22. Overview of Malignant Lymphoid Proliferations Flashcards
What is a haematologic malignancy
cancer of any of the formed elements in the blood
- can be classified into:
a) lymphomas - Hodgkins vs non-Hodgkins
b) leukaemias - acute/chronic and myeloid/lymphoid
What are monoclonal cells
a group of cells that are derived form a single ancestral cell by repeated cellular replication
- in malignancy, these cells divide unchecked due to cell cycle dysregulation
- form a single ‘clone’
Development of B cells
bone marrow: [stem cell -> pro-B cell -> pre-B cell] -> periphery: [naive B cell -> activated B cell -> memory Germinal Center B cell -> late plasmablast] -> plasma cells
Manifestations of lymphoma
- painless lymphadenopathy
- abdominal distention, ascities
- SOB, pleural effusion
- fatigue
- fever
- night sweats
- weight loss
- neurological symptoms, CNS disease
Ann Arbor Staging
Stage I -single lymph node region Stage II -one side of diaphragm Stage III -both side of diaphragm Stage IV -disseminated
A-no systemic symptoms
B-fever, night sweats weight loss
What is a lymphoma
malignant proliferation of lymphoid cells
What is Hodgkins lymphoma
Hodgkin Reed Sternberg Cells
- express CD30 and CD15
- rest of node: inflammatory cells - T-cells, plasma cells, macrophages
Clinical features of Hodgkin’s disease
- enlarged firm node (often cervical)
- fever
- night sweats
- weight loss
- pain after alcohol
- pruritis
Type of non Hodgkins lymphoma
- B-cell
a) high grade
-Burkitt’s lymphoma
-diffuse large B-cell lymphoma
-mantle cell lymphoma
b) low grade
-follicular
-marginal zone lymphoma
-lymphoplasmacytic lymphoma - T-cell
mostly behave as high grade
-peripheral T-cell lymphoma
-angioimmunoblastic T-cell lymphoma
-anaplastic large cell lymphoma
-enteropathy-associated T-cell non-Hodgkin lymphoma
-mycosis fungoides
-Sezary syndrome
What are the features of Burkitt’s lymphoma
- high grade B cell
- require very aggresive chemotherapy
- lymphoblastic
- EBV associated
- c-Myc translocation present
- “starry sky” appearance on histological section of marrow
- children and adolescent
What are the features of mantle cell lymphoma
- low grade B cell
- Cyclin D1 positive
What are the features of mycosis fungoides
- T cell lymphoma in skin
- adults
- 12 years survival
What are the features of Enteropathy Associated T-cell Lymphoma (EATL)
- complication of coeliac disease
- occurs in small bowel
- common in those with poor dietary control
What are the features of T lymphoblastic lymphoma
- young adults
- rapid enlargement of nodes
- large lymphoid cells react with T lymphocyte markers
What are the features of adult T-cell leukemia/lymphoma
- rare
- HTLV1 virus
- hypercalcaemia (PTH)
