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6. Immune abnormalities: Since lymphoid neoplasms arise  The salient features of the 4 histologic subtypes of HD  369
           from immune cells of the body, immune derangements  are summarised in Table 14.9.
           pertaining to the cell of origin may accompany these cancers.
           This is particularly so in B-cell malignancies and include  Reed-Sternberg Cell
           occurrence of autoimmune haemolytic anaemia, autoimmune
           thrombocytopenia and hypogammaglobulinaemia.        The diagnosis of Hodgkin’s disease rests on identification of
                                                               RS cells, though uncommonly similar cells can occur in
              With this background, we now turn to discussion of some  infectious mononucleosis and other forms of lymphomas.
           common and important examples of lymphoid malignancies.
                                                               Therefore, additional cellular and architectural features of
                                                               the biopsy must be given due consideration for making the
           HODGKIN’S DISEASE
                                                               histologic diagnosis.
           Hodgkin’s disease (HD) primarily arises within the lymph  There are several morphologic variants of RS cells which
           nodes and involves the extranodal sites secondarily. This  characterise different histologic subtypes of HD (Fig. 14.16):
           group comprises about 8% of all cases of lymphoid   1. Classic RS cell is a large cell which has characteristically
           neoplasms. The incidence of the disease has bimodal peaks—  a bilobed nucleus appearing as mirror image of each other
           one in young adults between the age of 15 and 35 years and  but occasionally the nucleus may be multilobed. Each lobe
           the other peak after 5th decade of life. The HD is more  of the nucleus contains a prominent, eosinophilic, inclusion-  CHAPTER 14
           prevalent in young adult males than females. The classical  like nucleolus with a clear halo around it, giving an owl-eye
           diagnostic feature is the presence of Reed-Sternberg (RS) cell  appearance. The cytoplasm of cell is abundant and
           (or Dorothy-Reed-Sternberg cell) (described later).
                                                               amphophilic.
           Classification                                      2. Lacunar type RS cell is smaller and in addition to above
                                                               features has a pericellular space or lacuna in which it lies,
           The diagnosis of HD requires accurate microscopic diagnosis
           by biopsy, usually from lymph node, and occasionally from  which is due to artefactual shrinkage of the cell cytoplasm.
           other tissues. Unlike NHL, there is only one universally  It is characteristically found in nodular sclerosis variety of
           accepted classification of HD i.e. Rye classification adopted  HD.
           since 1966. Rye classification divides HD into the following  3. Polyploid type (or popcorn or lymphocytic-histiocytic
           4 subtypes:                                         i.e. L and H) RS cells are seen in lymphocyte predominance
           1. Lymphocyte-predominance type                     type of HD. This type of RS cell is larger with lobulated
           2. Nodular-sclerosis type                           nucleus in the shape of popcorn.
           3. Mixed-cellularity type                           4. Pleomorphic RS cells are a feature of lymphocyte
           4. Lymphocyte-depletion type.                       depletion type. These cells have pleomorphic and atypical
              However, the WHO classification of lymphoid neoplasms  nuclei.
           divides HD into 2 main groups:                         The nature and origin of RS cells, which are the real neo-  Disorders of Leucocytes and Lymphoreticular Tissues
           I. Nodular lymphocyte-predominant HD (a new type).  plastic cells in HD, have been a matter of considerable debate.
           II. Classic HD (includes all the 4 above subtypes in the Rye  One main reason for this difficulty in their characterisation
           classification).                                    is that in HD, unlike most other malignancies, the number
              Central to the diagnosis of HD is the essential  of neoplastic cells (i.e. RS cells) is very small (less than 5%)
           identification of Reed-Sternberg  cell though this is not the sole  which are interspersed in the predominant reactive cells.  In
           criteria (see below).                               general, the number of RS cells is inversely proportional to


             TABLE 14.9: Modified WHO Classification of Hodgkin’s Disease.
              Histologic Subtype   Incidence     Main Pathology            RS Cells                Prognosis
           I.  CLASSIC HD
           Lymphocyte-predominance  5%           Proliferating lymphocytes,  Few, classic and polyploid  Excellent
                                                 a few histiocytes         type, CD15–, CD30–, CD20+
           Nodular sclerosis       70%           Lymphoid nodules,         Frequent, lacunar type,  Very good
                                                 collagen bands            CD15+, CD30+
           Mixed cellularity       22%           Mixed infiltrate          Numerous, classic type,  Good
                                                                           CD15+, CD30+
           Lymphocyte-depletion    1%            Scanty lymphocytes,       Numerous,               Poor
           (Diffuse fibrotic and                 atypical histiocytes, fibrosis  pleomorphic type,
           reticular variants)                                             CD15+, CD30+
           II. NODULAR LYMPHOCYTE-PREDOMINANT HD
                                   2%            Proliferation of small lympho-  Sparse number of RS cells,  Chronic
                                                 cytes, nodular pattern of growth  CD45+, EMA+, CD15-, CD30-  relapsing, may
                                                                                                   transform into
                                                                                                   large B cell NHL
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