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2.2 Haematology > Haematological Malignancies 2 > Flashcards

Haematological Malignancies 2 Flashcards

1
Q

What is Hodgkin lymphoma
(5)

A

Clonal B-cell malignant that develops within the lymphatic system

The malignant Reed-Sternberg Cell typically has a bilobed nucleus that gives an “owls eyes” appearance

Diagnosis, excisional lymph node biopsy

Spreads in an orderly fashion to adjacent nodes

Painless lymphadenopathy, constitutional “B” symptoms (fever, night sweat, weight loss), pruritus, hepatosplenomegaly

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2
Q

Write about Reed-Sternberg

A

Cell may contain more than one nucleus

Presence in peripheral blood indicate advanced stage of disease

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3
Q

What are the three main types of lymphoma

A

NK cell
B cell
T cell

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4
Q

What cells are involved in leukaemia

A

Neutrophil
Monocyte
RBC
Megakaryocyte

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5
Q

Write about acute lymphoblastic leukaemia

A

Usually occurs before 14 years of age

Peak incidence between 2 and 9 years

Less common in adults -> peak at about 50

Low RCC, Hb, Hct, platelet count, low normal or high WBC count

Accumulation of malignant, poorly differentiated lymphoid cells within the Bone marrow, peripheral blood and 20% of the time at extramedullary sites

Chromosomal aberrations are the hallmark of ALL, but are not sufficient to generate leukaemia.

Characteristic translocations

More recently, a variant with a similar gene expression profile to Ph positive ALL but without the BCR-ABL1 rearrangement has been identified (poor prognosis)

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6
Q

Comment on the prevalence of ALL

A

Usually occurs before 14 years of age

Peak incidence between 2 and 9 years

Less common in adults -> peak at about 50

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7
Q

What are the clinical findings of ALL?
(5)

A

Low RCC
low Hb
low Hct
low platelet count
low normal or high WBC count
Increased lymphoblast

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8
Q

What exactly happens in ALL

A

Accumulation of malignant, poorly differentiated lymphoid cells within the Bone marrow, peripheral blood and 20% of the time at extramedullary sites

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9
Q

What causes ALL

A

Chromosomal aberrations are the hallmark of ALL, but are not sufficient to generate leukaemia.

Characteristic translocations

More recently, a variant with a similar gene expression profile to Ph positive ALL but without the BCR-ABL1 rearrangement has been identified (poor prognosis)

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10
Q

Write about the clinical findings of bone marrow aspirate in ALL

A

Hypercellularity
High lymphoblasts greater than 20%

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11
Q

Write about acute myeloid leukaemia

A

Most common type of acute leukaemia in adults

Accounts for 30% of all leukaemia

300-400 cases of AML in Ireland/year

Outcome in patients with AML ranges from death within a few days of beginning treatment to likely cure

The major reason patients are not cured is resistance to treatment, often manifested as relapse from remission, rather than, even in older patients, treatment related mortality, whose incidence is decreasing

Knowledge of the pre-treatment mutation statis of various genes has improved our ability to assign initial treatment and, of particular importance, knowledge of whether patients apparently in remission have measurable residual disease should influence subsequent management

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12
Q

What is the most common type of leukaemia in adults

A

Acute myeloid leukaemia in adults

Accounts for 30% of all leukaemia in adults

300-400 cases per year

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13
Q

Comment on the severity of AML

A

One of the harder leukaemias to beat

Outcome in patients with AML ranges from death within a few days of beginning treatment to likely cure

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14
Q

Why is AML so hard to cure

A

The major reason patients are not cured is resistance to treatment, often manifested as relapse from remission, rather than, even in older patients, treatment related mortality, whose incidence is decreasing

Knowledge of the pre-treatment mutation statis of various genes has improved our ability to assign initial treatment and, of particular importance, knowledge of whether patients apparently in remission have measurable residual disease should influence subsequent management

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15
Q

How is AML classified

A

M0 -> M7

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16
Q

What is M0 AML

A

Undifferentiated acute myeloblastic leukaemia
5%

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17
Q

What is M1 AML

A

Greater number of myeloblasts with less than 10% granulocytic differentiation

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18
Q

What is M2 AML

A

Myeloblasts in great number with granulocytic differentiation >10%

NSE < 20%

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19
Q

What is M3 AML

A

Promyelocytes that are hyper granular with many Auer rods on CAE or Wright-stain and variant form cells with reniform nuclei, multilobed or bibbed, primeval cells with multiple Auer rods or relative scarcity of Hypergranular promyelocytes

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20
Q

What is M4 AML

A

> 20% but <80% NSE-butyrate positivity in Monocytic cells

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21
Q

What is M5 AML

A

Monocytic cells with >80% NSE positivity

a. Monocytic differentiated
b. Monocytic, differentiated

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22
Q

What is M6 AML

A

> 30% myeloblasts with more than 50% erythroblasts eliminating the erythrois cells

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23
Q

What is M7 AML

A

Acute megakaryoblastic leukaemia <5%

24
Q

Write about CLL
(7)

A

Most common leukaemia, usually B lineage

Normally B cells produce antibody in CLL they don’t

Often diagnosed on routine check up

BM aspirate not useful in early stages

Rai Binet staging system

Flow cytometry needed for diagnosis

Richters transformation: very rare, very severe, CLL> Prolymphocytic leukaemia

25
Q

What are the clinical symptoms of CLL

A

Lymphocytosis B
Smudge/smear cells up
Reticulocyte up
Haemoglobin down

26
Q

Write about the use of flow cytometry in CLL

A

Needed for diagnosis

Neoplastic B-cells with co-expression of CD19, CD5, CD23, with weak CD20 and monoclonal surface immunoglobulin expression

27
Q

What is a telltale feature of CLL

A

Hairy Cell

28
Q

What are some laboratory findings of peripheral blood?
(4)

A

Hairy cells

Pancytopenia

Neutropenia

Flow cytometry

29
Q

Explain how flow cytometry is used for CLL
(2)

A

The cells are strongly positive for CD20

In contrast to other B cell disorders they also express CD25, CD103 and CD123

They are CD5 negative

30
Q

Write about hairy cell trapping

A

Hairy cells are trapped in the bone marrow and that’s why you get a dry tap

They are also trapped everywhere else, therefore, they don’t show up in the lymph nodes and that’s why the clinical finding of lymphadenopathy is absent

31
Q

Write about the clinical findings of plasma cell myeloma
(7)

A

Rouleaux formation
RBCC down
Neoplastic plasma cells

Neoplastic plasma cells in BM aspirate
Bence-Jones in urine electrophoresis
Paraprotein in serum electrophoresis
IG up in serum electrophoresis

32
Q

Write about the use of flow cytometry in plasma cell myeloma
(3)

A

Usually lack surface light chain with monotypic cytoplasmic Ig
Express bright CD38, CD138, often CD56+ or CD117+
May have partial CD45, usually negative for CD20, CD19 and CD10

33
Q

What are MPNs

A

Myeloproliferative neoplasms

34
Q

What does Ph negative MPN mean

A

Philadelphia chromosome negative myeloproliferative neoplasms

35
Q

What are the four types of philadelphia chromosome negative MPNs

A

Myelofibrosis
Polycythemia vera
Essential thrombocythemia
Other

36
Q

What are some clinical findings of CML
(6)

A

RBCC down
Little to no platelets
WBCC up
Neutrophils myelocytes
PHL Chromosome in BM
Hypercellularity in bone marrow > 90%
Blasts

37
Q

Write about myelodysplastic Neoplasms

A

Cytopenias
Dysplasia in one or more of the major myeloid cell lines
Ineffective haematopoiesis
Increased risk of development of acute myeloid leukaemia (AML)

38
Q

What are the thresholds for crytopenias ?
(4)

A

The thresholds for crytopenias as recommended in the International Prognostic Scoring System (IPSS) for risk stratification in the MDS are:
- Haemoglobin < 10 g/dL
- Absolute neutrophil count (ANC) < 1.8 x10^9/L
- Platelets < 100 x 10^9/L

39
Q

What happens in MDS

A

Bone marrow does not produce enough healthy blood cells

Average age of diagnosis is between 60 and 75 years

Risk factors include smoking and exposure to automobile exhaust

40
Q

What are the symptoms of MDS

A

Fever
Fatigue
Weakness
Easy bruising

41
Q

How is MDS diagnosed

A

Blood tests
Bone marrow examination
Karyotyping

42
Q

Comment on the severity of MDS

A

Death is mostly caused by bleeding and infections

Average survival after diagnosis is 6-12 months

43
Q

How is MDS treated

A

Chemotherapy
Stem cell transplantations

44
Q

What morphological signs are there for MDS

A

Dysgranulopoiesis

Dyserythropoiesis

Dysmegakaryopoiesis

45
Q

What is meant by dysgranulopoiesis?

A

Formation of asynchr

46
Q

What is meant by dyserythropoiesis

A

Formation of macrocytic, megaloblastic cells

47
Q

What is meant by dysmegakaryopoiesis

A

Formation of Rund, non-lobules megakaryocytes

48
Q

How does MDS become AML

A

Normal stem cells undergo aberrant epigenetic programs to become MDS stem cells

MDS stem cells undergo aberrant growth signals and reduced apoptosis to become AML

This results in inhibited differentiation and uncontrolled blast cell proliferation

Thought to be due to epigenetics

49
Q

How do we diagnose and monitor haematological neoplasms
(5)

A

FBC and morphology

Flow cytometry

Cytogenetics, FISH, Karyotyping, Array CGH

Molecular sequencing

Molecular methods e.g. RQ-PCR, dPCR, RT-PCR

50
Q

How do we use PCR to quantify disease burden
(3)

A

Real time PCR used to quantify amount of disease that is there e.g. cycle numbers in covid

If you have to use cycle numbers then there is a very low disease dose

Real time PCR allows us to find out exactly how
much of a disease they have -> used to monitor disease and the effectiveness of drugs

The higher the disease burden, the more fusion transcripts present so the more template cDNA present and the earlier products appear during PCR cycles e.g PML-RAR, BCR-AbI

51
Q

Write about next generation sequencing

A

Making its way to all of the labs
A sequencing method
Allows us to discover mutations
Allows us to see what DNA base pairs are present

52
Q

What are the four types

A

Extraction
Library Prep
Sequencing
Analysis

53
Q

How are malignancies treated
(6)

A

Chemotherapy - usually combination of drugs is used, 7+3 regimen

Epigenetic therapies - demethylating agents and HDACs

Stem cell transplant (younger patients, allogeneic versus autologous)

Radiotherapy

Immunotherapy - stimulate the patients own immune system to mount a response against the malignant cells
- Monoclonal antibodies - examples include Rituximab
- CAR-T cell therapy

Small molecule inhibtiors e.g. Tyrosine Kinase inhibitors - Imatinib (Gleevec), JAK2 inhibitors, BTK inhibitors

54
Q

What is chimeric antigen receptor (CAR) T-cell therapy ?
(6)

A

Used in lymphoid leukaemias and lymphoma patients

Can cure patients

Patients own T cells are taken and genetically engineered to fight their own leukaemia

Patients remain cancer free afterwards

Works well in lymphoid => targets CD19 -> expressed on malignant cells

Phase 1 trials for AML (there isn’t one antigen i.e. CD molecule for myeloid)

55
Q

What is meant be hide and seek

A

Leukaemia cells can move to lymph nodes (lymphoma) or hide in the bone marrow

2.2 Haematology (38 decks)

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