Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

DMD2 sem 2 > Haematology/Oncology: Leukaemia and Lymphoma > Flashcards

Haematology/Oncology: Leukaemia and Lymphoma Flashcards

1
Q

What is leukaemia?

A

Leukaemia literally means “white blood”

Neoplastic proliferation of white blood cells

Cancer of the WBCs

Affects both the bone marrow and circulating blood

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What is the normal leukocyte count?

A

4500 - 11000/μL

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What is the leukocyte count in infections and what can this also be?

A

Increase in white blood cell count

Leukocyte count in acute infections is 15000 - 25000/μL

Less often leukocytosis is a sign of primary bone marrow abnormality related to leukaemia.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What kind of cells increase in number with time?

A

Myeloid or lymphoid cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What are the forms of leukaemia? What is the difference?

A

Acute: Rapidly progressive with accumulation of immature non-functional cells in the marrow and the blood.

Chronic: Slower onset and allows for more mature cells to be produced.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is the aetiology for leukaemia?

A

Unkown

Increased risk associated with radiation, chemical agents, oncogenic viruses, smoking, and genetic predisposition.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

How is leukaemia classified?

A

Based on primary haematopoietic cell line that is affected (Myeloid or lymphoid)

Based on clinical behaviour (Acute or chronic)

4 most simplistic categories are: AML, ALL, CML, CLL

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

How is leukaemia diagnosed?

A

FBC (Peripheral granulocyte count (ANC) increases in chronic leukaemia and increases or decreases in acure leukaemia)

Bone marrow biopsy

Additional techniques including flow cytochemical staining, cytometric immunophenotyping, and genetic analysis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What are the types of myeloid neoplasms?

A

Acute Myeloid Leukaemia

Myeloproliferative disorders (eg chronic myelogenous leukaemia)

MyeloDysplastic Syndromes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

How common is acute myelogenous leukaemia?

A

1/3 of all leukaemias

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What is the aetiology of AML?

A

De novo

Environmental causes (Tobacco, benzene containing compounds, chemotherapy)

Consequence of myelodysplastic syndrome (30% of MDS progress to AML)

Genetic factors (Translocation and rearrangement of genes, down syndrome, klinefelter syndrome, fanconi anaemia, recklinghausen disease)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What are the clinical signs and symptoms of AML?

A

Anaemia related symptoms such as shortness of breath, weakness, dyspnea on exertion.

Physical findings: Pallor, bleeding/bruising, hepatomegaly, and/or splenomegaly (<10% of patients), lymphadenopathy (Uncommon)

Unexplained headache or focal neurologic complaints

Pancytopaenia

Accumulation of leukemic forms in peripheral blood, bone marrow, and other tissues.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

How is AML diagnosed?

A

> 20% of BM cellular component is myeloid blasts/precursors

Myeloblasts contain delicate nuclear chromatin, fine azurophilic cytoplasmic granules, and 3 to 5 nucleoli.

Auer rods (Red staining rod-like structures that are numerous in acute promyelocytic leukaemia and are specific to neoplastic myeloblasts)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

How is AML treated?

A

In phases:

Induction therapy: Intensive combination therapy to achieve a CR through reducing number of leukaemia cells and restoring bone marrow function

Post-remission therapy: Includes chemotherapy, targetted therapies, and autologous or allogenic haematopoietic cell transplantation.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is the goal of AML therapy?

A

Sustain CR and achieve long-term disease-free survival.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is CML?

A

A myeloproliferative neoplasm

Dysregulated production and uncontrolled proliferation of mature and maturing granulocytes with fairly normal differentiation.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What causes CML?

A

BCR-ABL1 fusion gene:

t(9;22)(q34;q11)

Philadelphia chromosome is formed which produces an enzymatic domain from the normal ABL1 resulting in elevated and constitutive kinase activity.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What percentage of cases of CML are caused by BCR-ABL fusion?

A

95% of cases are a balanced 9;22 translocation

in 5% of cases it is caused by complex rearrangements.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

How is CML diagnosed?

A

FISH visualization of BCR-ABL

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

How common is CML?

A

Accounts for approximately 15 - 20% of adult leukaemias in adults

Annual incidence of 1 to 2 cases per 100000

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What age and gender is CML most common in?

A

Most common in males and median age of 50 years

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What are the risk factors for CML?

A

Only known risk factor is exposure to ionizing radiation.

No known familial disposition with exceptional familial CML involving JAK2 and Ph chromosome.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

What are the clincial findings often seen at diagnosis?

A

It is asymptomatic in 20 - 50% of cases

In the symptomatic patients it produces systemic symptoms such as fever (34%), malaise (3%), weight loss (20%), excessive sweating (15%), abdominal fullness (15%), and bleeding episodes due to platelet dysfunction (21%)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

How is CML treated?

A

Imatinib

Pre-blast phase palliative treatment

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Why is imatinib important for dentists?

A

Imatinib leads to hyperpigmentation of the mouth. Leads to staining of mouth (palate most commonly)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

When is CML hardest to treat?

A

CML exists in 3 phases. If diagnosed in late phase it is hard to treat.

27
Q

What is methotrexate used for?

A

It is the first line of therapy for rheumatoid arthritis and lupus as well as part of chemotherapy.

28
Q

Why is methotrexate relevant for dentists?

A

It causes mouth ulceration and severe mucositis.

29
Q

How do lymphomas present?

A

They show up as enlarged lymph nodes (important to physically examine lymph nodes in extra oral examination).

30
Q

What condition is ALL similar to?

A

Overlapping clinical symptoms between ALL and LymphoBlastic Lymphoma

Both pre-B and pre-T cell tumours take on the clinical appearance of ALL during their course.

31
Q

What cells typically cause LymphoBlastic Lymphoma?

A

B cell precursors in 85%

T cell precursors in 10 - 15%

Uncommon variants seen in <1% of cases are early T cells and NK cells.

32
Q

Who most commonly gets B/T-ALL/LBL?

A

Most common in children (2500 to 3500 new cases in children every year)

Peak incidence at 2 - 5 years of age.

More common in boys.

33
Q

What risk factors are associated with ALL/LBL?

A

Unknown cause

Genetic and environmental influences including advanced paternal age and maternal foetal loss, increased birth weight.

Not a familial disease.

34
Q

How is B/T-ALL/LBL diagnosed?

A

Characteristic morphology

Diagnostic immunophenotype of cells from the peripheral blood, bone marrow, lymph nodes, and other involved tissue.

Markers

35
Q

What markers are used for B-lymphoblasts?

A

CD19

Cytoplasmic CD79a

Cytoplasmic CD22

36
Q

What markers are used for T-lymphoblasts?

A

CD3

37
Q

Which people have bad prognosis when they have B/T-ALL/LBL?

A

Ages <1 y.o and >10 y.o

Leukocyte count >50000/microL

Race (black or hispanic) due to genomic variations and socioeconomic factors)

Males

38
Q

How are people with B/T-ALL/LBL treated?

A

Administration of a multidrug regimen: Induction, Consolidation, and maintenance. (takes 2 - 3 years to complete)

Transfusion support

Treatment of proven or suspected infections

Treatment of proven or suspected infections.

Correction of metabolic imbalances

Rarely leukophoresis

39
Q

What is the prognosis of treatment?

A

Induction: 90% of children/adolescents enter complete remission at the end of induction.

40
Q

What is the induction treatment and how is it done differently for different people?

A

BCR_ABL1 positive ALL (Rare and treated with TKI)

BCR-ABL1 negative (Weekly vincristine, daily corticosteroids, and asparaginas)

Down-syndrome ALL (Susceptible to adverse events and treatment-related mortality)

41
Q

What is the purpose of post-remission therapy?

A

It is started soon after complete remission to prevent leukemic regrowth, reduce residual tumour burden, and to prevent emergence of drug-resistance in remaining leukemic cells.

42
Q

What drugs are given for consolidation?

A

Lasts 4 - 8 months:

Cytarabine

Methotrexate

Anthracyclines (Daunorubicin, doxorubicin)

Alkylating agents (Cycllophosphamide, ifosfamide)

Epipodophllotoxins (etoposide, etopophosphamide)

43
Q

What is more common, Hodgkin’s or non-Hodgkin’s lymphoma?

A

Non-hodgkins lymphoma is more common than hodgkin’s lymphoma

44
Q

What are the symptoms of lymphomas?

A

Night sweats, unexpected weight loss, pallor, bone pain, increased infections, lumps, anaemia, thrombocytopaenia, leukopenia, M protein spike, hypercalcaemia, Bence Jones proteins, Genetic testing , biopsy, and PET-CT.

45
Q

What is the defining feature of Hodgkin’s lymphoma?

A

Reed-Sternberg cells

Remember: Owl eyes = Harry potter = Hodgkin’s

46
Q

What are the WHO classifications of Hodgkin’s lymphoma?

A

Nodular lymphocyte predominant Hodgkin lymphoma (distinctive B-cell immunophenotype)

Classic Hodgkin lymphoma:
Nodular sclerosis classic Hodgkin lymphoma
Lymphocyte-rich classic Hodgkin lymphoma
Mixed cellularity classic Hodgkin lymphoma
Lymphocyte-depleted classic Hodgkin lymphoma

47
Q

What causes Hodgkin lymphoma? (pathogenesis)

A

Somatic hypermutation and V(D)J recombination of Ig
gene of Reed-Sternberg cells

Establishment of a germinal centre or post-germinal
centre B cell

Activation of NF-kB through
• EBV infection -> LoF mutation of NF-kB gene

48
Q

Who most commonly gets Hodgkin lymphoma?

A

Bimodal age distribution:

Economically advantaged countries = 20 years and 65 years

Economically disadvantaged countries = peak in young boys and older adults (low in young adults)

49
Q

What are the clinical features of Hodgkin lymphoma?

A

Painless lymphadenopathy

B symptoms (More common in disseminated disease (stage 3 or 4)

50
Q

How is Hodgkin lymphoma treated?

A

Early stage:

RT (High dose increases risk of lung cancer, melanoma, and breast cancer)

ABVD (Doxorubicin, bleomycin, vinblastine, dacarbazine)

Advanced stages:

ABVD (longer and more intense)

BEACOPP (Bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, and prednisone)

51
Q

What is the prognosis like in Hodgkin lymphoma?

A

Stage dependent

52
Q

How are hodgkin and non-hodgkin lymphoma similar?

A

More often localized to a single axial group of nodes (cervical, mediastinal, and para-aortic) in non-hodgkin its more frequent in peripheral nodes

Orderly spread by contiguity whereas non-contiguous spread in non-hodgkin’s lymphoma

Mesenteric nodes and Waldeyer ring rarely involved whereas they are commonly involved in non-hodgkin lymphoma

Extranodal involvement is uncommon in hodgkin but not common in non-hodgkin

53
Q

What are the important types of non-hodgkin lymphomas?

A

Burkitt lymphoma

Multiple myeloma

54
Q

What is the cause of burkitt lymphoma?

A

Translocation and deregulation of the MYC gene

55
Q

What are the clinical forms of burkitt lymphoma?

A

Endemic (african)

Sporadic (non-endemic)

Immunodeficiency associated

56
Q

How are BL and Burkitt lymphoma classified in the WHO?

A

BL and Burkitt leukemia are considered different manifestations of
the same disease in the World Health Organization (WHO)
classification of hematologic malignancies

57
Q

What is burkitt lymphoma?

A

Highly aggressive B cell tumour

58
Q

Where are the types of Burkitt lymphoma seen most often?

A

Endemic: Equatorial Africa and New Guinea. 30 - 50% of all childhood cancer in equatorial Africa. Peak 4 - 7 years old. Male:female 2:1

Sporadic: US and Western Europe. (30% of paediatric lymphomas and <1% of adult non-hodkin lymphomas) Peak incidence at 11 y.o. Male:Female = 3-4:1

59
Q

How is burkitt lymphoma treated?

A

No standard tx:

– Tx is more intensive than other NHL
– Clinical trials
• Aggressive tx with CNS prophylaxis
– Examples of chemotherapy regimens
• CODOX-M plus IVAC (“Magrath regimen”)
– (cyclophosphamide, vincristine, doxorubicin, high-dose methotrexate) with
IVAC (ifosfamide, cytarabine, etoposide, and intrathecal methotrexate)
• HyperCVAD
– Cyclophosphamide, vincristine, doxorubicin, and dexamethasone
(HyperCVAD) with or without rituximab alternating with high-dose
methotrexate and cytarabine

60
Q

What are the signs and symptoms of multiple myeloma?

A

C - hypercalcaemia
R - Renal obstruction
A - Anaemia
B - Bone lesions “punched out”

61
Q

What markers are responsible for multiple myeloma?

A

Cyclin D1 and D3

KRAS+ and FGF+

Bence Jones Proteins

RANK-L induced bone resorption

62
Q

What are the clinical features of multiple myeloma?

A

Older age

Aggressive

Monoclonal gammopathy

63
Q

What are the features of multiple myeloma?

A

Bone pain

Pathologic fracture

Hypercalcemia

Renal failure

Recurrent infections

Anemia

Amyloidosis

Waldenstroms
Macroglobulinemia

DMD2 sem 2 (97 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions