Page 284 - Essential Haematology

270 / Chapter 20 Non-Hodgkin lymphoma

Angioimmunoblastic l ymphadenopathy S é zary s yndrome

Angioimmunoblastic lymphadenopathy usually In S é zary syndrome there is dermatitis, erythro-
occurs in elderly patients with lymphadenopathy, derma, generalized lymphadenopathy and circulat-
+

hepatosplenomegaly, skin rashes and a polyclonal ing T - lymphoma cells. The cells are usually CD4
increase in serum IgG. Patients are treated with and have a folded or cerebriform nuclear chromatin.
chemotherapy or other agents such as thalidomide Initial treatment of these conditions is by local irra-
or ciclosporin. diation, topical chemotherapy or photochemother-
apy with psoralen and ultraviolet light (PUVA).
Chemotherapy (e.g. CHOP) may be needed but is
Mycosis f ungoides
rarely effective for long.

Mycosis fungoides is a chronic cutaneous T - cell
lymphoma that presents with severe pruritus and Adult T - c ell l eukaemia/ l ymphoma

psoriasis - like lesions (Fig. 20.19 ). Ultimately, deeper

organs are affected, particularly lymph nodes, This is associated with human T - cell leukaemia/

spleen, liver and bone marrow. lymphoma virus type 1 (HTLV - 1) infection (see
Chapter 18 ) .

Enteropathy - a ssociated T - c ell l ymphomas
Enteropathy - associated T - cell lymphomas are asso-
ciated with coeliac disease and have a very poor
response to treatment. Trials using high - dose meth-
otrexate and autografts are in progress.

Anaplastic l arge c ell l ymphoma
Anaplastic large cell lymphoma is particularly
common in children and is usually of T - cell pheno-
+
type. The disease is CD30 and usually associated

with the t(2; 5) (p23; q35) translocation. Th is leads
to overexpression of anaplastic lymphoma kinase
(ALK). It has an aggressive course characterized by
systemic symptoms and extranodal involvement.
ALK – cases occur and have a worse prognosis.
ALK + large B - cell lymphoma is also described.

Histiocytic and d endritic c ell n eoplasms
Th ese are rare tumours including dendritic and
macrophage - derived sarcomas which may be local-
ized or disseminated. They usually present as

tumours at extranodal sites, especially the intestinal
tract, skin and soft tissues. Systemic symptoms are
present. The outlook is poor except for those with

small localized tumours who may do well.
Langerhans ’ cell histocytosis, a clonal disease of his-

Figure 20.19 Mycosis fungoides. tiocytes, is discussed on page 122 .