Haem - Leukaemia Flashcards

1
Q

What is leukaemia?

A

Cancer of the stem cells in the bone marrow

Leading to unregulated production of blood cells

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2
Q

What are the different types of leukaemia that affect children?

A

Acute lymphoblastic leukaemia (most common)
Acute myeloid leukaemia
Chronic myeloid leukaemia

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3
Q

What age are most patients affected by leukaemia?

A

60-70 in most patients

Acute lymphoblastic leukaemia most commonly under 5

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4
Q

What are the key differentiating features between different types of leukaemia?

A

AML
Transformation from a myeloproliferative disorder
Auer rods

CML
3 phases including a long chronic phase
Philadelphia chromosome

ALL
Children
Down Syndrome

CLL
Warm haemolytic anaemia
Richter’s transformation cells
Smudge cells

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5
Q

What is the pathophysiology of leukaemia

A

Mutation in precursor cells, leads to excessive production of abnormal white cells

Excessive production can lead to suppression of other cell lines leading to underproduction

This can cause a pancytopenia

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6
Q

How does leukaemia present?

A

Think cytopenia-based symptoms e.g. anaemia, leucopenia and thrombocytopenia

Fatigue
Fever
Pallor
Petechiae
Abnormal bleeding
Lymphadenopathy
Hepatosplenomegaly
Failure to thrive (kids)

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7
Q

What causes petechiae and abnormal bruising?

A

Thrombocytopenia

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8
Q

What are the different types of petechiae?

A

Petechiae
Less than 3mm
Burst capillaries

Purpura
3-10mm

Ecchymosis
> 1cm

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9
Q

What are the differentials for a non-blanching rash?

A

Leukaemia
Meningococcal sepsis
Vasculitis
HSP
Immune thrombocytopenic purpura
Thrombotic thrombocytopenic purpura
Traumatic or mechanical
Non-accidental injury

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10
Q

When is non-accidental injury considered as a differential for a non-blanching rash?

A

Children
Vulnerable adults

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11
Q

How is suspected leukaemia managed?

A

Suspected cancer

FBC within 48 hours

Children or young people with petechiae or hepatosplenomegaly sent for immediate specialist assessment

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12
Q

What investigations are used for leukaemia?

A

FBC - initial investigation

Blood film - abnormal cells and inclusions

LDH - non-specific marker of tissue damage, can be raised even after exercise

Bone marrow biopsy - analyse cells to establish leukaemia

CT and PET scans - stage the condition

Lymph node biopsy - assess abnormal lymph node

Genetic tests - chromosome and DNA changes

Immunophenotyping - look for specific proteins on surface of cells to guide treatment and prognosis

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13
Q

Outline bone marrow biopsy

A

Taken from iliac crest

Requires local anaesthetic

Either aspiration or trephine

Bone marrow aspiration
Liquid sample of cells from bone marrow

Bone marrow trephine
Solid core sample of bone marrow
Better assessment of cells and structure

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14
Q

Outline acute lymphoblastic leukaemia

A

Affects one of the lymphocyte precursor cells

Causes acute proliferation of a single type of lymphocyte usually B-lymphocytes excessive accumulation replaces other cell types in the bone marrow causing pancytopenia

Affects kids under 5 but also older adults

More common with Down’s Syndrome
Can be associated with Philadelphia chromosome (more associated with CML)

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15
Q

Outline chronic lymphocytic leukaemia

A

Slow proliferation of a single type of well-differentiated lymphocyte usually B-lymphocytes

Affects over 60s

Often asymptomatic but can present with infections, anaemia, bleeding and weight loss

Can cause a warm autoimmune haemolytic anaemia

Richter’s transformation - rare transformation of CLL into high-grade B-cell lymphoma

Smear or smudge cells - ruptured white blood cells that occur when preparing the blood film because they are aged or fragile

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16
Q

Outline chronic myeloid leukaemia

A

Chronic phase
Accelerated phase
Blast phase

Chronic phase
Asymptomatic
Patients diagnosed after incidental finding of raised WCC, can last several years before progressing

Accerlated phase
Abnormal blast cells take up high proportion of bone marrow (10-20%)
Patients are symptomatic and develop anaemia, thrombocytopenia and immunodeficiency

Blast phase
Even higher proprtion of blast cells (20%) in blood
Severe symptoms and pancytopenia often fatal

Associated with the Philadelphia chromosome

17
Q

What is the Philadelphia chromosome?

A

Abnormal chromosome 22

Caused by reciprocal translocation between chromosome 9 and 22

Creates an abnormal gene sequence- BCR-ABL1

Codes for tyrosine kinase enzyme that drives proliferation of abnormal cells

18
Q

Outline acute myeloid leukaemia

A

Many subtypes

Can present at any age but normally from middle age onwards

Can be due to transformation from a myeloproliferative disorder e.g. polycythaemia ruby vera or myelofibrosis

19
Q

What do blood films and bone marrow biopsy show in acute myeloid leukaemia?

A

High proportion of blast cells

Auer rods in cytoplasm of blast cells are characteristic of AML

20
Q

What is the general management of leukaemia?

A

Oncology and haematology MDT

Treated with chemotherapy, targeted therapies and other

Targeted therapies
CLL mainly
- Tyrosine kinase inhibitors (ibrutinib)
- Monoclonal antibodies (rituximab, targets B-cells)

Radiotherapy
Bone marrow transplant
Surgery

21
Q

What are the complications of chemotherapy?

A

Failure to treat cancer
Stunted growth and development in kids
Infections due to immunosuppression
Neurotoxicity
Infertility
Secondary malignancy
Cardiotoxicity
Tumour lysis syndrome

22
Q

What is tumour lysis syndrome?

A

Release of chemicals when cells are destroyed by chemotherapy causing :
- High uric acid
- High potassium
- High phosphate
- Low calcium (due to high phosphate)

23
Q

What does tumour lysis syndrome lead to?

A

Uric acid can form crystals in the interstitial space and tubules of kidneys causing AKI

Hyperkalaemia can cause cardiac arrhythmias

Release of cytokines can cause systemic inflammation

24
Q

How is tumour lysis syndrome managed?

A

Very good hydration and urine output prior to chemo required in patients at risk of tumour lysis

Allopurinol or Rasburicase to suppress uric acid levels