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Hematology > Plasma cell disorders > Flashcards

Plasma cell disorders Flashcards

1
Q

_______ % MM patients have no identifiable M component

A

1

Light chain myeloma pts in whom light chains are catabolised by kidney

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2
Q

Bone pain in MM

A

Back and ribs

precipitated by movement

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2
Q

Light chain subtype and survival

A

lambda secretors have shorter survival

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2
Q

fundus in WM

A

Vascular segmentation

dilatation of retinal veins

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3
Q

Gamma heavy chain disease

A

franklin

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3
Q

Blood picture in gamma heavy chain disease

A

thrombocytopenia

eosinophilia

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4
Q

Factors associated with poor prognosis in MM

A

Histologic atypia

elevated LDH levels

High labeling index

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5
Q

Treatment of hypercalcemia in MM

A

Hydration

Natriuresis

Glucocorticoids

bisphosphonates

Calcitonin

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6
Q

Only light chains are produced in _______ % of myelomas

A

20%

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6
Q

Staging system for MM

A
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6
Q

Rx of hyperviscosity symptoms

A

plasmapheresis

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6
Q

Difference between Myeloma and waldenstrom macroglobulinemia

A

Hepatosplenomegaly and lymphadenopathy in WM

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7
Q

Na+ levels in MM

A

Pseudohyponatremia

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8
Q

Anion gap in MM

A

low

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8
Q

ROTI

A

Serum calcium: Increase of more than 1mg/dl above ULN or >11mg/dl

Serum creatinine > 1.95 mg/dl

anemia: Hb less than 2g/dl below LLN or

Bone lesions: Lytic,osteoporosis

Others: Hyperviscosity,amyloidosis,recurrent bacterial infections(>2 episodes/year)

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8
Q

Serum markers playing a role in MM prognosis

A

IL-6 receptor

HGF

CRP

C-terminal cross-linked telopeptide of collagen I

TGF beta

syndecan 1

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9
Q

Complication of cryoglobulin formation in MM

A

Raynaud phenomenon

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10
Q

Median age at onset of MM

A

70 years

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11
Q

qualitative assessment of M component is made by

A

immunoelectrophoresis

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12
Q

Factors associated with higher incidence of progression of MGUS to myeloma

A

Non IgG subtype

abnormal Kappa/lambda free light chain ratio

serum M protein > 1.5 g/dl

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13
Q

Splenomegaly and lymphadenopathy in MM

A

Unusual

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14
Q

__________ % of MM pts will have only light chains in serum and urine

A

20%

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14
Q

POEMS syndrome

A

polyneuropathy

organomegaly

endocrinopathy

Multiple myeloma

skin changes

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15
Q

Diagnostic criteria for MGUS

A

M protein in serum

Bone marrow clonal plasma cells

No evidence of other B cell proliferative disorders

No myeloma-related organ or tissue impairment (no end organ damage, including bone lesions)

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16
Q

Time relation between fall in M component and symptomatic improvement in MM after therapy

A

Fall in M component lags behind symptomatic improvement

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17
Q

__________ % of pts present with biclonal or triclonal gammopathy

A

1

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17
Q

Bone pain due to METs differs from that due to MM by

A

nocturnal pain

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19
Q

MM is uncommon under the age of

A

40 years

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19
Q

Plasma cell leukemia

A

Plasma cells > 2000/µL

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19
Q

presentation of gamma heavy chain disease

A

fever

lymphadenopathy

hepatosplenomegaly

anemia

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20
Q

Difference btw IgM myeloma and WM

A

IgM myeloma:

Lytic bone lesions

CD138 positive cells in bone marrow

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22
Q

Autoimmune conditions associated with M component

A

RA

MG

Cold agglutinin disease

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22
Q

Skin changes in POEMS syndrome

A

hyperpigmentation

hypertrichosis

skin thickening

digital clubbing

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23
Q

Cd of plasma cells

A

CD138

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24
Q

Alpha heavy chain disease

A

seligman

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26
Q

Most common infections in MM

A

pneumonia

pyelonephritis

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26
Q

Neuropathy in MM

A

More often sensory

associated with IgM

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26
Q

Important feature of level of M protein after therapy

A

Rate of its increase after therapy

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27
Q

Symptoms of Hyperviscosity occur at ______ centipoise

A

4

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27
Q

Difference btw MM and MGUS

A

Labeling index >1% in Myeloma

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27
Q

Treatment of MM variants

A

Highly responsive to local radiation

40Gy

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28
Q

Type of anemia in multiple myeloma

A

Normocytic normochromic

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28
Q

Lymphadenopathy in poems syndrome resembles

A

castleman disease

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30
Q

Causes of recurrent infections in MM

A

Hypogammaglobulinemia

low CD4 count

defective neutrophil migration

therapy related(dexamethasone)

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31
Q

alpha heavy chain disease is characterised by

A

infiltration of the lamina propria of the small intestine with lymphoplasmacytoid cells that secrete truncated alpha chains

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32
Q

Heavy chain diseases

A

Gamma

alpha

Mu

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33
Q

Amount of light chains normally excreted per day

A

<10mg

34
Q

common pathogens causing infections in MM

A

Lungs: s.pneumoniae,s.aureus,klebsiella pneumoniae

Urinary tract: E.coli and other gram negative pathogens

35
Q

Antigenic determinants

A

Isotype

Allotype

idiotype

36
Q

Characteristics of MM variants

A

younger age at onset

survival > 10 yrs

M component in

38
Q

Neoplastic diseases associated with M component

A

CLL

Lymphomas of B or T cell origin

CML

Breast,colon Ca

39
Q

Diagnostic criteria for asymptomatic Myeloma (Smoldering Myeloma)

A

M protein in serum 30 g/L and/or

Bone marrow clonal plasma cells 10%

No myeloma-related organ or tissue impairment (no end organ damage, including bone lesions)a or symptoms

41
Q

Reliable marker of tumor burden in

A

M component

41
Q

Course of MM variants

A

Solitary bone plasmacytomas may recur in other bony sites or evolve into myeloma

Extramedullary plasmacytomas rarely recur or progress

42
Q

Causes for DVT in MM

A

Thalidomide or lenalidomide

43
Q

mu heavy chain disease is associated with

A

CLL

44
Q

Major clinical manifestation of WM

A

hyperviscosity syndrome

45
Q

Bone marrow in WM

A

>10% lymphoplasmacytic infiltrate(surface IgM+,CD19+,CD20+,CD22+,Cd10-,CD23-)

Mast cells

46
Q

Response to therapy in MM is assessed by

A

Reduction in bone pain,hypercalcemia,anemia,infections

47
Q

Which is effective in AKI in MM?

A

Plasmapheresis to remove light chains

49
Q

causes of bone changes in MM

A

Activation of osteoclasts

inhibition of osteoblasts

tumor expansion

50
Q

Cytogenetic alterations in MM

A

13q,17p deletions

t(11;14)

t(4;14)

t(14;16)

hypodiploidy

52
Q

Most common cause of renal failure in MM

A

hypercalcemia

53
Q

Earliest manifestation of tubular damage in MM

A

Type 2 RTA

53
Q

Most common heavy chain disease

A

alpha heavy chain disease

54
Q

Skin diseases associated with M component

A

Lichen myxedematosus(papular mucinosis)

Necrobiotic xanthogranuloma

55
Q

presentation of alpha heavy chain disease

A

chronic diarrhoea

weight loss

malabsorption

extensive mesenteric and paraaortic adenopathy

57
Q

% of myeloma preceded by MGUS

A

100%

58
Q

Diagnostic criteria for Nonsecretory Myeloma

A

No M protein in serum and/or urine with immunofixation

Bone marrow clonal plasmacytosis 10% or plasmacytoma

Myeloma-related organ or tissue impairment (end organ damage, including bone lesions)

58
Q

most distinctive symptom of gamma heavy chain disease

A

palatal edema due to involvement of waldeyer ring

59
Q

rule of 10 in MM

A

>10% plasma cells in BM

10% of hematologic neoplasms

10% have smoldering myeloma

59
Q

endocrine manifestations of POEMS syndrome

A

amenorrhea

Impotence

gynacomastia

Hyperprolactinemia

hypothyroidism

DM

adrenal insufficieny

61
Q

protenuria in multiple myeloma

A

nearly all light chains

no albumin as glomerulus is intact

62
Q

Light chain excretion falls within _______(time) of treatment

A

first week

63
Q

renal disease in WM

A

Uncommon(as only 20% excrete light chains and there are no bone lesions and hypercalcemia)

65
Q

Causes of anemia in MM

A

Marrow infiltration by plasma cells

Decreased erythropoietin secretion by kidney

hemolysis

Hematopoiesis inhibitory factors

Megaloblastic anemia due to B12 or folate

66
Q

_________ % of MGUS develop Myeloma

A

1% per year

67
Q

Malignancy associated with alpha heavy chain disease

A

mediterranean lymphoma

68
Q

M component in gamma heavy chain disease

A

Usually IgG1

found in both serum and urine

doesnot react with light chain antibody

70
Q

Normal relative serum viscosity

A

1.8

71
Q

Plasma Cell disorders

A

MM

WM

Primary amyloidosis

heavy chain diseases

73
Q

Plasma cell leukemia is common in

A

IgD(12%) and IgE(25%) myelomas

74
Q

Rx of heavy chain diseases

A

combination chemotherapy used to treat low-grade lymphoma

76
Q

osteoclast activating factors in MM

A

IL-6

TNF

VEGF

RANK

Lymphotoxin

MIP 1 alpha(macrophage inhibitory factor)

77
Q

Macroglobulin in WM has specificity for

A

Myelin associated Glycoprotein

78
Q

Untreated IPSID pts progress to

A

lymphoplasmacytic and immunoblastic lymphoma

79
Q

Serum alkaline phosphatase in MM

A

Normal

81
Q

Rx of infections in MM

A

IV gamma globulins

No role for vaccines or prophylactic antibiotic admn

83
Q

Sensory neuropathy in MM

A

IgM

Thalidomide

Bortezomib

84
Q

Other manifestations of POEMS syndrome

A

Peripheral edema

ascites

pleural effusion

fever

thrombocytosis

85
Q

Diagnostic criteria for Symptomatic Multiple Myeloma

A

M protein in serum and/or urine

Bone marrow (clonal) plasma cells or plasmacytoma

Myeloma-related organ or tissue impairment (end organ damage, including bone lesions)

86
Q

Ig subtypes in MM associated with hyperviscosity syndromes

A

IgM

IgG3

IgA

87
Q

Classic triad of Myeloma

A

Marrow plasmacytosis>10%

lytic bone lesions

serum and/or urine M component

88
Q

Neurological disease associated with Monoclonal component

A

5% of sensorimotor neuropathy

90
Q

Most common symptom in myeloma

A

Bone pain (70%)

92
Q

Diagnostic criteria of Solitary Plasmacytoma of Bone

A

No M protein in serum and/or urine

Single area of bone destruction due to clonal plasma cells

Bone marrow not consistent with multiple myeloma

Normal skeletal survey (and MRI of spine and pelvis if done)

No related organ or tissue impairment (no end organ damage other than solitary bone lesion)

94
Q

Non neoplastic conditions associated with M component

A

sarcoidosis

parasitic diseases

cirrhosis

gaucher

pyoderma gangrenosum

95
Q

gamma heavy chain disease is frequently associated with

A

RA

96
Q

alpha heavy chain disease

A

seligman disease

98
Q

Usual sites of extramedullary plasmacytosis

A

nasopharynx and nasal sinuses

99
Q

Minimum concentration of monoclonal antibody to be detected by electrophoresis

A

0.5 g/dl

100
Q

Partially reversible renal diseases in MM

A

Light chain deposition disease

light chain cast nephropathy

amyloidosis

102
Q

Causes of Renal failure in MM

A

Hypercalcemia

urate nephropathy

light chain deposition disease

amyloidosis

drug induced(NSAID,Bisphosphonates)

contrast dye

104
Q

role of radioisotope bone scanning in MM

A

No role as there is no osteoblastic activity

105
Q

cord compression in MM

A

Radiation therapy

106
Q

Causes of neurologic symptoms in MM

A

Hyperviscosity

cryoglobulinemia

amyloid deposits

hypercalcemia

antineuronal antibodies

POEMS

therapy related

nerve compression

107
Q

Features responsible for higher risk of progression from smoldering myeloma to MM

A

bone marrow plasmacytosis >30%

abnormal kappa/lambda free light chain ratio

serum M protein >30 g/L (3 g/dL)

108
Q

light chain isotype in WM

A

Kappa(80%)

109
Q

Suppression of osteoblatic activity in MM is due to

A

dickhoff(DKK-1)

110
Q

Rx of relapsed myeloma

A

Lenalidomide

bortezomib

bortezomib and liposomal doxorubicin

111
Q

Side effects of bisphosphonate Rx

A

osteonecrosis of jaw

renal dysfunction(rare)

112
Q

persistent localised pain in MM

A

pathological #

113
Q

Rx of WM

A

Fludaribine

Cladribine

rituximab

114
Q

Cell of origin of WM

A

Post germinal center B cell

115
Q

concentrations of different Ig at which hyperviscosity symptoms appear

A

IgM: 4g/dl

IgG3: 5g/dl

IgA: 7g/dl

116
Q

% of serum M components

A

IgG 53%

IgA 25%

IgD 1%

117
Q

Most common primary bone neoplasm of adults

A

Multiple myeloma

Hematology (9 decks)

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