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Haematology > Lymphoproliferative Disorders > Flashcards

Lymphoproliferative Disorders Flashcards

1
Q

Lymphoma Presentation

A
  • can present with enlarged lymph nodes (lymphadenopathy)
  • +/- extranodal involvement
  • +/- bone marrow involvement
  • systemic (B) symptoms: weight loss (> 10% in 6 months), fever, night sweats, pruritus, fatigue
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2
Q

how is a lymphoma or leukaemia diagnosed and staged?

A
  • defined by malignant cell characteristics
  • Diagnosis: Biopsy (e.g. lymph node, bone marrow), tells us what type it is
  • Staging: Clinical exam and imaging (e.g. CT) describes the location and extent of disease, gives info regarding prognosis, sometimes influences treatment.
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3
Q

list some lymphoproliferative disorders

A
  • acute lymphoblastic leukaemia (ALL)
  • chronic lymphocytic leukaemia (CLL)
  • Hodgkin lymphoma
  • non-hodgkin lymphoma (NHL): high grade vs low grade
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4
Q

Describe acute lymphoblastic leukaemia (ALL).

A

Acute lymphoblastic leukaemia (ALL) affects one of the lymphocyte precursor cells, causing acute proliferation of a single type of lymphocyte, usually B-lymphocytes. Excessive accumulation of these cells replaces the other cell types in the bone marrow, leading to pancytopenia.

ALL most often affects children under five but can also affect older adults. It is more common with Down’s syndrome. It can be associated with the Philadelphia chromosome (but this is more associated with chronic myeloid leukaemia).

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5
Q

how does acute lymphoblastic leukaemia present?

A
  • 75% cases occur in children < 6 years
  • 2-3 week history of bone marrow failure +/- raised white cell count
  • bone pain, infection, sweats
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6
Q

acute lymphoblastic leukaemia treatment

A

Treated with multi-agent intensive chemotherapy +/- allogenic stem cell transplant

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7
Q

what are some newer therapies which can be used to treat ALL?

A
  • Bi-specific T-cell engagers (BiTe molecules) e.g. Blinatumumab
  • CAR (Chimeric antigen receptor) T-cells
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8
Q

what are some key side effects of T-cell immunotherapy?

A
  • cytokine release syndrome: fever, hypotension, dyspnoea
  • neurotoxicity: confusion with normal conscious level, seizure, headache, focal neurology, coma
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9
Q

diagnosis of chronic lymphocytic leukaemia (CLL) requires a lymphocyte count of?

A

> 5 (normal is < 4)

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10
Q

describe chronic lymphocytic leukaemia (CLL)

A

Chronic lymphocytic leukaemia is where there is slow proliferation of a single type of well-differentiated lymphocyte, usually B-lymphocytes. It usually affects adults over 60 years of age.

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11
Q

what is the most common leukaemia worldwide?

A
  • chronic lymphocytic leukaemia (CLL)
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12
Q

CLL presentation

A
  • ofte asymptomatic at presentation

Frequent findings:
- bone marrow failure (anaemia, thrombocytopenia)
- lympahdenopathy
- splenomegaly (30%)
- fever and sweats (< 25%)

Less common findings:
- hepatomegaly
- infections
- weight loss

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13
Q

what are the key differentiating features of ALL?

A
  • most common leukaemia in children
  • associated with Down’s syndrome
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14
Q

what are the key differentiating features of CLL?

A
  • warm haemolytic anaemia
  • Richter’s transformation
  • smudge cells
  • immune paresis (loss of normal immunoglobulin production)
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15
Q

what is Richter’s transformation?

A

refers to the rare transformation of CLL into high-grade B-cell lymphoma

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16
Q

CLL staging

17
Q

CLL treatment

A
  • often nothing - ‘watch and wait’
  • cytotoxic chemotherapy e.g. fludarabine, bendamustine
  • monoclonal antibodies e.g. Rituximab, obinatuzumab
  • novel agents
18
Q

How is non-Hodgkin Lymphoma classified?

A

according to:
- lineage (B-cell or T-cell) - 90% are B-cell in origin
- ‘grade’ - high vs low

19
Q

what are the differences between low and high grade Non-Hodgkin lymphoma?

A

low grade:
- indolent, often asymptomatic
- responds to chemotherapy but incurable

high grade:
- aggressive, fast-growing
- require combination chemotherapy
- can be cured

20
Q

what are the most common types of non-hodgkin lymphoma?

A

Diffuse large B-cell lymphoma:
- most common subtype of lymphoma (of any kind)
- high-grade lymphoma

Follicular lymphoma:
- 2nd most common subtype of lymphoma
- low-grade lymphoma
- like CLL, ‘watch and wait’

Both are treated with combination chemotherapy - typically andti-CD20 monoclonal antibody + chemo

21
Q

what is the most common specific type of lymphoma?

A

Hodgkin’s lymphoma
- bimodal age distribution with peaks around 20-25 and 80 years

22
Q

risk factors Hodgkin’s lymphoma

A
  • HIV
  • Epstein-Barr virus
  • autoimmune conditions, such as RA and sarcoidosis
  • family history
23
Q

Hodgkin lymphoma treatment

A
  • combination chemotherapy
  • +/- radiotherapy
  • monoclonal antibodies (anti-CD30)
  • immunotherapy (checkpoint inhibitors)
  • PET scanning central to asessment of response to treatment

Haematology (11 decks)

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