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SH - Haematology > Haematological malignancies > Flashcards

Haematological malignancies Flashcards

1
Q

What would the growth fraction be in mature differentiated haematological malignancies

A

low

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2
Q

What would the growth fraction be in haematological malignancies involving the primitive stem cells

A

high

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3
Q

haematological malignancies are more common in young/elderly patients

A

elderly exception being ALL (children) and hodgkin lymphoma which affects young people

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4
Q

haematological malignancies are more common in male/females

A

males

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5
Q

how are haematological malignancies classified

A

Haematological malignancies are classified according to their:

  • Presentation:
    • Acute
    • Chronic
  • Tissue distribution:
    • Leukaemia (blood and marrow)
    • Lymphoma (lymph nodes and other tissues)
  • Cells of origin:
    • Myeloid
    • Lymphoid
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6
Q

define Acute Lymphoblastic leukaemia (ALL):

A
  • Acute Lymphoblastic leukaemia (ALL): the mutation occurs in the lymphoid progenitor, which means the stem cell can proliferate but not differentiate resulting in the production of uniform useless lymphoid progenitor cells.
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7
Q

define Acute Myeloid Leukaemia (AML)

A
  • Acute Myeloid Leukaemia (AML): the mutation occurs in the myeloid progenitor, which means the stem cell can proliferate but not differentiate resulting in the production of uniform useless myeloid progenitor cells.
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8
Q

define chronic myeloproliferative diseases and give examples

A

chronic myeloproliferative diseases: a mutation has occurred in the myeloid progenitor stem cell which enables it to grow and differentiate resulting in the accumulation of myeloid end products (neutrophils, eosinophils etc). An example of this is:

  • Chronic myeloid leukaemia (CML)
  • Myelodysplastic syndromes (MDS)
  • Myeloproliferative neoplasms (MPN)
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9
Q

define chronic lymphoproliferative diseases and give examples

A

The chronic lymphoproliferative diseases: a mutation has occurred in the lymphoid progenitor cell that has allowed it to grow and differentiate resulting in the accumulation of lymphoid progenitor cells, which can be categorised into one of the following groups:

  • Chronic lymphocytic leukaemia (CLL)
  • Lymphomas:
    • Non-Hodgkin lymphoma (NHL): describes the accumulation of cells occurs in the lymph nodes with an even distribution
    • Hodgkin lymphoma: describes when the accumulation of cells is unevenly distributes between the different lymph nodes
  • Multiple myeloma (MM)
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10
Q

what is the difference between hodgkin and non-hodgkin lymphoma

A

hodgkin lymphoma - uneven distribution of cells in the lymph tissue

Non-hodkin = even distribution in the lymph tissue

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11
Q

how to acute leukaemias present

A

pancytopenia (anaemia, low WBC, low platelets i.e. thrombocytopenia)

  • Anaemia:
    • Pallor
    • Abnormal bleeding
    • Fatigue
  • Thrombocytopenic bleeding:
    • Petechiae
    • Purpura
    • Abnormal bruising
  • Infection because of neutropenia (predominantly bacterial and fungal)
    • Fever
  • Failure to thrive (children)

Overspill of cells - splenomegaly and hepatomegaly

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12
Q

how should you investigate for acute leukaemia

A

FBC

blood film

lactate dehydrogenase

bone marrow biopsy (Blasts >20% of marrow cells in acute leukaemia)

Cytogenics and immunophenotyping

lumbar puncture if NS involvement

targeted molecular genetics

CXR

lymph node biopsy

CT, MRI, PET

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13
Q

what is the most common type of lymphocyte involved in ALL

A

B lymphocyte

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14
Q

what genetic condition is ALL associated with

A

Downs syndrome

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15
Q

What are the characteristics of ALL blast cells

A

large

express CD19 and CD34

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16
Q

how would you treat ALL

A
  • Standard treatment:
    • Induction chemotherapy to obtain remission
    • Consolidation therapy
    • CNS directed treatment
    • Maintenance treatment for 18 months
    • Stem cell transplantation (if high risk)
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17
Q

What are poor prognostic factors for ALL

A
  • Increasing age
  • Increased white cell count
  • Cytogenics/molecular genetics:
    • T(9;22), t(4;11)
  • Slow or poor response to treatment
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18
Q

What is the prognosis of ALL in adults and children

A
  • Adults with ALL:
    • Complete remission rate = 90%
    • Leukaemia free survival at 5years = 30-35%
  • Children:
    • 5 years overall survival = 90%
    • Poor risk factor patients 5 year survival = 45%
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19
Q

what is the most common acute leukaemia in adults

A

acute myeloblastic leukaemia

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20
Q

what are the treatment options for AML

A

Treatment for AML consists of:

  • Supportive care
  • Anti-leukaemia chemotherapy:
    • Remission induction – to achieve remission (1-2 cycles)
      • Normal blood counts and less than 5% blasts
      • Daunorubicin and cytosine arabinoside (DA)
      • Gemtuzumab ozogamicin
      • Gemtuzumab ozogamicin
      • CPX-351
    • Consolidation (1-3 cycles)
      • High dose cytosine arbinoside
      • Allogenic stem cell transplantation – to consolidate remission/potential cure
  • Maintenance therapy:
    • Midostaurin (FLT3 inhibitor)
    • Oral azacytidine (hypomethylating agent)
  • All-trans retinoic acid (ATRA) and arsenic trioxide (ATO) in low risk Acute Promyelocytic Leukaemia – ‘chemo-free’ – high cure rate ~90%
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21
Q

what causes chronic myeloid leukaemia

A

philadelphia chromosome (shortened 22 chromosome with translocation of chromosome 9) which results in the BCR-ABL protein that has TK activity and promoted proliferation, differentiation and survival of cells

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22
Q

what are the 3 phases of CML

A
  • A chronic phase: in which the disease is responsive to treatment and is easily controlled. This typically lasts around 5 years. This phase is often asymptomatic and patients are diagnosed incidentally with a raised white cell count.
  • An accelerated phase occurs where the abnormal blast cells take up a high proportion of the cells in the bone marrow and blood (10-20%). In the accelerated phase patients become more symptomatic, develop anaemia and thrombocytopenia and become immunocompromised.
  • The blast phase follows the accelerated phase and involves an even high proportion of blast cells and blood (>30%). This phase has severe symptoms and pancytopenia. It is often fatal.
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23
Q

what is a tell tale sign in CML

A

massive splenomegaly

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24
Q

what are the treatment options for CML

A
  • Tyrosine kinase inhibitors (first line):
    • Imatinib (Glivec)
    • Dasatinib (Sprycel)
    • Nilotinib (Tasigna)
    • Busitinib
    • Ponatinib
  • When TKI fail – allogenic transplantation
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25
Q

what laboratory features would you se ein CML

A
  • High WCC
  • High platelet count
  • Anaemia
  • Blood film shows all stages of white cell differentiation with increased basophils
  • Bone marrow is hypercellular
  • Bone marrow and blood cells contain the Philadelphia chromosome
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26
Q

compare acute and chronic leukaemia in terms of differentiation, bone marrow failure, fatality, curability

A
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27
Q

what type of haematological malignancy is polycythaemia rubera vera

A

Myeloprolierative neoplasm

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28
Q

what are some clinical features of Polycythaemia vera

A
  • Headaches
  • Itch
  • Vascular occlusion
  • Thrombosis
  • TIA, stroke
  • Splenomegaly
29
Q

what are some laboratory feature s of polycythaemia vera

A
  • A raised haemoglobin concentration and haematocrit
  • A tendency to also have a raised WCC and platelet count
  • Raised uric acid
  • True increase in red cell mass when blood volume is measured
30
Q

what is the management options for polycthaemia vera

A
  • Venesection to keep haematocrit below 0.45 in men and 0.43 in women
  • Aspirin
  • Hydrocarbamid (HC)/alpha interferon
  • Ruxolitinib (JAK2 inhibitor) in HC failures with systemic symptoms
31
Q

what are the complications of polycythaemia vera

A
  • Stroke and other arterial/venous thrombosis if poorly controlled
  • Bone marrow failure form the development of secondary myelofibrosis
  • Transformation to AML
32
Q

what mutations are common in essential thrombocythemia

A

JAK2 (50%) and CALR (25%)

33
Q

what are complications of essential thrombocythemia

A

arterial and venous thromboses

digital ischaemia

gout

headache

progress to myelofibrosis or AML

34
Q

what is the predominant feature of essential thrombocythemia

A

raised platelet count

35
Q

what is a common mutation in myeloproliferative neoplasms

A

V617F in JAK2 protein

36
Q

what cell type is most commonly implicated in CLL

A

B lymphocyte

37
Q

what is the commenest leukaemia worldwide

38
Q

CLL is more common in men/women

39
Q

how does CLL present

A

often asymptomatic

Frequent findings are:

  • Bone marrow failure (anaemia, thrombocytopenia)
  • Lymphadenopathy
  • Splenomegaly (30%)
  • Fever and night sweats (<25%)

Less common findings:

  • Hepatomegaly
  • Infections
  • Weight loss

CLL can cause:

  • Immune paresis (loss of normal immunoglobulin production)
  • Haemolytic anaemia
40
Q

what are the stages of CLL and their prognosis

A

CLL staging:

  • Clinical stage A:
    • <3 lymph node areas
    • Median survival is same as age matched controls
  • Clinical stage B:
    • 3 or more lymph node areas
    • Median survival is ~8 years
  • Clinical stage C
    • Stage B plus anaemia or thrombocytopenia
    • Median survival is ~6 years
41
Q

what is the management options for CLL

A
  • Often nothing – ‘watch and wait’
  • Cytotoxic chemotherapy e.g. fludarabine, bendamustine
  • Monoclonal antibodies e.g. Rituximab, obinatuzumab
  • Novel agents:
    • Bruton tyrosine kinase inhibitor e.g. ibrutinib
    • PI3K inhibitor
    • BCL-2 inhibitor e.g. venetoclax
42
Q

what are the indications for treatment in CLL

A
  • Progressive bone marrow failure
  • Massive lymphadenopathy
  • Progressive splenomegaly
  • Lymphocyte doubling time <6 months or <50% increase over 2 months
  • Systemic symptoms
  • Autoimmune cytopenias
43
Q

what are poor prognostic factors for CLL

A
  • Advanced disease (stage B or C)
  • Atypical lymphocyte morphology
  • Rapid lymphocyte doubling time (<12 months)
  • CD38 expression
  • Loss/mutation p53; del 11q23 (ATM gene)
  • Unmutated IgVH gene status
44
Q

what cell type do lymphomas most commonly originate from

A

B cell lineage

45
Q

what are systemic B symptoms

A

Fever

night sweats

loss of weight (>10% in 6 months)

Pruritis

Fatigue

46
Q

describe the stages of lymphoma

A

The Ann Arbor staging system is used for both Hodgkins and non-Hodgkins lymphoma. The system puts importance on whether the affected nodes are above or below the diaphragm. A simplified version is:

  • Stage 1: Confined to one region of lymph nodes.
  • Stage 2: In more than one region but on the same side of the diaphragm (either above or below).
  • Stage 3: Affects lymph nodes both above and below the diaphragm.
  • Stage 4: Widespread involvement including non-lymphatic organs such as the lungs or liver.

Also B is added to stage if systemic B symptoms accompany this

47
Q

a localised and painful lymphadenopathy suggests

A

bacterial infection in draining site

48
Q

a localised and painless lymphadenopathy could be

A
  • Rare infections such as catch scratch fever, TB
  • Metastatic carcinoma from draining site – hard
  • Lymphoma – rubbery
  • Reactive – no cause identified
49
Q

a generalised and painful lymphadenopathy suggests

A

viral infection

50
Q

a generalised and painless lymphadenopathy suggests

A
  • Lymphoma
  • Leukaemia
  • Connective tissue diseases, sarcoidosis
  • Reactive, no cause identified
  • Drugs
51
Q

what si the typical age of presentation for Hodgkin lymphoma

A

early adult (20 years) and elderly (75 years_)

52
Q

how would you manage hodkin lymphoma

A

chemotherapy +/- radiotherapy

Other therapies include monoclonal antibodies and immunotherapy

53
Q

how is hodkin lymphoma distributed

54
Q

how is non-hodkin lymphoma distributed

55
Q

what is the most common lymphoid malignancy

A

non-hodkin lymphoma

56
Q

what are the two grades of non-hodkin lymphoma

A

low and high grade

57
Q

compare low and high grade non-hodgkin lymphoma

A
  • Low grade:
    • Indolent, often asymptomatic
    • Responds to chemotherapy but incurable
  • High grade:
    • Aggressive fast growing
    • Requires combination chemotherapy
    • Can be cured
58
Q

what are the two most common types of non-hodkin lymphoma

A
  • Diffuse large B cell lymphoma:
    • Commonest subtype of lymphoma (of any kind)
    • High-grade lymphoma
  • Follicular lymphoma:
    • 2nd commonest subtype of lymphoma
    • Low-grade lymphoma
    • Like CLL, leave alone if it isn’t causing problems – ‘watch and wait’
59
Q

how are the common non-hodkin lymphomas managed

A

combination chemotherapy (chemo +monoclonal antibody typically CD20)

60
Q

define myeloma

A

a cancer of the plasma cells resulting in large quantities of a single type of antibody (called a paraprotein)

61
Q

what population is myeloma most common in

A

elderly black population

62
Q

define monoclonal gammopathy of undetermined significance

A

Monoclonal gammopathy of undetermined significance (MGUS) is where there is an excess of a single type of antibody or antibody components without other features of myeloma or cancer. This is often an incidental finding in an otherwise healthy person and as the name suggests the significance is unclear. It may progress to myeloma and patients are often followed up routinely to monitor for progression.

63
Q

what type of haematological cancer would you see an IgM antibody being elevated

A

lymphoma - do not get IgM antibody increase in myeloma

64
Q

what are the clinical features of myeloma

A

CRAB:

The four key features of myeloma to remember for exams (CRAB):

  • C – Calcium (elevated)
  • R – Renal failure
  • A – Anaemia (normocytic, normochromic) from replacement of bone marrow.
  • B – Bone lesions/pain
65
Q

what are the risk factors for myeloma

A

Risk Factors

  • Older age
  • Male
  • Black African ethnicity
  • Family history
  • Obesity
66
Q

what blood tests would you perform in someone that you suspected to have myeloma

A
  • FBC (low white blood cell count in myeloma)
  • Calcium (raised in myeloma)
  • ESR (raised in myeloma)
  • Plasma viscosity (raised in myeloma)
67
Q

after performing the initial investigations for myeloma (FBC, ESR, plasma viscosity and calcium) what tests would you perform next

A

You can remember these with the mnemonic “BLIP”. You cannot exclude myeloma with just one investigation.

  • B – Bence–Jones protein (superseded by light chain analysis)
    • Bence jones protein is part of the light chain subunit of antibodies
  • L – Serum‑free Light‑chain assay:
    • Assesses imbalance/excess of lift chains
  • I – Serum Immunoglobulins
    • Measures Ig subclasses by heavy chain/Fc portion
  • P – Serum Protein electrophoresis
    • Assesses antibody diversity, identifies paraprotein

Bone marrow biopsy is then required to confirm diagnosis

68
Q

what findings would you see in an Xray of someone with myeloma

A

lytic bone lesions - raindrop skull

69
Q

how would you manage someone with myeloma

A

First line treatment usually involves a combination of chemotherapy with:

  • Proteasome inhibitors (Carflizomib, Bortezomid)
  • Immunomodulator drugs (Thalidomide, lenalidomide, pomalidomide)
  • Monoclonal antibodies (Dexamethasone)

Stem cell transplantation can be used as part of a clinical trial where patients are suitable.

Management Myeloma Bone Disease

  • Myeloma bone disease can be improved using bisphosphonates. These suppress osteoclast activity.
  • Radiotherapy to bone lesions can improve bone pain.
  • Orthopaedic surgery can stabilise bones (e.g. by inserting a prophylactic intramedullary rod) or treat fractures.
  • Cement augmentation involves injecting cement into vertebral fractures or lesions and can improve spine stability and pain

SH - Haematology (6 decks)

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