Pleuropulmonary Manifestations of Waldenstrom’s Macroglobulinemia

Abstract

aldenstrom's macroglobulinemia ( WMG ) is a rare lymphoproliferative disorder of insidious onset and relatively benign clinical course which predominantly affects the elderly. WMG is characterized by anemia, an elevated erythrocyte sedimentation rate, lymphocytic tumor infiltration of the bone marrow, and a monoclonal IGM gammop athy.' Peripheral lymphadenopathy, hepatosplenomegaly, and ocular or central nervous symptoms may variably be present. In contrast to other tumors of lymphocytic origin, the recorded incidence of pleuropulmonary involvement in patients with macroglobulinemia is quite low. Excluding congestive heart failure and infection, pulmonary involvement with WMG has varied from 0 percent to 3 percent in several seriesSu This compares to a 30 percent incidence of thoracic tumor involvement in patients with other ly~nphomas.~ Recent experience with two patients with WMG who presented as challenging pulmonary diagnostic problems stimulated this review of our experience to define the incidence and clinical features of pulmonary involvement. Surprisingly, five of the U) patients reviewed demonstrated thoracic involvement. The details of these five patients and a review of ten previously reported patients with pulmonary syndromes secondary to WMG form the basis of this report. with abnormal chest x-ray pic- in the absence of lymphadenopathy, hepatosplenomegaly, and ocular changes and inchded two patients who were not anemic. Pulmonary biopay or necropsy specimens from four pa tients showed an infiltration of hutg, pleura, or hilar lymph nodes by lymphocytes and phacytoid forms. The infiltrate tended to be patchy with sheets of lymphocytes replacing the long parenchyma In some areas, the lympbocytk infiltrate was exclusively interstitial mimicing the histologic pattern of lymphocytic interstitial peemonia In the absence of therapy tbe pulmonary abnormalities were inshliowly progressive over yervs. Three of the four patients treated with chlorambucil achieved slgnificad radiographic clearing in one to three months. One patient died from dBose pl~hnonary involvement in her eighth year of chlorambodl tberapy.