scholarly journals Case Report: Lenalidomide as a Second-Line Treatment for Bortezomib-Ineffective Nephrotic Syndrome Caused by LCDD: 2 Case Reports and a Literature Review

2021 ◽  
Vol 8 ◽  
Author(s):  
Xin Zhang ◽  
Xiao-juan Yu ◽  
Su-xia Wang ◽  
Fu-de Zhou ◽  
Ming-hui Zhao
Keyword(s):  
Nephrotic Syndrome ◽  
Light Chain ◽  
Case Reports ◽  
Light Chain Deposition Disease ◽  
First Line Therapy ◽  
Hematological Response ◽  
Systemic Disorder ◽  
Light Chain Deposition ◽  
Stage Renal Disease ◽  
End Stage

Background: Light-chain deposition disease (LCDD) is a rare systemic disorder characterized by the deposition of monoclonal light chains in organs. The kidney is a prominent target of light-chain deposition, with a median time to end-stage renal disease (ESRD) of 2.7 years and 5-year ESRD-free survival of 37%. The therapeutic management of LCDD remains ill-defined. In addition to bortezomib-based therapy as first-line therapy, the effect of lenalidomide on LCDD is rarely reported.Case Presentation: This study describes two male LCDD patients in their 60s with nephrotic syndrome and moderately impaired renal function. One patient had monoclonal IgGλ with underlying MGRS, and another had monoclonal IgGκ with underlying monoclonal gammopathy that developed into symptomatic MM during follow-up. The hallmarks of this disease were consistent with previous reports. Both patients initially received BCD therapy, but no hematological response was observed. Consequently, the nephrotic syndrome was refractory. Sequential Rd therapy was initiated, and partial hematological response and nephrotic remission were observed in the IgGλ patient but absent in the IgGκ patient.Conclusion: Limited reports have demonstrated the effect of lenalidomide in LCDD. We report the outcome of lenalidomide in two cases of bortezomib-resistant LCDD. This treatment might be a beneficial supplement for those unresponsive or intolerant to bortezomib in LCDD, but the effect should be prospectively investigated.

scholarly journals Gastric antral vascular ectasia should not be overlooked in erythropoietin resistance: a series of case reports

2019 ◽  
Vol 25 (4) ◽  
pp. 219-225
Author(s):  
Laurynas Rimševičius ◽  
Domantas Galkauskas ◽  
Julius Lavinskas ◽  
Evelina Šestelinska ◽  
Ernesta Mačionienė ◽  
...  
Keyword(s):  
Medical Condition ◽  
Case Reports ◽  
Argon Plasma Coagulation ◽  
Argon Plasma ◽  
Gastric Antral Vascular Ectasia ◽  
Vascular Ectasia ◽  
First Line Therapy ◽  
Stage Renal Disease ◽  
End Stage ◽  
Erythropoietin Resistance

Background. Gastric antral vascular ectasia (GAVE) is currently recognized as an important cause of upper gastrointestinal (GI) haemorrhage, being responsible for about 4% of non-variceal upper GI haemorrhages and typically presents in middle-aged females. GAVE, also called “watermelon stomach”, is diagnosed through esophagogastroduodenoscopy and is characterized by the presence of visible columns of red tortuous enlarged vessels along the longitudinal folds of the antrum. The pathogenesis is still obscure and many hypotheses have been proposed such as mechanical stress, humoral and autoimmune factors. In the last two decades, numerous therapeutic strategies have been proposed, including surgical, endoscopic, and medical choices, yet successful treatment of GAVE continues to be a challenge. Currently, given the rapid response, safety, and efficacy, endoscopic ablative modalities have largely usurped medical treatments as first-line therapy, particularly using argon plasma coagulation. The actual GAVE prevalence in patients with end-stage renal disease (ESRD) is not clear, yet in difficult cases it should be considered as a cause of erythropoietin resistance. Case presentation. We report four clinical cases of GAVE syndrome patients diagnosed with stage 4 to 5 chronic kidney disease. All patients presented with anaemia and GI haemorrhage, the origin of which turned out to be GAVE syndrome. Conclusions. GAVE syndrome is a serious condition in ESRD patients, especially in those presenting with treatment-refractory anaemia. Realization of its aetiology and characteristics is essential to suspect, diagnose, and treat gastric ectasia. Only proper diagnosis and well-timed disease treatment can significantly improve a patient’s medical condition and future prognosis.

scholarly journals Kidney transplantation for active multiple myeloma or smoldering myeloma: a case–control study

2019 ◽  
Author(s):  
Raphaël Kormann ◽  
Claire Pouteil-Noble ◽  
Clotilde Muller ◽  
Bertrand Arnulf ◽  
Denis Viglietti ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Kidney Transplantation ◽  
Light Chain ◽  
Fungal Infections ◽  
Light Chain Deposition Disease ◽  
Cast Nephropathy ◽  
Amyloid Light Chain ◽  
Smoldering Myeloma ◽  
Light Chain Deposition ◽  
Stage Renal Disease

Abstract Background The increased survival of patients with multiple myeloma (MM) raises the question of kidney transplantation (KT) in patients with end-stage renal disease (ESRD). Methods We included 13 patients with MM or smoldering myeloma (SMM) and ESRD transplanted between 2007 and 2015, including 7 MM with cast nephropathy, 3 with MM-associated amyloid light chain amyloidosis or light chain deposition disease and 3 SMM and compared them with 65 control-matched kidney-transplanted patients. Nine of the MM patients with KT were also compared with 63 matched MM patients on haemodialysis. Results Pre-transplantation parameters were comparable, except for the duration of renal replacement therapy (57.8 versus 37.0 months; P = 0.029) in MM versus control patients, respectively. The median follow-up post-KT was 44.4 versus 36.4 months (P = 0.40). The median MM graft and patient survival were 80.1 and 117.2 months, respectively, and were not significantly different from control patients, although mortality tended to be higher in the 10 symptomatic MM patients (P = 0.059). MM patients had significantly more viral and fungal infections and immunosuppressive maintenance therapy modifications while they received lower induction therapy. Two MM patients relapsed and two SMM cases evolved to MM after KT. Three cast nephropathies occurred, two of them leading to ESRD. Moreover, survival of MM with KT increased relative to control haemodialysed patients (P = 0.002). Conclusions Selected MM patients may benefit from KT but need careful surveillance in the case of KT complications and MM evolution.

Management of light chain deposition disease: A systematic review.

2021 ◽  
Vol 39 (15_suppl) ◽  
pp. e20042-e20042
Author(s):  
Adeel Masood ◽  
Moazzam Shahzad ◽  
Ahsan Wahab ◽  
Pranali Santhoshini Pachika ◽  
Tehniat Faraz Ahmed ◽  
...  
Keyword(s):  
Plasma Cell ◽  
Light Chain ◽  
Retrospective Studies ◽  
Case Reports ◽  
Single Agent ◽  
Case Series ◽  
Cell Transplant ◽  
Light Chain Deposition Disease ◽  
Deposition Disease ◽  
Light Chain Deposition

e20042 Background: Light-chain deposition disease (LCDD) is part of a wide spectrum of plasma cell proliferation disorders. LCDD predominantly affects kidneys by systemic deposition of congo red negative non-fibrillar light chains. Due to the rarity of disease, no prospective data is available, and clear guidelines don’t exist for the treatment. We reviewed data to summarize effective treatment strategies. Methods: A literature search was conducted on PubMed, Cochrane, and ClinicalTrials.gov with final update in November 2020 using terms ‘light chain deposition disease’, ‘therapy,’ and ‘disease management’. Library was manually screened to remove articles on LCDD in transplant kidneys. We identified 63 case reports/series and 11 retrospective studies n=365. Results: Retrospective Studies: 9 studies (n=283) identified various combinations of LCDD and plasma cell dyscrasias given the rarity of idiopathic LCDD. Lorenz et al. reported 6 patients (pts) that received ASCT with MEL preconditioning with 92% improved proteinuria and 95% improved eGFR. Pozzi et al. reported 63 pts (32% idiopathic LCDD, 65% concurrent MM and 3% concurrent CLL) where all pts received a combination of vincristine, doxorubicin, dexamethasone/methylprednisolone. 5/63 patients received ASCT. The median survival was 4.1 years. Bortezomib (V) based regimens: Most experts favor V based regimens to treat LCDD, but no dedicated trials exist. 26 case reports/case series (37 pts) included; 23 pts with idiopathic LCDD and rest with a concurrent disorder. Daratumumab: Milani et al. treated 8 pts with LCDD and concurrent MM with single agent daratumumab who failed at least one regimen. Autologous stem cell transplant (ASCT): 13 case reports/case series (20 pts) identified who received ASCT with melphalan (MEL) conditioning with/without various combinations of chemotherapies before and after ASCT. 14 pts had LCDD, and 6 pts had concurrent clonal cell dyscrasia. Miscellaneous regimens: Various other regimens showed promising results with improved renal functions but not well studied e.g., thalidomide and dexamethasone (Haruyuki et al.). Conclusions: Dedicated clinical trials are difficult given rarity of LCDD but needed. Bortezomib based regimens seem to be good options, ASCT with MEL conditioning is a suitable choice for younger and fit pts.[Table: see text]

Recovery from LCDD-associated Severe Liver Cholestasis: a Case Report and Literature Review

2016 ◽  
Vol 25 (1) ◽  
pp. 99-103 ◽  
Author(s):  
Benoit Brilland ◽  
Johnny Sayegh ◽  
Anne Croue ◽  
Frank Bridoux ◽  
Jean-François Subra ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Light Chain ◽  
Intrahepatic Cholestasis ◽  
Relevant Literature ◽  
Gamma Glutamyl Transferase ◽  
Light Chain Deposition Disease ◽  
Deposition Disease ◽  
Uncommon Presentation ◽  
Light Chain Deposition ◽  
Glutamyl Transferase

Light chain deposition disease (LCDD) is a rare multisystemic disorder associated with plasma cell proliferation. It mainly affects the kidney, but liver and heart involvement may occur, sometimes mimicking the picture of systemic amyloidosis. Liver disease in LCDD is usually asymptomatic and exceptionally manifests with severe cholestatic hepatitis. We report the case of a 66-year-old female with κ-LCDD and cast nephropathy in the setting of symptomatic multiple myeloma who, after a first cycle of bortezomib-dexamethasone chemotherapy, developed severe and rapidly worsening intrahepatic cholestasis secondary to liver κ-light chain deposition. Intrahepatic cholestasis was attributed to LCDD on the basis of the liver histology and exclusion of possible diagnoses. Chemotherapy was maintained and resulted in progressive resolution of cholestasis. We report here an uncommon presentation of LCDD, with prominent liver involvement that fully recovered with bortezomib-based chemotherapy, and briefly review the relevant literature. Abbreviations: AKI: Acute kidney injury; ALP: alkaline phosphatase; ALT: alanine aminotransferase; AST: aspartate aminotransferase; CMV: Cytomegalovirus; EBV: Epstein–Barr virus; GGT: gamma-glutamyl transferase; HSV: Herpes simplex virus; LC: light chain; LCDD: Light chain deposition disease; MIDD: Monoclonal immunoglobulin deposition disease; MM: Multiple myeloma.

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