Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Haematology > Haematological Malignancy > Flashcards

Haematological Malignancy Flashcards

1
Q

What is Acute Lymphoblastic Leukaemia a malignancy of?

A

The lymphoid precursor cells in the bone marrow. (lymphoblasts)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

In which age group is this cancer most common?

A

Most common childhood cancer, peaks around 2 - 4 years.

Can also affect adults over 45 yrs.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What condition does ALL have an association with?

A

Downs syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What chromosome is ALL associated with?

A

The Philadelphia Chromosome - A translocation of chromosome 9 and 22.

30% of adults.
5% of children.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Which cell type is produced in ALL?

A

Single type of cell
- Usually B-Lymphocyte

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is it called when other cell types in the bone marrow are replaced in ALL?

A

Pancytopenia
- Excessive proliferation of B-Lymphocytes causes them to replace the other cell types being created in the bone marrow, leading to a pancytopenia.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What are the two most common subtypes of ALL?

A
  • B-cell (80%)
  • T-cell
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What are the typical symptoms for a patient with ALL?

A

The symptoms associated with Marrow failure include;
- Fatigue (due to anaemia)
- Abnormal bleeding/bruising (low platelets)
- and infections (Low white cells)

Symptoms from organ infiltration, such as bone pain.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What are some clinical signs of ALL?

A
  • Painless Lymphadenopathy
  • Hepatosplenomegaly
  • CNS involvement (cranial nerve palsies, meningism) (very common in ALL in contrast to AML)
  • Testicular infiltration (Painless unilateral testicular enlargement)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What are some differentials for ALL?

A

Aplastic anaemia
- Both cause thrombocytopenia
- However in Aplastic anaemia the bone marrow is HYPOcellular.

CLL
- Both Lymphoid malignancies
- Differ in terms of cell maturity and clinical features.
- CLL typically affects older adults.

Non-Hodgkin Lymphoma
- The lymph nodes are the typical sites of involvement rather than the Bone marrow in ALL.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What does an FBC and Blood film show in ALL?

A

FBC - Leucocytosis.
Blood film - Blast cells.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What type of investigation is done to differentiate the cell of origin in ALL?

A

Immunophenotyping.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What does Periodic acid-schiff (PAS) stain for in ALL?

A

Stains for carbohydrate material

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

How is ALL usually treated?

A
  • Combination chemotherapy.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is given to all patients with ALL?

A

CNS prophylactic agents.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What are some potential complications of ALL?

A
  • Infections
  • Bleeding
  • CNS involvement - ALL can infiltrate the CNS, leading to neurological symptoms. Intrathecal chemotherapy and CNS prophylaxis are employed to prevent and Treat CNS involvement.
  • Chemotherapy-related toxicities.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What is the prognosis in children with ALL on chemotherapy alone?

A

70-90% cure rate.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What cell type is proliferating in Acute Myeloid Leukaemia?

A

Myeloid Precursor cells (the progenitor for Granulocytes, Monocytes, erythrocytes or platelets.)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

How can AML arise and who does it typically affect?

A

Typically affects older adults (>60).

  • Can arise as progression from Myelodysplastic syndromes.
  • Can also Arise De-novo.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What are the clinical features of AML?

A
  • Similar to AML causes Bone Marrow Failure.
  • Subtypes of AML may have characteristic presentations… (Coagulation defects / DIC in acute promyelocytic leukeamia)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What are some signs and Symptoms of AML?

A

Signs of tissue infiltration;
- Hepatomegaly
- Splenomegaly
- Gum Hypertrophy
Bone marrow failure;
- Anaemia, Bleeding, Infections.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What are some differential diagnoses in AML?

A

Myelodysplastic syndromes (MDS)
- share similarities in bone marrow abnormalities and cytopenias.
- MDS typically have mild cytopenias and dysplastic features.
- AML presents with rapid proliferation of blasts in the bone marrow.

Acute Lymphoblastic Leukaemia (ALL)
- AML and ALL both involve Leukaemia,
- Differ in type of blood cell affected.

23
Q

What do blood tests show in AML?

A
  • Acute proliferation of Blast cells in either peripheral blood or bone marrow.
  • AUER RODS (blast cells have rods inside them names AUER Rods)
24
Q

What would a bone marrow biopsy show in AML?

A
  • Hypercellular marrow
  • The presence of blasts (usually >50%)
  • Sometimes Auer rods.
25
Q

What other tests can be used in AML?

A

Cytochemistry - “Sudan black stain” (stains lipid material in myoblasts)

Cytogenetics - translocation t(15,17) involving the RARA gene. (confirms acute promyelocytic leukaemia)

26
Q

What is the management of AML?

A

Multi-agent chemotherapy
- Between 2-4 cycles of chemo
- Prolonged Hospitalisation
- Targeted tx in subtypes.
- Hickman line used to provide long-term central venous access

27
Q

What are the complications for AML?

A

Same as ALL

  • Infections
  • Bleeding
  • CNS involvement - ALL can infiltrate the CNS, leading to neurological symptoms. Intrathecal chemotherapy and CNS prophylaxis are employed to prevent and Treat CNS involvement.
  • Chemotherapy-related toxicities.
28
Q

When would death occur without Tx in AML?

A

Typically within 2 months
(prognosis is still poor in those who undergo Tx)
- 3 yr survival only 20%

29
Q

What cells does Chronic Myeloid Leukaemia (CML) have an increased Proliferation of?

A

Myeloid cells (granulocytes and their precursors, other lineages (platelets))

30
Q

What is the Cytogenetic change characteristic of CML?

A

The cytogenetic change that is characteristic of CML is thePhiladelphia chromosome,which is a translocation of genes betweenchromosome 9 and 22: it is at(9:22) translocation

31
Q

What is the name of the new gene produced in CML as a result of the Philadelphia chromosome?

A

BCR-ABL1
- The gene produced is a tyrosine kinase which causes abnormal phosphorylation leading to the haematological changes in CML.

32
Q

What are the 3 typical phases in CML?

A
  • The Chronic phase
  • The Accelerated phase
  • The Blast Phase
33
Q

What is the Chronic phase in CML?

A

Thechronic phasecan last around 5 years, is often asymptomatic and patients are diagnosed incidentally with a raised white cell count.

34
Q

What is the Accelerated phase in CML?

A

Theaccelerated phaseoccurs where the abnormal blast cells take up a high proportion of the cells in the bone marrow and blood (10-20%)
- Patients become more Symptomatic
- Develop anaemia and thrombocytopenia

35
Q

What is the Blast phase in CML?

A

Theblast phasefollows the accelerated phase and involves an even high proportion of blast cells (>30%)
- This phase has severe symptoms and pancytopenia - it is often fatal.

36
Q

What are common signs in CML?

A
  • Massive Splenomegaly (>75% of patients)
  • Bleeding (due to thrombocytopenia)
  • Gout (caused by high cellular turnover causing high urate)
37
Q

What constitutional symptoms can CML present with?

A
  • Wt loss
  • Tiredness
  • Fever
  • Sweating (night sweats)
38
Q

What symptoms occur as a result of the hyperleukocytosis in CML?

A
  • Visual disturbance
  • Confusion
  • Priapism
  • Deafness
39
Q

What would a full blood count show in CML?

A

Normal or decreased Hb.
Increased White blood cells.
Platelets low, normal or raised.

40
Q

What is seen on a blood film of a patient with CML?

A

Neutrophilia + Myeloid blast cells.

41
Q

What test can be used to look for the Philadelphia chromosome in patients with CML?

A

FISH (Fluorescence In Situ Hybridization

42
Q

How is CML treated?

A

Tyrosine Kinase Inhibitors - improve prognosis of disease and reduce incidence of transformation to the acute blast crisis phase.

43
Q

What are some examples of Tyrosine Kinase inhibitors?

A
  • Imatinib
44
Q

What does Hydroxycarbamide do in CML?

A

Used to help normilise the blood count while awaiting genetic test results and helps reduce splenomegaly.

45
Q

What cell type is uncontrollably proliferated in Chronic Lymphocytic Leukaemia?

A

Occurs where there is chronic proliferation of a single type of well differentiated lymphocyte, usually B-Lymphocyte.

46
Q

Who does CLL usually effect?

A

Adults over 55 yrs of age

47
Q

What are the clinical features of CLL?

A

Often asymptomatic.
- Can present with infections, anaemia, bleeding and wt loss.
- Can cause warm autoimmune haemolytic anaemia.

48
Q

What would an FBC show in a patient with CLL?

A

Hb Normal or low.
Increased White cells. (may be very high)
platelets normal or low.

49
Q

What does A blood film show in CLL?

A

Increased Lymphocytes.
- May also show “smear” or “smudge” cells - these occur during the process of preparing the blood film where aged or fragile white blood cells rupture and leave a smudge on the film.

50
Q

What will Immunophenotyping show in CLL patients?

A

Mainly CD19/20 and CD5 B cells which may weakly express surface immunoglobulins.

51
Q

What is the typical management of CLL?

A
  • Depends on stage of the disease
  • Chemotherapeutic interventions in early-stage disease is not usually indicated.
  • watchful waiting.
52
Q

What is a complication of CLL?

A

Transformation into high-grade lymphoma.

53
Q
A

Haematology (10 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions