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Year 4 - Cancer Care SL > Leukaemias > Flashcards

Leukaemias Flashcards

1
Q

A 3yo girl presents with a 10/7 history of fatigue, malaise and easy bruising. O/E you note an enlarged spleen + liver as well as multiple bruises + petechiae.

What is the likely diagnosis and how would you investigate?

A

ALL

Bloods

  • FBC: anaemia, thrombocytopaenia, neutropenia, high or low WCC
  • peripheral blood film: blasts
  • coagulation screen: high risk of DIC
  • U+Es, LFTs, LDH

BMAT: BM blasts 25% or more
- also send for immunophenotyping (determine lineage e.g. AML vs ALL) + cytogenetic analysis

Other

  • LP: ?CNS disease
  • CXR: ?mediastinal mass typical of T cell disease
  • abdo. USS: ?intra-abdominal adenopathy or renal infiltration
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2
Q

Describe the supportive + specific management of a patient newly diagnosed with ALL.

A

Supportive:

  1. blood or platelet transfusion: if cytopaenic
  2. hydration + allopurinol (start 12hrs before chemo) OR rasburicase if high risk of TLS
  3. CO-TRIMOXAZOLE (2-3 consecutive days weekly): for P. jirovecci prophylaxis

Specific:
1. induction combination chemo (1/12) to include pred/dex
+/- intrathecal chemo e.g. METHOTREXATE if CNS disease

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3
Q

A 3yo child has been diagnosed with ALL (no CNS disease) and received induction combination chemo for 1/12. What will follow up treatment involve?

A
  1. consolidation: systemic chemo + intrathecal chemo

2. maintenance: e.g. daily oral mercaptopurine for 2 (F) or 3 (M) yrs

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4
Q

What test should be performed before prescribing mercaptopurine?

A

TPMT activity

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5
Q

What are the haematological causes of hepatosplenomegaly?

A
  1. leukaemias: AML, CML, ALL, CLL
  2. lymphomas
  3. myelofibrosis
  4. myeloproliferative disease e.g. essential thrombocytopaenia, polycythaemia
  5. haemolytic anaemias e.g. thalassaemia, sickle cell, spherocytosis, G6PD deficiency, pyruvate kinase deficiency
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6
Q

A 36yo woman presents with a 2/52 Hx of fatigue, palpitations and bruising.
What would a peripheral blood film show if the diagnosis is AML?

A

blasts with Auer rods

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7
Q

Suggest risk factors for the development of AML.

A
  1. previous chemo
  2. radiation exposure inc. radiotherapy
  3. haematological dyspoiesis e.g. MDS, aplastic anaemia and myeloproliferative disorders
  4. genetic factors
  5. age >65yrs
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8
Q

A patient who has been diagnosed with AML has a WCC of 128,000 cells/uL. How would you treat this hyperleukocytosis?

A

HYDROXYCARBAMIDE

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9
Q

Which mutation is characteristic of CML?

A

T(9;22) translocation (Philadelphia chromosome) causing BCR-ABL fusion - constitutively active tyrosine kinase (oncogene)

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10
Q

Which signs/symptoms may be present in a patient with AML?

A
  • Sx of anaemia e.g. lethargy
  • Sx of thrombocytopaenia e.g. bruising, bleeding
  • weight loss + night sweats
  • splenomegaly (may be marked) causing abdo. discomfort/fullness
  • +/- hepatomegaly or lymphadenopathy
  • hyperviscosity (due to leucocytosis) e.g. visual disturbances (due to retinal haemorrhages), confusion, cerebrovascular accident, priapism
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11
Q

A 56yo gentleman presents with fatigue, weight loss + bruising for the past 2/12. There is marked splenomegaly on examination and you suspect CML. How will you investigate and what will these show?

A

Bloods

  • FBC: raised WCC, anaemia, +/- thrombocytopaenia/cytosis
  • peripheral blood smear: raised granulocytes (all stages of dev.), >20% basophils, >20% eosinophils
  • U+Es: +/- raised urate
  • LDH: raised

BMAT:

  • aspirate: raised BM blasts
  • trephine: hypercellular marrow, raised myeloid + progenitor cells
  • cytogenetic analysis: t(9;22)
  • qRT-PCR: quantitative detection of BCR-ABL fusion
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12
Q

A 56yo gentleman is diagnosed with chronic phase AML.

What is 1st line treatment?

A

TKIs: IMATINIB (v. good cytogenetic response) - inhibits BCR-ABL tyrosine kinase and inhibits proliferation/induces apoptosis of AML cells

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13
Q

A 78yo asymptomatic man present with an incidental finding of a lymphocytosis on a recent blood test.

You suspect CLL. How would you confirm diagnosis?

A
  • FBC + differential: raised WCC with lymphocytes
  • peripheral blood smear: smudge cells
  • peripheral blood flow cytometry: typical CLL cell surface markers
  • peripheral blood FISH: to determine prognosis
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14
Q

What are the management options for CLL?

A
  1. watch + wait: if asymptomatic with no indications for Tx
  2. FCR chemotherapy: if favourable cytogenetics + good performance status
  3. BCR inhibitors e.g. IBRUTINIB + RITUXIMAB: if unfavourable cytogenetics

Indications for immediate Tx include: anaemia or thrombocytopaenia, symptomatic, rapidly progressive disease (<6/12 lymphocyte doubling time, rapidly enlarging lymph nodes or spleen, AIHA or ITP refractory to immunosuppresive therapy

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15
Q

Suggest possible complications of CLL.

A
  1. Richter’s transformation: transformation to high-grade lymphoma
  2. hypogammaglobulinaemia with higher risk of infection
  3. warm autoimmune haemolytic anaemia (10-15%)
  4. anaemia or thrombocytopaenia (poor prognosis)
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16
Q

What is your differential for lymphocytosis?

A
  1. infection e.g. EBV, CMV, TB, hepatitis
  2. smoking
  3. post-splenectomy
  4. CLL
  5. ALL
  6. lymphoma (leukaemic phase)
17
Q

What is myelodysplastic syndrome?

A

Haematopoietic stem cell clonal cytogenetic abnormality causing dysplastic haematopoiesis resulting in:

  • 1+ cytopenias
  • BM dysplasia with <20% blast cells
  • risk of AML development (1/3)
18
Q

A 73 yo man presents with an incidental finding of anaemia + thrombocytopaenia (no bleeding). You suspect myelodysplastic syndrome. How will you investigate?

A

Bloods

  • FBC + differential: 1+ cytopenia
  • reticulocyte count: inappropriately normal (if anaemia) or low
  • folate + B12: exclude deficiency as cause of anaemia
  • iron studies: exclude deficiency as cause of anaemia
  • HIV testing: exclude as cause of anaemia

BMAT

  • aspiration: single or multilineage dysplasia, BM blasts <20%
  • trephine: hypercellular marrow
  • cytogenetic analysis: chromosomal abnormalities characteristic of MDS
19
Q

What are the management option for a patient with myelodysplastic syndrome?

A

If acute high risk disease with suitable donor + good general health: allogeneic HSCT

If asymptomatic: watch + wait, regular FBC

Symptomatic cytopaenia(s) with chromo. 5q31 deletion:

  • LENALIDOMIDE (immunomodulatory agent)
  • RBC or platelet transfusions

Symptomatic cytopaenia(s) without chromo. 5q31 deletion:

  • G-CSF (e.g. filgrastim) or EPO
  • RBC or platelet transfusions

Year 4 - Cancer Care SL (12 decks)

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