Chapter 9- Leukemias and Lymphomas Flashcards

1
Q

What are some similarities shared between leukemias and lymphomas?

A
  • two groups of malignancies of the blood, bone marrow and lymphatic system
  • both neoplasms of the hematologic system
  • acquired gene mutation either in a stem cell or in early stage WBC.
  • all leukemics cells have the same gene mutation
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2
Q

What are some differences between leukemias and lymphomas?

A

lymphomas- solid tumours,

leukemias- malignant proliferation of WBC.

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3
Q

Where does hematopoisesis occur during

  1. fetal development
  2. childhood
  3. adulthood
A
  1. liver, spleen, lymph nodes, and bone marrow
  2. marrow of all bones
  3. bone marrow of sternum, ribs, vertebrae and ilia of the pelvis bones.
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4
Q

All blood cells begin as primitive stem cells called hemocytoblasts (in the marrow) and then develop into what types of immature cells? (5)

A
erythroblasts
myeloblasts
lymphoblasts
monoblasts
megakaryoblasts
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5
Q

What do these immature cells become once they mature?

  1. erythroblasts
  2. myeloblasts
  3. lymphoblasts
  4. monoblasts
  5. megakaryoblasts
A
  1. erythrocytes (RBC)
  2. granulocytes (WBC) [ neutrophils, eosinophils, basophils]
  3. lymphocytes (WBC)
  4. Monocytes (WBC)
  5. platelets
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6
Q

Which WBC in the immune system produce immunoglobulins?

A

Lymphocytes. There are 3 major types:

B-cells, C-cells, And natural killer (NK) cells.

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7
Q

What do B-cell, T-cells and NK cells do?

A
  1. make antibodies
  2. assit B cells in recognizing the foreign antigens and then secreting hormones to activate other cells to respond.
  3. attack and kill cells infected by foreign organism.
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8
Q

Leukemias can be divided into which two main groups based upon the type of WBC involved.

A
  1. Myeloid
  2. lymphocytic.
    * these groups are then further divided into acute and chornic forms.
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9
Q

What are the 4 major classifications of Leukemias?

A
  1. Acute Myelogenous leukemia (AML)
  2. Chronic myelogenous leukemia (CML)
  3. Acute lymphocytic leukemia (ALL)
  4. Chronic Lymphocytic Leukemia (CLL)
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10
Q

What diffentiates acute vs. chronic leukemia>

A

acute: result from block in cell differentiated process = proliferation of blasts (myeloblasts in AML and lynphoblsats in ALL) CBC showes 30% blasts in bone marrow.
Chronic: uncontrolled growth of more mature but usually immunoincompetent cells.

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11
Q

Is leukemia curable?

A

Acute is but also had a early mortality.

chronic show a slower and relapse course, with gradual mortality curve.

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12
Q

The signs and symptoms are similar for all types of leukemias. What are they?

A
  1. fatigue and SOB d/t anemia
  2. bruising and bleeding as a results of thrombocytopenia
  3. fever/infection d/t low number of WBC
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13
Q

How is leukemia diagnosed?

A
  1. CBC and differential analysis,
  2. bone marrow biopsy,
  3. bone smears
    * 4. cytogenic studies can see gene abN in cells.
    * 5. immunophenotyping subcategorises the type via antigens/antibody classification
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14
Q

How is leukemia treated?

A
  1. monitoring
  2. chemo
  3. bone marrow transplant (Stem cell transplant) *after chemo
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15
Q

Stem cell transplant can be allogeneic or autologous. What does this mean?

A
  1. allogeneic transplant- stem cells come from selected matched donor (sibling)
  2. Autologous transplant the stem cells are taken from the individual prior to chemo and purged or tx’ed, then transplanted back into the pt.
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16
Q

What is the cause of mortality (death) d/t leukaemia?

A

infiltration of vital tissues by leukemic cells (cytopenia) = infection and hemorrhage, or oragn vailure.

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17
Q

In regards to Acute Myelogenous Leukemia (AML) also known as acute non-lymphocytic leukemia (ANLL). What is the

  1. prevelance
  2. typical age of onset
  3. treatement
  4. prognosis.
A
  1. males = females. risk ^ with Radiation or benzene exposure or chemo in past with Alk agents.
    associateion to Downsyndrome, fanconi anemia, Klinfelter and Turner syndrome.
  2. after 50, risk ^ with age.
  3. Chemo- results in remission. will lapse occurs in 75% of cases in first two years. 20-30% have cure.
  4. FAB classification system divides AML into 7 or 8 subclasses M0 or M1 to M7 based on morphologic, histochemical and immunologic characteristics.
  5. Older dx = poorer prognosis
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18
Q

What are some examples of Alkylating agents which are a group of drugs that interfere with malignant cell’s DNA?

A

cytoxan
Leukeran
5-FU

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19
Q

Define the term Morphologic characteristics.

A

physical size and shape of the cell.

ie: myeloblastic, promyelocytic, myelomonocytic, monocytic, erythroleukemia and megakaryocytic groupings

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20
Q

Define the term histologic characteristics

A

microscopic anatomy of the cell.

- chemical components or activities of the cell

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21
Q

define the term immunologic characteristics

A

related to antigen/antibody receptors and other markers on the cell surface.

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22
Q

In Regards to Chronic Myelogenous leukemia. What is the

  1. prevelanc + typical age of onset
  2. epitology
  3. what are the signs and symptoms.
  4. treatment
A
  1. adults, ^ in age. Males > females.
  2. chromosome abN on Ph chromosome. shortened #22 following translocation with chromosome #9. > resulting gene produces abN protein/enzyme => leukemic myeloid cell.
  3. in addition to the ones mentioned, early satiety, Lt upper quadrant fullness/pain/ splenomegaly. weight loss.
  4. chemo, remission does not last long, radiation can be used for splenomegaly.
    alpha interferon can also be used during chronic phase. Bone marrow transplant hold the best hope.
    * young recipients have better mortality than older BMT recipients.
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23
Q

What is the typical 3 clinical phases of the normal course of CML disease?

A
  1. stable/chronic phase
  2. accelerated phase, where WBC fluctuates and show more immature forms
  3. blast crisis phase, the diease resembles acute leukemia.
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24
Q

In regards to Acute Lymphocytic Leukemia. What is

  1. The age of onset
  2. epitology
  3. signs and sxs
  4. prognosis
  5. treatment
A
  1. any age, children>adults.
    80% of children leukemias. peek age 3-4.
  2. Aquired DNA mutation in the DNA of a lymphocyte, leading to the overproduction of immature lymphoblast and disruption of Blood cell formation.
  3. fatigue and pallor, d/t anemia.
  4. doesnt use FAB but rather a class system based on cell origin and immunologic factors
  5. chemo, prophylacytic CNS therapy, mainenance chemo.
    bone marrow transplant can be considered for those tho replase or have worst prognosis. complete remission is more common in Children between 2-9. poor prognosis factors: dx >2-94 weeks to acheive remission, high leukocyte
25
Q

the French-American-British (FAB) classification system describes aLL into 3 subgroups, L1 through L3 based on what?

A
  1. morphologic factors (cell size)
  2. amount and type of cytoplasm
  3. shape/features of the nucleus
26
Q

What are the 5 ALL subclasses using the classification system based on cell origin and immunologic factors.

A
  1. early pre-B cell
  2. Pre-B cell
  3. Transitional pre-B cell
  4. B-cell
  5. T-cell (10-15% of cases)
27
Q
In regards to Chronic Lymphocytic Leukemia (CLL). What is 
1. the age of onset and prevelance?
2. epidemiology
3. signs and sxs 
4. diagnosis
5 . treatment 
6. prognosis
A
  1. adult - most common form, rare under 45yo. ^ with age. Males 2x>females.
    not associated with radiation or toxin exposure.
  2. results from Damage to the DNA of a lymphocyte in the bone marrow.
  3. develop gradually, fatigue, SOB or frequent infections.
  4. routine physical where high lymphocyte count was incidental findings.
  5. depends on stage and sxs, observation is common, chemo can be used in later stages for sxs control. Radiation can alleviate sxs arising from splenomegaly or lymph nodes. Imbruvica.
  6. related to stage.
28
Q

There are 2 commonly used classification systems for CLL, both based on physical and laboratory findings. Define the Ray system.

A

Has 5 stages, prognosis worsens with higher stages.
stage 0: lymphocutosis only
Stage 1: lymphocytosis and adenopathy
stage 2: lymphocytosis and hepatomegaly or splenomegaly
stage 3- lymphocytosis and anemia
Stage 4- lymphocystosis and thrombocytopenia.

29
Q

There are 2 commonly used classification systems for CLL, both based on physical and laboratory findings. Define the Binet classification system.

A

3 stages, prognosis worsens with higher stages
Stage A: lymphocytosis with enlagrement of 3 or less lymph node groups
Stage B: lymphocytosis with enlargement of more than 3 lymph node groups
Stage C: lymphocytosis with anemia or thrombocytopenia.

30
Q

What is HAir cell leukemia?

A

variant of CLL that gets it name from the tiny hair-like projections from the cell membrane of the lymphocyte.
- slow progressive malignancy, but with a median age of onset 50 yop.
males 4x> femalres.
- tx;ed with alpha interferon, not a cure.

31
Q

What needs to be reviewed when UW a history of Leukemias?

A

type, tx and response to tx.
Acute hx with 5 yr remission can be considered.
Chronic are not considered curable and should only be consideered if indolent/slow progress.

32
Q

Is morbidity increased with leukaemia?

A

yes, d/t disease and tx.

SA: nausea, diarrhea, fatigue, loss of apetite, weight loss and infections. Future anxiety and depression after tx.

33
Q

Immunotherapy is a promising area of research for cancer cures. What is this?

A

involves the use of abx, immune cells, or vaccines to fight disease.

34
Q

What are lymphomas?

A

tumours that originate in the lymphatic system. Neoplasm can metastasize to the lymph nodes or though the lymph system, but start in the nodes.

35
Q

What is lymphadenopathy.

A

enlarged lymph nodes d/t malignancies (hard, fixed location, mutiple, nontender) or infections (soft, tender, <2cm, in area of infection)

36
Q

What are sxs of Lymphoma?

A
lymphadenopathy 
fatigue
fever
cough 
night sweats
weight loss
37
Q

Lymphomas are generally divided into which two subgroups?

A
  1. hodgkins lymphoma,

2. non-hodgkin lymphoma

38
Q

What is the prevelance and age of onset for Hodgkin lymphoma?

A

incidence incrases after age 10 and peaks in 20’s. declines until rising again in mid 40’s then steady with age.

39
Q

What is the epidemiology of Hodgkins Lymphoma?

A

related to damage to DNA to a lymphocute after birth, not inherited
possibly infection cause (Epstein-Barr virus)

40
Q

What are the sxs and signs of Hodgkins Lymphoma?

A
painless swelling of the lymph nodes/upper chest/armpit or groin. 
Faigue 
malaise 
fever 
night sweats
weight loss 
itching
41
Q

how is Hodgkins Lymphoma diagnosed?

A

biopsy of lymph node via complete removal of the node. CT and MRIs can help determine the extent of the disease.

42
Q

what are the 4 major subgroups of Hodgkinds lymphoma based on histologic cell type?

A
  1. lymphocyte predominant - good prognosis
  2. nodular sclerosis - most common, good prognosis
  3. mixed cellularity - poor prognosis
  4. lymphocyte depleted. - poor prognosis
43
Q

What differentiates Hodgkinds from other types of lymphoma?

A

presence of a distinguished cell type known as Reed-Sternberg Cells.

44
Q

How is Hodgkins Lymphoma staged?

A

by extent of the disease, using clinical and patholgoical factors. There are 4 stages based on Ann Arbor stading system. They can be further categorized using A and B, based on absence (a) or presence (b) of sxs: fever, sweating, weight loss

Stage 1: involement of single lymph node region or single extralymphatic order or site.
Stage
Stage 2: involvement of two or mode node regions on the same side of the diaphragm.
Stage 3: involvement of several nodes regions on both sides of diaphragm
Stage 4: diffuse involvement of extralymphatic organ/site node regions.

45
Q

How is Hodgkins Lymphoma treated?

A

chemotherapy, radiation (if early stages),

if later stages, where diffuse lymph node is involved chemo and radiation is used.

46
Q

What is the prognosis of Hodgkins Lymphoma?

A

Most complications arrise from CHEMO and RAD AE. chemo can increase risk for acute leukemia or non-hodgkinds lymphoma.
Prognosis is based on:
1. stage (earlier the better)
2. histologic subtype (predom and nodular sclerosis better)
3. presence of absence of sxs (B is better)
4. age (young better)
5. replaces (poor)

47
Q

True or False Hodgkins Lymphoma is considered the most curable forms of cancer?

A

true,

specially if in complete remission for a number of years

48
Q

What is the prevalence and age of onset for Non-Hodgkin Lymphoma (NHL)?

A

incidence increases with age, males > females. number of incidences has increased since 1970s thought to be associated to viruses, people with suppressed immune systems.
- multicentric in origin (group of dieases originatinf from the malignant transformation of a lymphocute or lymphocyte precursor)

49
Q

What are the signs and symptoms of NHL?

A

nonspecific and usually undiagnosed. usually initiated by enlarged lymphnode, in the neck/armpit/groin.
possible weight loss, abdo pain/bloating, anemia, fatigue,fever, sweats and itching.

50
Q

how is NHL diagnosed?

A

lymph node biopsy or biopsy of involved organ.
additional testing done to determine extent of disease: CT and MRI of chest and abdo, Bone marrow study, CBC, gallium scan.

51
Q

What is a gallium scan?

A

radioactive isotope of gallium is injected into a vein and the radioactive gallium collects in the lymphoma lesions, showing where the tumors are located.

52
Q

How is NHL staged?

A

using Ann Arbor staging system. Stages 1-4 with A and B subclassification for sxs of fever, sweating and weight loss.
NHL does not spread to other organs.
- in addition to staging,

53
Q

How is NHL graded?

A

oncologist also classify the various types of NHL as low grade (slow progression), intermediate, or high grade (fast progression)

54
Q

How is NHL histologically classified?

A

immunophenotyping determines if the lymphoma is related to B-cells, T-cells, or NK. Also considered if cell is small or large, whether the architecture is nodular or follicular vs. Diffuse and whether the cell is well differentiated or poorly differentiated.

** Low-grade lymphomas are primarily B-cell,

55
Q

The Wroking Formulation system was developed in 1982 in an attempts to resolve differences in prior classification systems of NHL. What are the sub–types?

A
  1. Low Grade
    a) small lymphocytic
    b) follicular small cleaved lymphocytic
    c) mixed follicular small cleaved cell and large cell
  2. Intermediate grade
    a) follicular predominantly large cell
    b) diffuse small cleaved cell
    c) diffuse large cell
  3. High grade
    a) diffuse large cell immunoblasts
    b) lymphoblastic
    c) small noncleaved
56
Q

What is the primary treatement for NHL?

A

chemotherapy #1, and regim is based on histologic subtype, stage, and age.
Radiation sometime used to control localized disease or d/t sxs w/ advanced diseases.
Stem cell transplant- relapse

57
Q

what is the prognosis for those dx with NHL?

A

usually diagnosed in later stages, and is related to histologic subtype and grade (not stage)
intermediate/severe disease: survival curve drops sharply in first 2-4 yrs the levels off.
Low-grade survival curve is slower but consistent drop, these are usually chronic, progressive and incurable.

58
Q

True or False

low-grade NHL tumours have a better short-term prognosis than intermediate and high-grade lesions?

A

True, but those with intermediate or high-grade tumours who survived the 8 year mark, have better long-term prognosis

59
Q

What are some unfavourable prognostic factors for NHL?

A
>60 yo
lymphoma mass >10cm 
abnormal serum LDH, 
T-cell phenotype. 
lapses