Page 256 - Essential Haematology

242 / Chapter 18 Chronic lymphoid leukaemias

(a) (b)

Figure 18.7 Hairy cell leukaemia: (a) peripheral blood fi lm showing typical ‘ hairy ’ cells with oval nuclei and
fi nely mottled pale grey – blue cytoplasm with an irregular edge; (b) bone marrow trephine.

entation and the lymphocyte count is rarely over Lymphocytosis may also be seen in other types
9
20 × 10 /L. Monocytopenia is a distinctive feature. of non - Hodgkin lymphoma (e.g. follicular, mantle

The blood film reveals a variable number of unusual cell, diffuse large B cell) and are discussed further
large lymphocytes with villous cytoplasmic projec- in Chapter 20 .
tions (Fig. 18.7 ). Immunophenotyping shows
CD22, FMC7 and CD103 positivity in most cases

(Table 18.2 ). The hairy cells stain for tartrate - T - c ell d iseases

resistant acid phosphatase (TRAP). Th e bone
marrow trephine shows a characteristic appearance T - c ell p rolymphocytic l eukaemia

of mild fibrosis and a diffuse cellular infi ltrate

This presents as B - PLL with a high white cell count
(Fig. 18.7 ).
but lymphadenopathy is more marked and skin
There are several effective treatments for HCL

lesions and serous effusions are common. Most are

and a patient can expect a long - term remission. Th e +
CD4 .
treatment of choice is 2 - chlorodeoxyadenosine
(CDA) or deoxycoformycin (DCF) and both
agents achieve responses in over 90% of cases. In
Large g ranular l ymphocytic l eukaemia
two - thirds of cases no relapse occurs, even after
5 – 10 years. HCL was one of the fi rst diseases Large granular lymphocytic leukaemia (LGL - L) is
in which α - interferon was shown to be eff ective characterized by the presence of circulating lym-
and it remains an excellent treatment. Th ese phocytes with abundant cytoplasm and large
drugs have largely replaced the need for splenec- azurophilic granules (Fig. 18.8 a). Such cells may
tomy or combination chemotherapy. Rituximab be either T or natural killer (NK) cells and
can be combined with CDA or DCF for relapsed show variable expression of CD16, CD56 and
cases. CD57. Cytopenia, especially neutropenia, is the
main clinical problem although anaemia, splenom-
egaly and arthropathy with positive serology
Lymphocytosis in n on - Hodgkin
l ymphomas for rheumatoid arthritis are also common. Th e
mean age is 50 years. Treatment may not be needed
Some cases of splenic marginal zone lymphoma but, if required, steroids, cyclophosphamide,
show circulating monoclonal B lymphocytes with a ciclosporin or methotrexate may relieve the
villous cell outline and were previously termed cytopenia. Granulocyte colony - stimulating factor
‘ splenic lymphoma with villous lymphocytes ’ (see (G - CSF) has been used in cases associated with
p. 265 ) . neutropenia.