Cancers of the haematopoietic system

Published on 09/04/2015 by admin

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21 Cancers of the haematopoietic system

Hodgkin’s disease

Pathology

The WHO classification of HD is shown in Table 21.1. Nodular sclerosis is the common subtype in young adults (more common in females), which presents with early stage supradiaphragmatic disease whereas mixed cellularity presents with generalized lymphadenopathy or extranodal disease with B symptoms. Lymphocyte depletion type presents with advanced stage disease with extranodal involvement and aggressive clinical course.

Table 21.1 WHO classification of Hodgkin’s lymphoma

Classical Hodgkin’s lymphoma Frequency
Nodular sclerosis Hodgkin’s lymphoma (grades 1 and 2) 60–70%
Mixed cellularity Hodgkin’s lymphoma 20–30%
Lymphocyte-rich classical Hodgkin’s lymphoma (LRCHL) 3–5%
Lymphocyte-depleted Hodgkin’s lymphoma (LDHL) 0.8–1%
Nodular lymphocyte-predominant Hodgkin’s lymphoma (LPHL) 3–5%

LRCHL usually presents in young males with localized cervical node involvement with no B symptoms.

Histologically HD is characterized by the presence of Hodgkin and Reed–Sternberg (H-RS) cells in a background of non-neoplastic cells such as lymphocytes, histiocytes, neutrophils, eosinophils and monocytes. Classical HD is characterized by CD30 positive H-RS cells and the nodular lymphocyte predominant form of HL (LPHL) with CD20 positive lymphocytic and histiocytic (L and H) cells. In classical HD, RS cells stain positive for CD15 (80%) and CD30 (90%).

Investigations and staging

Staging

Cotswolds modification of the Ann Arbor Classification is used for staging (Table 21.2).

Table 21.2 The Cotswolds modification of Ann Arbor staging for lymphoma

Stage I Involvement of a single lymph node region or lymphoid structure or involvement of a single extralymphatic site (IE)
Stage II Involvement of two or more lymph node regions on the same side of the diaphragm; localized contiguous involvement of only one extranodal organ or site and lymph node region(s) on the same side of the diaphragm (IIE)
Stage III Involvement of lymph node regions on both sides of the diaphragm (III),which may also be accompanied by involvement of the spleen (IIIS) or by localized contiguous involvement of only one extranodal organ site (IIIE) or both (IIISE)
Stage IV Diffuse or disseminated involvement of one or more extranodal organs or tissues, with or without associated lymph node involvement
Designations applicable to any disease stage
A: No B symptoms
B: Presence of B symptoms
X: Bulky disease (a widening of the mediastinum by more than one third of the chest or presence of a nodal mass with a maximal dimension greater than 10 cm)

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

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.