Introduction to Lymphoid Malignancies Flashcards Preview

Sem 4: TI2 > Introduction to Lymphoid Malignancies > Flashcards

Flashcards in Introduction to Lymphoid Malignancies Deck (11):
1

Describe the epidemiology of Hodgkin's lymphoma. 

  • Peak incidence in young adults. 
  • Possible association with EBV - aka Human Herpes Virus 4 (HHV4).

2

Outline the histopathology, treatment and prognosis of Hodgkin's lymphoma. 

  • Presence of Reed-Sternberg cells - malignant B cells.
  • Typically 99% of cells are reactive non-malignant cells. 
  • Treatment - chemotherapy +/- radiotherapy. 
  • Prognosis - 5 year survival = 50-90%, depending on age, stage and histology.
  • Good prognosis in young adults. 

3

Outline the classification of Non-Hodgkin's lymphomas. 

Grade:

  • Low grade - indolent
  • High grade - aggressive

Type:

  • T-cell lymphomas
  • EBV (HHV4) driven lymphomas in immunosuppressed patients

4

What chromosomal translocation is responsible for most cases of follicular lymphoma, and how does this translocation result in disease?

  • t(14;18) - IgH locus on Chr14 translocated to BCL-2 gene on Chr18. 
  • IgH acts as an enhancer - activates BCL-2 promoter. 
  • Overexpression of BCL-2 protein.
  • BCL-2 is an apoptosis inhibitor. 

5

What is the consequence of the t(;8:14) chromosomal translocation?

  • MYC gene on Chr8 translocated to IgH locus on Chr14. 
  • MYC is a powerful proto-oncogene. 
  • Overactivation of MYC by IgH enhancer leads to uncontrolled B cell proliferation.
  • Results in high grade non-Hodgkin lymphoma - "Burkitt's lymphoma". 

6

Outline the presentation and diagnosis of low grade NHL. 

  • Enlarged lymph node(s).
  • Histology - normal tissue architecture partially preserved.
  • Normal cell of origin recognisable - used to name lymphoma - e.g. follicular.
  • Diagnosis - histology, immunocytochemistry, light-chain restriction, PCR. 
  • PCR - analyse clonal Ig gene rearrangement, chromosomal translocations. 

7

Outline the treatment and prognosis of low grade NHL. 

Treatment:

  • Chemotherapy
  • Glucocorticoids - e.g. prednisolone 
  • Radiotherapy
  • Monoclonal Ab therpay - e.g. rituximab

Prognosis:

  • Relatively indolent - slow growth
  • Responds well to therapy
  • Hard to cure

8

Outline the presentation and diagnosis of high grade NHL.

  • Enlarged lymph nodes. 
  • Loss of normal tissue architecture. 
  • Normal cell of origin hard to determine. 
  • Diagnosis - histology, immunocytochemistry, light-chain restriction, PCR. 

9

Describe the prognosis of high grade NHL. 

  • Prognosis variable depending on type, stage and other factors.
  • Long term survival ~ 65%. 
  • Easier to cure than low grade NHL. 

10

Outline the pathophysiology and presentation of multiple myeloma. 

  • Suppression of normal bone marrow, blood cell and immune cell function - anaemia, recurrent infections, bleeding. 
  • Bone resoprtion and calcium release - myeloma cells produce cytokines e.g. IL-6 > bone marrow cells produce RANKL > osteoclast activation - bone pain, fractures, hypercalcaemia - mental disturbance.
  • Pathological effects of paraprotein - monoclonal Ig in serum - causes renal failure, deposited as amyloid in many tissues - 2% of cases lead to hyperviscosity syndrome - increased viscosity of blood leading to heart failure/stroke. 

11

How is multiple myeloma diagnosed?

  • Serum electrophoresis for paraprotein. 
  • Urine electrophoresis - Bence-Jones protein represents free monoclonal light chains. 
  • Increased plasma cells in bone marrow. 
  • High erythrocyte sedimentation rate (ESR) - caused by rouleaux formation. 
  • Radiological investigation of skeleton for lytic lesions.