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Essential Haematology > Plasma Cell Disorders > Flashcards

Plasma Cell Disorders Flashcards

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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
What is the paraprotein observed in 20% of the multiple myeloma cases
IgA (20%)
26
What kind of formation is seen in a blood film for pts with Multiple Myeloma
Rouleaux formation
27
What percentage of Multiple Myeloma pts have serum calcium elevations
45%
28
What percentage of Multiple Myeloma pts have serum creatinine elevations
20%
29
Levels of B2- microglobulin less than what value indicates good prognosis for Multiple Myeloma
4mg:L
30
What are the two main types of treatment for Multiple Myeloma
Specific | Supportive
31
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)
Yupp
32
What are the two major treatments foe Multiple Myeloma
Intensive Therapy | Non-intensive therapy
33
What are the two main aims of Intensive therapy for Multiple Myeloma PS intensive therapy for <70 yrs and no co morbidity
Combination of several courses of chemotherapy to reduce the tumour burden Followed by stem cell collection and autologous SCT after high-dose chemotherapy
34
Repeated intravenous or oral chemotherapy cycles such as which drugs are used to treat Multiple Myeloma
``` Cyclophosphamide Dexmethasone Thalidomide Cyclophosphamide Vincristine Adriamycin Dexmethasone ```
35
What is the typical conditioning regime for Autologous SCT
High dose melphalan (bone marrow suppression)
36
NB// | Although allogenic transplantation may cure Multiple Myeloma it carries a high procedure- related mortality
Yup
37
Describe non-intesive therapy for elderly pts >70 yrs/ co morbidity
Monthly courses of MELPHALAN (oral alkylating agent), sometimes in combination with PREDNISOLONE/THALIDOMIDE are usually effective in reducing the tumour burden
38
If a plateau in paraprotein levels occurs during Multiple Myeloma treatment what is the approach
Stop treatment | Consider maintenance Thalidomide
39
If relapse of Multiple Myeloma (increasing paraprotein level) occurs in non invasive treatment, what is the approach
Further chemotherapy - cyclophosphamide - thalidomide - bortezomib
40
If relapse of Multiple Myeloma (increasing paraprotein level) occurs in invasive treatment, what is the approach
Another Autologous SCT | Chemotherapy
41
What is the international prognosis index based on serum B2-microglobulin
Patients with serum B2M > 5.5mg/L have poor prognosis
42
What is the international prognosis index based on albumin levels
<35g/L has poor prognosis
43
What is the median survival with non- intensive chemotherapy
3-4 yrs | (Improved by 1 yr with Autologous Transplantation)
44
Define Solitary Plasmacytoma
These are isolated plasma cell tumours , usually of bone or soft tissue
45
These are isolated plasma cell tumours , usually of bone or soft tissue
Solitary Plasmacytoma
46
What is Plasma Cell Leukemia
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
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))
Plasma Cell Leukemia
48
What is Heavy Chain Disease
In these rare disorders the Neoplastic cells secrete only incomplete immunoglobulin heavy chains (gamma, alpha, u)
49
What is the most common form of Heavy Chain disease
Alpha- heavy chain disease | (Which occurs mainly in the Mediterranean area)
50
Waldentröm’s macroglobulinaemia is seen most frequently in which age group and gender
Men over 50
51
What causes Waldentröm’s macroglobulinaemia
Lymphoplasmacytoid lymphoma which produces monoclonal IgM paraprotein
52
What are the clinical features of Waldentröm’s macroglobulinaemia
``` Fatigue Weight loss Hyperviscosity Visual changes Anemia Moderate Lymphadenopathy Enlargement of liver and spleen ```
53
Which is more likely to increase blood viscosity IgM, IgA, IgG
IgM
54
What is used to diagnose Waldentröm’s macroglobulinaemia
Made by finding of - A monoclonal serum IgM - Bone marrow / lymph node infiltration with lymphoplasmacytoid cells - Raised ESR - May be Peripheral blood lymphocytosis
55
Is therapy needed for Waldentröm’s macroglobulinaemia pts with symptoms ?
Nope
56
Which drugs are used for Waldentröm’s macroglobulinaemia therpay
Chlorambucil | Cyclophosphamide
57
What is the therapy for advanced Waldentröm’s macroglobulinaemia disease
Stem Cell Transplantation
58
How is Acute Hyperviscosity Syndrome treated
Repeated Plasmapheresis
59
A serum paraprotein detected without any evidence of myeloma or underlying disease
Monoclonal Gammopathy of undetermined Significance (MGUS)
60
Monoclonal Gammopathy of undetermined Significance (MGUS)
A serum paraprotein detected without any evidence of myeloma or underlying disease
61
Is treatment needed for Monoclonal Gammopathy of undetermined Significance
No
62
What is Amyloidosis
A heterogenous group of disorders characterized by the extra cellular depositions of protein in an abnormal fibrillar form
63
A heterogenous group of disorders characterized by the extra cellular depositions of protein in an abnormal fibrillar form
Amyloidosis
64
What is the classic diagnostic test for Amyloidosis
Red-green birefringence after staining with Congo red and viewing under polarized light
65
What caused Systemic Amyloid disease
Deposition of monoclonal light chains produced from a clonal plasma cell proliferation
66
What is the level of paraprotein in Systemic AL Amyloidosis
Very low and nit always detectable in urine or serum
67
Clinical Features of Systemic Amyloidosis
``` Heart Failure Macroglossia Peripheral neuropathy Carpal tunnel syndrome Renal Failure ```
68
What is the treatment for Systemic Amyloidosis
Chemotherapy | Autologous SCT
69
What is the most common cause of Hyperviscosity syndrome
Polycythemia
70
What is the term used to describe high RBC concentration
Polycythemia
71
What are clinical features of Hyperviscosity Syndrome
``` Visual disturbances Lethargy Confusion Muscle Weakness Nervous System symptoms and signs Congestive heart failure ```
72
List four types of Amyloidosis
Systemic AL Amyloidosis Reactive Systemic AA Amyloidosis Familial Amyloidosis Localized Amyloidosis

Essential Haematology (14 decks)

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