melphalan and Granuloma

The association of sarcoidosis with Hodgkin disease and non-Hodgkin lymphoma is well known. However, multiple myeloma also can occur rarely in association with sarcoidosis. We describe a patient with sarcoidosis who subsequently developed multiple myeloma. The patient was a 49-year-old woman with a 4-year history of severe, chronic, active sarcoidosis involving her lungs, lymph nodes, eyes, and bone marrow. During the initial clinical workup, a serum monoclonal paraprotein was detected and bone marrow examination revealed a slight increase in plasma cells (4%), in addition to noncaseating granulomas. Thus, the diagnoses of monoclonal gammopathy of undetermined significance and sarcoidosis were established simultaneously. She sought medical attention for her current illness when she developed low back pain and weakness of her lower extremities. Serum protein electrophoresis and immunofixation revealed a monoclonal paraprotein, immunoglobulin (Ig) G kappa type, and quantification revealed an IgG level of 46.67 g/L (normal, 5.88--15.73 g/L). Bone marrow aspiration and biopsy revealed multiple myeloma and sarcoidosis. Including this patient, 11 cases of sarcoidosis and multiple myeloma have been reported to date, including 3 patients with monoclonal gammopathy of undetermined significance preceding the onset of multiple myeloma. In this case, as in most of the cases reported previously, sarcoidosis preceded the development of multiple myeloma.

Topics: Antineoplastic Agents, Alkylating; Bone Marrow Cells; Drug Therapy, Combination; Female; Granuloma; Humans; Immunoenzyme Techniques; Immunoglobulin kappa-Chains; Melphalan; Middle Aged; Multiple Myeloma; Prednisone; Sarcoidosis; Treatment Outcome

We present a case report of necrobiotic xanthogranuloma (NXG) in a 76-year-old Caucasian lady occurring as a nodule in a blepharoplasty scar. NXG is a rare histiocytic disease with progressive orbital and systemic features. Management options of excision biopsy or chemotherapy are discussed.

Topics: Aged; Biopsy, Needle; Cicatrix, Hypertrophic; Drug Therapy, Combination; Eyelid Diseases; Female; Follow-Up Studies; Granuloma; Humans; Immunohistochemistry; Melphalan; Necrobiotic Disorders; Orbital Diseases; Prednisolone; Recurrence; Risk Assessment; Treatment Outcome; Xanthomatosis

Topics: Antineoplastic Agents, Alkylating; Combined Modality Therapy; Female; Glucocorticoids; Granuloma; Humans; Melphalan; Middle Aged; Necrobiotic Disorders; Paraproteinemias; Prednisolone; Skin; Skin Transplantation; Ulcer; Xanthomatosis

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 li

Topics: Antineoplastic Agents, Alkylating; Antineoplastic Combined Chemotherapy Protocols; Chlorambucil; Cyclophosphamide; Female; Glucocorticoids; Granuloma; Humans; Leukemia, Lymphocytic, Chronic, B-Cell; Lymphoma, Non-Hodgkin; Melphalan; Middle Aged; Necrobiotic Disorders; Paraproteinemias; Skin Diseases; Treatment Failure; Treatment Outcome; Xanthomatosis

Topics: Aged; Antineoplastic Agents, Alkylating; Female; Granuloma; Humans; Immunoglobulin lambda-Chains; Melphalan; Necrobiotic Disorders; Paraproteinemias; Treatment Outcome; Xanthomatosis

Topics: Aged; Female; Granuloma; Humans; Immunoglobulin G; Immunoglobulin kappa-Chains; Melphalan; Necrobiotic Disorders; Paraproteinemias; Prednisone; Xanthomatosis

A 66-year-old man with necrobiotic xanthogranuloma associated with paraproteinaemia is described. He also had long-standing normolipaemic plane xanthomata. Treatment was with melphalan 0.15 mg/kg body weight/day. Initially, three 5-day courses were given at 4-weekly intervals but a fourth course was necessary after a further 4 months because of a deterioration in both his skin and associated systemic symptoms.

Topics: Aged; Granuloma; Humans; Hypergammaglobulinemia; Immunoglobulin G; Male; Melphalan; Necrobiosis Lipoidica; Xanthomatosis

The conditions of three patients who had generalized granuloma annulare responded favorably to treatment with alkylating agents. This mode of therapy in two cases was chosen on the basis of recent evidence that implicates delayed hypersensitivity mechanisms as forming part of the pathogenesis of granuloma annulare. Two patients had rapid clearing of their lesions. The response in one of these patients was observed during therapy for a concomitant plasma cell dyscrasia. The third patient had an unusual form of granuloma annulare that had evolved during a period of 28 years, with hand deformities secondary to fascial and tendon involvement and the production of contractures and lymphedema, which responded partially to therapy. The results lend further support to data implicating cellular immunity to granuloma annulare.

Topics: Aged; Alkylating Agents; Chlorambucil; Female; Granuloma; Humans; Male; Melphalan; Middle Aged; Prednisone; Skin Diseases