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H&L High Yield > Chapter 13 > Flashcards

Chapter 13 Flashcards

1
Q 
Hodgkin lymphoma subtype:
Nonclassical subtype
Excellent prognosis
Express BCL6 (unique to this subtype)
Not associated with EBV
“Popcorn cells”
A

Lymphocyte-predominance subtype

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2
Q

Langerhans cell histiocytosis subtype:
2 year old boy has had a seborrheic eruption over the scalp and trunk over the past month. He then develops a right ear ache. On physical exam, the right tympanic membrane is erythematous and bulging. He has hepatosplenomegaly and generalized lymphadenopathy. Lab studies show Hgb 9.5 g/dL, Hct 28.7%, MCV 90 fL, platelet count 58,000/microliter, and WBC count 3540/microliter. Bone marrow biopsy shows 100% cellularity with extensive infiltration by cells resembling macrophages that express CD1a antigen and, by electron microscopy, have prominent HX bodies (Birbeck granules).

A

Letterer-Siwe disease and localized eosinophilic granuloma

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3
Q 
Hodgkin lymphoma subtype:
Uncommon
Reactive lymphocytes make up majority of cellular infiltrate
Lymph nodes diffusely effaced
Very good to excellent prognosis
A

Lymphocyte-rich subtype

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4
Q

AML class I (genetic aberrations) favorable vs poor prognosis

A

Favorable: t(8:21) and inv(16)
Poor: t(11:q23:v)

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5
Q

When a high level of M proteins causes RBCs in blood smears to stick together linearly
Characteristic of multiple myeloma

A

Rouleaux formation

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6
Q

HIV associated Burkitt lymphoma has __% of patients latently infected with EBV

A

25

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7
Q

Is CMML (chronic myelomonocytic leukemia) a myelodysplastic syndrome or a myeloproliferative disorder?

A

Myelodysplastic syndrome (MDS)

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8
Q

Thymocytes hypoplasia/aplasia:
What is the name of this syndrome?
Which pharyngeal pouches is there a problem with developing?

A

DiGeorge Syndrome; 3rd and 4th pharyngeal pouches

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9
Q

Immunophenotype (CD markers) of CLL

A

CD 19, 20, 23

ALSO CD 5 - B cell proliferation that is positive for CD 5 = CLL

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10
Q

Pentalaminar tubules with a dilated terminal end (“tennis racket” appearance)
What pathology are they seen in?

A

Birbeck granules

Seen in langerhans cell histiocytosis (Birbeck granules contain langerin)

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11
Q

Tumors (2) that present with CLA (+), CCR4 (+) and CCR10 (+)

A

Mycosis fungoides and Sezary syndrome

CLA = cutaneous leukocyte antigen

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12
Q

Immunophenotype of langerhans cell histiocytosis

A

HLA-DR (+)
S100 (+)
CD1a (+)

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13
Q

Multiple Myeloma:
Bence-jones proteinuria:
Excreted _____ chains are toxic to renal tubular epithelial cells -> kidney myeloma -> renal failure/insufficiency (second MCC of death in MM)

A

Light

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14
Q

Lymphoma with t(11:14) cyclin D1/IgH

Overexpression of cyclin D1 promotes G1->S phase progression

A

Mantle Cell Lymphoma

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15
Q

Leukemia/lymphoma with the following clinical features:
Massive splenomegaly
Exceptionally sensitive to genital chemotherapy -> long lasting remission
Excellent prognosis

A

Hairy cell leukemia

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16
Q

Leukemia/lymphoma with large anaplastic cells with horseshoe nuclei and voluminous cytoplasm as hallmark cells

A

Anaplastic large cell lymphoma (ALK+)

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17
Q 
Hodgkin lymphoma subtype:
Most common form
Adolescent/young adults
Excellent prognosis
Lacunar cells
Deposition of collagen bands that divide lymph nodes into circumscribed nodules
A

Nodular sclerosis subtype

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18
Q

Thymomas typically contain _____ (cell)

A

Thymocytes - immature T cells

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19
Q

Leukemia/lymphoma with the following features:
Destructive nasopharyngeal mass
More common in Asia
Tumor originates from a single EBV-infected cell
Small to large pythonic cells cuffing and invading small vessels with ischemic necrosis
CD21 (-) and CD3 (-)

A

Extranodal NK/T-cell lymphoma

Extra-nodal = extra-“nosal”

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20
Q 
Langerhans cell histiocytosis subtype:
Benign
Adolescents
Pathologic fracture
Skin is NOT involved
A

Eosinophilic granuloma

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21
Q

Most common non-random chromosomal abnormality seen in WBC neoplasms

A

Translocations

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22
Q

Most common plasma cell disorder/dyscrasia

most common plasma cell neoplasm/malignancy is multiple myeloma

A

Monoclonal Gammopathy of Uncertain Significance (MGUS)

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23
Q

Splenomegaly can cause hypersplenism characterized by ___, ____, and ____ either alone or in combination

A

Anemia, leukopenia, and thrombocytopenia

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24
Q

Bence-Jones proteins are light chains small in size that are excreted in the urine and are seen in _______

A

Plasma cell neoplasms (dyscrasias)

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25
Q

BCL6 is required for formation of normal germinal centers. What leukemia/lymphoma results from a dysregulation of BCL6?

A

DLBCL

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26
Q

AML class III (therapy related) favorable vs poor prognosis

A

Only very poor prognosis

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27
Q 
Langerhans histiocytosis subtype:
Malignant
Young children (>3 years old)
Triad of calvarial (skullcap) bone defects, diabetes insipidus, and exophthalmos
Scalp rash
A

Hand-Schuller-Christian disease

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28
Q

How do you differentiate between benign and malignant thymomas?
What are the 3 histologic subtypes of thymomas?

A

Cellular atypia;
Thymoma - cytologically benign and noninvasive
Invasive thymoma (type I malignant thymoma) - cytologically benign but locally invasive/metastatic (no cellular atypia)
Malignant thymoma (thymic carcinoma, type II malignant thymoma) - cytologically malignant (cellular atypia)

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29
Q

DLBCL subtype always associated with Kaposi’s

Tumor cells infected with KSHV/HHV8 appears to have a causal role

A

Primary Effusion Lymphoma

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30
Q

Leukemia/lymphoma presenting with BRAF (serine/threonine kinase) activating mutation (valine -> glutamate)

A

Hairy cell leukemia

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31
Q

Lymphoma characterized by an acquired MYD88 mutation

A

Lymphoplasmacytic lymphoma

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32
Q

Burkitt lymphoma subtype - ALL patients latently infected with EBV
Involves mandible/face

A

African (endemic)

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33
Q

Mechanism of pancytosis (high counts of all cells) in myeloproliferative disorders

A

Overactive (mutation) tyrosine kinase causing growth factor independence

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34
Q

Prognosis of Adult T-cell Luekemia/lymphoma (ATLL)

A

Rapidly progressive disease

Fatal in months-1 year even with aggressive chemotherapy

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35
Q

T/F - fever presents in Hodgkin lymphoma

A

TRUE!

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36
Q

Leukemia/lymphoma with the following:
HUGE spleen
Low LAP
Basophilic + eosinophilia

A

Chronic myeloid leukemia (CML)

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37
Q

Factors influence WBC neoplasia:

Where do oncogenic mutations occur most frequently in during attempted antibody diversification?

A

Germinal center B cells

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38
Q

Monoblasts/monocytes are positive for _________

A

Non-specific esterase

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39
Q

Which lymphoma spreads in an orderly fashion and staging is useful

A

Hodgkin’s

Has distinct pathologic features and is treated in a unique fashion

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40
Q

Myeloma with elevated serum M protein >3 g/dl but ASYMPTOMATIC

A

Smoldering myeloma

41
Q

Most common leukemia of adults in Western world

A

CLL

42
Q

Neoplasm that presents with lytic bone lesions, hypercalcemia, renal failure, and acquired immune problems

A

Multiple Myeloma

43
Q

Which leukemia/lymphoma has a waxing-waning course?

A

Follicular lymphoma

44
Q

DLBCL subtype that occurs with severe T-cell immunodeficiency (HIV/allogenic bone marrow transplant)

A

Immunodeficiency associated Large B-cell Lymphoma

45
Q

Kids with ______ have ALL until proven otherwise

A

A very low platelet count

46
Q

Are CNS symptoms such as nerve palsies seen in ALL or AML?

A

ALL

No nerve palsies seen in AML

47
Q

Are patients with polycythemia Vera prone to thrombosis or bleeding?

A

Both
Elevated hct -> increased blood viscosity + thrombocytosis and abnormal platelet function -> prone to both thrombosis and bleeding

48
Q 
Hodgkin lymphoma subtype:
Second most common type
Male predominance
Biphasic age distribution
Prognosis very good
A

Mixed cellularity subtype

49
Q

Burkett lymphoma immunophenotype

A

Immunophenoytpe = mature B cells
CD 10, 19 20 (+), BCL6 (+), surface IgM (+)
MYC (+)
*BCL2 (-)

50
Q

Myelodysplasias are characterized by ______

A

Thrombocytopenia

51
Q

t(11:18), t(14:18) or t(1:14) are specific for ___________

A

Extranodal marginal zone lymphomas (MALTomas)

52
Q

AML class II (MDS-like features, i.e. myelodysplastic syndrome) favorable vs poor prognosis

A

Only poor prognosis

53
Q

Thymic epithelial cells are _____ cells

A

Squamous

54
Q

Myeloproliferative disorder with valine -> phenylalanine at residue 617

A

Polycythemia Vera

55
Q

Most common form of non-Hodgkin’s lymphoma

A

Diffuse Large B-cell Lymphoma (DLBCL)

Poor prognosis

56
Q 
Langerhans cell histiocytosis subtype:
Malignant
Infant (<2 years old)
Skin rash (trunk and scalp) and cystic skeletal defects
Rapidly fatal
A

Letterer-Siwe disease

Remember 2 names = <2 years old

57
Q

Lymphoma with a substantial fraction of tumor cells that undergo terminal differentiation to plasma cells (distinctive factor from CLL)

A

Lymphoplasmacytic lymphoma

58
Q

What tumor commonly arises in Waldeyer’s Ring (oropharyngeal lymphoid tissue including tonsils AND adenoids)

A

DLBCL

59
Q 
Myelodysplastic syndromes (MDS) can be characterized by a pathogenesis that includes:
Monosomies 5 and 7
Deletions of 5q, 7q, and 20q
Trisomy 8
Which chromosome is the MYC gene on?
A

Chromosome 8

60
Q

Prognosis of anaplastic large cell lymphoma (ALK+)

A

Good prognosis

61
Q

Syndrome characterized by RA, splenomegaly, and neutropenia

Caused by large granular lymphocytic leukemia

A

Felty syndrome

62
Q

Favorable prognoses for ALL

A 
Hyper-diploidy
Age between 2 and 10
Low white cell count
Trisomy of chr 4, 7, or 10
Presence of t(12:21) - involves ETV6 and RUNX1 genes
63
Q

Most common plasma cell neoplasm

A

Multiple Myeloma

64
Q

The spent phase of myeloproliferative disorders is only seen in ______
Looks like primary myelofibrosis

A

Polycythemia vera

65
Q

B-cell tumors present with _____ tumors

A

Cyclic

66
Q

Follicular lymphoma chromosomal associations

A

Strongly associated with chromosomal translocations involving BCL2
t(14:18) BCL2/Ig heavy chain - seen in almost all follicular lymphomas
IGH locus on chromosome 14
BCL 2 locus on chromosome 18
BCL2 antagonizes apoptosis (promotes cell survival, devoid of apoptotic cells because BCL2 is anti-apoptotic)

67
Q

CLL effect on immune function

A

Hypogammaglobulinemia = bacterial infection

68
Q

Hodgkin lymphoma - EBV vs non-EBV associated types

A 
Classical types (nodular sclerosis, mixed cellularity, lymphocyte-rich, and lymphocyte-depleted) - associated with EBV infection
Lymphocyte-predominant is NOT associated with EBV infection
69
Q

Which myeloproliferative disorder are dacrocytes seen in (and what are they)?

A

Primary myelofibrosis

Dacrocytes (“daCRYocytes”) - tear drop shaped cells (damaged during release form fibrotic marrow)

70
Q

Lymphoplasmacytic lymphoma-related syndrome in which high levels of IgM leads to symptoms related to hyperviscosity of the blood

A

Waldenstrom macroglobulinemia

71
Q

Myeloproliferative disorder with the following:
Obliterating marrow fibrosis
Extensive deposition of collagen in marrow by non-neoplasticism fibroblasts (chief pathologic feature)
Inappropriately released PDGF and TGF-beta (cause fibrosis and scarring)

A

Primary myelofibrosis

72
Q

Lymphoid neoplasms are monoclonal meaning

A

All daughter cells are derived form the malignant progenitor share the same antigen receptor gene configuration and sequence

Monoclonal - all daughter cells express the same configuration and sequence, implies malignancy

73
Q

Most common cause of agranulocytosis/neutropenia

This causes patients to be more susceptible to ______

A

Drug toxicity

Bacterial infection

74
Q

Prognosis of Hodgkin lymphoma

A

Curable in most cases

75
Q

Which lymphoma spreads widely and less predictively

A

Non-Hodgkin’s

76
Q

Most common trigger for hemophagocytic lymphohistiocytosis (HLH)

A

Infection - especially EBV

77
Q

Reactive condition with cytopenias and signs/symptoms of systemic inflammation due to macrophage activation

A

Hemophagocytic lymphohistiocytosis

78
Q

Dilated ER that stains to look like a “sky blue puddle” = granulocyte lineage effect seen in _______

A

Myelodysplastic syndromes (MDS)

79
Q

Cortical thymomas rich in thymocytes are more likely to be associated with ____

A

Autoimmune disease

80
Q

Burkitt lymphoma subtype with GI involvement

15-20% latently infected with EBV

A

Sporadic (nonendemic)

AKA American Burkitt

81
Q

Lab findings in primary myelofibrosis

A

Normocyctic, normochromic anemia with leukoerythroblastosis

82
Q

Is LAP high or low in reactive states (e.g. infection)?

A

High

83
Q

Neoplasm that commonly causes timorous masses throughout the skeletal system causing a “moth eaten” appearance

A

Multiple Myeloma

84
Q

Splenic rupture is most commonly precipitated by ______

A

Blunt trauma (or by physician palpating)

85
Q

Lymphoma that can present with lymphomatoid polyposis and can be misdiagnosed as UC

A

Mantle Cell Lymphoma

86
Q

2 most common chromosomal rearrangements in acute myeloid leukemia (AML)

A

t(8:21) and inv(16)

87
Q

Leukemia/lymphoma with the following characteristics:
t(15:17)
May have early life-threatening bleeding
May have DIC (often fatal)

A

Acute promyelocytic leukemia (AML subtype)

88
Q

Prognosis of t-MDS (therapy-related myelodysplastic syndrome)

A

Terrible

Median survival 5 months

89
Q

Most important prognostic factor of Hodgkin lymphoma

A

Tumor stage

NOT histology

90
Q

Basophilic leukocytosis is seen in

A

CML

91
Q

In hairy cell leukemia, ____ have excellent tumor response in patients who fail chemotherapy

A

BRAF inhibitors

92
Q

Prognosis of polycythemia Vera

A

Death within months if untreated

Phlebotomy extends survival to 10+ years

93
Q

Multiple Myeloma:
Bence-jones proteinuria:
Excessive production and aggregation of M proteins, especially ___ or ___ -> hyperviscosity

A

IgA or IgG3

94
Q

STAT3 mutation is seen in ____

A

Large granular lymphocytic leukemia

95
Q

BRAF is characteristic of Hairy cell leukemia

BRAF is positioned immediately downstream of ___ in the ____ signaling cascade

A

RAS; MAPK

96
Q

Most common leukemia of childhood

Most common cancer of children

A

ALL

97
Q

Pathognomonic morphology of CLL

A

Proliferation centers - larger lymphocytes gathered in loose aggregates that contain mitotically active cells

98
Q

Leukemia/lymphoma with the following pathogenesis/immunophenotype:
ALK rearrangement on chromosome 2p23
Presence of ALK is pathognomic
CD30+, CD 8 is anaplastic

A

Anaplastic large cell lymphoma (ALK+)

99
Q 
Hodgkin lymphoma subtype:
Least favorable prognosis
Least common subtype
Elderly
HIV+ and EBV+
Non-industrialized countries
A

Lymphocyte-depleted subtype

H&L High Yield (3 decks)

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