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Blood & Lymph > Unit 3 Lectures > Flashcards

Unit 3 Lectures Flashcards

1
Q

What does it mean for a lymphoma to be nodal or extra nodal?

A

nodal: presenting as enlarged lymph nodes
extranodal: presenting in skin, brain, GI tract, etc.

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2
Q

A myeloid sarcoma presents as a __________ and is very (common/rare)

A

solid mass, rare

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3
Q

T/F: Many hematopoietic neoplasms contain both a leukemic and lymphomatous component

A

true

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4
Q

Acute leukemia often has ____ RBCs, ____ platelets, and ____ neutrophils

A

low, low, low

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5
Q

Chronic leukmeia has ____ WBCs

A

increased

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6
Q

The chromosomal abnormality that is most commonly seen in hematologic malignancies is _______________

A

balanced translocation

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7
Q

_____________ is implicated in the development of Hodgkin lymphoma, Burkitt lymphoma, and other B-cell non-Hodgkin lymphomas

A

Epstein-Barr virus

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8
Q

_____________ is implicated in the development of adult T-cell leukemia/lymphoma

A

Human T-cell Leukemia Virus-1 (HTLV-1)

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9
Q

_____________ is implicated in primary effusion lymphoma

A

Kaposi Sarcoma Herpesvirus/Human Herpesvirus 8

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10
Q

What are some criteria of the WHO for classifying hematologic malignancies?

A
  • microscopic appearance of malignant cells
  • histologic growth patterns
  • relative amount of malignant cells
  • presence/absence of cell surface markers
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11
Q

What cells are neoplastic cells in Hodgkin lymphoma derived from?

A

B cells

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12
Q

Non-Hodgkin lymphoma refers to all the lymphomas of mature-appearing ____________

A

lymphocytes (excluding classic Hodgkin lymphomas)

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13
Q

T/F: Surgery is often the first therapy used for hematologic malignancies

A

False (almost never used!)

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14
Q

DNA alkylating agents and topoisomerase II inhibitors are risk factors for developing _________________

A

acute leukemia

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15
Q

75% of cases of ALL occur in ________________

A

children less than 6 years old

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16
Q

What is the generic marker of lymphocyte immaturity?

A

CD34

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17
Q

What is the common lymphoblast marker (not on mature cells)?

A

TdT

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18
Q

What are the markers of B cell lineage?

A

CD19, CD22

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19
Q

What are the markers of T cell lineage?

A

CD3, CD7

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20
Q

Is B or T lymphoblastic leukemia more common?

A

B (80-85%)

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21
Q

B-ALL with t(9:22) is more often seen in (children/adults) and has a (favorable/unfavorable) prognosis

A

adults, unfavorable

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22
Q

B-ALL with t(11q23) has a (favorable/unfavorable) prognosis and B-ALL with t(12;21) has a (favorable/unfavorable) prognosis

A

unfavorable, favorable

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23
Q

T-ALL presents with _____________ more frequently in ____________

A

mediastinal mass, teenagers/young adults

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24
Q

What is the average age at diagnosis with AML?

A

65

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25
Q

What are the generic markers of immaturity seen with AML?

A

CD34

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26
Q

What are the common myeloid markers?

A

CD117 (C-Kit), myeloperoxidase

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27
Q

What is a classic histological finding in AML?

A

auer rods

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28
Q

What translocation is associated with “AML with maturation” (still some normally developing neutrophils)?

A

t(8;21), RUNX1/RUNX1T1

RUNX1 needed for differentiation

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29
Q

Does AML with t(8;21) have a good prognosis?

A

yes

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30
Q

Does AML with inv(16) or t(16; 16) have a good prognosis?

A

yest

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31
Q

AML with inv(16) or t(16; 16) is associated with the presence of abnormal ____________ precursors.

A

eosinophilic (“baso-eos”)

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32
Q

In AML with t(15;17), cells are blocked in the ____________ stage

A

promyelocytic (hence, Acute Promyelocytic Leukemia rather than just AML)

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33
Q

In APL, cells have a ____________ morphology

A

hypergranular

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34
Q

What type of acute leukemia can be treated with retinoic acid?

A

APL

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35
Q

AML with t(1;22) usually shows ____________ differentiation and is often seen in infants with _____________

A

megakaryoblastic, Down syndrome

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36
Q

What is the latency period from therapy to AML after using an alkylating agent or radiation?

A

2-8 years

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37
Q

What is the latency period from therapy to AML after using a topoisomerase inhibitor?

A

1-2 years

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38
Q

What are the typical cytogenetic abnormalities in AML after using an alkylating agent or radiation?

A

whole or partial deletion of chr5, 7

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39
Q

What are the typical cytogenetic abnormalities in AML after using a topoisomerase inhibitor?

A

rearrangement of 11q23; MLL

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40
Q

Does cancer progress to AML through an MDS stage after using an alkylating agent or radiation?

A

usually yes

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41
Q

Does cancer progress to AML through an MDS stage after using a topoisomerase inhibitor?

A

usually no

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42
Q

What is the prognosis if the following molecular abnormalities are found?

  • FLT3 internal tandem duplication
  • nucleophosmin-1 (NPM1) mutation
  • CEBPA mutation
A

poor
good
good

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43
Q

SCID is a syndrome in which you lack _____________

A

T and B cells

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44
Q

The block for SCID occur between _____________ and _____________

A

hematopoietic stem cells, early lymphoid precursors

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45
Q

inheritance pattern for SCID

A

x-linked recessive

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46
Q

A block in the transformation from pre-B cell to immature B cell results in _____________

A

Bruton’s x-linked hypoimmunoglobulinemia

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47
Q

In Bruton’s, you can recover from ______ infections because you have functioning ____ cells even though you lack ____ cells

A

viral, T, lack B cells

48
Q

Hyper IgM occurs due to a block in…

A

Tfh cells (CD40L) being able to activate B cells (CD40)

49
Q

Transient hypoglobulinemia occurs due to too little ____

A

IgG

50
Q

IVIg can be used to treat ____________ immunodeficiency

A

secondary

51
Q

For people with the adenosine deaminase version of SCID, what treatment is available?

A

blood transfusion (but you have to irradiate the blood to be sure you’re killing any lingering T cells)

52
Q

Two main characteristics of myelodysplastic syndrome

A
  • ineffective hematopoiesis

- increased risk of transforming to AML

53
Q

In myelodysplastic syndrome, __________ is replaced by a __________

A

marrow, malignant clone

54
Q

MDS is diagnosed in the setting of persistent peripheral ____________

A

cytopenia

55
Q

Dyshematopoiesis occurs when more than ___% of cells in one lineage appear dysplastic

A

10%

56
Q

Small, hypolobated or non-lobated nuclei are morphologic evidence of _______________

A

dysmegakaryopoiesis

57
Q

MDS can be diagnosed by presence of…

A
  • whole or partial deletion of chr5, 7
  • isolated deletion 5q
  • trisomy 8
  • others
58
Q

What are some other possible differential diagnoses for patients with suspected MDS?

A
  • vitamin deficiency (B12, folate)
  • toxin exposure
  • exposure to certain drugs
  • viral infections
59
Q

In low grade MDS, blasts are

A
60
Q

Refractory cytopenia with unilineage dysplasia and refractory cytosine with multilineage dysplasia are examples of ______ grade MDS

A

low

61
Q

Refractory anemia with excess blasts-1 (5-9%) and refractory anemia with excess blasts-2 (10-19%) are examples of ______ grade MDS

A

high

62
Q

Myeloproliferative neoplasms are characterized by an increase in _________, with a corresponding increase in __________

A

1+ blood cell types, marrow cellularity

63
Q

______ is associated with persistent neutrophilic leukocytosis

A

CML

64
Q

CML has ________ WBCs, platelets, and basophils

A

increased

65
Q

In CML, the marrow is _________ and the nuclei are ___________

A

hypercellular, non-lobated

66
Q

If left untreated, CML progresses to a ________ phase

A

blast

67
Q

In (CML/Ph+-ALL), the BCR breakpoint is usually in the minor breakpoint cluster region

A

Ph+-ALL (190kd vs. 210kd in CML)

68
Q

Gleevac is a _______________ used to treat ____

A

protein tyrosine kinase inhibitor, CML

69
Q

Polycythemia vera is a _____ characterized by an increase in _________

A

myeloproliferative neoplasm, RBC mass (erythrocytosis)

70
Q

Polycythemia vera contains a _______ mutation

A

JAK2 (V617F)

71
Q

If a patient seems to have polycythemia vera but lacks a JAK2 mutation, consider…

A

secondary erythrocytosis (seen in smokers, chronic hypoxia, Hb disorders)

72
Q

PV transitions from a ________ phase (________ blood cell count) to a _______ phase (_________ blood cell count)

A

polycythemic, increased

spent, decreased

73
Q

Thrombosis of the mesenteric vein, portal vein, or splenic vein should always raise concerns for ________

A

polycythemia vera

74
Q

The main treatment for polycythemia vera is _________

A

phlebotomy

75
Q

Primary myelofibrosis is characterized by __________ and __________ but no _____________

A

granulocytic, megakaryocytic hyperplasia; no erythrocytosis

76
Q

What mutations are found in primary myelofibrosis?

A

JAK2 (50%)

MPL, CALR

77
Q

Primary myelofibrosis progresses from a pre-fibrotic to a __________ stage, which is characterized by _________________ and enlargement of organs (due to _______________________)

A

fibrotic
leukoerythroblastosis
extramedullary hematopoiesis

78
Q

Entrapped bizarre megakaryocytes on H&E suggest an ______________

A

MPN (myeloproliferative neoplasm)

79
Q

What mutations are found in essential thrombocythemia?

A

JAK2, MPL, CALR

80
Q

T/F: splenomegaly is common in essential thrombocythemia

A

False

81
Q

In myasthenia gravis, antibody is made against _____ receptors

A

Ach

82
Q

What is the treatment for myasthenia gravis?

A

IVIg

83
Q

What is goodpasture syndrome?

A

autoantibody reacts with basement membrane of capillaries in kidney and lung

84
Q

What is the primary antigen in goodpasture syndrome?

A

collagen epitope

85
Q

In Graves, antibody is stimulatory to the _____ receptor on the surface

A

TSH

86
Q

Hashimoto is an autoimmune _________ disease

A

thyroid

87
Q

The mantle zone of a lymph node is comprised entirely of ____ cells

A

B cells

88
Q

The dark zone of a lymph node germinal center is comprised of ________ cells and the light zone is ___________ cells

A

dark: B cells
light: B cells, T cells, macrophages, etc.

89
Q

CD19 and CD22 are ___ cell markers

A

B cell

90
Q

CD3, CD4, CD5, and CD8 are ___ cell markers

A

T cell

91
Q

What are two markers specifically expressed in normal germinal B cells?

A

CD10, BCL6

92
Q

Germinal center B-cell lymphomas include…(3)

A

follicular, Hodgkin, Burkitt

93
Q

In CLL cells, the nucleus has (round, condensed/loose) chromatin and the cells are (small/large)

A

round, condensed; small

94
Q

Is there effacement of lymph node architecture in CLL/SLL?

A

yes

95
Q

CLL and SLL are strongly positive for… (3)

A

CD5, CD19, CD23

96
Q

Describe the histology of follicular lymphoma

A

homogenous tumor cells with no dark zone, light zone, or tangible body macrophages

97
Q

BCL2’s function is to ___________ and is (unregulated/downregulated in normal germinal centers)

A

suppress apoptosis, downregulated

98
Q

Follicular lymphoma is strongly positive for…

A

B-cell markers: CD19, CD20
Germinal center B-cell marker: CD10, BCL6
BCL2

99
Q

Mantle cell lymphoma differs from other B-cell lymphomas in that it (is/is not) particularly aggressive

A

is aggressive

100
Q

Mantle cell lymphoma is positive for…

A

B-cell markers: CD19, CD20
CD5 (also positive in CLL/SLL)
cyclin D1 (BCL1) ***

101
Q

Cyclin D1 (BCL1) is important because its function is to…

A

activate genes required for cell cycle progression (G1/S transition)

102
Q

In mantle cell lymphoma, there is a _____ translocation that renders cyclin D1 continually on

A

t(11;14)

103
Q

Burkitt lymphoma: Describe the typical patient, location of lymphoma, and % EBV involvement:

  • endemic BL
  • sporadic BL
  • immunodeficiency-associated BL
A
  • endemic BL: children, jaw or abdomen, 95%
  • sporadic BL: children/young adults, iliocecal area, 30%
  • immunodeficiency-associated BL: HIV patients, 25-40%
104
Q

Burkitt’s lymphoma presents with deeply _________ cytoplasm with lipid vacuoles and H&E stain has a ___________ pattern

A

basophilic, “starry sky” pattern

105
Q

Burkitt’s lymphoma is positive for…

A

B-cell markers: CD19, CD20
germinal center B-cell markers: CD10, BCL6
MYC ***

106
Q

T/F: Diffuse large B-cell lymphoma is the most common of the non-Hodgkin lymphomas

A

true

107
Q

List five factors that affect prognosis of ALL

A
  • age
  • WBC count
  • response to therapy
  • number of chromosomes
  • B cell vs. T cell
108
Q

What is the most common immunodeficiency disease?

A

selective IgA deficiency

109
Q

In low grade myelodysplastic syndrome, myeloblasts (are/are not) increased in frequency

A

are not

110
Q

Name 3 sites where DIC could occur with polycythemia vera

A
  • mesenteric vein
  • portal vein
  • splenic vein
111
Q

A type II immune reaction involves what immunoglobins?

A

IgG, IgA, IgM

112
Q

What is the immunofluorescent pattern in Goodpasture syndrome?

A

linear

113
Q

What is the only immunopathology that does not require B cells or antibodies?

A

type IV

114
Q

_____ and _____ down regulate Tregs and up regulate Th1, Th2, and Th17

A

TGFbeta, IL-6

115
Q

_____ favors Treg development

A

IL-10

Blood & Lymph (3 decks)

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