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immunoproliferative disorders Flashcards

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1
Q

monoclonal gammopathies

A

-uncontrolled proliferation of single clone of plasma cells

-cancerous

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2
Q

monoclonal gammopathies also called

A

plasma cell myeloma

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3
Q

monoclonal gammopathies more likely in

A

african americans

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4
Q

most common monoclonal gammopathy

A

IgG Kappa

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5
Q

what monoclonal gammopathy has worse prognosis

A

lambada chain

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6
Q

what is monoclonal gammopathy preceded by

A

smoldering MM- buildup of plasma cells

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7
Q

symptoms of monoclonal gammopathy

A

bone pain in lone bones
increased calcium
plasma cell infiltration in BM
increased protein

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8
Q

why increased calcium in monoclonal gammopathy

A

due to tumor in bone

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9
Q

why increased protein in in monoclonal gammopathy

A

-antibodies, specifically gamma region

-decreased albumin

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10
Q

bence jones proteins are over proliferation of

A

light chains

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11
Q

what is the weird solubility of bence jones proteins

A

-Soluble at RT
-Insoluble at 60 -Resoluble at 80

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12
Q

IFE- immunofixation is based on

A

electrophoresis and precipitation

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13
Q

waldenstrom macroglobulinemia serum is

A

extremely viscous- think

IgM

people faint because no blood flow to the brain

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14
Q

blood smear of Waldenström’s Macroglobinemia can see

A

rouleaux

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15
Q

in Waldenström’s Macroglobinemia can also see

A

cryoglobulins

-IgM precipitate in the cold

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16
Q

difference between Waldenström’s Macroglobinemia and MM

A

treatment

-Waldenström’s Macroglobulinemia often treated with plasmapheresis to get rid of the serum IgM

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17
Q

Waldenström’s Macroglobinemia seen on electrophoresis

A

alpha 2 beta region

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18
Q

are there a high amount of plasma cells in the BM in Waldenström’s Macroglobinemia

A

no

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19
Q

heavy chain disease

A

excess of heavy chain in serum

-doesn’t allow heavy and light chains to join together

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20
Q

heavy chain disease is most common is what chain

A

alpha

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21
Q

symptoms of heavy chain disease

A

-malabsorption- plasma cell infiltrate in intestine
-abdominal pain
-weight loss

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22
Q

polyclonal gammopathy electrophoresis

A

plateau in gamma region

smudge

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23
Q

is polyclonal a good thing

A

yes if response is controlled

-goes up when sick and down when normal

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24
Q

most common immunodeficiency disorder

A

b cell

primary or secondary

-recurrent infections

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25
defect of X-linked hypogammaglobinemia
maturation of b cells
26
another name for X-linked hypogammaglobinemia
bruton's disease -seen about 6 months of age
27
SPE result in X-linked hypogammaglobinemia
huge decreased in gamma region
28
most mature b cell in X-linked hypogammaglobinemia
pre-b cell no CD19 no plasma cells no germinal centers
29
what causes X-linked hypogammaglobinemia
defect of tyrosine kinase enzyme
30
what other part of immune system will also be decreased in X-linked hypogammaglobinemia
t cells because b cells are APCs
31
treatment of X-linked hypogammaglobinemia
antibiotics passive immunity
32
how is selective IgA deficiency acquired
congenital but can also be secondary to blood transfusion
33
IgA found in
mucosal areas
34
symptoms of selective IgA deficiency
respiratory GI allergies malabsorption
35
in selective IgA deficiency you produce
anti-IgA -will not affect until blood transfusion-- hemolytic
36
does passive immunity work in selective IgA deficiency
no -IgA supplements don't work
37
only true t cell deficiency
DiGeorge Syndrome
38
defect in DiGeorge Syndrome
thymus development -underdevelopment of parathyroid gland
39
physical appearance of DiGeorge Syndrome
wide set eyes low set ears -cardiac anomalies due to low calcium
40
only cure in DiGeorge Syndrome
fetal thymus transplantation or BM transplantation
41
DiGeorge Syndrome also called
CATCH 22 syndrome -deletion found on chromosome 22
42
physical characteristic of Chediak-Higaski
hypopigmented
43
blood smear of Chediak-Higaski
giant lysosomal granules found in granulocytes, lymphs, monos fusion of primary and secondary granules decreased neutrophils and plts
44
in Chediak-Higaski granules don't release
lysosomes which they should in order to become phagocytic
45
in Chronic Granulomatous Disease neutrophils look
normal unlike Chediak-Higaski
46
test for Chronic Granulomatous Disease
nitro blue tetrazolium reduction test
47
in normal people for nitro blue tetrazolium reduction test
breakdown tetrazolium to formazan
48
in abnormal people nitro blue tetrazolium reduction test
will be not see blue black granules cause no breakdown
49
flow cytometry for Chronic Granulomatous Disease
neutrophils labeled with DHR -we will see decrease DHR due to breakdown IN DISEASE - no fluorescence= no breakdown
50
Alder-Reilly Anomaly seen in mucopolysaccharide disorders such as
Hurler's syndrome
51
what occurs in Alder-Reilly Anomaly
incomplete breakdown of mucopolysaccharides -- result in large purple granules (lipids) halo around granules
52
Alder-Reilly Anomaly can be confused with
toxic granulation
53
granules in Alder-Reilly Anomaly seen in
lymphs and monos
54
May- Hegglin Anomaly inclusions
Dohle-body like inclusions in granulocytes RNA
55
if see dohle bodies in monos it is
not true May-hegglin because they are not normally seen here
56
another blood smear feature in May-Hegglin
giant platelets -non functional or decreased funcion
57
SCID
-collection of immunodeficiencies BOY IN BUBBLE
58
SCID affects
all t and b cell function
59
only treatment for SKID
BM transplant die early in life
60
Wiskott-Aldrick Syndrome main characteristics
-eczema -decreased platelets -deficiencies in isohemagglutinins
61
Wiskott-Aldrick Syndrome isohemagglutinins issue
NO IgM Abs to ABO antigens
62
increased levels of ____________ in Wiskott-Aldrick Syndrome
alpha fetoprotein IgE
63
only cure for Wiskott-Aldrick Syndrome
BM transplant
64
Ataxia Telangiectasia word breakdown
-Ataxia- affects muscle coordination -Telangiectasia- spider life veins defect in vascular system
65
increased risk of _______ in Ataxia Telangiectasia
lymphoid cancer TCR cluster in t cell affected deficient t and b cell lines
66
in Complement Deficiencies have
recurrent bacterial infections
67
C1 esterase inhibitor
-complement in check -herediatry angioedema -unchecked cleaving
68
most common complement def
C2 -affect classical cascade
69
C3 def affects
all pathways
70
C5-C9
increased infections

immuno (12 decks)

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