Osteosclerotic myeloma is a rare entity, characterized by single or multiple osteosclerotic bone lesions and usually accompanied by a polyneuropathy syndrome (POEMS). Multiple myeloma with osteosclerotic lesions without polyneuropathy is exceedingly rare. We report a case of multiple myeloma associated with multifocal osteosclerotic lesions without any evidence of POEMS. A 48-year-old woman presented with incidentally found osteosclerosis of 8th thoracic vertebra on a plain chest film. Bone survey, CT scan, MR scan, and radioisotope scintigraphy revealed multiple localized osteoclerosis; serum protein immunofixation showed IgG, lambda monoclonal gammopathy. A biopsy of T8 vertebral body disclosed plasma cell myeloma. Given that there was no organ or tissue damage other than multifocal osteosclerosis, the patient was placed on close observation with regular examination. This case indicates that although rare, multiple myeloma should be included in the differential diagnosis of sclerotic bone lesions.
Biopsy ; Diagnosis, Differential ; Female ; Humans ; Immunoglobulin G ; Middle Aged ; Multiple Myeloma ; Osteosclerosis ; Paraproteinemias ; Polyneuropathies ; Spine ; Thorax

A large tumor located at right temporoparietal lobe was found in 25-years-old female. The brain CT scan and MR imaging revealed a large multi-lobulated mass which was composed of solid and cystic portions involving right temporoparietal lobe. Preoperative routine laboratory check showed microcytic hypochromic anemia and monoclonal gammopathy with bate-gamma bridging. The mass was removed totally by operation and histologic diagnosis resulted in chordoid meningioma surrounded by massive polyclonal lymphoplasmacellular infiltrates. After the mass was removed, the blood picture of the patient normalized and the patient discharged with neurologically free stage. We report this case with relative literatures.
Anemia, Hypochromic ; Brain ; Diagnosis ; Female ; Humans ; Magnetic Resonance Imaging ; Meningioma* ; Paraproteinemias ; Tomography, X-Ray Computed

The paraproteinemia is a disorder in which a single clone of plasma cells (monoclonal gammopathy) is responsible for the proliferation of monoclonal proteins (M-proteins). Approximately 10% of patients with idiopathic peripheral neuropathy have monoclonal gammopathy. Some M-proteins have the properties of an antibody to the components of peripheral nerve myelin, but the pathophysiological relationship between the neuropathy and the M-protein is often obscure. The relationship between peripheral neuropathy and monoclonal gammopathy requires the appropriate neurological and hematological investigations for precise diagnosis and treatment. In this review, we provide an update on the causal associations between peripheral neuropathy and monoclonal gammopathy as well as characteristics of clinical and electrophysiologic features.
Clone Cells ; Diagnosis ; Humans ; Myelin Sheath ; Paraproteinemias ; Peripheral Nerves ; Peripheral Nervous System Diseases ; Plasma Cells ; Polyneuropathies

Diffuse plane xanthoma (DPX) presents with symmetric yellow-orange plaques primarily on the neck, upper trunk, flexural folds, and the periorbital region. Based on serum lipid and lipoprotein levels, these xanthomas are classified as normolipemic or hyperlipoproteinemic DPX. Diffuse normolipemic plane xanthoma (DNPX) is a rare condition that is not well studied yet. It is associated with reticulo-endothelial diseases, particularly multiple myeloma and monoclonal gammopathy of unknown significance (MGUS). A 62-year-old woman developed yellowish hyperpigmented papules and diffuse patches in the medial canthal area of her neck. Based on a skin biopsy and laboratory analyses, she was diagnosed with DNPX associated with multiple myeloma. This diagnosis demonstrates that dermatological lesions should be carefully assessed as they may be the first manifestation of an underlying hematological disease. We report herein a rare case of diffuse plane xanthoma associated with multiple myeloma and review the relevant literature.
Biopsy ; Diagnosis ; Female ; Hematologic Diseases ; Humans ; Lipoproteins ; Middle Aged ; Multiple Myeloma ; Neck ; Paraproteinemias ; Skin ; Xanthomatosis

Amyloidosis is a systemic disorder associated with clonal plasma cell dyscrasia. Nephrotic syndrome, congestive heart failure, autonomic and peripheral neuropathy is often associated features in amyloidosis. Early diagnosis is most important because of different prognosis by stage. The diagnosis can be delayed since symptoms of amyloidosis may vary or nonspecific. We describe a patient of myeloma-associated amyloidosis, who showed orthostatic intolerance as the first symptom of the disease.
Amyloidosis* ; Diagnosis ; Early Diagnosis ; Heart Failure ; Humans ; Nephrotic Syndrome ; Orthostatic Intolerance* ; Paraproteinemias ; Peripheral Nervous System Diseases ; Prognosis

BACKGROUND: Immunoglobulins exist in the serum, mostly in a union type of heavy and light chains. Free light chain types exist in an extremely small quantity and are useful in the diagnosis and follow up of multiple myeloma, but are also increased in autoimmune diseases such as SLE. The aim of this study was to evaluate the usefulness of the serum free light chain in discriminating between monoclonal and polyclonal gammopathy. METHODS: Between January and June of 2003, we identified 15 patients with monoclonal gammopathy and 12 patients with polyclonal gammopathy on serum protein electrophoresis (SPEP) and immunofixation electrophoresis (IFE). We measured the serum concentration of the free light chain using Beckman Coulter IMMAGE(TM) analyzer with FREELITE(TM) reagents and calculated the kappa/lambda (kappa/lambda) ratio. We also measured the free light chain of 35 healthy controls to establish a reference range. RESULTS: The reference ranges established in this study were 4.97-12.84 mg/L for kappa light chains, 6.71-18.09 mg/L for lambda light chains, and 0.46-1.01 for the kappa/lambda ratio. The free light chains were abnormal in all 27 but 2 patients with polyclonal gammopathy on SPEP. The kappa/lambda ratio was abnormal in 12 of the 15 patients with monoclonal gammopathy and in none of the 12 patients with polyclonal gammopathy. CONCLUSIONS: Our results suggest that the kappa/lambda ratio can be a useful tool to discriminate between monoclonal and polyclonal gammopathy, especially in the case of vague SPEP results, or when monoclonal gammopathy is suspected in SPEP.
Autoimmune Diseases ; Diagnosis ; Discrimination (Psychology)* ; Electrophoresis ; Follow-Up Studies ; Humans ; Immunoassay* ; Immunoglobulins ; Indicators and Reagents ; Multiple Myeloma ; Paraproteinemias* ; Reference Values

Extramedullary plasmacytoma is a rare presentation of plasma cell dyscrasia. Most such tumors arise on the upper aerodigestive tract and renal plasmacytoma is very rare. The patient was 44 years old female presented with a 3 month-history of palpable mass in the right flank. There was a past history of complete remission after a chemotherapy for multiple myeloma (6 cycles of VAD chemotherapy) for the two years following the first diagnosis. After surgical resection, histologic and immunofluorescence studies of resected specimens revealed that the renal parenchyma was destroyed by sheets of mature plasma cells producing monoclonal protein (IgG-lambda) and by deposits of amorphous eosinophilic substance stained with anti-lambda antisera. Treatment with chemotherapy of Hyper-CVAD and local irradiation was done. The patient has been disease-free for 3 months after treatment. We report a case of relapsed renal plasmacytoma after complete remission of multiple myeloma.
Adult ; Diagnosis ; Drug Therapy ; Eosinophils ; Female ; Fluorescent Antibody Technique ; Humans ; Immune Sera ; Multiple Myeloma* ; Paraproteinemias ; Plasma Cells ; Plasmacytoma*

Extramedullary plasmacytoma is a rare malignancy, composed of plasma cell dyscrasias localized to an extramedullary site in the soft tissue. The great majority of extramedullary plasmacytoma occurs in the head and neck region, mainly in the upper respiratory tract and oral cavity. Extramedullary plasmacytoma arising in the kidney, termed a primary renal plasmacytoma, is a very rare malignancy. Primary renal plasmacytoma is not distinguished from a renal cell or transitional cell carcinoma using conventional imaging studies. The diagnosis of a primary renal plasmacytoma is only made on histopathological examination. Herein, a case of primary renal plasmacytoma, in a 39-year-old male patient, is reported.
Adult ; Carcinoma, Transitional Cell ; Diagnosis ; Head ; Humans ; Kidney ; Kidney Neoplasms ; Male ; Mouth ; Neck ; Paraproteinemias ; Plasmacytoma* ; Respiratory System

Monoclonal gammopathies are associated with a wide range of renal diseases, including cast nephropathy, light chain amyloidosis, monoclonal immunoglobulin deposition diseases, and so on. We describe seven cases of monoclonal gammopathies involving kidney. The mean age was 61.6+/-3.6 years and male to female ratio was 1:1.3. Among 7 patients, diagnoses were cast nephropathy with light chain deposition disease, two light chain deposition diseases, three light chain amyloidosis and light chain deposition disease with light chain amyloidosis. Two cases were monoclonal gammopathy of undetermined significance and three cases were multiple myeloma in five cases underwent bone marrow biopsy. It showed that renal function was severly decreased in light chain deposition disease. It is clear that monoclonal gammopathies show various renal disease and clinical course in our cases. It is necessary to do renal biospy for adequate diagnosis and treatment even to old patients suspecting monoclonal gammopathy.
Amyloidosis ; Biopsy ; Bone Marrow ; Diagnosis ; Female ; Humans ; Immunoglobulins ; Kidney* ; Male ; Monoclonal Gammopathy of Undetermined Significance ; Multiple Myeloma ; Paraproteinemias*

BACKGROUND AND OBJECTIVES: Cardiac involvement is frequent in systemic amyloidosis and is the most important determinant of the clinical outcome. The aims of this study were to assess the incidence and prognosis of cardiac amyloidosis and discuss the diagnostic issues related to cardiac amyloidosis. SUBJECTS AND METHODS: We retrospectively studied all patients diagnosed with systemic amyloidosis who presented to our institution from January 1999 to December 2011. RESULTS: Of the 129 patients with systemic amyloidosis, cardiac amyloidosis was diagnosed in 62 patients. At the 3 years' follow-up of the patients with systemic amyloidosis, there was a statistically significant difference in mortality between patients with cardiac amyloidosis and the rest of the patients (58.1% vs. 37.3%, p=0.008). In the Cox proportional hazard model, old age {hazard ratio (HR) 18.336, p=0.006}, elevation of cardiac troponin I (cTNI) (HR 13.246, p=0.020), left ventricular (LV) systolic dysfunction (HR 5.137, p=0.041) and diastolic dysfunction (HR 64.595, p=0.022) were independently associated with survival in cardiac amyloidosis. In the diagnosis of monoclonal gammopathy, serum or urine protein electrophoresis was not sensitive enough to be used clinically compared to serum free light chain assay (35.8% vs. 96.4%). CONCLUSION: In systemic amyloidosis, cardiac involvement was the most important determinant of the prognosis, and old age, elevation of cTNI, LV systolic dysfunction and diastolic dysfunction were independently associated with survival in cardiac amyloidosis.
Amyloidosis* ; Diagnosis* ; Electrophoresis ; Follow-Up Studies ; Humans ; Incidence* ; Mortality ; Paraproteinemias ; Prognosis* ; Proportional Hazards Models ; Retrospective Studies ; Troponin I