1/8. Monoclonal gammopathy after low-grade MALT lymphoma: evidence for a second neoplasm.We report the case of a patient with lymphoma of the salivary gland, at first diagnosed as lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) but later found to infiltrate the bone marrow. At diagnosis, the patient had a polyclonal increase of gamma-globulins. Five years after initial diagnosis, the patient presented with monoclonal gammopathy and infiltration of the bone marrow with neoplastic cells. Initially, the patient had received chemotherapy with different protocols (including etoposide, cyclophosphamide, fludarabin, methotrexate, and vincristine), none of which induced a lasting response. Therapy with rituximab (chimeric anti-CD20 monoclonal antibody) finally led to partial remission. Eighteen months after rituximab, progressive lymphoma in the abdomen and a monoclonal gammopathy developed. The bone marrow showed infiltration by lymphoplasmacytoid cells (monoclonal expression of the light-chain type lambda, positive for CD20, heterogeneous expression of CD45). The patient achieved another short clinical response with 4 cycles of the CHOP-protocol, but soon the lymphoma progressed again. Five years and 8 months after the initial diagnosis, the patient died from septicemia and progressive lymphoma. By polymerase chain reaction (PCR) for the IgH gene it was shown that lymphoma cells were initially oligoclonal in the salivary gland and, later, biclonal in the bone marrow. Sequencing of two bands of apparently same length showed that these manifestations of lymphoma were not identical. Taken together, our data show that the initial low-grade oligoclonal MALT lymphoma was no longer present and a more aggressive biclonal lymphoma with plasmacytoid differentiation had developed. The new lymphoma was clonally distinct and produced high amounts of monoclonal IgG lambda by immunoelectrophoresis. The relationship of the second lymphoma to the initial MALT lymphoma is discussed.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
2/8. necrobiotic xanthogranuloma associated with paraproteinemia and non-Hodgkin's lymphoma developing into chronic lymphocytic leukemia: the first case reported in the literature and review of the literature.A 56-year-old married female presented in May 1998 with a 5-month history of xanthelasma of the eyelids, followed 4 months later by two enlarged lymph nodes of the left side of the neck and three of the left axilla. At the same time, she developed xanthomatous patches on the face, neck, and shoulders (Fig. 1). The cutaneous lesions were xanthomatous nodules and plaques, affecting the periorbital regions. Later, the whole face was affected, followed by ulcerated lesions on the scalp, chest, back, and extremities (Fig. 2). The skin lesions became painful, pruritic, ulcerated tumors (Fig. 3). In July 1998, computed tomography (CT) scans of the chest and abdomen with contrast medium showed pretracheal, bilateral axillary, right retrochural, paracaval, aortocaval, and para-aortic lymph node enlargement. These findings were suggestive of lymphoma. CT scan also showed slight heterogeneous hypodensity in the upper part of the right lobe of the liver, suggesting fatty infiltration. The spleen, pancreas, and suprarenal glands appeared normal. One cervical and two left axillary lymph nodes were excised. They revealed total replacement of the nodular architecture by a diffuse proliferation of mature lymphoid cells having small nuclei and a crumbled chromatin pattern, and very rare mitosis. It was concluded from the lymph node biopsies that these changes were typical of non-Hodgkin's lymphoma, diffuse and small cell type, of low-grade malignancy. A bone marrow aspirate showed a marrow heavily infiltrated by lymphoid cells with some immaturity. The megakaryopoiesis was adequate. Trephine biopsies showed similar changes. iron stores appeared to be absent. The bone marrow picture was consistent with diffuse, well-differentiated non-Hodgkin's lymphoma, developing into chronic lymphocytic leukemia (CLL). endoscopy showed antral-type gastric mucosa exhibiting mild chronic gastritis. skin biopsy from a fresh lesion on the back showed a diffuse inflammatory cell infiltrate with collections of histiocytic cells. It also showed necrobiotic foci, surrounded by mixed inflammatory cells, dark palisaded foamy histiocytes, and a few Touton giant cells. These findings are compatible with necrobiotic xanthogranuloma (NXG) (Figs 4 and 5). blood film showed normochromic, normocytic erythrocytes with anisopoikilocytotic leukocytes and normal platelets. The sedimentation rate was 90 mm in the first hour. The blood picture also showed monoclonal IgG paraprotein (3170 mg/dL) of the kappa light chain type. The patient was treated by the oncologist for her lymphoma, and was given Cytoxan, prednisolone, endoxan, Leukeran, and melphalan. She showed an excellent response to pulsed treatment with steroids (60 mg prednisolone orally daily for 5 days, repeated every month for 6 months). She also responded to Leukeran at a dose of 5 mg daily for 5 days every month for 6 months, and showed regression in the size of the lymph nodes. The treatment of her skin lesions was unsatisfactory in spite of the fact that she was given cyclosporine and both systemic and topical corticosteroids.- - - - - - - - - - ranking = 0.5keywords = gland (Clic here for more details about this article) |
3/8. Extramedullary plasmacytoma of the thyroid associated with a serum monoclonal gammopathy.Extramedullary plasmacytoma is a malignant plasma cell dyscrasia that is most commonly found in the head and neck. Progression to disseminated disease in the form of multiple myeloma occurs in 17% to 32% of cases. Herein, we describe a patient with extramedullary plasmacytoma of the thyroid gland in whom a serum monoclonal gammopathy resolved with surgery and irradiation of the primary. Although rare, this diagnosis should be considered in patients presenting with obstructive thyromegaly and autoimmune thyroiditis.- - - - - - - - - - ranking = 0.5keywords = gland (Clic here for more details about this article) |
4/8. Plasma-cell dyscrasia with polyneuropathy and endocrine disorders associated with dysfunction of salivary glands.A case of plasma-cell dyscrasia with polyneuropathy and endocrine disorders that showed dysfunction of the salivary glands is reported. A 49-year-old Japanese man noticed swelling of the cervical lymph nodes and numbness in the lower extremities in May 1983. Histological examination of the enlarged cervical lymph nodes revealed many follicles penetrated by radial capillaries and proliferation of capillaries and plasma cells in the interfollicular area, forming Castleman disease-like lesions. The patient complained of a dry mouth and noticed swelling of the submandibular salivary gland in April 1984. Microscopic examination of this gland revealed an angiofollicular lymphoid lesion resembling that in the lymph nodes. He also suffered from an endocrine disturbance characterized by increased serum adrenocorticotropic hormone and impotence. This is the second reported case of plasma-cell dyscrasia with polyneuropathy and endocrine disorders that showed dysfunction of exocrine secretion. This case indicates that attention must be paid to organs of exocrine secretion as well as of endocrine secretion in this disease.- - - - - - - - - - ranking = 3.5keywords = gland (Clic here for more details about this article) |
5/8. Benign lymphoepithelial lesion in a patient with Sjogren's disease and an IgG paraprotein.Clinical and laboratory investigations were performed on a patient presenting with xerostomia and swelling of the parotid gland. Lymphoid infiltration of the salivary glands (parotid and labial) and paraproteinaemia were found. Immunoperoxidase studies of labial and parotid glands revealed that local immunoglobulin synthesis was essentially polyclonal although monoclonal staining of Russell bodies within the labial tissue suggested that at least some of the serum paraprotein was being synthesised locally. The implications of these findings are discussed in terms of diagnosis and treatment.- - - - - - - - - - ranking = 1.5keywords = gland (Clic here for more details about this article) |
6/8. An autopsy case of light chain deposition disease.This report describes a case of light chain deposition disease (LCDD) with unusual findings of fibrillar structures in the deposits and marked calcification in several organs. A forty-year-old man was initially diagnosed with LCDD in 1987, and died of sepsis three and one-half-years later. Histological examination of autopsy specimens demonstrated eosinophilic amorphous materials, which differed from amyloid, in vessel walls or around parenchymal cells in almost every organ examined. Ultrastructurally, in addition to granular deposits, fibrillar structures were also seen in the deposits. Marked calcification was present in the myocardium, skeletal muscles, adrenal glands and arteries.- - - - - - - - - - ranking = 0.5keywords = gland (Clic here for more details about this article) |
7/8. Salivary gland lymphoplasmacytoid lymphoma with nodular cutaneous amyloid deposition and lambda chain paraproteinaemia.We report the case of an 83-year-old woman who developed a lymphoplasmacytoid lymphoma of the salivary glands with extension to the skin of the cheeks and laterocervical area. The most remarkable feature was the massive amyloid deposition that gave the cutaneous lesions a distinctive clinical appearance, similar to that observed in cases of nodular cutaneous amyloidosis. An IgM-lambda paraprotein was also detected in the serum.- - - - - - - - - - ranking = 2.5keywords = gland (Clic here for more details about this article) |
8/8. Systemic involvement in scleredema of Buschke associated with IgG-kappa paraproteinaemia.Scleredema is a rare primary cutaneous mucinosis. Systemic involvement is uncommon and histological confirmation is often lacking. We report a case of a 60-year-old man with scleredema and evidence of mucin deposition on biopsies from multiple extracutaneous sites. The bone marrow, nerve, hepatic and salivary gland involvement seen on histology in our patient has not, to our knowledge, been previously reported in this condition.- - - - - - - - - - ranking = 0.5keywords = gland (Clic here for more details about this article) |