Plasma cell disorders (dyscrasias) Flashcards

1
Q

Which cell proliferates in multiple myeloma?

A

plasma cells in the bone marrow

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2
Q

What may be found in the serum of someone with multiple myeloma?

A

IL6 which stimulates plasma cell growth and immunoglobulin production

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3
Q

Clinical features of multiple myeloma

A
Bone pain with hypercalcemia
Elevated serum protein
Increased risk of infection
Rouleaux formation
Primary AL amyloidosis
Proteinuria
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4
Q

Pathophys of bone pain in multiple myeloma?

A

the neoplastic plasma cells activate RNK receptor on osteoclasts –> bone destruction, punched out bone lesions, increased risk of fracture

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5
Q

What will you see on XRAY of someone with multiple myeloma?

A

punched out lesions of bone

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6
Q

What will you see on serum electrophoresis of someone with multiple myeloma and why?

A

M spike -represents monoclonal IgG or igA produced by neoplastic plasma cells

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7
Q

Why will someone with multiple myeloma have increased risk of susceptibility to infection?

A

Monoclonal antibody production lacks antigenic diversity

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8
Q

What will you see on blood smear of someone with multiple myeloma and why?

A

Rouleaux formation of RBCs - increased protein that decreases charge of RBCs

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9
Q

Why may someone have primary AL amyloidosis in multiple myeloma?

A

free light chains circulate in serum and deposit in tissue

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10
Q

What effect will multiple myeloma potentially have on the kidney?

A

free light chains excreted in urine as benze jones protein may deposit in tubules and lead to risk of renal faliure

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11
Q

Explain monoclonal gammopathy of undetermined significant (MGUS)

A

increased serum protein with M spike without other features of multiple myeloma

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12
Q

Who is the common population to get MGUS

A

elderly

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13
Q

complication of MGUS

A

May progress to multiple myeloma

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14
Q

What will you see on electrophoresis of MGUS?

A

M spike

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15
Q

What is over produced in Waldenstrom macroglobulinemia?

A

igM

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16
Q

Clinical features of waldenstrom macroglobulinemia

A

Generalized lymphadenopathy
Increased serum IgM (M spike)
Visual and neurologic deficits – from hyperviscosity
Bleeding -viscous serum causes defective platelet aggregation

17
Q

How to treat acute complications waldenstrom macroglobulinemia?

A

plasmapheresis which removes IgM

18
Q

What are the types of langherhans cell histiocytosis?

A

Letterer-siwe disease
Eosinophilic granuloma
Hand-Schuller-Christian disease

19
Q

Where are langherhans cells primarily found?

A

the skin

20
Q

What is the characteristic finding in langherhans cells histiocytosis on EM?

A

Birbeck granules

21
Q

What do the cells of langherhans cells histiocytosis stain positive for?

A

CD1a+ and S-100+

22
Q

Explain letterer-siwe disease

A

Malignant proliferation of langherhans clls
Classically presents as skin rash and cystic skeletal defects, rapidly fatal
children <2 years old

23
Q

Explain eosinophilic granuloma

A

Benign proliferation of langerhans cells in bone

Classic presentation is pathological fracture (does not involve skin)

24
Q

Explain Hand-Schuller-Christian disease

A

Malignant proliferation of langherhans cells that presents with scalp rash, lytic skull defects, diabetes insipidus and exopthalamus in child >3