common in certain racial groups - Afro Caribbean)

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13
Q

The diagnosis for Multiple Myeloma depends on what three principal findings

A
  • Monoclonal protein in serum/urine
  • Increased plasma cells in the bone marrow
  • Related organ or tissue impairment
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14
Q

In the diagnosis for Multiple Myeloma, if the bona marrow cell count is >10% but there is no evidence of tissue damage what is the new term for the disease

A

Asymptomatic or Smouldering Myeloma

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15
Q

List 7 clinical features of Multiple Myeloma

A 
CRAB
C- elevated calcium
R- Renal Dysfunction 
A- anemia
B- bone disease
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16
Q

What causes bone pain (especially backache) in Multiple Myeloma

A

Vertebral Collapse and pathological fractures

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17
Q

List five features of anemia

A 
Pallor
Lethargy 
Fatigue
Weakness
Dyspnoea
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18
Q

What is the cause of recurrent infections in Multiple Myeloma

A

Related to deficient antibody production
Abnormal cell-mediated immunity
Neutropenia

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19
Q

What are 6 features of renal failure/ hypercalcemia

A 
Polydipsia
Polyuria
Anorexia
Vomiting
Constipation
Mental disturbance
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20
Q

What is the cause if abnormal bleeding tendency in Multiple Myeloma

A

Myeloma protein may interfere with platelet function and coagulation factors

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21
Q

What percentage of persons with Multiple Myeloma present with Amyloidosis

A

5%

( features as macroglossia, carpal tunnel syndrome and diarrhoea )

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22
Q

What percentage of Multiple Myeloma cases present with hyperviscosity syndrome?

A

2%

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23
Q

List 9 laboratory findings for Multiple Myeloma

A
  • Presence of paraprotein
  • Normochromic, normocytic or macrocytic anemia
  • High erythrocyte sedimentation rate
  • Increased plasma cells in the bone marrow
  • bone lesions
  • serum calcium elevation
  • Serum creatinine raised
  • Low serum albumin
  • Serum B2- microglobulin often raised
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24
Q

What is the paraprotein observed in 60% of the multiple myeloma cases

A

Immunoglobulin G (IgG)

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25
Q

What is the paraprotein observed in 20% of the multiple myeloma cases

A

IgA (20%)

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26
Q

What kind of formation is seen in a blood film for pts with Multiple Myeloma

A

Rouleaux formation

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27
Q

What percentage of Multiple Myeloma pts have serum calcium elevations

A

45%

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28
Q

What percentage of Multiple Myeloma pts have serum creatinine elevations

A

20%

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29
Q

Levels of B2- microglobulin less than what value indicates good prognosis for Multiple Myeloma

30
Q

What are the two main types of treatment for Multiple Myeloma

A

Specific

Supportive

31
Q

Note//
At the current time Multiple Myeloma remains incurable, except for those very few, mostly younger , patients who may be cured by allogenic stem cell transplantation (SCT)

32
Q

What are the two major treatments foe Multiple Myeloma

A

Intensive Therapy

Non-intensive therapy

33
Q

What are the two main aims of Intensive therapy for Multiple Myeloma

PS intensive therapy for <70 yrs and no co morbidity

A

Combination of several courses of chemotherapy to reduce the tumour burden

Followed by stem cell collection and autologous SCT after high-dose chemotherapy

34
Q

Repeated intravenous or oral chemotherapy cycles such as which drugs are used to treat Multiple Myeloma

A 
Cyclophosphamide 
Dexmethasone
Thalidomide 
Cyclophosphamide 
Vincristine
Adriamycin
Dexmethasone
35
Q

What is the typical conditioning regime for Autologous SCT

A

High dose melphalan (bone marrow suppression)

36
Q

NB//

Although allogenic transplantation may cure Multiple Myeloma it carries a high procedure- related mortality

37
Q

Describe non-intesive therapy for elderly pts >70 yrs/ co morbidity

A

Monthly courses of MELPHALAN (oral alkylating agent),

sometimes in combination with PREDNISOLONE/THALIDOMIDE

are usually effective in reducing the tumour burden

38
Q

If a plateau in paraprotein levels occurs during Multiple Myeloma treatment what is the approach

A

Stop treatment

Consider maintenance Thalidomide

39
Q

If relapse of Multiple Myeloma (increasing paraprotein level) occurs in non invasive treatment, what is the approach

A

Further chemotherapy

  • cyclophosphamide
  • thalidomide
  • bortezomib
40
Q

If relapse of Multiple Myeloma (increasing paraprotein level) occurs in invasive treatment, what is the approach

A

Another Autologous SCT

Chemotherapy

41
Q

What is the international prognosis index based on serum B2-microglobulin

A

Patients with serum B2M > 5.5mg/L have poor prognosis

42
Q

What is the international prognosis index based on albumin levels

A

<35g/L has poor prognosis

43
Q

What is the median survival with non- intensive chemotherapy

A

3-4 yrs

(Improved by 1 yr with Autologous Transplantation)

44
Q

Define Solitary Plasmacytoma

A

These are isolated plasma cell tumours , usually of bone or soft tissue

45
Q

These are isolated plasma cell tumours , usually of bone or soft tissue

A

Solitary Plasmacytoma

46
Q

What is Plasma Cell Leukemia

A

This occurs either as a late complication of Myeloma or as a primary disease characterized by the presence of 20% or more plasma cells in the blood

((Outlook poor))

47
Q

This occurs either as a late complication of Myeloma or as a primary disease characterized by the presence of 20% or more plasma cells in the blood

((Outlook poor))

A

Plasma Cell Leukemia

48
Q

What is Heavy Chain Disease

A

In these rare disorders the Neoplastic cells secrete only incomplete immunoglobulin heavy chains (gamma, alpha, u)

49
Q

What is the most common form of Heavy Chain disease

A

Alpha- heavy chain disease

(Which occurs mainly in the Mediterranean area)

50
Q

Waldentröm’s macroglobulinaemia is seen most frequently in which age group and gender

A

Men over 50

51
Q

What causes Waldentröm’s macroglobulinaemia

A

Lymphoplasmacytoid lymphoma which produces monoclonal IgM paraprotein

52
Q

What are the clinical features of Waldentröm’s macroglobulinaemia

A 
Fatigue 
Weight loss
Hyperviscosity
Visual changes
Anemia
Moderate Lymphadenopathy 
Enlargement of liver and spleen
53
Q

Which is more likely to increase blood viscosity

IgM, IgA, IgG

54
Q

What is used to diagnose Waldentröm’s macroglobulinaemia

A

Made by finding of

  • A monoclonal serum IgM
  • Bone marrow / lymph node infiltration with lymphoplasmacytoid cells
  • Raised ESR
  • May be Peripheral blood lymphocytosis
55
Q

Is therapy needed for Waldentröm’s macroglobulinaemia pts with symptoms ?

56
Q

Which drugs are used for Waldentröm’s macroglobulinaemia therpay

A

Chlorambucil

Cyclophosphamide

57
Q

What is the therapy for advanced Waldentröm’s macroglobulinaemia disease

A

Stem Cell Transplantation

58
Q

How is Acute Hyperviscosity Syndrome treated

A

Repeated Plasmapheresis

59
Q

A serum paraprotein detected without any evidence of myeloma or underlying disease

A

Monoclonal Gammopathy of undetermined Significance (MGUS)

60
Q

Monoclonal Gammopathy of undetermined Significance (MGUS)

A

A serum paraprotein detected without any evidence of myeloma or underlying disease

61
Q

Is treatment needed for Monoclonal Gammopathy of undetermined Significance

62
Q

What is Amyloidosis

A

A heterogenous group of disorders characterized by the extra cellular depositions of protein in an abnormal fibrillar form

63
Q

A heterogenous group of disorders characterized by the extra cellular depositions of protein in an abnormal fibrillar form

A

Amyloidosis

64
Q

What is the classic diagnostic test for Amyloidosis

A

Red-green birefringence after staining with Congo red and viewing under polarized light

65
Q

What caused Systemic Amyloid disease

A

Deposition of monoclonal light chains produced from a clonal plasma cell proliferation

66
Q

What is the level of paraprotein in Systemic AL Amyloidosis

A

Very low and nit always detectable in urine or serum

67
Q

Clinical Features of Systemic Amyloidosis

A 
Heart Failure
Macroglossia
Peripheral neuropathy 
Carpal tunnel syndrome
Renal Failure
68
Q

What is the treatment for Systemic Amyloidosis

A

Chemotherapy

Autologous SCT

69
Q

What is the most common cause of Hyperviscosity syndrome

A

Polycythemia

70
Q

What is the term used to describe high RBC concentration

A

Polycythemia

71
Q

What are clinical features of Hyperviscosity Syndrome

A 
Visual disturbances
Lethargy
Confusion
Muscle Weakness
Nervous System symptoms and signs
Congestive heart failure
72
Q

List four types of Amyloidosis

A

Systemic AL Amyloidosis
Reactive Systemic AA Amyloidosis
Familial Amyloidosis
Localized Amyloidosis

Essential Haematology (14 decks)

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