Cancers of the haematopoietic system

21 Cancers of the haematopoietic system




Hodgkin’s disease







Investigations and staging








Management


HD is a highly curable disease with long-term disease-free survival (DFS) exceeding 80%. Current efforts are to improve the chances of cure with the least long-term toxicity. Measures to preserve fertility should be discussed with all young patients prior to starting chemotherapy.


Patients with early stage disease (clinical stage I/II) are categorized as favourable and unfavourable group based on risk factors (Table 21.3). Prognosis of patients with advanced-stage (stage III and IV) disease is defined using the International Prognostic Score (IPS) (Box 21.1).


Table 21.3 EORTC risk grouping of early stage HD















Treatment group Definition
Favourable CS I–II without risk factors
Unfavourable CS I–II with ≥ 1 risk factors
Risk factors




* Erythrocyte sedimentation rate (≥50 mm/h without or ≥30 mm/h with B-symptoms).





Classical HD



Early-stage favourable


The current standard treatment is combined modality treatment, consisting of two to four cycles of ABVD chemotherapy (Boxes 21.2 and 21.3), followed by 30–35 Gy in 15–20 fractions of involved field radiotherapy (IF-RT).













Non-Hodgkin’s lymphoma




Pathology


The cell of origin is different for each subtype of lymphoma. Classification is by the updated WHO modification of the REAL (revised European and American lymphoma) classification system which is broadly divided into B-cell, T-cell/natural killer cell and Hodgkin’s disease (Table 21.4). Table 21.5 shows cytogenetic and molecular characteristics of NHL.


Table 21.4 Abridged WHO/REAL classification of NHL











B-cell neoplasms


T-cell and putative NK-cell neoplasms



Table 21.5 Cytogenetic and molecular characteristics of NHL



























Lymphoma Cytogenetics Genes
Burkitt’s



Follicular lymphoma t(14:18)(q32;q21)

Diffuse large B-cell lymphoma t(14:18)(q32;q21)+others such as p53, p16, p15  
Mantle cell lymphoma t(11;14)(q13;q32) BCL-1 or PRAD1 (11q) Ig heavy chain (14q)
Anaplastic lymphoma t(2;5)(p23:q35)






Treatment



Follicular lymphoma


Follicular lymphoma accounts for approximately 20% of all lymphomas and has a variable clinical course. The median age at presentation is 50 years. Most patients present with advanced disease and median survival is 10–15 years from diagnosis. Spontaneous regression can occur and 40% of patients transform to an aggressive histologic type. Over 90% have rearrangement of the bcl-2 gene which inhibits apoptosis.



Jun 18, 2016 | Posted by in ONCOLOGY | Comments Off on Cancers of the haematopoietic system

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