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There are a number of plasma cell and lymphoplasmacytic neoplasms characterized by specific damaging effects of a paraprotein rather than by the more usual features of a lymphoid neoplasm [1,2]. Sometimes the haematological and pathological features would lead to a diagnosis of monoclonal gammopathy of undetermined significance (MGUS) if it were not for the effects of the paraprotein. In other patients, there is an overt neoplasm at the onset but, in addition, the damaging effects of a paraprotein are apparent. An overt neoplastic condition may emerge some years later in patients in whom none was apparent at onset. The words ‘primary’ or ‘essential’ are sometimes used when there is no overt associated neoplasm, for example ‘primary amyloidosis’ or ‘essential cryoglobulinaemia’.

Clinical features

The main clinical features are those resulting from the specific effects of the paraprotein in an individual condition [1–7]. These are summarized in Table 27.1. Patients with polyneuropathy, organomegaly (hepatomegaly, splenomegaly, lymphadenopathy), endocrinopathy, M-protein and skin (POEMS) syndrome may not have all the manifestations. Mandatory diagnostic criteria are polyneuropathy and a monoclonal M band together with one or more major criteria (Castleman disease, osteosclerotic lesions, elevated VEGF) and one or more minor criteria (organomegaly, endocrinopathy, skin changes, thrombocytosis, extravascular volume overload) [8]. Alpha heavy chain disease is a form of MALT lymphoma (see Chapter 9).

Table 27.1Syndromes resulting from synthesis of a monoclonal paraprotein.

Haematological and pathological features

Haematological and pathological features also differ, according to the characteristics ...

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