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LYMPHOMA (Non-Hodgkin Lymphoma (Presentation: (Microscopic: (Loss of…

- - - - HTLV1
      - HHV8
      - EBV
      - HIV
    - - Secondary
        
        Aids
        
        Transplatation
      - Primary
        
        X-Linked immunodeficiency
  - - - Tissues involved by lymphoma have uniform fish flesh appearance & consistency.
    - - Loss of normal nodal architecture
      - If follicles are still recognisable, lymphoma is follicular.
      - If follicles are no longer recognisable, lymphoma is diffuse.
  - - - Mature cell neoplasms
        
        Mycosis fungoides & Sezary syndrome ( These are cutaneous)
        
        Has cerebriform nucleus on histology
        
        Patches on skin resembling fungal infection
        
        Others
        
        Anaplastic large cell (ALC) lymphoma
      - Lymphoblastic precursor cell neoplasms
    - - Mature cell neoplasms
        
        Small lymphocytic
        
        Pattern is diffuse
        
        Their ciourse is slow
        
        Affects over 50s
        
        Eventually transform to higher grade lymphoma
        
        Diffuse large cell
        
        Immunoblasts are recognisable as large cells with round vesicular nuclei & a large, centrally placed eosinophilic nucleolus.
        
        Cyto. is fairly abundant
        
        Poor prognosis but is curable
        
        Can arise:
        
        De novo
        
        In pre-existing low grade lymphoma
        
        Associated with immunodeficiency (esp. AIDS & post-transplant)
        
        Immunoblasts & centroblasts each provide a category of diffuse B large cell
        
        Follicular
        
        Commonest non-hodgkin
        
        Presents with painless generalized lymphadenopathy
        
        Has centroblasts/centrocytes at centre of follicles
        
        And form IgA & IgG
        
        Centroblasts then mature into centrocytes which differentiate into:
        
        Memory B cells which go to blood, LN, spleen / MALT
        
        Plasma cells which go to medulla
        
        Proliferate to form germinal centres
        
        Low grade lymphoma
        
        Good prognosis
        
        Burkitt lymphoma
        
        Consists of diffuse sheets of primitive medium sized blastic lymphoid cells with many histiocytes producing a " Starry Sky" appearance
        
        The appearance is due to tangible bodies which are macrophages that have pahgocytosed neoplastic cells.
        
        If untreated, the prognosis is poor.
        
        t(8,14) leading to increased cell proliferation
        
        Is curable
        
        Epidemiology types:
        
        Non-endemic
        
        Intestinal involvement id most common (ileum) with mesenteric nodes
        
        Older children
        
        Common in the Western part of world
        
        AIDS associated
        
        Endemic
        
        Affects 4 to 8 year olds
        
        Enlarged jaws & ovaries
        
        Common in Central Africa
        
        Nodal enlargement not characteristic
        
        Strong association with EBV
        
        t(14,18) resulting in the overexpression of bcl2 which blocks apoptosis
      - Lymphoblastic precursor cell neoplasms
        
        Diffuse primitive blastic lymphocytes
        
        Affects under 15s
        
        Mediastinal
        
        Aggresive
- - - - Pattern of fibrosis i.e. if present whether bands / diffuse
      - Proportion of reactive lymphocytes to neoplastic cells
      - Type & no. of neoplastic cells i.e. whether RS cells are classical / variants
    - - Axillary / cervical LN
      - B cell neoplasm
      - Male
      - May transform to high grade diffuse B cell lymphoma
      - Adults (30 50)
      - Has RS variants, popcorn cells
        
        Large cell
        
        Foldeed, multilobed, small nucleoli
        
        Pale cytoplasm
      - Plenty lymphocytes
      - Good prognosis
    - - Nodular Sclerosing
        
        Mediastinal involvement
        
        RS variants are Lacunar
        
        Appear to be lying in a space caused by cytoplasmic retraction
        
        Plenty present
        
        Young females
        
        LN divided into nodules by broad bands of fibrous tissue origination in the capsule
        
        Most common
        
        Many lymphocytes & eosinophils present
      - Mixed Cellularity
        
        Common in men
        
        Plenty RS cells & reactive component of lymphocytes, plasma cells, eosinophils
        
        More than 50% are stage 3 / 4 (Arbor Staging)
        
        Focal necrosis & granulomas
        
        Adults ( about 38 years old)
      - Lymphocyte Rich
        
        Affects older patients than Nodular Lymphocyte(NL)
        
        Less favorable prognosis than NL
        
        Early stage disease (stage 1 / 2)
      - Lymphocyte Depleted
        
        (-) no. of lymphocytes
        
        Plenty atypical RS cells
        
        Fibrosis present
        
        Common in older men presenting with advanced disease
  - - - Major cause of death due to:
        
        Splenectomy ( which facilitates for pneumococcal sepsis)
        
        Persistentce of immunological defect
        
        Opportunistic infections
    - - Irradiation &/ chemotherapy( bleomycin) produce pulmonary inflammatory & fibrosis
      - Chemotherapy ( adriamycin) produces pericardial fibrosis& myocardial damage
      - Endocrine dysfunction may result from irradiation( hypothyroidism) or chemotherapy (hypogonadism)
      - Second malignancies:
        
        High grade non-hodgkin lymphoma
        
        Bone & soft tissue sarcoma
        
        Acute myeloid leukemia
        
        Breast Cancer
      - Corticosteroids & immunosuppressives aggravate the already defective immunity
  - - - Constitutional symptoms
      - Disease bulk
      - Stage
      - Extent of splenic involvement