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AU2017 Gen Path Exam 2 > Lecture 5- WBC disorders > Flashcards

Lecture 5- WBC disorders Flashcards

1
Q

… are malignant proliferations of cells native to lymphoid tissue– lymphocytes abd their precursors and derivatives. These tumors usually arise in lymphoid tissue and can spread to involve solid tissue, marrow, and blood.

A

lymphomas

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2
Q

2 types of lymphomas

A

Hodgkins

Non-Hodgkins

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3
Q

…. are malignant proliferations of cells native to the bone marrow which often spillover to blood. Can spread to involve solid organs (spleen and liver)

A

leukemias

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4
Q

leukemias can be … or … and also … or …

A

acute or chronic

myeloid or lymphoid

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5
Q

T/F Lymphomas usually start with a solid tumor and leukemias don’t but can result in a solid tumor later

A

true

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6
Q

… is defined morphologically by the presence of Reed-Sternberg cells admixed with a variable inflammatory infiltrate

A

Hodgkin lymphoma

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7
Q

Which lymphoma (Hodgkins or Non-hodgkins) is often accompanied by a fever, arises in a single lymph node or chains of nodes, is more common in young adults (30) and is characterized by spread within lymph node groups

A

Hodgkins

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8
Q

Which lymphoma (HL or NHL) is highly curable in most cases with chemotherapy and/or radiotherapy

A

HL

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9
Q

HL lymphoma has a …. age distribution

A

bimodal

20-30 yo and >50 yo

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10
Q

Is HL common?

A

no, only about 8300 cases in 2017

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11
Q

HL usually presents first with ….

or …. which can be detected through an MRI

A

painless lymphadenopathy (often cervical, supraclavicular, mediastinal)

splenomegaly

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12
Q

What causes HL?

A

unknown but EBV has been implicated in playing a role

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13
Q

… is necessary for diagnosis of HL and diagnosis also requires identification of … in the appropriate…

A

Lymph node biopsy

Reed-Sternberg cells

background

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14
Q

How many different types of HL are recognized each with their own clinical presentations and histopathologic features?

A

5

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15
Q

Can RS-like cells be seen in other disorders besides HL?

A

Yes, can be seen in infectious mononucleosis but they are ALWAYS present in HL

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16
Q

The histologic background of RS cells seen in HL have variable numbers of …, … and ….

A

lymphocytes
plasma cells
eosinophils

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17
Q

Reed-Sternberg cells are large cells with … nuclei and …. nucleoli

A

mirror-image

prominent

(owl eyes)

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18
Q

RS cells are the malignant cell of HL but often comprises … of cells within involved lymph node

A

less than or equal to 2%

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19
Q

The origin of RS cells was unknown but just recently was discovered that its origin is

A

a B lymphocyte arising from a germinal center

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20
Q

EBV is often present in RS cell in HL (….%)

A

70%

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21
Q

spread of disease is predicatble in HL, …

A

lymph nodes
spleen
liver
bone marrow

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22
Q

Stage I of HL is when the tumor is in …. or … contiguous anatomic regions on the …. of the diaphragm

A

one or two

same side

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23
Q

Stage II of HL is when the tumor is in … regions on the …. of the diaphragm

A

more than 2 anatomic regions or non-contiguous regions

same side

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24
Q

Stage III of HL is when tumor is on …. of the diaphragm but not extending beyond….

A

both sides

lymph nodes, spleen or Waldeyers ring

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25
Q

Stage IV of HL is when the tumor is in …..outside of the ….

A

bone marrow, lung, any site outside lymph nodes, spleen or waldeyers ring

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26
Q

Stages of HL can also be categorized by A or B. A is the absence of symptoms and B symptoms are …

A

fever, night sweats, and unexplained weight loss

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27
Q

what is the 5 year survival rate of stage I and IIa HL

A

almost 100%

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28
Q

what is the 5 year survival rate of stage IV HL

A

50%

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29
Q

In NHL, there are over 2 dozen types currently recognized. Most are of … origin (…%), the remainder are of … origin

A

B cell (85%)

T cell

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30
Q

Incidence of NHL rises steadily after age…. there were approximately … cases in 2017

A

40

72,000

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31
Q

In contrast to HL, NHLs tend to have …. involvement , more frequent … spread and …. involvement, and affect all ages. Like HL, histologic examination of involved tissue is required for diagnosis

A

multiple node involvement

extranodal

peripheral blood involvement

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32
Q

Clinical presentation for NHL:

painless ….

systemic symptoms in …% of patients

frequent …. abnormalities

splenomegaly

may involve…

A

painless lymph node enlargement

systemic symptoms in 30% on patients

frequent immune abnormalities

may involve GI tract, bones, CNS

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33
Q

NHL classification first is by cell type and then is classified by … and …

A

growth pattern (nodular vs diffuse)

cell size (small vs large)

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34
Q

Which NHL characteristics have a better prognosis?

small or large

nodular or diffuse

A

small and nodular are better

35
Q

Staging with NHL is either localized disease which is … stage or numerous sites of involvement or bone marrow involvement which is … stage

A

low

high

36
Q

With NHL, is prognosis based more on the subtype or stage?

A

subtype (which is an exception to the general rule, “grading” or subtyping is critical with NHL)

37
Q

Treatment for NHL?(3)

A

chemotherapy

possibly radiation

bone marrow transplantation

38
Q

Classification of leukemias:

they can be … or …

and

… or …. (which cells are affected)

A

acute or chronic

39
Q

Acute leukemias have a … onset with … in the blood and either … or … cells can be affected

A

rapid
blast
myeloid
lymphoid

40
Q

Chronic leukemias have a … onset and tends to involve more … cells and either … or … cells can be affected

A

indolent
mature
myeloid
lymphoid

41
Q

Which leukemia is fatal and has a survival of a few months usually?

A

acute (chronic has survival of years or decades)

42
Q

Acute and chronic leukemias usually have an … WBC count

A

increased (acute sometimes is decreased)

43
Q

the 4 classifications of leukemias are

A

Acute Lymphoblastic Leukemia (ALL)

Chronic Lymphocytic Leukemia (CLL)

Acute Myelogenous Leukemia (AML)

Chronic Myelogenous Leukemia (CML)

44
Q

The proliferating cell in acute lymphoblastic leukemia (ALL) is a ….

this type of leukemia accounts for about … of the acute leukemias

A

primitive lymphoid cell

40%

45
Q

Which kind of leukemia is most frequent type in children less than 15yo and is the principal cause of cancer deaths in childhood

A

ALL (acute lymphoblastic leukemia)

46
Q

what is the peak age of incidence of ALL

A

4 years old

47
Q

How many subtypes of ALL are there and what are they

A

early B precursor
pre-B
mature B
T cell

48
Q

In ALL, prognosis is related to ….

A

cytogenetics

49
Q

With ALL, which have a better prognosis? children or adults?

A

children

50
Q

what is the treatment of ALL?

A

chemotherapy mainstay with bone marrow transplantation considered at relapse

51
Q

AML is seen to have clonal…

A

proliferation of a primitive myeloid cell

52
Q

AML is seen to have an … WBC count and is often accompanied by … and …

A

increased

anemia
thrombocytopenia

53
Q

Is AML more common in children or adults?

A

adults

54
Q

Which leukemia sometimes has lesional cells that proliferate into soft tissue (including gingivae) producing what is termed granulocytic sarcoma?

A

AML

55
Q

Which leukemia is myeloperoxidase present in the cytoplasm?

A

AML

56
Q

What is the treatment of AML?

A

chemotherapy

57
Q

While … % of patients may have a remission of AML, many relapse

A

70%

58
Q

Prognosis of AML is influenced by …

A

cytogenetics

59
Q

CLL (chronic Lymphocytic leukemia) is a clonal proliferation of…. identical to small lymphocytic lymphoma

A

mature (immunologically incompetent) B lymphocytes

60
Q

…. is the most common adult leukemia in the US

A

Chronic Lymphocytic Leukemia (CLL)

61
Q

CLL usually occurs in adults over…. and … are favored 2:1

A

60 yo

males

62
Q

CLL usually expresses … with kappa light chain restriction

A

surface IgM

63
Q

There is a high expression of …. in CLL

A

BCL2

64
Q

In CLL, patients often present with … and … and anemia and thrombocytopenia eventually develop as disease progresses.

A

splenomegaly

lymphadenopathy

65
Q

In CLL, tumor cells suppress normal B cells, leading to…

A

hypogammaglobulinemia

66
Q

Treatment and survival of CLL?

A

chemo

median survival is 4-6 years

67
Q

In CLL, all cases eventually terminate into… grade process, either … or …

A

high
acute leukemia
high grade lymphoma

68
Q

In CML (chronic myelogenous leukemia) the clonal proliferation is of ….

A

immature granulocytes - a stem cell disorder

69
Q

… has a marked increase in WBC count with eosinophilia and or basophilia- left shifted granulocytes

A

CML

70
Q

In CML, there may be … and … and splenomegaly is typically present

A

thrombocytosis

anemia

71
Q

Which disorder has a specific chromosomal abnormality, the Philadelphia chromosome t(9;22)

A

CML

72
Q

The philadelphia chromosome results in fusion of the … genes which mimic the effects of growth factor activation, driving the proliferation of CML

A

BCR-ABL genes

73
Q

T/F The BCR-ABL fusion is always present in CML and is unique to CML

A

False. It is always present in CML but it is NOT unique to this disorder

74
Q

Treatment for CML is… which induces complete remission in a high percentage of patients

A

targeted inhibitors of the BCR-ABL tyrosine kinase

75
Q

If the CML does not respond to targeted inhibitors of the bcr-abl tyrosine kinase what is the next step

A

bone marrow transplant (risky in older patients)

76
Q

…. is the clonal proliferation of monoclonal plasma cells.

A

multiple myeloma

77
Q

Which is the most common immunoglobin in multiple myeloma

A

IgG

78
Q

how many cases predicted of multiple myeloma in 2016 in the US

A

30,000

79
Q

what age do you see multiple myeloma in usually

A

late middle age to elderly

80
Q

which disease often presents with bone pain, hypercalcemia and renal disease

A

multiple myeloma

81
Q

Which disease causes increased serum monoclonal protein while normal immunoglobulins are suppressed and Bence-Jones proteinuria (light chains)

A

multiple myeloma

82
Q

What disase shows RBC rouleaux formation (stacking)

A

multiple myeloma

83
Q

median survival of multiple myeloma

A

4-6 years

84
Q

multiple myeloma treatment has had recent success with ….

A

chemotherapy with thalidomide analogs, anti-resorptives

AU2017 Gen Path Exam 2 (9 decks)

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