Clinical Lymphoma, Myeloma & Leukemia

Multiple myeloma (MM) and non-Hodgkin’s lymphoma (NHL) are lymphoproliferative diseases. The occurrence of both MM and other B cell lymphoproliferative disorder in the same patient is very vare and only a few cases have been described previously1-6). We report here a case of a patient who had both MM and NHL with IgA lambda monoclonal gammopathy at the time of presentation and we discuss the possible pathogenetic mechanism of the two disorders.

A 58-year-old man presented with lower extremity petechiae, melena and weight loss during the previous 1 year. On examination he appeared acutely ill and pale. There was no adenopathy or hepatosplenomegaly. Complete blood count showed hemoglobin 8.3g/dl, protein 7.2g/dl, albumin 2.0g/dl, creatinine 0.9mg/dl. Peripheral bood smear revealed moderately increased Rouleaux formation and presence of plasma cells. Chest X-ray showed minimal pleural effusion in both hemithoraces. Serum electrophoresis revealed a monoclonal peak in the gamma globulin region, identified IgA lambda on immunoelectrophoresis. Free lambda light chain was present in the urine as well. Serum IgG was 333mg/dl, IgA 5850mg/dl, IgM 52mg/dl. Skeletal X-ray survey demonstrated no osteolytic lesion.

Bone marrow aspiration smears revealed 0.6% of plasmablasts and 21.8% of plasma cells and the histological examination demonstrated a diffuse infiltration of atypical plasma cells coexisting with localized collections of monotonous neoplastic lymphoid cells. Surface and intracytoplasmic immunoglo bulin were evaluated by a direct immunofluo rescence method using goat-antihuman Ig labeled with FITC. Immunofluorescent studies revealed lymphoid populations with bright surfacefluorescence for IgA lambda, as well as the presence of IgA lambda in the cytoplasm of plasma cells. Pleural fluid contained atypical plasma cells and neoplastic small lymphocytes and its immunoelectrophoresis revealed IgA lambda monoclonal gammopathy. Esophagogastroduodenoscopic examination was normal. Contrast enhanced small bowel radiography demonstrated only mucosal irregularities and luminal narrowing of the jejunum. Abdominal CT scan with oral contrast revealed an irregular mass in the jejunum with multiple lymph node enlargement. Exploratory laparotomy was performed, revealing anunresectable mass in the jejunum and a small amount of ascites. The characteristics of ascites were similar to the pleural fluid. Biopsy of mesenteric lymph node disclosed malignant lymphoma of diffuse small cell type, and its immunochemical studies showed diffuse positivity for pan-B marker. 

In terms of patient management, treatment must be directed to the more life-threatening condition. In our case, lymphoma was a more serious condition because of intestinal bleeding.

This case offers additional support to the theory of malignant transformation of MM and NHL. Opportunities to study and understand the unique natural history and evolutional dynamics for this transformation are rare. We would recommend further multicenter comparisons of slimilar cases in a broad effort to better define this complicated disease process.

Regards

Amalia Azzariti

Managing Editor

Journal of Clinical Oncology and Cancer Research