Lymphoid Malignancy Flashcards

1
Q

What is acute lymphoblastic leukaemia?

A

Haematological malignancy in lymphoid progenitor/non-differentiated lymphoid cells, resulting in increased production of lymphoblasts

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2
Q

In what age group is acute lymphoblastic leukaemia more common?

A

Most common childhood cancer

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3
Q

What is the prognosis of ALL in children?

A

Good, 90% 5 year survival

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4
Q

What is the prognosis of ALL in adults?

A

Poor, 30–35% 5 year survival

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5
Q

What are risk factors for poor prognosis ALL?

A

>Age

>WCC

Slow/poor response treatment

Cytogenetics: T(9;22) and t(4;11)

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6
Q

How does ALL present?

A

Thrombocytopenic bleeding

  • Bruising
  • Menorrhagia
  • Epistaxis

Lethargy/malaise

Anaemia

Dyspnoea

Arthralgia

Recurrent infection: Neutropenia

CNS infiltration

Testicular disease

Weight loss

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7
Q

What investigations are used in ALL diagnosis?

A

Bone Marrow Biopsy

FBC, within 48 houts in young people with suspicious symptoms

  • Increased WCC

Clotting

Blood film

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8
Q

What biopsy sign is present in ALL?

A

90% B lymphoblasts

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9
Q

How is ALL managed?

A

Supportive

  • Treat infection
  • Manage bleeding

Chemotherapy

Stem cell transplantation

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10
Q

Give complications of ALL

A

Haemorrhage

Thrombosis

Tumour lysis syndrome

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11
Q

What is tumour lysis syndrome?

A

Cell break down secondary to chemotherapy, resulting in the release of intracellular compounds such as potassium, phosphate and uric acid, resulting in renal failure

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12
Q

What drug is used to prevent tumour lysis syndrome?

A

Allopurinol

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13
Q

What is chronic lymphocytic leukaemia?

A

Malignancy characterised by monoclonal proliferation of well-differentiated/mature lymphocytes, resulting in functionally incompetent lymphocytes

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14
Q

What is the most common leukaemia worldwide?

A

Chronic lymphocytic leukaemia

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15
Q

What age group is CLL more common?

A

60+

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16
Q

What are the poor prognostic factors for CLL?

A

Advanced disease (Binet B or C)

Atypical lymphocyte morphology

Rapid lymphocyte doubling time (<12 months)

CD38+ expression

Loss/mutation p53; del 11q23 (ATM gene)

Unmutated IgVH gene status

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17
Q

How does CLL present?

A

Often asymptomatic at presentation

Recurrent infection

Weight loss

Bleeding

Sweating

Malaise

Abdominal discomfort

Lymphadenopathy, more marked than CML

Splenomegaly

Hepatomegaly

Petechiae

Pallor

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18
Q

What investigations are used in CLL?

A

Immunophenotyping

Bone marrow biopsy

Lymph node biopsy

FBC

  • Warm autoimmune haemolytic anaemia
  • Lymphocytosis

Blood film

  • Smudge B cells/smear cells
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19
Q

How does Richter’s transformation present?

A

Lymph node swelling

Fever without infection

Weight loss

Night sweats

Nausea

Abdominal pain

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20
Q

What biopsy sign is seen in CLL?

A

>30% lymphocytes

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21
Q

What blood film sign is seen in CLL?

A

Smudge B cells/smear cells

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22
Q

What are the indications for treatment of CLL?

A

Progressive bone marrow failure

Massive lymphadenopathy

Progressive splenomegaly

Lymphocyte doubling time <6 months or >50% increase over 2 months

Systemic symptoms

Autoimmune cytopenias

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23
Q

How is CLL managed?

A

Often nothing/watch and wait

Supportive

  • Blood transfusions
  • Antibiotics

Cytotoxic chemotherapy

Monoclonal antibodies

Novel agents

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24
Q

Give complications of CLL

A

Transformation to high-grade lymphoma/Richter’s transformation

Autoimmune warm haemolytic anaemia

Idiopathic thrombocytopenic purpura

Hypogammaglobulonaemia

25
Q

Give examples of Monoclonal antibodies used in CLL treatment

A

Rituximab

Obinatuzumab

26
Q

What staging classification is used for CLL?

A

Binet

27
Q

Describe Binet stage A

A

<3 lymph node areas

28
Q

Describe Binet stage B

A

3 or more lymph node areas

29
Q

Describe Binet stage C

A

Stage B plus anaemia or thrombocytopenia

30
Q

What is the prognosis of Binet stage A?

A

Same as age matched controls

31
Q

What is the prognosis of Binets stage B?

A

8 years

32
Q

What is the prognosis of Binet stage C?

A

6 years

33
Q

What is Richter’s transformation?

A

Occurs when leukaemia cells enter the lymph node and change into a high-grade, fast-growing non-Hodgkin’s lymphoma, causing patients to become unwell very suddenly

34
Q

What is Hodgkin’s lymphoma?

A

Hodgkin’s lymphoma is a malignant proliferation of lymphocytes characterised by the presence of the Reed-Sternberg cell

35
Q

Give types of hodgkins lymphoma

A

Nodular scleroising, most common

Mixed cellularity

Lymphocyte predominant, best prognosis

Lymphocyte depleted, worst prognosis

36
Q

What age does Hodgkin’s peak at?

A

Bimodal age distribution

  • 1st peak at 15-35 years
  • 2nd peak 50-60
37
Q

How does Hodgkin’s present?

A

Painless, non-tender, asymmetrical lymphadenopathy

Weight loss

Pruritus

Night sweats

Fever

alcohol pain

Normocytic anaemia, eosinophilia

LDH raised

38
Q

What is the most common site of lymphadenopathy in Hodgkin’s?

A

Cervical chain

39
Q

What cells are characteristic of Hodgkin’s?

A

Reed-Sternberg cells (mirror image nuclei)

40
Q

How is Hodgkin’s managed?

A

Combination chemotherapy

Radiotherapy

Monoclonal antibodies: Anti-CD30

Immunotherapy

41
Q

What staging system is used in lymphoma?

A

Ann Arbor system

With A or B to indicate the presence or absense of B symptoms

42
Q

Describe stage 1 lymphoma

A

One node affected

43
Q

Describe stage 2 lymphoma

A

More than one node affected on the same side of the diaphragm

44
Q

Describe stage 3 lymphoma

A

Nodes affected on both sides of the diaphragm

45
Q

Describe stage 4 lymphoma

A

Extra-nodal involvement such as spleen, bone marrow or CNS

46
Q

Give an example of a low grade non-hodgkin’s lymphoma subtype

A

Follicular, marginal zone

47
Q

Give examples of high grade non-hodgkin’s lymphoma sub-types

A

Diffuse large B cell lymphoma/Burkitt’s lymphoma

T cell lymphoma

48
Q

What is the prognosis of non-hodgkins lymphoma?

A

Low-grade has a better prognosis

High-grade has a worse prognosis but a higher cure rate

49
Q

Give risk factors for non-hodgkin’s

A

Elderly

Caucasians

FH

Viral infection

  • EBV, Burkitt’s
  • HSV

Immunosuppression

  • HIV, Burkitt’s
  • DM
  • Transplant

History of chemotherapy or radiotherapy

Autoimmune disease

  • SLE
  • Coeliac
50
Q

How does non-hodgkin’s present?

A

Lymphadenopathy

Hepatosplenomegaly/palpable abdominal mass

B Symptoms occur later than hodgkins

Testicular mass

Weight loss

Fever

Extra nodal disease is more common

51
Q

What investigations are used in non-hodgkins lymphoma diagnosis?

A

Excisional node biopsy

  • Starry sky appearance in Burkitt’s

CT chest, abdomen and pelvis for staging

HIV test

FBC and blood film

ESR useful as a prognostic indicator

LDH useful as a prognostic indicator

52
Q

What is the management of non-hodgkins lymphoma?

A

Watchful waiting

Chemotherapy

Radiotherapy

All patients will receive flu/pneumococcal vaccines

Patients with neutropenia may require antibiotic prophylaxis

Consider autologous bone marrow transplantation in unresponsive or recurrent disease

53
Q

Give complications of non hodgkins lymphoma

A

Bone marrow infiltration causing anaemia, neutropenia or thrombocytopenia

Superior vena cava obstruction

Metastasis

Spinal cord compression

Complications related to treatment

54
Q

What is the most common lymphoma?

A

High grade Non-Hodgkin lymphoma is most common lymphoma, with diffuse large B-cell lymphoma most common subtype of lymphoma

55
Q

Give complications of tumour lysis syndrome

A

hyperkalaemia

hyperphosphataemia

hypocalcaemia

hyperuricaemia

acute renal failure

56
Q

What is given before chemotherapy to prevent tumour lysis syndrome?

A

Rasburicase

  • Recombinant version of urate oxidase, an enzyme which catalyses the conversion of uric acid to allantoin, increasing renal extraction
57
Q

How does neutropenic sepsis present?

A

Commonly occurs 7-14 days after chemotherapy

Neutrophil count of < 0.5 * 109

Temperature higher than 38ºC

Anorexia

Malaise

Lethargy

Sweats

Fever

Chills

58
Q

How is neutropenic sepsis managed?

A

Broad spectrum antibiotics immediately

  • Tazocin (Piperacilin and Tamzobactin) + Gentamicin
  • Alternate therapy if still febrile after 48 hours

G-CSF (Granulocyte Colony Stimulating Factor)