Two women with lymphoplasmocytoid immunocytoma (Waldenstrom's macroglo
bulinaemia) have been followed up, one for 23 years and the other for
18. In the first patient the disease presented at the age of 40 years
with lassitude and paraesthesiae. Investigation revealed monoclonal Ig
M-lambda with a serum IgM of 3350 mg/dl and marrow infiltration by lym
phoplasmocytoid cells. As there were no symptoms, apart from migraine
attacks which responded well to plasmapherese, and because haemopoiesi
s was unaffected and the lymph nodes were not enlarged, no cytostatic
has so far been given. The proportion of lymphoplasmoid cells in the m
arrow is at present 50%. In the second patient the disease presented a
t the age of 33 with involvement of stomach and lung, and a conglomera
te tumour in the upper abdomen. The serum showed an M-gradient togethe
r with raised IgM (3056 mg/dl). Chemotherapy (COPP protocol) and local
irradiation of the stomach achieved full remission. At intervals of s
everal years there have been histologically confirmed recurrences in t
he hypopharynx, ovaries, hilum of left lung and, most recently, in the
upper abdomen once more. These have been successfully treated by rese
ction and local radiotherapy (hypopharynx), operation followed by chem
otherapy (COP protocol) (ovaries, upper abdomen) or by radiotherapy al
one (hilum of lung - 50 Gy).