W4 Lect2: intro to lymphoproliferative

Question 1

what are the subset of lymphocytes?

Answer 1

B-cells (Bone marrow / Bursa derived)
*
T-cells (Thymus derived)
*
Natural Killer cells –innate immune responders, remove virally infected or damaged cells.

Question 2

what are thee histiocyte subset?

Answer 2

• Macrophages / Histiocytes Monocytes
• Dendritic cells -Follicular (B-cells)
• Interdigitating -(T-cells)
• Langerhans cells -(skin)

Question 3

WHAT is the normal b- cell differiantion process?

Answer 3

1. PRIMITIVE B-CELL (BLAST) -bone marrow
2. MATURE (antigen naïve) B-CELL -bone marrow, blood, secondary lymphoid organs
3. ANTIGEN
4. PLASMA CELL OR MEMORY B-CELL (antigen experienced)

Question 4

what is monoclonal?

Answer 4

Cells which arise from a single abnormal cell and proliferate to form a clone with the same abnormality. Indicates a malignancy.

Question 5

what are lymphoproliferative disorders?

Answer 5

Monoclonal proliferation or accumulation of abnormal lymphocytes (primitive or mature; B-or T-cells).

Question 6

what are the differences between hodgkin and non- hodgkin’s lyphoma?

Answer 6

1. HODGKIN LYMPHOMA
   Reed Sternberg cell in an inflammatory
   background
2. NON-HODGKIN LYMPHOMA
   Arise from stages of arrested development in B-or T-cell ontogeny

Question 7

what is multiple myeloma?

Answer 7

Monoclonal proliferation of abnormal plasma cells.
Systemic disease.

Question 8

Localisedplasma cell neoplasm?

Answer 8

plasmacytoma

Question 9

what are the histocytic neoplasms?

Answer 9

1. Langerhans cell histiocytosis
   * Eosinophilic granuloma (20yrs)
   * Hand-Schuller-Christian disease (12yrs)
   * Letterer-Siwe disease (2yrs)
2. Histiocytic sarcoma

Question 10

what are the characteristics of classic hodgkin lymphoma?

Answer 10

• 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

Question 11

outline Lymphocyte predominant Hodgkin lymphoma:

Answer 11

• 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

Question 12

non hodgkin’s lymphoma:

Answer 12

– Multicentric disease presentation
- Unpredictable pattern of spread
- Early marrow involvement

Question 13

1. Commonly a tumour of children –ages 4 to 9 years, but seen in all age groups
2. Present with bleeding (low platelets), infection (low neutrophils), tiredness (anaemia), bone pain, lymphadenopathy and hepatosplenomegaly
3. In younger children = good outcome on treatment (80%+ cure rates
   this are the clinical feature of which leukemia?

Answer 13

acute lymphoblastic leaukemia

Question 14

what are the features of chronic lymphocytic leukemia

Answer 14

1. Elderly
2. Lymphadenopathy (focal or generalised)
3. Hepatosplenomegaly or other organ involvement
4. Focal tumours
5. Peripheral blood involvement
6. Complications

Question 15

what are the outcomes and pathogenesis of B- CLL?

Answer 15

1. Failure of normal cell death (apoptosis)
2. Typically indolent (slowly progressive) tumours
3. Not able to cure them currently

Question 16

what are the features of follicular lymphoma?

Answer 16

1. Recapitulates the germinal centre
2. Consists of small cleaved (centrocytes) and large (centroblasts) follicle centre cells
3. Graded as I (predominantly centrocytes), II (mixed centrocytes and centroblasts) and III (predominantly centroblasts)
4. All considered an INDOLENT NHL
5. t(14;18) and BCL2 oncogene expression

Question 17

1. Endemic in areas in Africa
2. Associations:
   * Malaria
   * Epstein-Barr Virus
   * HIV
   * t(8;14) and MYCtranslocation, with over-activity of this oncogene
   * Jaw mass
   what lymphoma is this?

Answer 17

Burkitt lymphoma

Question 18

what is the diffused large B- cell myphoma

Answer 18

1. Commonest NHL
2. Aggressive tumour
3. Adults and children
4. Increased incidence in AIDS
   6 Chemotherapy may be curative

Question 19

pathogenesis of muitiple myeloma?

Answer 19

1. Proliferate in marrow, bone, CNS or other organs, where they displace or damage normal structures
2. Secrete cytokines, particularly ones which cause bone breakdown
   3.Secrete monoclonal antibodies (immunoglobulins) → M-protein, with a decrease in normal immunoglobulins(immune paresis)

Question 20

clinical picture of MM?

Answer 20

1. Disease of elderly, in general cannot be cured
2. Anaemia, thrombocytopenia and neutropenia
3. Bone pain, fractures (pathological) and lytic lesions
   Infection:
   *Immune paresis
   *Neutropaenia
   *Organ involvement –Myeloma kidney