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

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

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

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

OBJECTIVETo investigate the incidence of serum immunoglobulin (Ig) paraprotein in chronic lymphocytic leukemia (CLL), and to explore its clinical associated laboratory features and prognostic implication.

METHODSSerum protein electrophoresis and immunofixation electrophoresis were performed by automatic electrophoresis apparatus to identify serum Ig paraprotein. Immunonephelometry was used to measure serum Ig levels.

RESULTSOut of 101 CLL patients, serum Ig paraprotein detection was found in 20 (19.8%) cases, 13 (12.9%) patients with IgG paraprotein, 7 (6.9%) patients with IgM paraprotein and 1(1.0%) patient with IgA paraprotein. Among these 20 cases, 1 patient had both IgG and IgM paraprotein, 2 patients had both κ and λ light chains. The incidence of serum IgG paraprotein was high in the group of advanced Binet stage (P = 0.032) and high level of thymidine kinase 1 (TK1) (P = 0.013). The incidence of serum IgM paraprotein was high in the group of advanced Binet stage (P = 0.037), high level of TK1 (P = 0.017) and cytogenetic abnormalities of del(11q22.3) (P = 0.006). With a median follow-up of 30 months (range 1 - 101 months), 66 patients received therapy after initial diagnosis. Survival analysis showed that the patients with serum Ig paraprotein had significantly shorter treatment-free survival (TFS) times than the patients without serum Ig paraprotein (P = 0.024). And the patients with serum IgM paraprotein had significantly shorter treatment-free survival (TFS) times than the patients without serum Ig paraprotein (P = 0.013). However, serum Ig paraprotein or IgM paraprotein was not independent prognostic factor.

CONCLUSIONSerum Ig paraprotein can be detected in a subset of patients with CLL, which could be of value as a prognostic factor in CLL.

Adolescent ; Adult ; Aged ; Aged, 80 and over ; Female ; Humans ; Immunoglobulins ; blood ; Leukemia, Lymphocytic, Chronic, B-Cell ; blood ; diagnosis ; Male ; Middle Aged ; Paraproteins ; metabolism ; Prognosis ; Young Adult

BACKGROUND: Angiogenesis and osteoclastogenesis are increased in the bone marrow of multiple myeloma (MM) patients in parallel with the tumor progression. Osteopontin (OPN) is a multifunctional protein that is involved in angiogenesis and bone destruction and, eventually, in tumor progression in MM. OPN is known to increase in MM patients as the disease progresses and bone is destroyed. We studied the clinical usefulness of OPN as a monitoring marker for treatment response in patients with MM. METHODS: We obtained 70 serial sera from 27 MM patients and 14 sera from healthy individuals. OPN was measured by a sandwich ELISA method. The hospital records were reviewed, and the clinically important markers for monitoring the treatment response, such as monoclonal component, immunoglobulin, free light chain, and hemoglobin, etc, were analyzed together with OPN levels. RESULTS: There was no significant difference in OPN levels between MM patients and healthy controls. OPN showed no significant correlations with the markers used for monitoring of treatment response such as M component, immunoglobulin, and free light chain levels. There was no difference in OPN levels between the 3 groups classified by the amount of M component. In addition, OPN levels showed no compatible changes to the treatment response of MM patients. CONCLUSIONS: Although OPN has been known to have an important role in the formation and progression of MM by involving angiogenesis and bone destruction, our results show that OPN is not valuable as a clinical marker for monitoring the treatment response in MM patients because of inconsistency in its levels in MM patients.
Adult ; Aged ; Disease Progression ; Enzyme-Linked Immunosorbent Assay ; Female ; Humans ; Male ; Middle Aged ; Multiple Myeloma/*diagnosis/therapy ; Osteopontin/*blood ; Paraproteins/analysis ; Regression Analysis ; Tumor Markers, Biological/*blood

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

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*