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Case 20: Haematological malignancies > Leukaemia > Flashcards

Leukaemia Flashcards

1
Q

What is leukaemia?

A

Leukaemia is a progressive, malignant disease of the blood-forming organs, characterised by distorted proliferation and development of leukocytes and their precursors in the blood and bone marrow.

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2
Q

How can leukaemia be classified?

A

Acute or chronic, according to the degree of cell differentiation (not the duration of disease).

Myelogenous or lymphocytic, according to the predominant type of cell involved (myeloid or lymphoid)..

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3
Q

Aetiology of leukaemia

A

The exact cause is unknown

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4
Q

Signs and symptoms of leukaemia

A 
Fatigue
Weight loss
Fever
Pallor
Ecchymoses
Petechiae
Dyspnoea
Dizziness
Palpitations
Bleeding
Recurrent infections
Each sub-type has its distinguishing features.
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5
Q

Which tests are essential for the definitive diagnosis of leukaemia?

A

Definitive diagnoses often require bone marrow biopsy and/or blood analysis.

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6
Q

Types of leukaemia

A 
Acute lymphocytic leukaemia (ALL) 
Chronic lymphocytic leukaemia (CLL) 
Acute myelogenous leukaemia (AML)
Chronic myelogenous leukaemia (CML) 
Hair cell leukaemia (HCL)
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7
Q

What is ALL?

A

A malignant clonal disease that develops when a B/T-precursor-stage lymphoid progenitor cell becomes genetically altered through somatic changes and undergoes uncontrolled proliferation.

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8
Q

Which type of leukaemia is the most common in paediatrics?

A

Acute lymphocytic leukaemia (ALL) is the most common leukaemia in paediatrics, accounting for up to 80% of leukaemias in this group and 20% of leukaemias in adults

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9
Q

Signs and symptoms of ALL

A 
Most cases present with signs and symptoms associated with cytopenias. 
Symptoms of anaemia include:
fatigue
weakness
shortness of breath
poor concentration
dizziness or feeling lightheaded
cold hands and feet
Symptoms of leukopenia include:
frequent infections
fever
Symptoms of thrombocytopenia include:
bleeding and bruising easily
difficulty with stopping bleeding
internal bleeding
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10
Q

What is the initial cause of seeking medical attention in a patient with ALL?

A

Enlarged lymph nodes

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11
Q

What is CLL?

A

A cancer of B lymphocytes. Failure of B lymphocytes to undergo maturation and full differentiation leads to a monoclonal population of dysfunctional but self-renewing B lymphocytes.

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12
Q

Which organs does CLL infiltrate most commonly?

A

These lymphocytes can infiltrate lymphatic tissues and haematopoietic organs such as the liver, spleen and bone marrow.

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13
Q

What is a key risk factor in the development of CLL?

A

Age over 60 years

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14
Q

What are the key diagnostic factors in a patient with CLL?

A

Lymphadenopathy, splenomegaly (in 50% of cases), and shortness of breath and fatigue

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15
Q

Which tests are essential for the definitive diagnosis of CLL?

A

It is diagnosed by FBC with differential, blood smear showing smudge cells, and flow cytometry.
Most cases are diagnosed on a routine FBC for an unrelated reason.

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16
Q

What is AML?

A

The clonal expansion of myeloid blasts in the bone marrow, peripheral blood, or extra-medullary tissues.

17
Q

Who is a typical patient with AML?

A

Occurs predominantly in older adults.

18
Q

Common signs and symptoms of AML

A

Common findings are pallor, ecchymoses and petechiae.

19
Q

What is acute promyelocytic leukaemia?

A

Acute promyelocytic leukaemia (APML) is a form of acute myelogenous leukaemia with distinct cytological and clinical features, which has specific management.

20
Q

What is CML?

A

A malignant clonal disorder of the haematopoietic stem cell that results in marked myeloid hyperplasia of the bone marrow.

21
Q

What are the other names for CML?

A

May also be called chronic granulocytic leukaemia, chronic myelocytic leukaemia, or chronic myeloid leukaemia.

22
Q

Who is a typical patient with CML?

A

Median age at presentation is around 53 years, and the only known risk is exposure to ionising radiation.

23
Q

Common signs and symptoms of CML?

A

Many cases are asymptomatic but possible symptoms include fever, chills, malaise, weight loss, and night sweats. Around 75% of patients have splenomegaly.

24
Q

Which tests aids the definitive diagnosis of CML?

A

All patients will have an elevated WBC count.
Diagnosis should be confirmed by the presence of the Philadelphia chromosome and/or the BCR-ABL rearrangement in peripheral blood or bone marrow cells.

25
Q

What is the blast crisis?

A

Blast crisis refers to the transformation of chronic myelogenous leukaemia (CML) from the chronic or accelerated phase to the blast phase.

26
Q

Common signs and symptoms of blast-phase CML

A

Anaemia, infections, abnormal bleeding, bone pain, and constitutional symptoms (night sweats, weight loss, fever) are common presenting complaints of blast-phase CML.

27
Q

What is the goal in a patient with blast-phase CML?

A

The goal is to achieve a complete haematological remission or at least return to the chronic phase in order to perform stem cell transplant.

28
Q

What is HCL?

A

A B-cell malignancy characterised commonly by symptoms of fatigue, a markedly enlarged spleen and a distinctive histological appearance on peripheral blood smear and bone marrow biopsy.

29
Q

What are the most important characteristics of HCL?

A

Hairy cell leukaemia (HCL) is characterised by B-cells with delicate cytoplasmic projections resembling hair.
There is marked geographic variation in the frequency of HCL (also known as leukaemic reticuloendotheliosis) and it is relatively uncommon.

30
Q

Who is a typical patient with HCL?

A

The median age of patients is 50 years and HCL affects more men than women.
It often presents with abdominal discomfort or fullness.

31
Q

What is the abdominal discomfort in HCL attributed to?

A

This is attributed to splenomegaly, which is present in around 60% to 90% of patients.

32
Q

Is HCL curable?

A

The disease is not curable. However, it is highly responsive to therapy and maybe managed successfully for a decade or more.

33
Q

How can leukaemia cause pancytopenia?

A

Leukaemias may cause pancytopenia through decreased production, or increased destruction or sequestration, of blood cells.

34
Q

The main complication of neutropenia

A

Neutropenia contributes to some of the typical symptoms of leukaemia, including recurrent infections.

Case 20: Haematological malignancies (4 decks)

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