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Diseases of White Blood Cells, Lymph Nodes, Spleen, and Thymus > Robbins > Flashcards

Robbins Flashcards

1
Q

Blood cell progenitors first appear during the third week of embryonic development in the

A

yolk sac

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2
Q

Cells derived from the _____ are the source of long-lived tissue macrophages such as microglial cells in the brain and Kupffer cells in the liver

A

yolk sac

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3
Q

During the third month of embryogenesis, HSCs migrate to the ______, which becomes the chief site of blood cell formation until shortly before birth

A

liver

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4
Q

By birth, _____ hematopoietically active, and hepatic hematopoiesis dwindles to a trickle, persisting only in scattered foci that become inactive soon after birth.

A

bone marrow

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5
Q

T/F After puberty, hematopoiesis continuous throughout the entire skeleton.

A

False:
After puberty, hematopoiesis ceases in distal bones and becomes restricted to the axial skeleton.

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6
Q

refers to the ability of a single hematopoietic stem cell (HSC) to generate all mature blood cells.

A

Pluripotency

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7
Q

T/F In adulthood, HSCs are immobile and remain in the bone marrow

A

False

Under conditions of stress, such as severe anemia or acute inflammation, HSCs are mobilized from the bone marrow and appear in the peripheral blood

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8
Q

some divisions of HSCs give rise to cells referred to as ________, which are more proliferative than HSCs but have a lesser capacity for self-renewal

A

multipotent progenitors

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9
Q

T/F In acute viral infections, lymphopenia stems from an overall decrease in the number of lymphocytes in the body

A

False

In acute viral infections, lymphopenia actually stems from lymphocyte redistribution (sequestration of activated T cells in lymph nodes and increased adherence to endothelial cells)

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10
Q

2 causes of neutropenia

A

inadequate or ineffective granulopoiesis

increased destruction or sequestration of neutrophils in the periphery

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11
Q

The most common cause of agranulocytosis is

A

drug toxicity

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12
Q

mechanisms of leukocytosis (4)

A

Increased Marrow Production

Increased Release From Marrow Stores

Decreased Margination

Decreased Extravasation Into Tissues

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13
Q

T/F exercise may cause leukocytosis as it decreases margination

A

True

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14
Q

In _________ leukocytosis is often accompanied by morphologic changes in neutrophils, such as toxic granulations, Döhle bodies, and cytoplasmic vacuoles

A

sepsis or severe inflammatory disorders

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15
Q

This morpholigic change in neutrophils called______ are coarser and darker than normal neutrophilic granules and represent abnormal azurophilic (primary) granules

A

toxic granules

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16
Q

This morpholigic change in neutrophils called ____ are patches of dilated endoplasmic reticulum that appear as sky-blue cytoplasmic “puddles.”

A

Döhle bodies

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17
Q

hyperplasia caused by stimuli that activate humoral immune responses. It is defined by the presence of large oblong germinal centers (secondary follicles), which are surrounded by a collar of small resting naive B cells (the mantle zone)

A

follicular hyperplasia

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18
Q

macrophages in the germinal centers containing the nuclear debris of B cells are called

A

tingible-body macrophages

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19
Q

When do B cells in the germinal centers undergo apoptosis?

A

B cells in the germinal centers undergo apoptosis if they fail to produce an antibody with a high affinity for the presented antigen

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20
Q

Features favoring a reactive follicular (nonneoplastic) hyperplasia (3)

A
  1. preservation of the lymph node architecture, including the interfollicular T-cell zones and the sinusoids
  2. marked variation in the shape and size of the follicles
  3. presence of frequent mitotic figures, phagocytic macrophages, and recognizable light and dark zones, all of which tend to be absent from neoplastic follicles
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21
Q

hyperplasia caused by stimuli that trigger T-cell–mediated immune responses, such as acute viral infections

A

Paracortical hyperplasia

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22
Q

is marked by an increase in the number and size of the endothelial cells that line lymphatic sinusoids and increased numbers of intrasinusoidal macrophages, which expand and distort the sinusoids

A

Sinus histiocytosis

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23
Q

Three lymphotropic viruses implicated as causative agents in particular lymphomas

A

HTLV-1
EBV
HHV-8

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24
Q

World Health Organization (WHO) classification scheme uses morphologic, immunophenotypic, genotypic, and clinical features to sort the lymphoid neoplasms into five broad categories namely

A
  1. Precursor B-cell neoplasms
  2. PeripheralB-cellneoplasms
  3. Precursor T-cell neoplasms
  4. Peripheral T-cell and NK-cell neoplasms
  5. Hodgkin lymphomas
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25
Q

T/F Lymphoid neoplasia can be diagnosed based on clinical features

A

False
Lymphoid neoplasia can be suspected based on clinical features, but histologic examination of lymph nodes or other involved tissues is required for diagnosis

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26
Q

T/F all daughter cells derived from the malignant progenitor share the same antigen receptor gene configuration and sequence and synthesize identical antigen receptor proteins

A

true

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27
Q

T/F vast majority of lymphoid neoplasms are of B-cell origin

A

True

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28
Q

T/F Lymphoid neoplasms are often associated with immune abnormalities

A

True

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29
Q

T/F Non-Hodgkin lymphoma spreads in an orderly stepwise fashion, whereas most forms of Hodgkin lymphomas disseminate widely and somewhat unpredictably early in their course

A

False

Hodgkin lymphoma spreads in an orderly stepwise fashion, and most forms of NHL disseminate widely and unpredictably early in their course

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30
Q

the most common cancer of children

A

Acute Lymphoblastic Leukemia/Lymphoma (ALL)

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31
Q

T/F Peak incidence of B-ALL is in adolescence, the age when the thymus reaches maximum size

A

False, T-ALL

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32
Q

Most T-ALLs have mutations in ___ a gene that is essential for T-cell development

A

NOTCH1

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33
Q

a high fraction of B-ALLs have mutations affecting genes such as ___(4) , all of which are required for the proper differentiation of early hematopoietic precursors

A

PAX5, TCF3, ETV6, and RUNX1

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34
Q

Histochemical stains are helpful in distinguishing ALL from AML, that (in contrast to myeloblasts) lymphoblasts are myeloperoxidase (positive or negative?)

A

negative

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35
Q

_____ is a specialized DNA polymerase that is expressed only in pre-B and pre-T lymphoblasts and ihc staining for this is positive in 95% of cases of ALL

A

terminal deoxynucleotidyl transferase (TdT)

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36
Q

B-ALL lymphoblasts usually express the pan B-cell marker CD19 and the transcription factor PAX5 as well as CD10. However, in very immature B-ALLs, one of the 3 is negative.

A

CD10

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37
Q

in T-ALLs these usual T-cell anitgens are negative in more immature tumors (3)

A

CD3, CD4, and CD8

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38
Q

factors are associated with a worse prognosis in ALL (3)

A
  1. <2 years of age
  2. presentation in adolescence or adulthood
  3. peripheral blood blasts greater than 100,000
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39
Q

factors are associated with a favorable prognosis in ALL (5)

A
  1. age between 2 and 10 years
  2. low white cell count
  3. hyperdiploidy
  4. trisomy of chromosome 4, 7, and 10
  5. presence of t(12;21)
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40
Q

T/F The BCR-ABL protein in B-ALL with a t(9;22), has stronger tyrosine kinase activity than the form of BCR-ABL that is found in chronic myeloid leukemia

A

True

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41
Q

T/F Clinical presentations of ALL and AML vary widely

A

False

they are clinically very similar

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42
Q

The most common genetic anomalies in CLL/SLL are (4)

A

deletions of 13q14.3, 11q, and 17p and trisomy 12q

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43
Q

T/F. Chronic lymphocytic leukemia (CLL) and small lymphocytic lymphoma (SLL) differ only in the degree of peripheral blood lymphocytosis

A

True

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44
Q

overexpression of the anti-apoptotic protein ______, is uniformly observed in CLL/SLL

A

BCL2

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45
Q

pathognomonic for CLL/SLL

A

proliferation centers

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46
Q

In this peripheral B-Cell neoplasm, lymph nodes are diffusely effaced by predominantly small lymphocytes 6 to 12 μm in diameter with round to slightly irregular nuclei, condensed chromatin, and scant cytoplasm.

A

CLL/SLL

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47
Q

In CLL/SLL the blood contains variable numbers of small round lymphocytes with scant cytoplasm. Some of these cells are usually disrupted in the process of making smears, producing so-called

A

smudge cells

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48
Q

CLL/SLL has a distinctive immunophenotype. The tumor cells express the pan B-cell markers CD19 and CD20, as well as _______(2)

A

CD23 and CD5

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49
Q

Transformation of CLL/SLL to diffuse large B-cell lymphoma (DLBCL) is called _____ syndrome

A

Richter syndome

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50
Q

Richter syndrome is often heralded by the development of a rapidly enlarging mass within a lymph node or the spleen. It is often associated with acquisition of new abnormalities involving the genes _____(2)

A

TP53 and MYC

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51
Q

Follicular lymphoma is strongly associated with chromosomal translocations involving

A

BCL2

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52
Q

hallmark translocation of folliulcar lymphoma which leads to overexpression of BCL2

A

t(14;18)

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53
Q

Two principal cell types present in varying proportions in follicular lymphoma

A

centrocytes

centroblasts

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54
Q

in follicular lymphoma large cells with open nuclear chromatin, several nucleoli, and modest amounts of cytoplasm, are referred to as

A

centroblasts

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55
Q

in follicular lymphoma small cells with irregular or cleaved nuclear contours and scant cytoplasm are referred to as

A

centrocytes

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56
Q

follicular lymphoma ihc:
CD20
CD10
BCL6
BCL2
CD5

A

CD20 +
CD10 +
BCL6 +
BCL2 +
CD5 -

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57
Q

T/F Follicular lymphoma is incurable

A

True

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58
Q

90% of follicular lymphomas have a mutation in

A

KMT2D

59
Q

drives Warburg metabolism and rapid cell growth

A

MYC mutation

60
Q

the most common form of NHL in adults

A

Diffuse large B-cell lymphoma (DLBCL)

61
Q

One frequent pathogenic event in DLBCL is dysregulation of _____, a DNA-binding zinc-finger transcriptional repressor that is required for the formation of normal germinal centers

A

BCL6

62
Q

T/F DLBCLs with MYC translocations have a worse prognosis

A

True

63
Q

All forms of Burkitt lymphoma are associated with translocations of the ___ gene on chromosome ___

A

MYC gene on chromosome 8

64
Q

MYC is a master transcriptional regulator that increases the expression of genes that are required for aerobic glycolysis called the ____ effect

A

Warburg effect

65
Q

Essentially all endemic Burkitt lymphomas are latently infected with

A

EBV

66
Q

This tumor exhibits a high mitotic index and contains numerous apoptotic cells, the nuclear remnants of which are phagocytosed by interspersed benign macrophages.These phagocytes have abundant clear cytoplasm, creating a characteristic “starry sky” pattern.

A

Burkitt lymphoma

67
Q

category of burkitt lymphoma that often presents as a mass involving the mandible and shows an unusual predilection for involvement of abdominal viscera, particularly the kidneys, ovaries, and adrenal glands

A

endemic

68
Q

category of burkitt lymphoma that often appears as a mass involving the ileocecum and peritoneum

A

sporadic

69
Q

Virtually all mantle cell lymphomas have this translocation

A

t(11;14)

70
Q

is over expressed in mantle cell lymphoma

A

cyclin D1

71
Q

up-regulation of cyclin D1 in mantle cell lymphoma promotes ______ progression during the cell cycle

A

G1- to S-phase

72
Q

NHL most likely to present with mucosal involvement of the small bowel or colon produces polyp-like lesions (lymphomatoid polyposis)

A

mantle cell lymphoma

73
Q

Typically, the proliferation in this NHL consists of a homogeneous population of small lymphocytes with irregular to occasionally deeply clefted (cleaved) nuclear contours. Large cells resembling centroblasts and proliferation centers are absent

A

Mantle cell lymphoma

74
Q

Mantle cell IHC
cyclin D1
CD20
CD5
CD23

A

cyclin D1 +
CD20 +
CD5 +
CD23 -

75
Q

T/F mucosa-associated lymphoid tumors (or MALTomas) often arise within tissues involved by chronic inflammatory, remain localized for prolonged periods, and do not regress even if the inciting agent is eradicated

A

False

first 2 descriptions are true but the last is false. They may regress if the inciting agent (e.g., H. pylori) is eradicated.

76
Q

hairy cell leukemia is associated most commonly with this point mutation

A

BRAF V600E

77
Q

is the most common clinical and sometimes the only abnormal physical finding in hairy cell leukemia

A

splenomegaly

78
Q

most common indolent lymphoma of adults

A

follicular lymphoma

79
Q

This uncommon entity is defined by the presence of rearrangements in the ALK gene on chromosome 2p23

A

Anaplastic Large-Cell Lymphoma (ALK Positive)

80
Q

has a worse prognosis
ALK-/ALK+ anaplastic large cell lymphoma

A

ALK-

81
Q

This neoplasm of CD4+ T cells is observed only in adults infected by HTLV-1

A

Adult T-Cell Leukemia/Lymphoma

82
Q

are different manifestations of a tumor of CD4+ helper T cells that home to the skin (2)

A

Mycosis fungoides and Sézary syndrome

83
Q

is a variant of CD4+ helper T cell tumors in which skin involvement is manifested as a generalized exfoliative erythroderma

A

Sézary syndrome

84
Q

Approximately 30% to 40% of large granular lymphocytic leukemias have acquired mutations in the transcription factor

A

STAT3

85
Q

T-cell variants of large granular lymphocytic leukemias express CD3 on their surfaces, whereas NK-cell variants lack surface CD3 and express NK markers such as

A

CD56

86
Q

some with this syndrome, characterized by the triad of rheumatoid arthritis, splenomegaly, and neutropenia, have large granular lymphocytic leukemias as an underlying cause

A

Felty syndrome

87
Q

white blood cell neoplasm which most frequently presents as a destructive nasopharyngeal mass. The tumor cell infiltrate typically surrounds and invades small vessels, leading to extensive ischemic necrosis. In touch preparations, large azurophilic granules are seen in the cytoplasm of the tumor cells that resemble those found in normal NK cells.

A

Extranodal NK/T-Cell Lymphoma

88
Q

Extranodal NK/T-cell lymphoma is highly associated with this virus

A

EBV

89
Q

surface protein that serves as the B-cell EBV receptor

A

CD21

90
Q

In most patients with plasma cell tumors, the level of free light chains is elevated and markedly skewed toward one light chain (e.g., κ) at the expense of the second (e.g., λ). Because free light chains are small in size, they are also excreted in the urine, where they are referred to as

A

Bence Jones proteins

91
Q

the most important plasma cell neoplasm, usually presents as tumor masses scattered throughout the skeletal system associated with lytic bone lesions, hypercalcemia, renal failure, and acquired immune abnormalities

A

Multiple myeloma (plasma cell myeloma)

92
Q

plasma cell neoplasm that presents as a single mass in bone or soft tissue

A

Solitary myeloma (plasmacytoma)

93
Q

plasma cell neoplasm defined by a lack of symptoms and a high plasma M component >3g/dL

A

Smoldering myeloma

94
Q

is a syndrome in which high levels of IgM lead to symptoms related to hyperviscosity of the blood. It occurs in older adults, most commonly in association with lymphoplasmacytic lymphoma

A

Waldenström macroglobulinemia

95
Q

plasma cell disorder wherein patients have no signs or symptoms but have small to moderately large M components <3g/dL in their blood.

A

Monoclonal Gammopathy of Undetermined Significance (MGUS)

96
Q

Virtually all cases of lymphoplasmacytic lymphoma are associated with acquired mutations in

A

MYD88

97
Q

In this B-cell neoplasm, the marrow contains an infiltrate of lymphocytes, plasma cells, and plasmacytoid lymphocytes in varying proportions, often accompanied by mast cell hyperplasia

A

Lymphoplasmacytic lymphoma

98
Q

Multiple myeloma is genetically heterogeneous and is associated with frequent rearrangements involving the

A

IGH locus

99
Q

Morphologically, the distinctive feature of Hodgkin lymphoma is the presence of neoplastic giant cells called

A

Reed-Sternberg cells

100
Q

T/F Hodgkin lymphoma arises in a single node or chain of nodes and spreads first to anatomically contiguous lymphoid tissues

A

true

101
Q

first human cancer to be successfully treated with radiation therapy and chemotherapy

A

Hodgkin lymphoma

102
Q

out of the 5 subtypes of Hodgin lymphoma, 4 make up the “classic” forms namely

A
  1. Nodular sclerosis
  2. Mixed cellularity
  3. Lymphocyte-rich
  4. Lymphocyte depletion
103
Q

In the hodgkin lymphoma subtype, lymphocyte predominance, the Reed-Sternberg cells have a (B/T?)-cell immunophenotype that differs from that of the classic types.

A

B

104
Q

T/F Due to their B-cell origin, the Reed-Sternberg cells of classic Hodgkin lymphoma express most B-cell– specific genes, including the Ig genes.

A

False, despite their B-cell origin the classic forms do not express B-cell specific genes

105
Q

common event in classic Hodgkin lymphoma and turns on genes that are believed to promote the growth and survival of Reed-Sternberg cells

A

Activation of the transcription factor NF-κB

106
Q

Several Reed-Sternberg cell variants are also recognized namely (3)

A

mononuclear variant
lacunar variant
lymphohistiocytic variant, L&H variant (popcorn cell)

107
Q

most common form of Hodgkin lymphoma. It is characterized by deposition of collagen in bands that divide involved lymph nodes into circumscribed nodules

A

Nodular Sclerosis Type

108
Q

Reed-Sternberg cell variant found characteristically in the nodular sclerosis subtype

A

lacunar variant

109
Q

characteristic immunophenotype of Reed-Sternberg cells in the 4 classic Hodgkin lymphoma subtypes
PAX5
CD15
CD30
CD20
LCA

A

PAX5 +
CD15 +
CD30 +
CD20 -
LCA -

110
Q

Reed-Sternberg cells in HL of the mixed cellularity subtype are infected with ____ in about 70% of cases

A

EBV

111
Q

least common subtype of Hodgkin lymphoma

A

lymphocyte depletion type

112
Q

The Reed-Sternberg cells of the HL lymphocyte depletion subtype are infected with _____ in over 90% of cases

A

EBV

113
Q

Reed-Sternberg cell variant commonly found in the nodular lymphocyte predominance subtype

A

L&H variant (popcorn cell)

114
Q

Reed-Sternberg cells of L&H variant found in nodular lymphocyte predominance subtype of hodgkin lymphoma express
CD20
BCL6
CD15
CD30

A

CD20 +
BCL6 +
CD15 -
CD30 -

115
Q

The spread of Hodgkin lymphoma is remarkably stereotypic (5)

A

nodal
splenic
hepatic
bone marrow
other tissues

116
Q

most important prognostic variable in HL

A

tumor stage

117
Q

Clinical Staging of Hodgkin and Non-Hodgkin Lymphomas (Ann Arbor Classification)
1
2
3
4

A
  1. Involvement of a single lymph node region (I) or a single extralymphatic organ or site (IE)
  2. Involvement of two or more lymph node regions on the same side of the diaphragm alone (II) or localized involvement of an extralymphatic organ or site (IIE)
  3. Involvement of lymph node regions on both sides of the diaphragm without (III) or with (IIIE) localized involvement of an extralymphatic organ or site
  4. Diffuse involvement of one or more extralymphatic organs or sites with or without lymphatic involvement
118
Q

Pathogenic mechanism:
Burkitt lymphoma
Follicular lymphoma
Hodgkin lymphoma
Mantle cell lymphoma
Acute lymphoblastic leukemia
Marginal zone lymphoma

A

Burkitt lymphoma
dysregulation of MYC

Follicular lymphoma
dysregulation of BCL2

Hodgkin lymphoma
PD-1 ligand gene amplification

Mantle cell lymphoma
loss of cell cycle control (cyclin D1 rearrangements)

Acute lymphoblastic leukemia
loss of the CDKN2A

Marginal zone lymphoma
chronic immune stimulation

119
Q

Three broad, major categories of myeloid neoplasms

A

Acute myeloid leukemias

Myelodysplastic syndromes

Myeloproliferative neoplasms

120
Q

a tumor of hematopoietic progenitors caused by acquired oncogenic mutations that impede differentiation, leading to the accumulation of immature myeloid blasts in the marrow

A

Acute myeloid leukemia (AML)

121
Q

distinctive subtype of AML associated with the t(15;17) that create the chimeric protein PML-RARa

A

acute promyelocytic leukemia

122
Q

the two most common chromosomal rearrangements in AML and the polypeptides they disrupt

A

t(8;21) - RUNX1
inv(16) - CBFB

123
Q

distinctive needle-like azurophilic granules, present in many cases; they are particularly numerous in AML with the t(15;17) (acute promyelocytic leukemia)

A

auer rods

124
Q

The diagnosis of AML is based on the presence of at least __% myeloid blasts in the bone marrow.

A

20%

125
Q

AML immunophenotype
CD34
CD64
CD33
CD15

A

CD34 + marker for multipotent stem cells
CD64 - marker for mature myeloid cells
CD33 + marker for immature myeloid cells
CD15 +/- marker for more mature myeloid cells

126
Q

AML arising de novo in younger adults is commonly associated with balanced chromosomal translocations, particularly (3)

A

t(8;21), inv(16), and t(15;17)

127
Q

AML following MDS or exposure to DNA-damaging agents (such as chemotherapy or radiation therapy) often has deletions or monosomies involving (2)

A

chromosome 5 and 7

128
Q

AML following MDS or exposure to DNA-damaging agents (such as chemotherapy or radiation therapy) usually lack chromosomal translocations EXCEPT for those treated with ___

A

topoisomerase II inhibitors, which are strongly associated with translocations involving the KTM2A gene

129
Q

AML subtype with the best prognosis, being curable in more than 90% of patients

A

AML with t(15;17); RARa fusion gene

130
Q

refers to a group of clonal stem cell disorders characterized by maturation defects that are associated with ineffective hematopoiesis and a high risk of transformation to AML

A

myelodysplastic syndrome

131
Q

All forms of MDS can transform to AML, but transformation occurs with highest frequency and most rapidly in

A

t-MDS or MDS secondary to previous genotoxic drug or radiation therapy

132
Q

clonal stem cell disorder wherein the most characteristic finding is disordered (dysplastic) differentiation affecting the erythroid, granulocytic, monocytic, and megakaryocytic lineages to varying degrees

A

myelodysplastic syndrome

133
Q

_____ are neutrophils with only two nuclear lobes are often seen in MDS

A

Pseudo–Pelger-Hüet cells

134
Q

is distinguished from other myeloproliferative neoplasms by the presence of a chimeric BCR-ABL gene derived from portions of the BCR gene on chromosome 22 and the ABL gene on chromosome 9

A

Chronic Myeloid Leukemia (CML)

135
Q

In more than 90% of cases of CML, BCR-ABL is created by a reciprocal (9;22)(q34;q11) translocation, the so-called ____

A

Philadelphia chromosome

136
Q

characterized by increased marrow production of red cells, granulocytes, and platelets (panmyelosis), but it is the increase in red cells that is responsible for most of the clinical symptoms

A

Polycythemia vera (PCV)

137
Q

In PCV, the transformed progenitor cells have markedly decreased requirements for erythropoietin and other hematopoietic growth factors due to activating mutations in the tyrosine kinase

A

JAK2

138
Q

pathway which lies downstream of multiple hematopoietic growth factor receptors, including the erythropoietin receptor

A

JAK/STAT pathway

139
Q

serum erythropoietin levels in PCV are low/high?

A

low

140
Q

serum erythropoietin levels in secondary polycythemia are low/high?

A

high

141
Q

substitution that occurs in JAK2 mutation

A

valine to phenylalanine

142
Q

most common mutation in Langerhans cell histiocytosis

A

BRAFV600E

143
Q

substitution in BRAFV600E

A

valine to glutamate

144
Q

______ in the cytoplasm is characteristic in Langerhans cell histiocytosis. They are pentalamiar tubules, often with a dilated terminal end producing a tennis racket–like appearance which contain the protein langerin

A

Birbeck granules

Diseases of White Blood Cells, Lymph Nodes, Spleen, and Thymus (1 decks)

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