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Clinical Immunology > Immunoproliferative Disorders > Flashcards

Immunoproliferative Disorders Flashcards

1
Q

What type of cell produces most of the immunoglobulin G or A in normal adult serum?

A

Plasma Cells

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2
Q

Are hypergammaglobulinemias monoclonal or polyclonal in nature?

A

They are both monoclonal and polyclonal

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3
Q

What are a few key factors about Monoclonal gammopathy?

A

Can be benign or malignant
results from single clone of plasma cells producing a single class elevation of immunoglobulins (ex. monoclonal protein/paraprotein)
Multiple myeloma and Waldentrom macroglobulinemias

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4
Q

What are some key factors about Polyclonal Gammopathy?

A

classified as a secondary disease
characterized by the elevation of two or more immunoglobulins of several clones of plasma cells

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5
Q

General Characteristics of Monoclonal Gammopathies include…?

A

production of monoclonal immunoglobulins
associated w/ suppressed uninvolved immunoglobulins and dysfunctional t cell responses
MM-prototypical monoclonal gammopathy
Serum and urine electrophoresis can show abnormal results

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6
Q

Do symptomatic pt show characteristic monoclonal band/spike in protein patterns?

A

some pt do not show characteristic monoclonal spike in protein patterns - seen with light chain disease

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7
Q

what is a common abnormality in polyclonal gammopathies?

A

common protein abnormality

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8
Q

What is increased in general characteristics of polyclonal gammopathies?

A

there is an increase in more than one immunoglobulin, involving several clones of plasma cells

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9
Q

True or false: Polyclonal Gammopathies consist of one or more heavy chain classes and both light chain types.

A

true

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10
Q

What are some secondary manifestations of infection or inflammation of Polyclonal Gammopathies?

A

chronic infections, chronic liver disease, lymphoproliferative disorders and rheumatoid connective diseases

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11
Q

Multiple Myeloma is characterized by?

A

Plasma cell Neoplasms.
accumulation of malignant plasma cells within the bone marrow microenvironment, monoclonal protein in blood or urine, associated organ dysfunction

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12
Q

What is the normal % of plasma cells in Bone Marrow vs Multiple Myeloma?

A

BM -1%
MM -90%

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13
Q

MM Etiology

A

cause is uncertain, possible causes include radiation, viral, environmental stim: LIKELY A GENETIC FACTOR

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14
Q

true or false: plasma cells produce one of five heavy-chain types together with K and lambda (no key for that rip) molecules

A

true

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15
Q

True or false: There is approximately 30% excess production of free LC over HC synthesis to allow proper conformation of intact immunoglobulin molecules

A

False: it is approximately 40%

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16
Q

What is the most likely cause of MM?

A

Most likely is Genetic factors that cause Multiple myeloma

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17
Q

What cells do myelomas arise from?

A

They arise from asymptomatic premalignant proliferation of monoclonal plasma cells

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18
Q

True or false: MM myelomas arise from cells that are often immature and may have the appearance of plasmablasts

A

true

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19
Q

In MM, most patients have what kind of abnormality? (Pathophysiology)

A

most pt have complex karyotype abnormalities - chromosomal gains, deletions and translocations

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20
Q

Do myeloma cells proliferate slowly or quickly? Where do they proliferate?

A

They proliferate slowly in the marrow - absolute number of these cells correlates with disease activity and predicts progression in SMOLDERING MM (wtf is smoldering mm)

Smoldering MM made me think of the Flynn Rider smolder lol

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21
Q

What Interleukin is essential for survival and growth of myeloma cells?

A

IL-6 - promotes survival of myeloma cells and prevents apoptosis

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22
Q

What is the most common form of dysproteinemia?

A

Multiple myeloma

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23
Q

What is the average age of onset of MM? When is the peak?

A

onset is around 40-70 yrs with a peak incidence in 70s, less than 2% under 40yrs old

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24
Q

What is the most common immunoglobulin in MM?

A

IgG is the most common (IgG3 likely to experience hyperviscosity syndrome)

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25
Q

What is the most common cause of death in pt with MM?

A

Most patients die from infections and renal insufficiency, MM is progressive and pt usually die within 1-3 yrs :(

26
Q

What are the most common signs/symptoms of MM?

A

bone pain in chest/back is the most common
also weakness, fatigue, pallor, renal issues
20% hepatomegaly
5% splenomegaly

27
Q

What are some hematologic features of MM?

A

Increased plasma cells in BM or biopsy
Bleeding, abnormal PLTs and thrombocytopenia

28
Q

What % of pt have broadly disseminated destruction of the skeleton in MM?

A

90% of pts, responsible for the bone pain (how does it hurt if you dont have bones?) 80% of pt have punched out bones/lytic areas

29
Q

True or false: Renal failure is common and occurs more with IgD and light chain myeloma in MM?

A

True

30
Q

Proteinuria is common in MM. Over half of MM pt’s excrete what protein abnormally to cause this?

A

half of all MM pt excrete abnormal amounts of Bence-Jones Proteins (light chains)

31
Q

What are the two ways Bence Jones proteins cause issues in MM?

A

Via intratubular precipitation and intrarenal obstruction (referred to as myeloma kidney)
or Via direct tubular cell injury (renal acidification is observed)

32
Q

True or false: MM causes compression of the spinal cord and or nerves, as well as bladder incontinence

A

True

33
Q

Immunologic manifestations of MM include defects in what type of immunity?

A

Humoral but not cellular immunity

34
Q

What is the most consistent immunologic manifestation of MM?

A

incessant synthesis of dysfuncional single monoclonal protein or immunoglobulin chains of fragments

35
Q

True or false: Normochromic normocytic anemia is present in 2/3rd of pt at diagnosis of MM

A

True

36
Q

What is a common rbc morphology found in MM pts?

A

Rouleaux rbcs

37
Q

What CD classes (ex CD4+ or CD8+) are increased/decreased in MM?

A

CD4+ is reduced (helper) and CD8+ is increased (Cytotoxic)

38
Q

What is a molecular testing method used in the identification of MM?

A

FISH (fluorescent in situ hybridization) test of bone marrow ESSENTIAL

39
Q

One is an important diagnostic marker in MM?

A

Bence jones proteins are an important marker, seen with typical monoclonal gammopathy and in free LCD

40
Q

True or false: Very small amounts of BJ proteins in serum are not associated with clinical significance in MM

A

False they are very significant

41
Q

Bence jones proteins are soluble at RT, become insoluble (form precipitate) at ____ degrees, then dissolve at ____ degrees. (this is unique to BJ proteins)

A

become insoluble at 60 degrees celsius and dissolve at 100 degrees celsius and the pattern reverses when cooled

42
Q

FLC (free light chains) occurs in what cells?

A

B cell development and plasma cells, normally rapidly cleared and metabolized by the kidneys

43
Q

Immunoelectrophoresis for MM determines the blood levels of what immunoglobulin classes?

A

IgM, IgA, IgG
(also 90% of pt will have a monoclonal protein)

44
Q

Assume the diagram on the left is normal, what disease is present in the diagram on the right?

A

MM

45
Q

Treatment of MM includes what three supportive treatments?

A

biophosphonates to reduce risk of fractures
Plasmaphoresis to remove abnorm proteins
Surgery to remove tumors/prevention of fractures

46
Q

What kind of cell disorder is associated with Waldenstrom Macroglobulinemia?

A

B cell disorder characterized by infiltration of lymphoplasmacytic cells and IgM monoclonal gammopathy

47
Q

True or false: Waldenstrom Macroglobulinemia is considered a lymphoplasmacytic lymphoma

A

True

48
Q

What is the cause of Waldenstrom Macroglobulinemia?

A

Unknown cause- possibly genetic, but has malignant lymphs-plasma cells with abnormal amounts of IgM

49
Q

What age does Waldenstrom Macroglobulinemia mostly occur in?

A

mostly 60-64yr olds, disease onset is insidious and survival is approx 3yrs post diagnosis

50
Q

The pace of manifestation of Waldenstrom Macroglobulinemia is determined by the rate of profileration of what clone?

A

IgM-secreting Clones

51
Q

In Waldenstrom Macroglobulinemia, bleeding from what areas causes the most concern?

A

Bleeding from the gums/nose

52
Q

What are some hematologic abnormalities associated with Waldenstrom Macroglobulinemia?

A

chronic anemia/bleeding, bruising, disturbed PLT function, increases ESR..etc (idk how important this is so i picked the most important sounding ones)

53
Q

True or false: Pickles are good

A

true (if you said false you’re dead to me)

54
Q

What is the predominant form of renal injuries in Waldenstrom Macroglobulinemia?

A

Glomerular lesions

55
Q

What is the basic abnormality in Waldenstrom Macroglobulinemia?

A

Uncontrolled prolif of B lymphs and plasma cells, heavy accum of IgM in plasma and plasmacytoid in BM

56
Q

Assume left is normal, what disease is present on the right?

A

Waldenstrom Macroglobulinemia

57
Q

What are some treatment methods for Waldenstrom Macroglobulinemia?

A

Plasmapheresis, drugs, and various therapies

58
Q

Monoclonal Gammopathy of Undetermined Significance (MGUS) has presence of what kind of Protein? What gender is it most frequent in?

A

Monoclonal proteins, increases with age and more common in males

59
Q

What is the most common Ig class in MGUS?

A

IgG followed by IgM

60
Q

What are some facts about Light-Chain disease?

A

10-15% of monoclonal gammopathies
only K or lambda light chains or BJ proteins produced

61
Q

What are some facts about Heavy-Chain disease?

A

Franklins disease - IgG heavy chain disease
Alpha Heavy-chain is most common (men w mediterranean descent)
Mu heavy-chain disease is rare

Clinical Immunology (14 decks)

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