White Blood Cell Disorders Flashcards

1
Q

What are lymphomas?

A

malignant proliferations of cells native to lymphoid tissue (lymphocytes, lymph precursors and derivatives)

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2
Q

Lymphomas usually arise in lymphoid tissue and can spread to involve ____ tissue, ______, and _____.

A

solid tissue
marrow
blood

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3
Q

What are the two main types of lymphomas?

A
  1. Hodgkin

2. Non-Hodgkin

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4
Q

What are leukemias?

A

malignant proliferations of cells native to the bone marrow, which often spill over into the blood

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5
Q

True or False: Only lymphomas can involve lymphoid tissue.

A

False,

both leukemia and lymphoma can involve lymphoid tissue at any site

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6
Q

Where do leukemias typically spread?

A

to solid tissues/organs (particularly the spleen and liver)

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7
Q

Hodgkin Lymphoma is defined morphologically by the presence of _______ cells.

A

Reed-Sternberg

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8
Q

True or False: Both forms of lymphoma present with fever, arise from a single lymph node, and are more common in young adults.

A

False, that is Hodgkin ONLY

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9
Q

True or False: The cause of HL is unknown.

A

True, but EBV has been implicated in playing a role

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10
Q

How is the Reed-Sternberg cell distinguished?

A

it is a LARGE neoplastic cell with mirror image nuclei and prominent nucleoli (Owl’s Eye)

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11
Q

True or False: Large numbers of RS cells can be found in the HL involved node.

A

False, small numbers of RS cells are present in the involved node of HL (less than 2%)

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12
Q

True or False: RS cells alone are adequate for the diagnosis of HL.

A

False, RS cells can be seen in non-neoplastic disorders like noninfectious mononucleosis

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13
Q

How many types of HL are recognized? Do they present the same clinically?

A

5 types

different clinical presentations and histopathologic features

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14
Q

______ is the term that refers to the assessment of the amount of tumor burden and its distribution in the body.

A

Staging

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15
Q

The ______ virus is present in 70% of RS cells.

A

Epstein-Barr

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16
Q

Choice of therapy is based on ______.

A

stage

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17
Q

Describe a “low stage” disease.

A

localized lymph node involvement without systemic signs (fever, weight loss)
*better prognosis

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18
Q

Describe “high stage” disease.

A

widespread disease, often with bone marrow involvement

*worse prognosis

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19
Q

Treatment for HL consists of a combination of ______ and, now to a lesser extent, ______.

A

chemotherapy

radiotherapy

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20
Q

Describe the “predictable spread” of HL.

A
  1. lymph nodes
  2. spleen and liver involvement (assessed with MRI)
  3. Bone Marrow (decreased immunity, anemia, thrombocytopenia, etc)
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21
Q

True or False: There is a risk for developing acute leukemia after treating HL with chemotherapy and radiotherapy.

A

True, because bone marrow toxicities of the drugs

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22
Q

What are the Stages of HL?

A

I - tumor in one anatomic region or two on same side
II - tumor in more than two regions or two regions on opposite sides of the diaphragm
III - tumor on both sides of the diaphragm, not extending past nodes, spleen or Waldeyer’s ring
IV - tumor in bone marrow, lung, etc; any organ outside nodes, spleen or Waldeyer’s ring

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23
Q

How are stages further divided?

A

A or B
A = absence
B = presence of systemic symptoms (fever, night sweats, and unexplained significant weight loss) = worse prognosis

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24
Q

True or False: Both chemotherapy and radiotherapy are used to treat high and low level stages of HL.

A

False,
chemo or radio = low stage (localized)
chemo = high stage (widespread)

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25
Q

Which stages are more likely to have “B” symptoms?

A

Stage III and IV

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26
Q

What is the 5 year survival rate for Stage I through Stage IIA of HL?

A

almost 100%

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27
Q

What is the 5 year survival rate for Stage IV HL?

A

50%

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28
Q

Non-Hodgkin Lymphomas (NHL) arise in _______ tissue and have the capacity to spread into __1___, ___2___, ___3__, and __4__.

A

lymphoid tissues (either nodes, or lymph tissue of solid organs)

  1. other nodes
  2. solid organs
  3. bone marrow
  4. blood
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29
Q

True or False: There is less morphological diversity in NHL than in HL.

A

False, there is more morpho diversity in NHL

*more than 2 dozen subtypes are recognized

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30
Q

How common are HL and NHL?

A

HL ~ 9k cases in 2015

NHL ~71k cases in 2015

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31
Q

About _____ % of NHL are clonal neoplasms of _____.

A

85

B-cell origin

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32
Q

If 85% of NHL are of B-cell origin, where do the other 15% originate?

A

T cells

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33
Q

How do NHL differ from Hodgkins?

A

NHL have:

  • multiple node involvement
  • more frequent extranodal spread and peripheral blood involvement
  • affect at all ages
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34
Q

All lymphoid neoplasms are considered to arise from a single transformed cell. Describe how this pertains to NHL.

A

B-Lymphocytes
-normally have the capacity to differentiate into plasma cells as part of the immune response

T-Lymphocytes
-become activated as part of the normal immune response and secrete cytokines

**Lymphoma = monoclonal expansion of lymphocytes that have been arrested at a particular stage = cells proliferate without normal regulatory mechanisms

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35
Q

How are NHL’s classified (4 components)?

A
  1. morphology
  2. cell of origin
  3. clinical features
  4. genotype
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36
Q

NHL shows systemic symptoms in ____% of patients.

A

30

37
Q

True or False: NHL is associated with painful lymph node enlargement.

A

False, the nodes enlarge but it is PAINLESS

38
Q

There is less correlation between stage and prognosis with NHL (unlike HL). What are better determinants of prognosis?

A
  1. cell type (aka: NHL Subtype = grading)

2. tumor proliferative index

39
Q

NHL can involve the ______, _____, and _____.

A

GI Tract
Bones
CNS

40
Q

Describe the Stages of NHL.

A

I - single node or extralymphatic organ/site
II - two or more nodes on same side of diaphragm or with involvement of extralymphatic organ
III - lymph nodes on both sides of diaphragm (could include spleen)
IV - multiple or disseminated foci of one or more organs/tissues, or tissues with/without lymphatic involvement

**However: remember NHL prognosis is more based on grading than staging (HL is opposite)

41
Q

True or False: Bone marrow involvement is more common in HL than in NHL.

A

False, more common in NHL

42
Q

Which lymphoma type can be treated with bone marrow transplants?

A

highly resistant NHL, transplant with high dose chemo

43
Q

NHL can be classified by its growth pattern: _____ or _____. Which has a worse prognosis?

A

Nodular or Diffuse

nodular is better, diffuse is bad

44
Q

NHL can be classified based on _____: small or large. Which gives a better prognosis?

A

cell size

small is better than large

45
Q

How are leukemias classified?

A
  1. Acute

2. Chronic

46
Q

True or False: Leukemias arise in the bone marrow and often spill over into the blood or other organs.

A

True

47
Q

What is the difference between acute and chronic leukemia?

A

Acute: RAPID onset with BLASTS in the blood
Chronic: INDOLENT onset with more MATURE cells

48
Q

True or False: In both acute and chronic leukemia, myeloid and lymphoid cells are affected.

A

True

49
Q

What are the four types of leukemia?

A
Lymphoblastic:
ALL 
CLL
Myelogenous:
AML
CML
50
Q

What is the most frequent type of leukemia in children less than 15 years old? What are other characteristics of this leukemia?

A

Acute Lymphoblastic Leukemia (ALL)

  • accounts for about 40% of acute leukemias
  • clonal growth of PRIMITIVE LYMPHOID cell
  • both B and T cell types
  • increased WBC and decreased platelets
  • OFTEN involves the CNS
  • enlargement of nodes, liver, and spleen
51
Q

Which type of ALL has the best prognosis?

A

pre-B type

age 2-10 group in particular

52
Q

What are the subtypes of ALL?

A
early B precursor
pre-B
mature B
T cell
hyperdiploidy (more than 50 chromosomes per cell)
53
Q

What is the proliferating cell type in AML?

A

primitive myeloid cell

54
Q

True or False: AML is more common in adults.

A

True, median age is 50

differentiation includes more mature myeloid cells with granules (myeloperoxidase)

55
Q

When present, the cytoplasmic inclusions called ______ are diagnostic for AML.

A

Auer Rods

56
Q

AML cells will sometime proliferate the soft tissue, including the ______, and produce ________ sarcoma.

A

granulocytic

57
Q

Is remission common in AML after chemotherapy?

A

yes, 70%

58
Q

The proliferating cell in CLL is an ________ _____ lymphocyte that appears mature.

A

immunologically incompetent

59
Q

More than 95% of Chronic Lymyphocytic Leukemias are for which cell type? Most commonly expressing which surface immunoglobulin?

A

B Lymphocyte

surface IgM with Kappa light chain restriction

60
Q

Which leukemia is most common in adults over 60 years old?

A

CLL

accounts for 2/3 of chronic leukemias

61
Q

What is the male to female ratio for CLL?

A

2:1 (male to female)

62
Q

In CLL there is an increased white cell count and increased expression of ______.

A

BCL2

63
Q

The median survival rate for CLL is 4-6 years as the tumor cells suppress normal ______ actions and cause ________ in most patients.

A

B cell

hypogammaglobulinemia

64
Q

True or False: All cases of CLL eventually terminate into a higher grade process, such as acute leukemia or high grade lymphoma.

A

True

65
Q

What is the proliferating cell in Chronic Myelogenous Leukemia?

A

immature hematopoietic cell

66
Q

CML is associated with an increase in white blood cell count with _______ and/or _______.

A

eosinophilia

basophilia

67
Q

Which chromosome abnormality occurs in proliferating cells of CML?

A

Philadelphia Chromosome (9 and 22) which causes fusion of the BCR-ABL tyrosine kinase genes that mimic the effects of growth factor activation (driving proliferation)

68
Q

What age range is affected by CML and CLL?

A
CML = 25-60
CLL = over 60 (later leukemia)
69
Q

What anatomic alteration is almost always present with CML?

A

enlargement of the spleen (splenomegaly) due to proliferation of abnormal cells

70
Q

What is “blast crisis” and with which form of leukemia is it related?

A

-a relative increase in immature cells in peripheral blood/bone marrow and decreased response to treatment, in the terminal phase of CML

71
Q

From what two events do the clinical features of leukemia arise?

A
  1. impairment of marrow function as abnormal cells suppress growth of normal cells
  2. infiltration of body organs due to proliferation of the abnormal cells
72
Q

How does anemia manifest?

A

pallor, weakness, and fatigue

73
Q

Thrombocytopenia causes _____ and _____.

A

bleeding

bruising

74
Q

Why do infections occur in conjunction with leukemia?

A

decreased production of mature granulocytes and production of non-functional granulocytes and/or lymphocytes

75
Q

Fever with leukemias can be due to infection of increased _______.

A

metabolism of proliferating cells

76
Q

True or False: Chest pain or obstruction of vascular and lymphatic channels can result from leukemia.

A

False, abdominal pain not chest pain

77
Q

Infiltration of the gingiva is a feature commonly associated with ______ leukemias.

A

acute myelo-monocytic

78
Q

What are “plasma cell disorders?”

A
  • clonal expansion of immunoglobulin-secreting cells that result in increased serum monoclonal protein
  • increases in serum monoclonal protein (M component) which may have adverse effects on renal and neurologic function
79
Q

What is Multiple Myeloma?

A
  • a plasma cell disorder

- proliferating cell is a PLASMA CELL that produces one immunoglobulin type (heavy or light chain)

80
Q

What are the three most common immunoglobulins that proliferate in multiple myeloma?

A

IgG (60%)
IgA (20-25%)
kappa or lamda light chain (the remaining 15%)

81
Q

Multiple Myeloma is a disease that affects those of ________ age.

A

late middle age (to elderly)…average around 70 years

82
Q

How many cases of Multiple Myeloma are predicted for 2016?

A

30,000

83
Q

When only light chains are produced, patients can excrete the _____ molecular weight chains in the urine. This is referred to as _______.

A

low

Bence Jones proteinuria

84
Q

_______ bone lesions characterize myeloma.

A

multifocal destructive (punched out, honey-comb areas of bone)

85
Q

Proteinaceous casts may form in the ______ due to multiple myeloma.

A

kidneys

86
Q

What are the blood and marrow features associated with MM?

A
  • RBC stacking (rouleaux formation)

- marrow plasma cell infiltrates in single cells and sheets

87
Q

True or False: The survival time of the typical form of Multiple Myeloma is 10 years.

A

False, 4-6 years typically

88
Q

How is multiple myeloma treated?

A
  • chemotherapy (thalidomide analogs; anti-resorptives)
  • radiotherapy (palliation for bone pain)
  • bone marrow transplant