W4 Lect2: intro to lymphoproliferative Flashcards
what are the subset of lymphocytes?
B-cells (Bone marrow / Bursa derived)
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T-cells (Thymus derived)
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Natural Killer cells –innate immune responders, remove virally infected or damaged cells.
what are thee histiocyte subset?
- Macrophages / Histiocytes Monocytes
- Dendritic cells -Follicular (B-cells)
- Interdigitating -(T-cells)
- Langerhans cells -(skin)
WHAT is the normal b- cell differiantion process?
- PRIMITIVE B-CELL (BLAST) -bone marrow
- MATURE (antigen naïve) B-CELL -bone marrow, blood, secondary lymphoid organs
- ANTIGEN
- PLASMA CELL OR MEMORY B-CELL (antigen experienced)
what is monoclonal?
Cells which arise from a single abnormal cell and proliferate to form a clone with the same abnormality. Indicates a malignancy.
what are lymphoproliferative disorders?
Monoclonal proliferation or accumulation of abnormal lymphocytes (primitive or mature; B-or T-cells).
what are the differences between hodgkin and non- hodgkin’s lyphoma?
- HODGKIN LYMPHOMA
Reed Sternberg cell in an inflammatory
background - NON-HODGKIN LYMPHOMA
Arise from stages of arrested development in B-or T-cell ontogeny
what is multiple myeloma?
Monoclonal proliferation of abnormal plasma cells.
Systemic disease.
Localisedplasma cell neoplasm?
plasmacytoma
what are the histocytic neoplasms?
- Langerhans cell histiocytosis
* Eosinophilic granuloma (20yrs)
* Hand-Schuller-Christian disease (12yrs)
* Letterer-Siwe disease (2yrs) - Histiocytic sarcoma
what are the characteristics of classic hodgkin lymphoma?
- Nodular sclerosing HL (lacunar cell = RS cell)
- Mixed cellularity HL
- Lymphocyte-rich cHL
- Lymphocyte depleted
- Reed Sternberg cell –“owl’s eye”, binucleate cell
- Majority are actually B-lymphocytes with abnormal function
- Inflammatory background: eosinophils, plasma cells, lymphocytes, histiocytes
outline Lymphocyte predominant Hodgkin lymphoma:
- B-cell lymphoma, young adult males
- 10% tumour: Progressively transformed germinal centres (PTGC), progression to diffuse large B-cell lymphoma (DLBCL)
- “L&H” Lymphocytic and Histiocytic cell/ popcorn cell in a lymphocyte rich background
- Unifocal/unicentric presentation
- Orderly, predictable pattern of spread
- Late bone marrow involvement
- Ann Arbor Clinical Staging:
A. Localised, single focus of disease
B. Two/ more disease foci, same side of diaphragm
C. Two/ more disease foci, both sides of diaphragm
4. Disseminated disease
non hodgkin’s lymphoma:
– Multicentric disease presentation
- Unpredictable pattern of spread
- Early marrow involvement
- Commonly a tumour of children –ages 4 to 9 years, but seen in all age groups
- Present with bleeding (low platelets), infection (low neutrophils), tiredness (anaemia), bone pain, lymphadenopathy and hepatosplenomegaly
- In younger children = good outcome on treatment (80%+ cure rates
this are the clinical feature of which leukemia?
acute lymphoblastic leaukemia
what are the features of chronic lymphocytic leukemia
- Elderly
- Lymphadenopathy (focal or generalised)
- Hepatosplenomegaly or other organ involvement
- Focal tumours
- Peripheral blood involvement
- Complications
what are the outcomes and pathogenesis of B- CLL?
- Failure of normal cell death (apoptosis)
- Typically indolent (slowly progressive) tumours
- Not able to cure them currently
what are the features of follicular lymphoma?
- Recapitulates the germinal centre
- Consists of small cleaved (centrocytes) and large (centroblasts) follicle centre cells
- Graded as I (predominantly centrocytes), II (mixed centrocytes and centroblasts) and III (predominantly centroblasts)
- All considered an INDOLENT NHL
- t(14;18) and BCL2 oncogene expression
- Endemic in areas in Africa
- Associations:
* Malaria
* Epstein-Barr Virus
* HIV
* t(8;14) and MYCtranslocation, with over-activity of this oncogene
* Jaw mass
what lymphoma is this?
Burkitt lymphoma
what is the diffused large B- cell myphoma
- Commonest NHL
- Aggressive tumour
- Adults and children
- Increased incidence in AIDS
6 Chemotherapy may be curative
pathogenesis of muitiple myeloma?
- Proliferate in marrow, bone, CNS or other organs, where they displace or damage normal structures
- Secrete cytokines, particularly ones which cause bone breakdown
3.Secrete monoclonal antibodies (immunoglobulins) → M-protein, with a decrease in normal immunoglobulins(immune paresis)
clinical picture of MM?
- Disease of elderly, in general cannot be cured
- Anaemia, thrombocytopenia and neutropenia
- Bone pain, fractures (pathological) and lytic lesions
Infection:
*Immune paresis
*Neutropaenia
*Organ involvement –Myeloma kidney