Page 281 - Essential Haematology

Chapter 20 Non-Hodgkin lymphoma / 267

infi ltration. The cells have characteristically angular

nuclei in histological sections (Fig. 20.4 ) and often
circulate in the blood (Fig. 20.6 ).
Current treatment regimens include:
1 R - CHOP
2 Intensive combinations such as R - Hyper -
CVAD
3 Cytosine arabinoside, rituximab and autologous
SCT and
4 Purine analogues with or without cyclophos-

phamide and rituximab.

The roles of bortezomid and lenalidomide are being
investigated. Allogeneic SCT may also be consid-
(a)

ered in some patients. The prognosis is usually poor
and overall survival is 4 – 6 years, although 15% of
patients show an indolent course similar to CLL.

Heavy - c hain d iseases
These are rare disorders in which neoplastic cells

secrete only incomplete ‘ immunoglobulin ’ heavy

chains ( γ , α or μ ). The most common is α chain
disease which occurs in the Mediterranean area and
starts as a malabsorption syndrome which may
progress to systemic lymphoma.

High - g rade n on - Hodgkin l ymphoma
(b)

Diffuse l arge B - c ell l ymphomas (DLBCL)
Figure 20.15 Follicular lymphoma: immunostain.

DLBCL are a heterogeneous group of disorders rep-
(a) CD20 expressed in tumour cells. (b) CD3 is
confi ned to reactive T cells. resenting the classic ‘ high - grade ’ lymphomas. As
such they typically present with rapidly progressive
lymphadenopathy associated with a fast rate of cel-
lular proliferation. Progressive infi ltration may
Mantle c ell l ymphoma

affect the bone marrow, gastrointestinal tract, brain
Mantle cell lymphoma is derived from pre - germinal (Fig. 20.16 ), spinal cord, the kidneys or other
centre cells localized in the primary follicles or in organs.
the mantle region of secondary follicles. It has a A variety of clinical and laboratory fi ndings are
+
+
characteristic phenotype of CD19 and CD5 (like relevant to the outcome of therapy. According to
+
−
CLL) but in contrast to CLL is CD22 and CD23 . the International Prognostic Index these include

A specific t(11; 14) translocation juxtaposes the age, performance status, stage, number of extran-
cyclin D1 gene to the immunoglobulin heavy - chain odal sites and serum LDH (Table 20.4 ). Bulky
gene, and leads to increased expression of cyclin D1. disease (major mass > 5 cm in diameter) and prior

Presence of this translocation is required for diag- history of low - grade disease or AIDS are also associ-

nosis. Clinical presentation is typically with lym- ated with a poorer prognosis. There are a variety of
phadenopathy and often there is bone marrow histological patterns including centroblastic, immu-