Ch 13: WBC, Lymph Node, Spleen, & Thymus Pt 1. Flashcards

1
Q

what are the two essential properties of hematopoietic stem cells

A

pluripotency
self-renewing

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2
Q

what are the two primary lymphoid organs

A

thymus
bone marrow

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3
Q

what are the five types of secondary lymphoid organs

A

lymph nodes
spleen
tonsils and adenoids
peyers patches
appendix

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4
Q

what is the most widely distributed and easily accessible lymphoid tissue

A

lymph nodes

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5
Q

spleen weight (16-20 yrs)

A

150-200g (170g)

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6
Q

spleen weight (20-65 yrs)

A

155 g

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7
Q

spleen weight (over 80 yrs)

A

100 g

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8
Q

what are the two broad categories of white cell disorders

A

proliferative disorders
leukopenias

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9
Q

what is leukopenia

A

deficiency of leukocytes

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10
Q

what is neutropenia

A

deficiency of neutrophils

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11
Q

what is a major consequence of leukopenia

A

agranulocytosis which makes you most susceptible to bacterial and funcal infections

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12
Q

what is the most common cause of agranulocytosis

A

drug toxicity

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13
Q

what are some consequences of agranulocytosis

A

ulcerating necrotizing lesions of the gingiva, floor of mouth, buccal mucosa, and pharynx
can cause severe, invasive bacterial or fungal infections in the lungs, urogenitital tract, or kidneys

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14
Q

a neutropenic patient is at high risk for what

A

deep fungal infections caused by candida and Aspergillus

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15
Q

what is lymphadenitis

A

activation of resident immune cells which leads to morphologic changes in lymph nodes
primary follicles enlarge and develop germinal centers

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16
Q

what are germinal centers

A

found in lymph nodes
highly dynamic structures in which B cells acquire the capacity to make high-affinity antibodies against specific antigens

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17
Q

unfiltered lymph flows into a lymph node through which vessel

A

afferent vessel

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18
Q

filtered lymph fluid flows out of a lymph node through which vessel

A

efferent vessel

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19
Q

what is follicular hyperplasia

A

presence of large germinal centers (secondary follicles) which are surrounded by naive B cells (mantle zone)
caused by stimuli that activate humoral immune responses

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20
Q

what is acute myeloid leukemia (AML)

A

immature progenitor cells accumulate in the bone marrow

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21
Q

what is myelodysplastic syndrome (MDS)

A

ineffective hematopoiesis and resultant peripheral blood cytopenias

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22
Q

what are myeloproliferative neoplasms

A

increased production of one or more terminally differentiated myeloid elements like granulocytes

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23
Q

what most commonly causes white cell neoplasms

A

translocations, specifically Philadelphia chromsome

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24
Q

what are three types of lymphotropic viruses

A

human T-cell leukemia virus-1 (HTLV-1)
EBV
human herpesvirus-8 (HHV-8)

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25
Q

what type of lymphoma accounts for 2/3rds of all T-cell lymphomas and is closely linked to celiac disease

A

enteropathy-associated T-cell lymphoma (EATL)

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26
Q

what is associated with and known to cause gastric B-cell lymphomas

A

H. pylori

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27
Q

what is associated with and known to cause anaplastic large cell lymphoma

A

breast implants

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28
Q

what are leukemias

A

neoplasms that present with widespread involvement of the bone marrow and peripheral blood

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29
Q

what are lymphomas

A

proliferations of white cells, usually lymphocytes, that usually present as discrete tissue masses like in the tonsils

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30
Q

what are the five types of lymphoid neoplasms

A

precursor B-cell
peripheral B-cell
precursor T cell
peripheral T and NK cell
hodgkin lymphoma

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31
Q

what is a type of T-cell associated marker that is also expressed by lymphocytic leukemia and mantle cell lymphoma

A

CD5 antibody

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32
Q

what does the receptor of a B cell look like

A

has two antigen binding sites

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33
Q

what does the receptor of a T cell look like

A

has just one antigen binding site

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34
Q

85-90% of lymphoid neoplasms are of which cell origin

A

B-cell

35
Q

what are some features of acute lymphoblastic leukemia/lymphomas (ALL)
-how does it typically show up

A

neoplasms composed of immature B or T cells (lymphoblasts)
most common cancer of children
aggresive and shows up as bone marrow failure or as a mass
75% cure rate with aggresive chemotherapy

36
Q

what is the most common cancer of children

A

acute lymphoblastic leukemia/lymphoma

37
Q

what are the three major symptoms of ALL

A

anemia
neutropenia (fever)
thrombocytopenia (bleeding)

38
Q

what is a nuclear protein widely used as a marker for pre-B and pre-T lymphoblast leukemias

A

terminal deoxynucleotidyl transferase antibody (TdT)

39
Q

how is flow cytometry used

A

helps identify cells in solution
used to evaluate peripheral blood, bone marrow, and other body fluids
identify and quantify immune cells and characterize hematological malignancies

40
Q

what are some features of chronic lymphocytic leukemia (CLL)

A

most common leukemia of adults in Western World
median survival is 4-6 years

41
Q

what symptoms are present in chronic lymphocytic leukemia (CLL)

A

usually asymptomatic but can present with weight loss, lymphadenopathy, and hepatosplenomegaly

42
Q

what is the most common leukemia of adults in the western world

A

chronic lymphocytic leukemia (CLL)

43
Q

what are the four pan B-cell markers expressed in CLL tumors

A

CD 5, 19, 20, and 23

44
Q

how can chronic lymphocytic leukemia affect the spleen

A

causes small, white lesions that are scattered through the nodular areas of the white pulp
looks like sago spleen but sago spleen is caused by deposition of amyloid - nodules are more gray-translucent

45
Q

what is the most common form of indolent non-hodgkins lymphoma in the US

A

follicular lymphoma

46
Q

what does indolent mean

A

causing little or no pain

47
Q

what causes follicular lymphoma

A

chromosomal
14:18 translocation
leads to overexpression of BCL2

48
Q

what does BCL-2 do

A

anti-apoptotic protein seen in follicular lymphoma that promtoes the survival of follicular lymphoma cells

49
Q

what is follicular lymphoma

A

type of peripheral B-cell neoplasm
germinal centers of nodes have many B cells that are not getting apoptosed as they should be
too much BCL-2

50
Q

BCL-2 is expressed in more than what percentage of follicular lymphoma cases

A

90%

51
Q

what lymphoid organs are most affected by follicular lymphoma

A

lymph nodes and bone marrow
can also been seen in liver and spleen sinusoids

52
Q

what are the two major histomorphologic patterns seen in follicular lymphomas

A

centrocytes and centroblasts

53
Q

what are centrocytes

A

small cells with irregular or cleaved nuclear contours and scant
seen in follicular lymphoma

54
Q

what are centroblasts

A

larger cells with open nuclear chromatin, several nucleoli, and modest amount of cytoplasm
seen in follicular lymphoma

55
Q

how does follicular lymphoma affect the spleen

A

white pulp follicles are expanded by follicular lymphoma cells

55
Q

what is the most common form of non-hodgkin lymphoma

A

diffuse large B-cell lymphoma (DLBCL)

56
Q

which sites are most affected by diffuse large B-cell lymphoma

A

extra nodal sites of the GI, CNS, skin, mediastinum, and bone

57
Q

what chromosome abnormality is seen in 20-30% of diffuse large B-cell lymphoma cases

A

rearragnements of BCL2 and BCL6 genes

58
Q

what are some features of diffuse large B-cell lymphoma

A

a type of peripheral B-cell neoplasm
presents as an enlarging mass at a node or extranodal site
have large cell size and diffuse pattern of growth
waldeyer ring commonly involved
very aggressive - 50% are cured with chemotheraphy

59
Q

what is the waldeyer ring

A

oropharyngeal lymphoid tissue that includes the tonsils and adenoids

60
Q

are the three main types of burkitt lymphoma

A

african (endemic)
sporadic (nonendemic)
subset of aggressive lymphomas

61
Q

what are some features of burkitt lymphoma

A

a type of peripheral B-cell lymphoma
extremely fast growing
shows infiltrate of medium sized lymphoid cells with round nuclei and several nucleoli
high mitotic index with apoptotic cells

62
Q

what causes burkitt lymphoma

A

caused by translocation of MYC gene on chromosome 8 which leads to increased MYC protein levels

63
Q

almost all endemic Burkitt lymphomas are infected with which virus

A

EBV

64
Q

what is the “starry sky” pattern and in which disease is it seen

A

macrophages that have phagocytosed oval nuclei with lots of nucleoli
seen in burkitt lymphoma

65
Q

where are african (endemic) burkitt lymphoma cases mostly seen (3 places)

A

jaw, orbit, and retroperitoneal

66
Q

where are sporadic (non-endemic) burkitt lymphoma cases mostly seen

A

GI tract, especially the ICV

67
Q

what types of neoplasms are mantle and marignal cell lymphoma and hairy cell leukemia

A

peripheral B-cell neoplasms

68
Q

what are three types of peripheral T and NK cell neoplasms

A

anaplastic large cell lymphoma
adult T-cell leukemia/lymphoma
mycosis fungoides/sezary syndrome

69
Q

what is anaplastic large-cell lymphoma

A

a type of peripheral T and NK cell neoplasm
presence of rearrangements in the ALK gene
breast implant associated ALCL which is extremely rare

70
Q

what is the incidence of breast implant associated ALCL

A

1 per 500,000-3 million women with implants

71
Q

what is adult T-cell leukemia/lymphoma

A

a type of peripheral T and NK cell neoplasm
neoplasm of helper T cells that is only observed in adults infected with the human T-cell leukemia virus type 1

72
Q

what was the first oncogenic human retrovirus to be discovered

A

adult T-cell leukemia/lymphoma

73
Q

how is human T-cell leukemia virus type 1 (HTLV-1) spread

A

through blood, breast milk, and semen

74
Q

what is mycosis fungoides/sezary syndrome

A

a type of peripheral T and NK cell neoplasm
primary cutaenous T-cell lymphoma seen in those that are 40+
tumor of helper T cells in the skin

75
Q

what is multiple myeloma (plasma cell myeloma)

A

a type of plasma cell neoplasm where the mutated plasma cells make abnormal antibodies that harm the body
presents as tumor masses throughout the skeletal system

76
Q

what are four symptoms of multiple myeloma

A

lytic bone lesions
hypercalacemia
renal failure
aquired immune abnormalities

77
Q

how does multiple myeloma affect the kidneys

A

neoplastic plasma cells suppress normal humoral immunity and secrete partial immunoglobulins that are toxic to the kidney
excretion of bence jones proteins cause myeloma kidney

78
Q

what is hodgkin’s lymphoma

A

lymphoma that arises in a single node or chain of nodes that spreads firm to anatomically contigous lymphoid tissues
diagnosed around 32 years

79
Q

what is the distinctive feature of hodgkin’s lymphoma

A

reed-sternberg cells

80
Q

what is one of the most common cancers of young adults and adolescents

A

hodgkin’s lymphoma

81
Q

hodgkin’s lymphoma accounts for what percentage of all new cancers in the US

A

0.7%

82
Q

what is the most common type (70%) of classic hodgkin lymphoma

A

nodular sclerosis

83
Q

what are the five subtypes of hodgkin’s lymphoma

A

nodular sclerosis
mixed cellularity
lymphocyte rich
lymphocyte depletion
nodular lymphocyte predominance