Lecture 5- WBC disorders

Question 1

… 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.

Answer 1

lymphomas

Question 2

2 types of lymphomas

Answer 2

Hodgkins

Non-Hodgkins

Question 3

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

Answer 3

leukemias

Question 4

leukemias can be … or … and also … or …

Answer 4

acute or chronic

myeloid or lymphoid

Question 5

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

Answer 5

true

Question 6

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

Answer 6

Hodgkin lymphoma

Question 7

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

Answer 7

Hodgkins

Question 8

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

Answer 8

HL

Question 9

HL lymphoma has a …. age distribution

Answer 9

bimodal

20-30 yo and >50 yo

Question 10

Is HL common?

Answer 10

no, only about 8300 cases in 2017

Question 11

HL usually presents first with ….

or …. which can be detected through an MRI

Answer 11

painless lymphadenopathy (often cervical, supraclavicular, mediastinal)

splenomegaly

Question 12

What causes HL?

Answer 12

unknown but EBV has been implicated in playing a role

Question 13

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

Answer 13

Lymph node biopsy

Reed-Sternberg cells

background

Question 14

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

Answer 14

5

Question 15

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

Answer 15

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

Question 16

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

Answer 16

lymphocytes
plasma cells
eosinophils

Question 17

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

Answer 17

mirror-image

prominent

(owl eyes)

Question 18

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

Answer 18

less than or equal to 2%

Question 19

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

Answer 19

a B lymphocyte arising from a germinal center

Question 20

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

Answer 20

70%

Question 21

spread of disease is predicatble in HL, …

Answer 21

lymph nodes
spleen
liver
bone marrow

Question 22

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

Answer 22

one or two

same side

Question 23

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

Answer 23

more than 2 anatomic regions or non-contiguous regions

same side

Question 24

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

Answer 24

both sides

lymph nodes, spleen or Waldeyers ring

Question 25

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

Answer 25

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

Question 26

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

Answer 26

fever, night sweats, and unexplained weight loss

Question 27

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

Answer 27

almost 100%

Question 28

what is the 5 year survival rate of stage IV HL

Answer 28

50%

Question 29

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

Answer 29

B cell (85%) T cell

Question 30

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

Answer 30

40 72,000

Question 31

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

Answer 31

multiple node involvement extranodal peripheral blood involvement

Question 32

Clinical presentation for NHL: painless .... systemic symptoms in ...% of patients frequent .... abnormalities splenomegaly may involve...

Answer 32

painless lymph node enlargement systemic symptoms in 30% on patients frequent immune abnormalities may involve GI tract, bones, CNS

Question 33

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

Answer 33

growth pattern (nodular vs diffuse) cell size (small vs large)

Question 34

Which NHL characteristics have a better prognosis? small or large nodular or diffuse

Answer 34

small and nodular are better

Question 35

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

Answer 35

low high

Question 36

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

Answer 36

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

Question 37

Treatment for NHL?(3)

Answer 37

chemotherapy possibly radiation bone marrow transplantation

Question 38

Classification of leukemias: they can be ... or ... and ... or .... (which cells are affected)

Answer 38

acute or chronic

Question 39

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

Answer 39

rapid blast myeloid lymphoid

Question 40

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

Answer 40

indolent mature myeloid lymphoid

Question 41

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

Answer 41

acute (chronic has survival of years or decades)

Question 42

Acute and chronic leukemias usually have an ... WBC count

Answer 42

increased (acute sometimes is decreased)

Question 43

the 4 classifications of leukemias are

Answer 43

Acute Lymphoblastic Leukemia (ALL) Chronic Lymphocytic Leukemia (CLL) Acute Myelogenous Leukemia (AML) Chronic Myelogenous Leukemia (CML)

Question 44

The proliferating cell in acute lymphoblastic leukemia (ALL) is a .... this type of leukemia accounts for about ... of the acute leukemias

Answer 44

primitive lymphoid cell 40%

Question 45

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

Answer 45

ALL (acute lymphoblastic leukemia)

Question 46

what is the peak age of incidence of ALL

Answer 46

4 years old

Question 47

How many subtypes of ALL are there and what are they

Answer 47

early B precursor pre-B mature B T cell

Question 48

In ALL, prognosis is related to ....

Answer 48

cytogenetics

Question 49

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

Answer 49

children

Question 50

what is the treatment of ALL?

Answer 50

chemotherapy mainstay with bone marrow transplantation considered at relapse

Question 51

AML is seen to have clonal...

Answer 51

proliferation of a primitive myeloid cell

Question 52

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

Answer 52

increased anemia thrombocytopenia

Question 53

Is AML more common in children or adults?

Answer 53

adults

Question 54

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

Answer 54

AML

Question 55

Which leukemia is myeloperoxidase present in the cytoplasm?

Answer 55

AML

Question 56

What is the treatment of AML?

Answer 56

chemotherapy

Question 57

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

Answer 57

70%

Question 58

Prognosis of AML is influenced by ...

Answer 58

cytogenetics

Question 59

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

Answer 59

mature (immunologically incompetent) B lymphocytes

Question 60

.... is the most common adult leukemia in the US

Answer 60

Chronic Lymphocytic Leukemia (CLL)

Question 61

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

Answer 61

60 yo | males

Question 62

CLL usually expresses ... with kappa light chain restriction

Answer 62

surface IgM

Question 63

There is a high expression of .... in CLL

Answer 63

BCL2

Question 64

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

Answer 64

splenomegaly | lymphadenopathy

Question 65

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

Answer 65

hypogammaglobulinemia

Question 66

Treatment and survival of CLL?

Answer 66

chemo | median survival is 4-6 years

Question 67

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

Answer 67

high acute leukemia high grade lymphoma

Question 68

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

Answer 68

immature granulocytes - a stem cell disorder

Question 69

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

Answer 69

CML

Question 70

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

Answer 70

thrombocytosis | anemia

Question 71

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

Answer 71

CML

Question 72

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

Answer 72

BCR-ABL genes

Question 73

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

Answer 73

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

Question 74

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

Answer 74

targeted inhibitors of the BCR-ABL tyrosine kinase

Question 75

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

Answer 75

bone marrow transplant (risky in older patients)

Question 76

.... is the clonal proliferation of monoclonal plasma cells.

Answer 76

multiple myeloma

Question 77

Which is the most common immunoglobin in multiple myeloma

Answer 77

IgG

Question 78

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

Answer 78

30,000

Question 79

what age do you see multiple myeloma in usually

Answer 79

late middle age to elderly

Question 80

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

Answer 80

multiple myeloma

Question 81

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

Answer 81

multiple myeloma

Question 82

What disase shows RBC rouleaux formation (stacking)

Answer 82

multiple myeloma

Question 83

median survival of multiple myeloma

Answer 83

4-6 years

Question 84

multiple myeloma treatment has had recent success with ....

Answer 84

chemotherapy with thalidomide analogs, anti-resorptives