ABSTRACT
Objective To explore the clinical value of serum κ/λ ratio in the differential diagnosis of multiple myeloma (MM) and primary nephritic,and to explore its relationship with MM other laboratory markers.Methods We obtained 88 cases of MM patients with serotyping by immunofixation and 109 cases of primary nephritic patients.In accordance with the composition of protein M in immunofixation electrophoersis (IFE),88 patients were divided into 4 groups:IgGκ,IgGλ,IgAκ and IgAλ.In addition,45 serum samples of health examination were collected as control samples.The levels of serum IgG,IgA,IgM,light chain κ and λ in each group were tested by immune turbidimetry,and κ/λ ratio was calculated.The levels of serum β2-microglobin (β2-MG),albumin (ALB),serum urea (BUN),creatinine (CRE),and M protein percentage were also detected.Results (1) In MM group,there was no significant difference of sex and International Staging System (ISS) stages between each type(P ＞ 0.05),while there was significant difference in age distribution between each type (P ＜ 0.05).(2) Compared to control,the levels of se-rum light chain κ,κ/λ ratio,and matched Ig were significantly higher and the level of light chain λ and other Ig were significantly lower in κ typed MM patients,while the levels of serum light chain λ were significantly higher and the levels of serum light chain κ,and κ/λ ratio were significantly lower in λ typed MM patients(P ＜0.01).The levels of light chain κ,λ,and IgG in primary nephritic patients were significantly lower than control(P ＜0.01),while there was no significant difference in κ/λ ratio between two group (P ＞ 0.05).There were also significant difference of light chain,Ig,BUN and CRE levels between MM patients and primary nephritic patients (P ＜ 0.05).(3) The κ/λ ratio correlated with serum ALB (r =-0.264,P =0.013) and β2-MG (r =0.235,P =0.040) levels in MM.Conclusions Serum levels of light chain κ,λ,and κ/λ ratio have great significance in the differential diagnosis of multiple myeloma and primary nephritic.The κ/λ ratio correlated with several tumor markers in MM progresses,rendering it as a promising method for diagnosis and prognosis monitoring of MM.
ABSTRACT
Fanconi syndrome (FS) is a rare condition that is characterized by defects in the proximal tubular function. A 48-year-old woman was admitted for evaluation of proteinuria. The patient showed normal anion gap acidosis, normoglycemic glycosuria, hypophosphatemia, and hypouricemia. Thus, her condition was compatible with FS. The M peak was found behind the beta globulin region in urine protein electrophoresis. Upon bone marrow examination, we found that 24% of cells were CD138+ plasma cells with kappa restriction. From a kidney biopsy, we found crystalline inclusions within proximal tubular epithelial cells. Thereafter, she was diagnosed with FS accompanied by multiple myeloma. The patient received chemotherapy and autologous stem cell transplantation, and obtained very good partial hematologic response. However, proximal tubular dysfunction was persistent until 1 year after autologous stem cell transplantation. In short, we report a case of FS accompanied by multiple myeloma, demonstrating crystalline inclusion in proximal tubular cells on kidney biopsy.
Subject(s)
Female , Humans , Middle Aged , Acid-Base Equilibrium , Acidosis , Beta-Globulins , Biopsy , Bone Marrow Examination , Crystallins , Drug Therapy , Electrophoresis , Epithelial Cells , Fanconi Syndrome , Glycosuria , Hypophosphatemia , Immunoglobulin kappa-Chains , Kidney , Multiple Myeloma , Plasma Cells , Proteinuria , Stem Cell TransplantationABSTRACT
The authors present a rare case of plasmacytoma of the orbit involving lacrimal gland with secondary transformation into multiple myeloma in a 42-year-old woman. The lesion was surgically removed and analyzed. Histopathological examination with immunostaining revealed it to be positive for immunoglobulin G and Kappa chains, demonstrating monoclonality. However, no abnormality was observed on serum electrophoresis, skeletal survey and bone marrow aspiration. Therefore, the tumor was diagnosed solitary plasmacytoma of bone. The patient was treated with external beam radiotherapy and has remained disease free for 5 years and 6 months until 2007, when she presented a pathological fracture due to multiple myeloma. Extensive medical work-up to rule out multiple myeloma or other malignant lymphoproliferative conditions involving orbit or ocular adnexa is needed when the diagnosis of solitary plasmacytoma of bone is suspected because treatment and prognosis are very different.
Os autores relatam um raro caso de plasmocitoma da órbita envolvendo glândula lacrimal com transformação secundária para mieloma múltiplo em uma paciente de 42 anos. A lesão foi removida cirurgicamente e o exame anatomopatológico, aliado à imuno-histoquímica, revelou positividade para imunoglobulina G e cadeias leve tipo Kappa. Após extensa avaliação sistêmica, nenhuma anormalidade foi observada, sendo feito o diagnóstico de plasmocitoma solitário ósseo. A paciente foi submetida à radioterapia, permanecendo livre da doença por 5 anos e 6 meses até 2007, quando apresentou uma fratura patológica devido à infiltração plasmocitária na coluna lombar, sendo feito diagnóstico de mieloma múltiplo. Uma vez que o plasmocitoma solitário ósseo pode ser a primeira manifestação do mieloma múltiplo, esses pacientes necessitam de adequado diagnóstico e seguimento a longo prazo, pois o tratamento e prognóstico dessas duas condições é diferente.