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Essential Hematology > Chapter 18: Multiple Myeloma > Flashcards

Chapter 18: Multiple Myeloma Flashcards

1
Q

What is paraproteinemia?

A

The presence of a monoclonal immunoglobulin band in the serum.

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2
Q

What is Multiple myeloma?

A

Multiple myeloma is a neoplastic proliferation of plasma cells usually in the bone marrow.

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3
Q

What is the epidemiology of multiple myeloma?

A

98% of cases occur over the age of 40 with a peak incidence in the seventh decade (60’s). More common among afro-caribbeans.

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4
Q

What are the characteristics of the myeloma cell?

A

Myeloma cells are post-germinal center plasma cells that have undergone class switching and somatic hypermutation. The immunophenotype is

(1) CD38 high
(2) CD138 high
(3) CD 45 low.

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5
Q

What is the cause of multiple myeloma?

A

The genetic changes involved in multiple myeloma are complex, but they usually involve

(1) increased or dysregulated expression of cyclin D
(2) Hyperploidy
(3) translocation of the IgH locus.
(4) 13q deletion.

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6
Q

What growth factor is important for myeloma cells?

A

IL-6

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7
Q

How is multiple myeloma diagnosed?

A

(1) monoclonal protein in serum or urine.
(2) increased plasma cell in the bone marrow
(3) Related organ or tissue impairment

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8
Q

What sorts of organ impairment occur in multiple myeloma?

A

(1) bone disease/hypercalcemia
(2) renal impairment
(3) anemia
(4) hyperviscosity (2%)
(5) amyloidosis (5%)
(6) infection

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9
Q

What is smouldering or asymptomatic myeloma?

A

Myeloma that is characterized greater than 10% plasma cell count in the bone marrow without organ impairment.

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10
Q

What causes the bone pain and hypercalcemia associated with multiple myeloma?

A

In multiple myeloma over expression of RANKL by the bone marrow stroma leads to activation of oseoclasts that breakdown bone leading to hypercalcemia and pathologic fractures.

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11
Q

What are the four major signs and symptoms of multiple myeloma?

A

CRAB

(1) Calcium increased
(2) renal failure
(3) anemia
(4) bone lesions.

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12
Q

Why do patients with multiple myeloma experience recurrent infections?

A

Because multiple myeloma leads to deficient antibody production and neutropenia.

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13
Q

What leads to the renal failure associated with multiple myeloma?

A

Deposition of light chains and calcium in the kidneys.

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14
Q

Why do multiple myeloma patients experience increased bleeding?

A

Because myeloma proteins interfere with platelet function and coagulation factors.

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15
Q

How often does amyloidosis occur secondary to multiple myeloma?

A

Amyloidosis occurs in about 5% of multiple myeloma patients.

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16
Q

How is the presence of paraprotein in the serum assayed?

A

By immunoglobulin electrophoresis. (usually IgG or IgA)

17
Q

What is unusual about the blood cells in patients with multiple myeloma?

A

Rouleaux formation (with normocytic/normochromic anemia)

18
Q

What abnormal laboratory values are associated with multiple myeloma?

A

(1) High ESR
(2) High CRP
(3) elevated serum calcium
(4) Elevated creatinine (20%)
(5) Serum Beta-2-microglobulin increased (associated with a poor prognosis)

19
Q

Is multiple myeloma curable?

A

Usually not! only younger patients who can undergo a successful allogenic stem cell transplant have a hope for a cure.

20
Q

How is intensive therapy used to treat multiple myeloma?

A

Intensive therapy is reserved for patients under the age of 65. it involves chemotherapy and autologous stem cell transplant.

21
Q

How is non-intensive therapy used to treat multiple myeloma?

A

In older patients melphalan is used in conjunction with prednisolone. Thalidomide or cyclophosphamide may also be used.

22
Q

What is the effect of bortezomib on multiple myeloma?

A

Bortezomib is a proteosome inhibitor that stops NFkB activation

23
Q

How is radiotherapy used to treat multiple myeloma?

A

Radiotherapy is useful for treating the symptoms of multiple myeloma including bone pain

24
Q

What supportive care is given to patients with multiple myeloma to manage renal failure, bone disease, paraplegia, anemia, bleeding, and infections?

A

(1) rehydration and dialysis for renal failure
(2) Bisphosphonates for bone disease and hypercalcemia
(3) radiation for compression paraplegia
(4) transfusion/EPO for anemia
(5) Plasmapharesis helps with bleeding
(6) Prophylaxis with antimicrobials and Ig infusions.

25
Q

What is the prognosis for multiple myeloma?

A

High albumin and low beta-2 microglobulin are associated with a better prognosis. High beta-2 microglobulin and low albumin are associated with a poor prognosis. median survival is 3-4 years with non-intensive therapy

26
Q

What are solitary plasmacytomas?

A

isolated plasma cell tumors usually of bone, respiratory tract, or GI tract. Lesions disappear after radiotherapy.

27
Q

What is plasma cell leukemia?

A

20% or more plasma cells in the blood can be primary or secondary to multiple myeloma. The prognosis is poor

28
Q

What is heavy chain disease?

A

A disorder in which cells secrete and incomplete heavy chain. Most common in mediterranean areas and starts with a malabsorbtion syndrome

29
Q

What is Waldenstrom’s macroglobulinemia (WM)?

A

A rare condition characterized by lymphoplasmacytoid lymphomas that secrete monoclonal IgM. Occurs most commonly in men over 50.

30
Q

How does WM usually present?

A

(1) fatigue
(2) weight loss
(3) Neurological symptoms
(4) dyspnea
(5) heart failure

31
Q

What secondary complications can result from WM?

A

(1) hyperviscosity (due to IgM)
(2) Visual problems (engorged retina)
(3) Anemia (from increased plasma volume)
(4) Bleeding (due to inhibition of platelets)

32
Q

How is WM diagnosed?

A

(1) monoclonal IgM
(2) Lymph node in filtration by lymphoplasmacytoid cells.
(3) peripheral blood lymphocytosis.

33
Q

What specific treatments are used for WM?

A

Chlorambucil or cyclophosphamide are the mainstays of therapy. Rituximab may also be used. SCT may be considered in severe cases.

34
Q

What supportive therapies are used for WM?

A

(1) plasmapheresis for hyperviscosity

(2) EPO for anemia.

35
Q

What is monoclonal gammopathy of undetermined significance (MGUS)?

A

MGUS is characterized by transient or persistent paraproteins in the serum without evidence of myeloma or other underlying disease.

36
Q

Why does systemic amyloidosis sometimes develop secondary to myeloma?

A

Because the deposition of Ig light chains can cause amyloidosis.

37
Q

What are the clinical features of amyloidois?

A

(1) Heart failure
(2) macroglossia
(3) peripheral neuropathy
(4) carpel tunnel
(5) renal failure

Essential Hematology (22 decks)

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