Paraproteinemias ; Paraproteins/therapeutic use*

When analyzing samples containing paraproteins, various interference effects are encountered in the clinical laboratory. Precipitation of paraproteins mostly interferes with the assays that use photometric detection. Herein, we present a case of a patient with multiple myeloma who had paraproteins and spuriously elevated total bilirubin levels (31.1 mg/dL), which were measured by using Roche total bilirubin assay on the Modular DPE (Roche Diagnostics, Switzerland) chemical analyzer. The total bilirubin concentration reduced from 31.1 mg/dL to 1.5 mg/dL, when tested after three fold dilution of the sample on Modular DPE chemical analyzer.
Bilirubin ; Humans ; Hyperbilirubinemia* ; Multiple Myeloma* ; Paraproteinemias ; Paraproteins

Papular mucinosis, also known as lichen myxedematosus or scleromyxedema, is a chronic cutaneous disorder characterizecl by infiltration of mucin in the dermis without abnormalities of thyroid function. It often combines serum monoclonal paraproteins. Various drugs have been used for the treatment without consistent results. We report a case of papular mucinosis in a 51-year-old man, who started the disease on his scalp 10 years before the first examination. Subsequent development of the characteristic glistening papular lesions on his entire scalp, eye brows, ears and upper back continued for 5 years thereafter until complete resolution. Treatment with corticosteroids and melphalan showed only limited effects during the period.
Adrenal Cortex Hormones ; Dermis ; Ear ; Humans ; Melphalan ; Middle Aged ; Mucins ; Paraproteins ; Scalp ; Scleromyxedema* ; Thyroid Gland

Hyperphosphatemia is an unusual manifestation in patients with multiple myeloma without a significantly reduced glomerular filtration rate. Serum phosphate may be falsely elevated when a large amount of paraproteins is present in the serum, because ultraviolet light absorbance is elevated with the phosphomolybdate ultraviolet assay, which is most commonly used for serum phosphate measurement. This pseudohyperphosphatemia can be confirmed by deproteinization of the serum of patients. We report a case of multiple myeloma presenting with spurious hyperphosphatemia revealing pseudohyperphosphatemia by deproteinization of serum using sulfosalicylic acid.
Glomerular Filtration Rate ; Humans ; Hyperphosphatemia ; Multiple Myeloma* ; Paraproteinemias ; Paraproteins ; Ultraviolet Rays

Crystalline nephropathy is a rare yet well-known condition associated with multiple myeloma and other light chain–secreting disorders. Paraproteins that are resistant to proteolysis crystallize within proximal tubular cells and cause light-chain proximal tubulopathy, which presents clinically as Fanconi syndrome. Podocytes are rarely affected, and the crystalline inclusions within podocytes are typically precipitated, yielding significant glomerular proteinuria. Here we report a case of extensive crystalline inclusions primarily within podocytes and proximal tubules that presented only with Fanconi syndrome and renal insufficiency. Despite the presence of extensive crystalline inclusions in podocytes and diffuse foot process effacement, the patient had no clinical evidence suggestive of podocyte injury.
Crystallins* ; Fanconi Syndrome ; Foot ; Humans ; Multiple Myeloma* ; Paraproteins ; Podocytes ; Proteinuria* ; Proteolysis ; Renal Insufficiency

Multiple myeloma is a monoclonal plasma cell proliferation disorder with various symptoms and signs caused by paraproteinemias. Among these signs, a bleeding tendency is one of the major fatal causes. However, significant severe bleeding is rare in most cases. In this study, we report a case of multiple myeloma in a patient who had a severe recurrent bleeding tendency due to platelet dysfunction caused by paraproteins. After being treated with therapeutic plasma exchange and chemotherapy, the patient's monoclonal protein level decreased and the bleeding stopped.
Blood Platelets ; Hemorrhage ; Hemostatic Disorders ; Humans ; Multiple Myeloma ; Paraproteinemias ; Paraproteins ; Plasma Cells ; Plasma Exchange ; Plasmapheresis ; Platelet Function Tests

Ascites is a rare complication of multiple myeloma. When it develops, it is usually associated with extensive liver infiltration with plasma cells, infectious peritonitis or myelomatous peritoneal infiltration. Ascites caused by peritoneal infiltration is even less frequent than others. The majority of previously reported cases were characterized by an IgA paraprotein and lack of skeletal lesions. This rare extramedullary complication of myeloma has been unresponsive to therapy and rapidly fatal. Therefore, it is important to recognize myeloma as a cause of ascites and the presence of ascites heralds a poor prognosis of myeloma. We recently experienced a case of myeloma with ascites and reviewed the relevant literature of human myeloma presenting with the triad of ascites, relative or absolute sparing of the skeleton, and an IgA paraprotein. A 76-year-old man was presented with ascites early in the course of myeloma. He had no evidence of intra-abdominal plasmacytoma and skeletal lesions. Myelomatous ascites was demonstrated by the monoclonal immunoglobulin of IgA type in ascitic fluid. He was treated by plasmapheresis due to hyperviscosity syndrome and VAD combination chemotherapy. He was discharged with the improved clinical condition.
Aged ; Ascites* ; Ascitic Fluid ; Drug Therapy, Combination ; Humans ; Immunoglobulin A ; Immunoglobulins ; Liver ; Multiple Myeloma* ; Paraproteins ; Peritonitis ; Plasma Cells ; Plasmacytoma ; Plasmapheresis ; Prognosis ; Skeleton

Scleromyxedema is a rare fibromucinous disorder that is often difficult to treat and is associated with significant morbidity and mortality. Clinically, it shows white or flesh colored, dome-shaped waxy papules on the hands, arms, face, neck and upper trunk. There are proliferation of fibroblasts and mucin deposition in dermis and presence of serum paraproteins. It should be differentiated from scleroderma because of sclerotic skin changes. Patients were a 70 year old male and a 45 year old female. They showed multiple confluent or linearly arranged waxy papules on the head, neck and extremities. Sclerotic plaques were also seen. Abnormal findings were not detected in laboratory tests and bone marrow biopsy. Histologically, mucin deposition and marked proliferation of fibroblasts were seen in dermis. Therapy was performed with 200 mg/day of cyclophosphamide. Both patients showed good response and had no recurrence. The latter one had shown improvement of skin lesions but subsequently she developed leukocytopenia. Cyclophosphamide was stopped temporarily until the abnormal leukocytes count recovered. During therapy, mild hemiparesis developed. We could not find the cause of the symptom through physical examination and radiologic studies.
Aged ; Arm ; Biopsy ; Bone Marrow ; Cyclophosphamide ; Dermis ; Extremities ; Female ; Fibroblasts ; Hand ; Head ; Humans ; Leukocytes ; Leukopenia ; Male ; Middle Aged ; Mortality ; Mucins ; Neck ; Paraproteins ; Paresis ; Physical Examination ; Recurrence ; Scleromyxedema* ; Skin

BACKGROUND/AIMS: Multiple myeloma (MM)–associated cardiac damage, particularly according to the type of monoclonal (M) protein has not been elucidated. We sought to investigate relationship between elevated serum M protein levels and echocardiographic indices of cardiac structure and function in patients with MM. METHODS: We evaluated a total of 184 consecutive MM patients who underwent echocardiography for bone marrow pre-transplant screening. Serum levels of intact immunoglobulin M protein and free light chain kappa/lambda (FLC-κ/-λ) were measured. RESULTS: One hundred thirty-nine patients were non-light chain MM (non-LCMM) and 45 patients belonged to LCMM. In patients with non-LCMM, significant correlations were found between serum M protein and left atrial volume index (LAVi; r = 0.720, p < 0.0001), E/e’ (r = 0.511, p < 0.0001), and systolic pulmonary arterial pressure (r = 0.485, p < 0.0001). In patients with LCMM, log-transformed FLC-λ (log-λ) was correlated with left ventricular ejection fraction (LVEF, r = –0.536, p = 0.010), left ventricular (LV) end-systolic dimension (r = 0.500, p = 0.018), and LV end-systolic volume (r = 0.444, p = 0.038). On multivariate analyses, hematocrit and serum M protein were independent predictors of LAVi in patients with non-LCMM. In patient with LCMM, FLC-λ isotype was only found to be an independent determinant of LVEF. CONCLUSIONS: An increase in serum M protein was associated with LV diastolic dysfunction, whereas an increase in serum FLC-λ concentration showed a negative correlation with the echocardiographic parameters of LV systolic function. These findings also suggest that serum M protein has different effects on LV function according to the type of paraproteins in patients with MM.
Arterial Pressure ; Bone Marrow ; Echocardiography ; Hematocrit ; Humans ; Immunoglobulin M ; Mass Screening ; Multiple Myeloma* ; Multivariate Analysis ; Paraproteins ; Stroke Volume ; Ventricular Function, Left*

BACKGROUND/AIMS: Multiple myeloma (MM)–associated cardiac damage, particularly according to the type of monoclonal (M) protein has not been elucidated. We sought to investigate relationship between elevated serum M protein levels and echocardiographic indices of cardiac structure and function in patients with MM. METHODS: We evaluated a total of 184 consecutive MM patients who underwent echocardiography for bone marrow pre-transplant screening. Serum levels of intact immunoglobulin M protein and free light chain kappa/lambda (FLC-κ/-λ) were measured. RESULTS: One hundred thirty-nine patients were non-light chain MM (non-LCMM) and 45 patients belonged to LCMM. In patients with non-LCMM, significant correlations were found between serum M protein and left atrial volume index (LAVi; r = 0.720, p < 0.0001), E/e’ (r = 0.511, p < 0.0001), and systolic pulmonary arterial pressure (r = 0.485, p < 0.0001). In patients with LCMM, log-transformed FLC-λ (log-λ) was correlated with left ventricular ejection fraction (LVEF, r = –0.536, p = 0.010), left ventricular (LV) end-systolic dimension (r = 0.500, p = 0.018), and LV end-systolic volume (r = 0.444, p = 0.038). On multivariate analyses, hematocrit and serum M protein were independent predictors of LAVi in patients with non-LCMM. In patient with LCMM, FLC-λ isotype was only found to be an independent determinant of LVEF. CONCLUSIONS: An increase in serum M protein was associated with LV diastolic dysfunction, whereas an increase in serum FLC-λ concentration showed a negative correlation with the echocardiographic parameters of LV systolic function. These findings also suggest that serum M protein has different effects on LV function according to the type of paraproteins in patients with MM.
Arterial Pressure ; Bone Marrow ; Echocardiography ; Hematocrit ; Humans ; Immunoglobulin M ; Mass Screening ; Multiple Myeloma* ; Multivariate Analysis ; Paraproteins ; Stroke Volume ; Ventricular Function, Left*