melphalan and Sjogren-s-Syndrome

Light chain deposition disease is a systemic disorder characterised by tissue deposition of monoclonal immunoglobulin light chains without tinctorial properties. It has been exceptionally reported with salivary involvement mimicking Sjögren's syndrome and peripheral neuropathy.. We report a case of light chain deposition disease associated with plasma cell dyscrasia presenting as sicca syndrome with salivary glands hypertrophy and polyneuropathy successfully treated by high dose melphalan and autologous blood stem transplantation.. Light chain deposition disease should be recognized as an aetiology of sicca syndrome and peripheral neuropathy. Further studies should assess the prevalence of sicca syndrome in light chain deposition disease and better characterise the neurological manifestations.

Topics: Biopsy; Diagnosis, Differential; Electromyography; Female; Humans; Hypertrophy; Immunoglobulin kappa-Chains; Melphalan; Paraproteinemias; Peripheral Nervous System Diseases; Salivary Glands; Sialography; Sjogren's Syndrome; Stem Cell Transplantation; Tomography, X-Ray Computed; Transplantation, Autologous

A 67-year-old woman was admitted to our hospital because of breathlessness. Systemic amyloidosis had been diagnosed 5 years previously. Her chest X-ray film showed multiple nodules in both lung fields. Chest computed tomography (CT) revealed some of the nodules had calcifications. Bronchoscopy demonstrated amyloid deposits in the bronchial walls. The serum titer of anti-SS-A antibody was high. Results of both the Schirmer Test and the Rose-Bengal Test were positive. The final diagnosis was systemic amyloidosis with Sjögren's syndrome. She was treated by chemotherapy using high dose melphalan with autologous peripheral blood stem cell transplantation (PBSCT). It was obvious that her chest X-ray film findings and bronchoscopic findings had improved 9 months after high dose chemotherapy with PBSCT. The disappearance of M protein and improvement of thirst, a symptom of Sjögren's syndrome, were also observed.

Topics: Aged; Amyloidosis; Antineoplastic Combined Chemotherapy Protocols; Dexamethasone; Doxorubicin; Drug Administration Schedule; Female; Humans; Melphalan; Peripheral Blood Stem Cell Transplantation; Sjogren's Syndrome; Solitary Pulmonary Nodule; Transplantation, Autologous; Vincristine

There have been a number of reports on the improvement of concomitant autoimmune disease (AID) after autologous hematopoietic stem cell transplantation (SCT) performed for hematologic malignancy. However, in some cases of hematologic malignancy with AID, exacerbation of AID after autologous SCT has been reported. We have treated 27 adults with multiple myeloma with single or tandem autologous SCT. After peripheral blood stem cells were collected and stored without CD34+ cell selection or T-cell depletion, all patients received melphalan (200 mg/m2) as a conditioning regimen. In 2 patients with a history of AID (one with rheumatoid arthritis [RA] and the other with bullous pemphigoid [BP]) and in 1 patient with Sjögren syndrome, AID recurred 7 to 12 months after autologous SCT. The RA and BP were in durable remission before SCT, and no Sjögren syndrome-related disease activity was clinically documented at the time of SCT. No progression of the myeloma was observed when the AIDs recurred. The patients required systemic steroid therapy for their AID, and successful control of the disease was achieved. Our experience suggests that autologous SCT with unmanipulated stem cells for myeloma is unlikely to cure preexisting AID; rather, the AID may worsen. Transplantation physicians should be aware of this possible complication.

Topics: Aged; Antigens, CD34; Arthritis, Rheumatoid; Autoimmune Diseases; Female; Humans; Lymphocyte Depletion; Male; Melphalan; Middle Aged; Multiple Myeloma; Pemphigoid, Bullous; Peripheral Blood Stem Cell Transplantation; Recurrence; Sjogren's Syndrome; Steroids; Transplantation Conditioning; Transplantation, Autologous

Topics: Aged; Amyloidosis; Anti-Inflammatory Agents; Humans; Male; Melphalan; Prednisolone; Sjogren's Syndrome