melphalan and Osteosclerosis

POEMS syndrome is a clonal plasma cell disease characterized by polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes. Significant advances have been made in the diagnosis and treatment of POEMS syndrome over the last decade. Herein, the diagnostic criteria and characteristic features are reviewed, focusing the role of characteristic features in early diagnoses. Autologous peripheral blood stem cell transplantation has become the first-line treatment for younger patients with normal organ function. Autologous transplantation has resulted in a high response rate and durable remission. Moreover, transplantation-related morbidity and mortality has been significantly reduced over the past 5 years. Induction therapy before transplantation may improve the harvest of stem cells and decrease transplantation-related morbidity. Melphalan and dexamethasone is an effective and well-tolerated treatment for older patients or those with organ dysfunction. Novel agents may also offer benefits to patients with a poor performance status or renal dysfunction, and transform transplantation eligibility.

Topics: Antibodies, Monoclonal, Humanized; Bevacizumab; Boronic Acids; Bortezomib; Castleman Disease; Chromosome Aberrations; Combined Modality Therapy; Dexamethasone; Disease Management; Drug Therapy, Combination; Genes, Immunoglobulin; Humans; Hypertension, Pulmonary; Lenalidomide; Melphalan; Osteosclerosis; Peripheral Blood Stem Cell Transplantation; Plasma Cells; POEMS Syndrome; Prognosis; Pyrazines; Severity of Illness Index; Symptom Assessment; Thalidomide; Transplantation, Autologous; Vascular Endothelial Growth Factor A

Topics: Alkylating Agents; Amyloidosis; Anemia; Anemia, Refractory; Animals; Antibodies, Monoclonal; Antineoplastic Combined Chemotherapy Protocols; Bacterial Infections; Blood Transfusion; Bone and Bones; Bone Marrow Examination; Bone Marrow Transplantation; Bone Neoplasms; Calcium; Combined Modality Therapy; Diagnosis, Differential; Heavy Chain Disease; Humans; Immunoglobulin D; Immunotherapy; Interferons; Kidney Failure, Chronic; Leukemia; Melphalan; Mice; Monoclonal Gammopathy of Undetermined Significance; Multiple Myeloma; Myeloma Proteins; Osteolysis; Osteosclerosis; Paraproteinemias; Plasma Cells; Plasmacytoma; Prednisone; Radionuclide Imaging; Waldenstrom Macroglobulinemia

A randomized double-blind study was carried out in 26 patients with multiple myeloma to compare the therapeutic effect of sodium fluoride (50 mg twice daily) plus calcium carbonate (1 g four times daily) and placebo. All patients also received melphalan and prednisone for one week every six weeks. Bone biopsies for microradiography and histology, and videodensitometry as well as conventional roentgenograms, 99mTc-polyphosphate bone scans, and bone densitometry of the mid and distal radius, were done initially and one year after therapy. Microradiography and videodensitometry studies revealed significant increases in bone formation (P less than 0.01) and bone mass (P less than 0.005) in the fluoride-calcium group. Bone trabeculae appeared thickened on roentgenograms of six of 13 fluoride-calcium-treated patients (P less than 0.02). Technetium bone scans and bone densitometry determinations proved insensitive for detection of skeletal changes. Fluoride calcium should be considered a useful adjunct in the treatment for multiple myeloma.

Topics: Bone and Bones; Bone Diseases; Calcium Carbonate; Clinical Trials as Topic; Fluorides; Humans; Melphalan; Multiple Myeloma; Osteosclerosis; Prednisone; Prospective Studies

Topics: Adult; Bone Diseases; Combined Modality Therapy; Diagnosis, Differential; Diffusion Magnetic Resonance Imaging; Female; Femur; Humans; Infarction; Melphalan; Multiple Myeloma; Osteosclerosis; Pelvic Bones; Severity of Illness Index; Stem Cell Transplantation; Thoracic Vertebrae; Treatment Outcome

Topics: Aged; Antineoplastic Combined Chemotherapy Protocols; Female; Humans; Melphalan; Multiple Myeloma; Osteosclerosis; Prednisolone; Thalidomide

Sclerotic lesions are rare in malignant monoclonal gammopathies, although they are occasionally associated with POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes). In most cases, osseous lesions in POEMS syndrome present as an isolated sclerotic deposit or a combination of both lytic and sclerotic lesions. Diffuse osteosclerosis is extremely rare and may lead to the diagnosis of multiple myeloma, classically known to present as lytic lesions in the skeleton, with or without diffuse osteoporosis. We report a 74-year-old woman with widespread and substantial osteosclerotic lesions, associated with IgA-lambda myeloma, and with no other criteria for POEMS syndrome, and who was rapidly diagnosed with compression of the spinal cord. Detailed knowledge of imaging features in myeloma emphasises the need to consider plasma cell neoplasm in the differential diagnosis of any pattern of bone sclerosis. Although exceptional, multiple myeloma must be borne in mind in the presence of diffuse bone sclerosis.

Topics: Aged; Antineoplastic Agents, Alkylating; Antineoplastic Agents, Hormonal; Antineoplastic Combined Chemotherapy Protocols; Chemotherapy, Adjuvant; Combined Modality Therapy; Diagnosis, Differential; Female; Humans; Immunoglobulin A; Immunoglobulin Allotypes; Immunoglobulin lambda-Chains; Melphalan; Multiple Myeloma; Osteosclerosis; Prednisone; Radiotherapy; Spinal Cord Compression; Treatment Outcome

A patient with a severe polyneuropathy in association with osteosclerotic myeloma improved after melphalan and prednisone treatment. Aggressive treatment with chemotherapy is appropriate in this type of patient.

Topics: Drug Therapy, Combination; Humans; Male; Melphalan; Middle Aged; Multiple Myeloma; Neural Conduction; Osteosclerosis; Peripheral Nervous System Diseases; Prednisone

A patient with a severe remote-effect polyneuropathy and other paraneoplastic features of osteosclerotic myeloma improved dramatically with melphalan and prednisone treatment. Serial electrodiagnostic studies provided an objective means of following the response to therapy and documented the improvement. We believe this represents the first reported patient with multifocal osteosclerotic myeloma and a myelomatous polyneuropathy responding to melphalan and prednisone.

Topics: Adult; Drug Therapy, Combination; Electrodiagnosis; Female; Humans; Melphalan; Multiple Myeloma; Osteosclerosis; Peripheral Nervous System Diseases; Prednisone

Sixteen cases of osteosclerotic myeloma and peripheral neuropathy (SM-PN) were reviewed. The neuropathy resembled chronic inflammatory-demyelinating polyneuropathy with predominantly motor disability, high CSF protein levels, and low motor nerve conduction velocities. Twelve of the 16 patients had detectable levels of monoclonal serum proteins, all with lambda light chains, but results of other laboratory studies were usually normal. Most of the patients also had organomegaly, endocrine abnormalities, or both. Treatment of solitary lesions with tumoricidal irradiation usually improved the neuropathy and reversed the nonneurologic abnormalities; chemotherapy for multiple osteosclerotic lesions was less helpful.

Topics: Adult; Aged; Antibodies, Monoclonal; Bone Neoplasms; Female; Humans; Immunoglobulin kappa-Chains; Male; Melphalan; Middle Aged; Multiple Myeloma; Osteosclerosis; Peripheral Nervous System Diseases; Prednisone

Topics: Female; Humans; Melphalan; Middle Aged; Osteosclerosis; Pulmonary Fibrosis

A case of IgG myeloma with eburnation of the femoral and ileoischiopubic bones is described. A review of the literature confirmed the rarity of osteosclerotic myeloma, especially if other possible causes of bone eburnation or the typical contemporary existence of osteolysis are excluded. The hypothesis that osteosclerotic myeloma may constitute an autonomous form of the disease, probably related to the type and/or extent of the proliferating bone is discussed.

Topics: Adrenal Cortex Hormones; Bone and Bones; Female; Femur; Humans; Immunoglobulin G; Ischium; Melphalan; Middle Aged; Multiple Myeloma; Osteosclerosis; Pubic Bone

A report on two patients with osteoclerotic myeloma is presented (myeloma with osteoblastic lesions). In case one, metastatic bone tumor of unknown origin was wrongly diagnosed initially. Because of increasing neurological symptoms laminectomy was performed. Biopsy led to the correct diagnosis of multiple myeloma of IgA-type. Patient also had a very severe peripheral neuropathy. The second patient had pancytopenia and osteosclerotic lesions of the pelvis. Bone marrow aspiration revealed so-called "empty marrow". Based on these findings, myelofibrosis was wrongly diagnosed at another hospital. Bone marrow aspiration, paper- and immunoelectrophoresis subsequently produced the correct diagnosis of multiple myeloma of IgG-type. Multiple myeloma usually is characterized by osteolytic lesions of the bones. However, the literature contains some 50 cases with osteosclerotic multiple myeloma, three different forms of which are described. In a fairly large percentage osteosclerotic multiple myeloma is combined with periphereal polyneuropathy. It would appear that in IgE-myeloma the incidence of sclerotic lesions is higher. Osteosclerotic multiple myeloma is very rare. It should however be considered if the differential diagnosis of osteosclerotic bone lesions is established.

Topics: Aged; Cyclophosphamide; Female; Humans; Immunoglobulin A; Immunoglobulin G; Male; Melphalan; Multiple Myeloma; Osteolysis; Osteosclerosis; Prednisone

Topics: Aged; Allopurinol; Animals; Blood Transfusion; Bone Marrow Examination; Cyclophosphamide; Electrophoresis; Female; Goats; Humans; Immune Sera; Immunochemistry; Immunodiffusion; Immunoglobulin E; Melphalan; Multiple Myeloma; Myeloma Proteins; Osteosclerosis; Pelvis; Rabbits; Radiography