BACKGROUND: We report a case of light chain proximal tubulopathy associated with lupus nephritis in a patient known to have systemic lupus erythematosus. The kidney can be injured in several ways in any of these disorders. Light chain proximal tubulopathy is a rare form of renal tubular injury that may occur in and complicate plasma cell dyscrasia, characterized by cytoplasmic inclusions of the monoclonal light chain within proximal tubular cells. Lupus nephritis is a common form of renal injury as it occurs in about 25-50% of adult patients with systemic lupus erythematosus.
METHODS: We present a 57-year-old African patient known to have systemic lupus erythematosus and hypertension presented with a new complaint of microscopic hematuria. A renal biopsy was performed and revealed lupus nephritis class II concurrently associated with light chain induced proximal tubulopathy. A subsequent bone marrow biopsy was performed, which revealed multiple myeloma.
CONCLUSIONS: We report a case of coincidental lupus nephritis and proximal tubulopathy featuring a combined constellation of rare histopathological features that might add to the relationship between systemic lupus and paraproteinemia.

Non-secretory myeloma (NSM) is a rare form of myeloma. It is defined as monoclonal plasmocytic proliferation of the bone marrow with the same clinical and radiological manifestations of myeloma. However, plasma cells are unable to secrete immunoglobulin (serum and urinary electrophoresis are negative and free light chain measurement is unquantifiable). This variant of multiple myeloma (MM) usually poses a diagnostic challenge to the biologist and clinician. We report a rare case of non-secretory myeloma in a 76-year-old patient who was diagnosed at the Mohammed V University Hospital Center in Oujda, Morocco.

Light chain amyloidosis has very rarely been reported in association with chronic lymphocytic leukemia (CLL). We reported on a 76-years-old female who presented with simultaneous kappa-restricted chronic lymphocytic leukemia (CLL) and a lambda-restricted multiple myeloma with plasma cells causing AL amyloidosis involving the heart. While monoclonal immunoglobulins occasionallyproduced by CLL have previously been implicated in AL amyloidosis, there only a few cases reported of AL amyloidosis resulting from a distinct plasma cell dyscrasia that is not clonally related to the concurrent CLL.

Monoclonal gammopathies are characterized by serum monoclonal component (MC) plus an intact immunoglobulin and a free light chain (FLC), or a combination of both. The measurement of FLC with Freelite® is the standard practice recommended by International Myeloma Working Group guidelines. Recently, Hevylite® heavy/light chains (HLC) assays were introduced to specifically target junctional epitopes between the heavy and light chains of intact immunoglobulins, allowing the independent quantification of the involved (MC) and uninvolved (polyclonal immunoglobulin background) HLC isotype. Between January 2012 and March 2014, 90 patients were examined: 49 multiple myeloma (MM), 6 smoldering MM (SMM) and 35 monoclonal gammopathy of undetermined significance (MGUS). Of these 90 patients, 300 samples were collected at different times. The diagnostic and monitoring contribution of Hevylite A and G assays was assessed in all 90 patients examined. Additionally, 3 representative cases were selected. The Hevylite absolute values and ratio demonstrated high sensitivity and specificity with respect to serum protein electrophoresis and serum immunofixation. The combined use of Hevylite A and G with Freelite was particularly useful in dubious cases with more than one MC or with co-migrating components, as well as in the course of monitoring to assess the independent change of FLC and HLC, possibly reflecting the presence of clonal heterogeneity in the cohort. From this study, it can be concluded that FLC and HLC are independent, useful markers to monitor the MC and to assess with greater specificity and sensitivity the effect of therapy, thereby providing clinical support. Further studies are required to assess the prognostic potential of Hevylite in MGUS and SMM.

To report endotoxemia presented in a case with multiple myeloma (MM) treated by high cutoff hemodialysis (HCO-HD) being prevented by using ultrapure dialysate. A female inpatient with MM received six times HCO-HD (HCO 2100 dialyzer) within 3 weeks after initiation of a chemotherapy based on vincristine+epirubicin+dexamethasone protocol. Conventional dialysate was used in the first three times and then changed to ultrapure dialysate due to elevation of body temperature after HCO-HD. Free light chains (FLC) and endotoxin levels in blood and dialysate were monitored. After six times HCO-HD, her serum FLC λ decreased from 4689 mg/L to 492.7 mg/L, with a trend of decline of serum creatinine. The clearance, reduction ratio, and removal amount of FLC λ was 38.4 mL/min, 71.0-85.2%, and 9.06-18.02 g, respectively, in the setting of dialysate flow rate 500 mL/min, while in the setting of dialysate flow rate 200 mL/min, the removal efficacy of FLC λ was lower than the former. A rise of body temperature up to 38.5°C after treatment and endotoxemia (endotoxin levels 0.122 EU/mL) was found when using conventional dialysate (endotoxin levels 0.112-0.145 EU/mL), but not seen after changing to ultrapure dialysate. Combined with appropriate chemotherapy, HCO-HD can effectively remove and reduce blood FLC. Attention should be paid to the endotoxemia and the rise of temperature after treatment when conventional dialysate is used, which can be prevented by using ultrapure dialysate.