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gen-path 2 > WBC disorders > Flashcards

WBC disorders Flashcards

1
Q

Hodgkins lymphoma is a lymphoma of a ______________

A

distinctive cell type

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2
Q

which specific cells are the malignant cells of hodgkins lymphoma?

A

Reed-Sternberg cells

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3
Q

what are the pathological characteristics of hodgkins lymphoma?

A

Arises in a single lymph node or spreads contiguously to involve a chain of nodes

Highly curable in most cases with chemotherapy and/or radiotherapy

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4
Q

what are the CLINICAL signs/symptoms of Hodgkins lymphoma? (theres 3 points)

A

1) Bimodal age distribution: 20-30 years old and >50 years old
2) Painless lymphadenopathy (often cervical, supraclavicular, mediastinal)
3) Splenomegaly (detected by MRI)

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5
Q

how are cases of Hodgkins lymphoma diagnosed?

A

Lymph node biopsy necessary for diagnosis

Diagnosis requires identification of Reed-Sternberg cells in appropriate background

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6
Q

there are _____ different types of Hodgkins lymphoma. How are they recognized?

A

Five different types

each is recognized with their own clinical presentations and histopathologic features

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7
Q

what are the characteristics of Reed-Sternberg (RS) Cells?

A
  • Large cell with mirror-image nuclei and prominent nucleoli
  • derived from B lymphocytes
  • The malignant cell of Hodgkin lymphoma, but comprises only 2% of cells in the involved lymph node
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8
Q

what virus is often found in reed-sternberg cells involved in Hodgkins lymphoma?

A

Epstein-Barr virus (70%)

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9
Q

____________ is used to determine treatment and prognosis for patients with Hodgkins lymphoma

A

staging

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10
Q

what are the two stages of Hodgkins lymphoma?

A

Low stage:
- Localized involvement

High stage:
- Wide spread disease with distant or bone marrow involvement

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11
Q

what is the definition of stage 1 hodgkins lymphoma? what about stage 2?

A

Stage 1: Tumor in one anatomic region or two contiguous anatomic regions on the same side of the diaphragm

Stage 2: Tumor in more than two anatomic regions or two non-contiguous regions on the same side of the diaphragm

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12
Q

how do stages 3 and 4 of hodgkins lymphoma differ?

A

stage 3:
- Tumor on both sides of the diaphragm not extending beyond lymph nodes, spleen or Waldeyer’s ring (tonsils)

stage 4:
- Tumor in bone marrow, lung, etc.- any organ site outside of the lymph nodes, spleen or Waldeyer’s ring

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13
Q

what are the “B” signs/symptoms of Hodgkins lympoma?

A

Fever, night sweats, and significant unexplained weight loss

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14
Q

the treatment for Hodgkins lymphoma is based primarily on ___________

A

the stage of the disease

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15
Q

what are the treatments for low stage (localized) hodgkins lymphoma? what about high stage?

A

Low stage: Chemotherapy and radiotherapy

high stage: Chemotherapy

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16
Q

T/F: patients with hodgkins lymphoma are at a high risk for development of secondary treatment-related acute leukemia

A

FALSE

they are at low risk

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17
Q

hodgkins lymphoma Patients without _____________ have better prognosis

A

“B” signs/symptoms

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18
Q

what is the survival rate for stage 1 & 2 hodgkins lymphoma? how about later stages?

A

5-year survival - Stage I and IIA - almost 100%

5-year survival rate in Stage IV is 50%

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19
Q

what are the characteristics of Non-Hodgkin Lymphoma (NHL)?

A
  • Neoplastic lymphocytes originating in lymph nodes or extranodal lymphoid tissue
  • Most (85%) are of B cell origin
  • Most of remainder are of T cell origin
  • Incidence rises steadily after age 40
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20
Q

how many different types of NHL are there?

A

Over 2 dozen types

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21
Q

list the clinical signs of NHL

A

Painless lymph node enlargement

Systemic symptoms in 30% of patients

Frequent immune abnormalities

Splenomegaly

May involve GI tract, bones, central nervous system

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22
Q

how are different types of NHL classified?

A

Growth pattern
- Nodular vs diffuse

Cell size
- Small vs large

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23
Q

is nodular or diffuse Non-Hodgkins lymphoma better? how about small vs large cell size?

A

Nodular disease better than diffuse

Small cell disease better than large

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24
Q

how is the prognosis for NHL determined?

A

Prognosis often based more on the sub-type of lymphoma than stage
(exception to the general rule, “grading” or subtyping is critical)

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25
Q

what are the treatments for non-hodgkins lymphoma?

A

Chemotherapy
Possible radiation therapy
Bone marrow transplantation

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26
Q

what are the characteristics of acute leukemia?

A

Rapid onset with blasts in the blood

Myeloid and lymphoid cells affected

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27
Q

what are the characteristics of chronic leukemia?

A

Indolent onset and tends to involve more mature cells

Myeloid and lymphoid cells affected

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28
Q

___________ are malignant neoplasms of hematopoietic tissue that arise in the bone marrow

A

Leukemias

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29
Q

what are the 4 basic classes of leukemia?

A

acute lymphoblastic leukemia (ALL)

chronic lymphocytic leukemia (CLL)

acute myelogenous leukemia (AML)

chronic myelogenous leukemia (CML)

30
Q

what are the general characteristics of acute lymphoblastic leukemia (ALL)?

A
  • Clonal growth of primitive lymphoid cell
  • Both B and T cell types exist
  • often involves the central nervous system
  • Increased white blood cell count often accompanied by thrombocytopenia
31
Q

in what population is acute lymphoblastic leukemia most common?

A

Most common in children

most frequent cancer in those < 15 yrs of age*

32
Q

the Prognosis of ALL is related to ___________

A

cytogenetics

hyperdiploidy (>50 chromosomes per leukemic cell)
the subtype with best prognosis (most are children)

33
Q

what population usually has a poor prognosis for acute lymphoblastic leukemia? who usually has a good prognosis?

A

Children have good prognosis

Adults often have bad prognosis

34
Q

list the general characteristics of Acute Myelogenous Leukemia (AML)

A
  • Clonal proliferation of primitive myeloid cell
  • Increased WBC count often accompanied by anemia and thrombocytopenia
  • More common in adults
35
Q

during acute myelogenous leukemia, granules of ______________ are present in the cytoplasm of more mature myeloid cells

A

Myeloperoxidase

36
Q

the prognosis for AML is influenced by __________

A

cytogenetics

the chromosome mutation is the most important factor in prognosis

37
Q

list the general characteristics of Chronic Myelogenous Leukemia (CML)

A
  • Clonal proliferation of mature lymphocyte, typically a B lymphocyte
  • The B cells are immunologically incompetent
  • Monoclonal expression of surface immunoglobulin
38
Q

CML is most common in what population?

A

adults over 60 years old

39
Q

during CML, there is an Increased white cell count with ___________

A

lymphocytosis

40
Q

what are the pathogenic characteristics of CML?

A
  • Increased white cell count with lymphocytosis
  • splenomegaly and lymphadenopathy
  • Anemia and thrombocytopenia eventually develop as disease progresses
41
Q

All CML cases eventually terminate into a higher grade process - either __________ or _________

A

acute leukemia or high grade lymphoma

42
Q

as CML progresses, __________ may develop (anemia and thrombocytopenia) as the marrow is overrun by leukemic cells

A

cytopenias

43
Q

what is the proliferating cell of CML?

A

an immature hematopoietic cell

a stem cell from which all other hematopoietic cells arise

44
Q

During CML, a specific chromosomal abnormality in the ________________, occurs in all the proliferating cells

A

Philadelphia chromosome t(9;22)

45
Q

The chromosomal abnormality of t(9;22) results in fusion of the __________ genes

A

BCR-ABL

46
Q

what molecule is targeted during chemotherapy for CML treatment?

A

Targeted chemotherapy blocks the bcr-abl tyrosine kinase

  • induces remission in most patients
47
Q

what treatments are available for relapsed/resistant CML?

A

Bone marrow transplant

48
Q

what are the 3 common characteristics of all leukemias?

A
  • Abnormal cells suppress growth of normal cells
  • Infiltration of body organs by abnormal cells
  • Immune dysfunction
49
Q

Multiple Myeloma is the result of ___________ proliferations

A

plasma cell proliferations

50
Q

what are the general pathological characteristics of multiple myeloma?

A
  • A clonal proliferation of monoclonal plasma cells
  • Monoclonal heavy and or light chain production
  • A disease of late middle age to elderly
51
Q

which immunoglobulin is most commonly produced during multiple myeloma?

A

IgG

52
Q

Plasma cell disorders result from clonal expansion of ______________-secreting cells

A

immunoglobulin

53
Q

what are the Clinical/Laboratory Findings for multiple myeloma?

A
  • Multiple lytic bone lesions, hypercalcemia
  • Bone marrow infiltration by plasma cells
  • ↑↑ serum monoclonal protein, but normal immunoglobulins are suppressed
  • Renal failure
  • Infections
54
Q

Bence-Jones proteinuria is caused by what kind of immunoglobulin?

A

light chain Ig’s

55
Q

what are the Blood and Marrow Features of patients with Multiple myeloma?

A
  • Circulating plasma cells in blood are uncommon
    (they are mostly found in the marrow)
  • RBC show rouleaux formation (stacks of clumped cells)
  • Marrow plasma cell infiltrates in single cells and sheets
56
Q

what are the prognosis for the indolent and typical forms of multiple myeloma?

A

Indolent form: survival for years (don’t need treatment)

Typical form: median survival – 4-6 years

57
Q

what are the treatments available for multiple myeloma?

A

Chemotherapy – recent success with thalidomide analogs, anti-resorptive tx

Radiotherapy (palliation – for bone pain)

Bone marrow transplant

58
Q

patients with multiple myeloma often present with what clinical signs?

A

Patients often present with bone pain, hypercalcemia, and renal disease

The "CRAB" symptoms
C- calcium (elevated)
R- renal failure
A- anemia
B- Bone pain/lesions
59
Q

what is the group with the best prognosis for Acute Lymphoblastic Leukemia?

A

children aged 2-10 with pre-B cell type

60
Q

Enlargement of lymph nodes, liver and spleen is more common in ALL than _______

A

AML

acute myelogenous leukemia

61
Q

In cases of AML, Cytoplasmic inclusions called _________, when present, are diagnostic

A

Auer rods

62
Q

the ___________ of AML cells is the most predictive factor for prognosis

A

The karyotype is most predictive of prognosis

63
Q

what is responsible for granulocytic sarcomas?

A

AML lesional cells proliferate into soft tissue (including the gingivae)

64
Q

T/F: remission of AML is usually transient, not permanent

A

true

65
Q

More than 95% of CLL are of what type?

A

B cell type

66
Q

what immunoglobulin do most CLL cells produce?

A

IgM kappa surface immunoglobulin

67
Q

This type of leukemia accounts for about 2/3 of chronic leukemias

A

Chronic Lymphocytic Leukemia

68
Q

____ accounts for about 1/3 of chronic leukemias

A

CML

69
Q

what is blast crisis?

A
  • occurs when treating terminal phase CML

- its a relative increase in immature cells in peripheral blood and bone marrow, and decreased response to treatment

70
Q

This stage of CML is equivalent to an acute leukemia

A

terminal phase with blast crisis

gen-path 2 (7 decks)

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