Haem - Leukaemia Flashcards

1
Q

Leukaemia - what is the difference between acute leukaemia and chronic leukaemia?

A

Acute leukaemia is a result of impaired cell differentiation, resulting in large numbers of malignant precursor (blast) cells in the bone marrow

Chronic leukaemia is the result of excessive proliferation of mature malignant cells, but cell differentiation is unaffected

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2
Q

Leukaemia - what is the difference between myeloid and lymphocytic leukaemia?

A

The difference between myeloid leukaemia and lymphocytic leukaemia is that myeloid leukaemia commonly arises from a myeloid precursor cell, such as neutrophils

Lymphocytic leukaemia arises from a lymphoid precursor, such as B-cells

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3
Q

What do myeloblasts and lympoblasts differentiate into?

A
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4
Q

What is the general pathophysiology of leukaemia?

A
  1. Leukaemia is a form of cancer of the cells in the bone marrow
  2. A genetic mutation in one of the precursor cells in the bone marrow leads to excessive production of a single type of abnormal white blood cell
  3. The excessive production of a single type of cell can lead to suppression of the other cell lines causing underproduction of other cell types
  4. This results in a pancytopenia, which is a combination of low red blood cells (anaemia), white blood cells (leukopenia) and platelets (thrombocytopenia)
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5
Q

What is the mnemonic to remember the progressive ages of the different leukaemias?

A

ALL CeLLmates have CoMmon AMbitions” to remember the progressive ages of the different leukaemia from 45-75 in steps of 10 years

Remember that ALL (the first in the mnemonic) most commonly affects children under 5 years.

  • Under 5 and over 45 – acute lymphoblastic leukaemia (ALL)
  • Over 55 – chronic lymphocytic leukaemia (CeLLmates)
  • Over 65 – chronic myeloid leukaemia (CoMmon)
  • Over 75 – acute myeloid leukaemia (AMbitions)
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6
Q

What are differential facts to help differentiate between the 4 leukaemia’s?

A

Acute lymphoblastic leukaemia: Most common leukaemia in children. Associated with Down syndrome

Chronic lymphocytic leukaemia: Most common leukaemia in adults overall. Associated with warm haemolytic anaemia, Richter’s transformation into lymphoma and smudge / smear cells

Chronic myeloid leukaemia: Has three phases including a 5 year “asymptomatic chronic phase”. Associated with the Philadelphia chromosome

Acute myeloid leukaemia: Most common acute adult leukaemia. It can be the result of a transformation from a myeloproliferative disorder. Associated with Auer rods

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7
Q

Differential diagnosis of Petechiae?

A

One of the key presenting features of leukaemia is bleeding under the skin leading to bruising and petechiae. This is caused by thrombocytopenia (low platelets)

  • Leukaemia
  • Meningococcal septicaemia
  • Vasculitis
  • Henoch-Schonlein Purpura (HSP)
  • Idiopathic Thrombocytopenia Purpura (ITP)
  • Non-accidental injury
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8
Q

Acute myeloid leukaemia (AML) - what is it?

A

Most common acute leukaemia in adults

May occur as primary disease or secondary transformation of a myeloproliferative disorder

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9
Q

AML - what are the clinical features?

A

Bone marrow failure symptoms:

Fatigue - due to anaemia

Easier bleeding - due to thrombocytopenia

More infections - due to leukopenia

Pain and tenderness in bones

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10
Q

AML - what are the signs of infiltration?

A

Hepatomegaly

Splenomegaly

Gum hypertrophy

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11
Q

AML - investigations, and diagnostic finding?

A

Blood film:

Will show high proportions of blast cells and Auer rods

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12
Q

AML - management?

A

Chemotherapy regimens

Bone marrow transplant

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13
Q

AML - prognosis?

A

Death occurs within 2 months without treatment; however, it is still poor in those who undergo treatment, with a 20% 3-year survival rate

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14
Q

Acute Lymphoblastic Leukaemia (ALL) - what is it?

A

ALL is the most common cancer of childhood

Caused by the abnormal clinical proliferation of lymphoid progenitor cells

These lymphoid precursors infiltrate normal haematopoietic cells of the bone marrow and other organs of the body

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15
Q

ALL - what is the peak age incidence?

A

2-5 years old in children

Adults over age of 45

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16
Q

ALL - what cell most commonly proliferates?

A

B-lymphocytes

17
Q

ALL - what genetic condition is is commonly associated with?

A

Downs syndrome

18
Q

ALL - what chromosome and translocation is it associated with?

A

It is associated with the Philadelphia chromosome (t(9:22) translocation) in 30% of adults and 3-5% of children with ALL

19
Q

ALL - what are some clinical features?

A

Bone marrow failure symptoms of anaemia including fatigue, abnormal bleeding/bruising caused by low platelets, and infections caused by low white cells

Bone pain caused by infiltration

20
Q

ALL - what are some signs?

A

Painless lymphadenopathy

Hepatosplenomegaly

CNS involvement (e.g. cranial nerve palsies, meningism)

Testicular infiltration (resulting in painless unilateral testicular enlargement)

21
Q

ALL - how do you diagnose?

A

Bone marrow analysis and blood film - blast cells

Blood results - leukocytosis

22
Q

ALL - management?

A

Chemotherapy regimens

23
Q

ALL - prognosis in children?

A

Cure rate of 70-90%

24
Q

Chronic myeloid leukaemia (CML) - what is it?

A

It is a leukaemia with three phases:

  1. Chronic phase - lasts around 5 years, often asymptomatic, patients diagnosed incidentally with raised WCC
  2. Accelerated phase - abnormal blast cells take up high proportion of the cells in BM and blood (10-20%), more symptomatic, develop anaemia and thrombocytopenia
  3. Blast phase - even higher proportion of blast cells and blood (>30%)
25
Q

CML - what is the chromosomal defect present in more than 95% of patients?

A

Philadelphia chromosome, translocation between the long arm of chromosome 9 and 22

26
Q

CML - common age of presentation?

A

60-70 years

27
Q

CML - what are the clinnical features?

A
  • weight loss and sweating are common
  • anaemia: lethargy
  • massive splenomegaly → abdo discomfort
  • bleeding - due to thrombocytopenia
    • gout
      *
28
Q

CML - diagnosis?

A

Leukocytosis - raised myeloid cell which include, neutrophils, monocytes, basophils, eosinophils

Granulocytes at different stages of maturation +/- thrombocytosis

29
Q

CML - management?

A

1st line - Imatinib, very high response rate in chronic phase

Hydroxyurea

Interferon-alpha

allogenic BM transplant

30
Q

Chronic lymphocytic leukaemia (CLL) - what is it?

A

Chronic lymphocytic leukaemia (CLL) is caused by a chronic monoclonal proliferation of well-differentiated lymphocytes which are almost always B-cells (99%)

It is the most common form of leukaemia seen in adults

31
Q

CLL - how does it typically present?

A

Typically presents asymptomatically

32
Q

CLL - what are the clinical features?

A

Non-tender lymphadenopathy (may be more marked than CML)

Hepatosplenomegaly

B symptoms - weight loss, night sweats, and fever

BM failure features - bleeding, infections, anaemia (less common than in acute Leukaemias)

33
Q

CLL - investigations?

A

FBC - lymphocytosis, anaemia

Blood film - smudge cells (also known as smear cells)

Immunophenotyping - key Ix

34
Q

CLL - characteristic finding on blood film?

A

Smudge or Smear cells

35
Q

CLL - what are three complications?

A
  • hypogammaglobulinaemia leading to recurrent infections
  • warm autoimmune haemolytic anaemia in 10-15% of patients
  • transformation to high-grade lymphoma (Richter’s transformation)
36
Q

CLL - what is Richter’s transformation?

A

CLL can transform into high-grade lymphoma

37
Q

CLL - when does Richter’s transformation occur?

A

Occurs when leukaemia cells enter the lymph node and change into a high-grade, fast-growing non-Hodgkin’s lymphoma

38
Q

CLL - how does Richter’s transformation present?

A

Presents suddenly

39
Q

CLL - what is Richter’s transformation indicated by?

A
  • lymph node swelling
  • fever without infection
  • weight loss
  • night sweats
  • nausea
    • abdominal pain