Amyloid cast nephropathy: A rare presentation of multiple myeloma–associated light chain amyloidosis

2021 ◽  
pp. 239936932110638
Author(s):  
Marina Reis ◽  
Ana Rodrigues ◽  
Joana Cristovão Maques ◽  
Mário Góis ◽  
Helena Sousa ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Light Chain ◽  
Kidney Injury ◽  
Rare Presentation ◽  
Light Chain Amyloidosis ◽  
Cast Nephropathy ◽  
Amyloid Deposits ◽  
Response To Chemotherapy ◽  
Histologic Pattern ◽  
Light Chain Nephropathy

Introduction: Monoclonal immunoglobulins can cause a variety of histologic patterns of kidney injury, depending on the physicochemical properties. Multiple myeloma manifests more often as light-chain nephropathy. On the other hand, light-chain amyloidosis leads to glomerular and vascular amyloid deposits, but a less common presentation with amyloid casts has also been described. Rarely, more than one histologic pattern can be present in the same patient. Case report: We report a case of a 73-year-old man, diagnosed with multiple myeloma that 8 months after achieving partial response to chemotherapy develops acute kidney injury and nephrotic syndrome. Kidney biopsy revealed features of light-chain nephropathy, amyloid cast nephropathy, and glomerular and vascular amyloid deposits. Immunofluorescence was positive for IgA (++) and lambda chains (+++) and negative for kappa chains. After the diagnosis of multiple myeloma-associated light-chain amyloidosis, chemotherapy was initiated; unfortunately, the patient died 1 month after the diagnosis. Conclusion: Amyloid casts, isolated or accompanied by other renal or extra-renal amyloid deposits, are another form of tubular toxicity caused by dysproteinemias and should be systematically screened in kidney biopsies.

MO182CRYSTALLINE LIGHT CHAIN CAST NEPHROPATHY IS ASSOCIATED WITH EARLY MORTALITY IN PATIENTS WITH MULTIPLE MYELOMA

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Zishan Lin ◽  
Xu Zhang ◽  
Xiaojuan Yu ◽  
Suxia Wang ◽  
Xinan Cen ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Acute Kidney Injury ◽  
Median Time ◽  
Light Chain ◽  
Kidney Injury ◽  
Early Mortality ◽  
Monoclonal Immunoglobulin ◽  
Cast Nephropathy ◽  
Significant Difference ◽  
Ordinary Light

Abstract Background and Aims Light chain cast nephropathy is the most common paraprotein-associated kidney lesion in patients with multiple myeloma (MM). Rarely, light chain cast nephropathy could show crystalline appearance in patients with multiple myeloma, also known as crystalline light chain cast nephropathy. We here report the first retrospective study of crystalline light chain cast from a single centre. Method All native kidney biopsies were retrospectively studied in the Peking University First Hospital from 2000 to 2020. Newly diagnosed MM patients with were enrolled. Patients with light chain cast nephropathy at least one cast with crystalline appearance were identified as crystalline light chain cast nephropathy (Figure 1, n = 8), others were identified as ordinary light chain cast nephropathy (n = 18). Results The cohort of crystalline light chain cast nephropathy consisted of 6 men and 2 women with a median age of 59.5 (range, 41-73) years. All patients suffered from advanced multiple myeloma (1 with ISS staging II, 7 with ISS staging III) and acute kidney injury with a median eGFR of 5.59 (range, 2.27-26.04) mL/min/1.73m2. All patients except 1 required emergency dialysis at admission. Microhematuria was presented in 3 patients. Median proteinuria was 2.13 (rang, 0.83-3.59) g/24h and median serum albumin was 38.2 (30.7-46.7) g/L. No one presented with nephrotic syndrome. Monoclonal immunoglobulin, detected in all patients on serum protein immunofixation electrophoresis, was λ alone in 5 patients, κ alone in 1 patient, IgG λ in 1 patient, IgA λ in 1 patient. The 8 patients were followed up with a median time of 8 (range, 2-24) months. Three patients received VAD chemotherapy and 5 patients received bortezomib based regimens. At the time of last follow-up, 2 of 7 patients who needed emergency dialysis got rid of dialysis and the rest remained dialysis-dependent. Five patients died with a median time of 5 (range, 2-19) months, 2 patients achieved partial remission and 1 patient achieved complete remission. There was no significant difference in clinical features, treatments and main outcomes between crystalline light chain cast nephropathy patients and ordinary light chain cast nephropathy patients (Table 1). However, crystalline light chain cast nephropathy patients had higher early mortality than ordinary light chain cast nephropathy patients (50.0% vs 11.1%, p = 0.03). Conclusion Crystalline light chain cast nephropathy patients usually presented with acute kidney injury requiring emergency dialysis. Although various types of monoclonal immunoglobulin were detected, there was a dominance of the λ isotype. Compared to ordinary light chain cast nephropathy patients, crystalline light chain cast nephropathy patients had higher early mortality.

A case of multiple myeloma presenting with uric acid kidney stones

2019 ◽  
Vol 3 (2) ◽  
pp. 98-102
Author(s):  
Hassan Salameh ◽  
Christophe Sirac ◽  
Sébastien Bender ◽  
Abdurrahman Hamadah ◽  
Lynn D Cornell ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Uric Acid ◽  
Kidney Disease ◽  
Light Chain ◽  
Plasma Cells ◽  
Fractional Excretion ◽  
Rare Presentation ◽  
Genetic Sequencing ◽  
Cast Nephropathy ◽  
Monoclonal Immunoglobulin Deposition Disease

Kidney disease is common in patients with multiple myeloma. Patients often present with acute kidney failure most commonly the result of light chain cast nephropathy. Presentation can also be in the form of nephrotic syndrome associated with immunoglobulin light chain amyloidosis or monoclonal immunoglobulin deposition disease and tubulopathy as in acquired light chain Fanconi syndrome. We present a case of a 56-year-old male with multiple myeloma who presented with chronic kidney disease and uric acid stones. Chemical analysis of the nephrolithiasis showed it to be entirely composed of uric acid. Fractional excretion of uric acid was elevated at 35.9%. Kidney biopsy was consistent with light chain proximal tubulopathy. Bone marrow biopsy showed 30% kappa light chain–restricted plasma cells. Genetic sequencing identified the light chain to be from the Vk1-33 subgroup. The patient was treated with bortezomib, cyclophosphamide, and dexamethasone, with improvement of his kidney function. This case illustrates a rare presentation of multiple myeloma.

Cast Nephropathy in Plasma Cell Dyscrasias

2019 ◽  
pp. 347-358
Author(s):  
Sandhya Manohar ◽  
Nelson Leung
Keyword(s):  
Multiple Myeloma ◽  
Renal Failure ◽  
Plasma Cell ◽  
Light Chain ◽  
Wide Spectrum ◽  
Kidney Injury ◽  
Cast Nephropathy ◽  
Clinical Presentations ◽  
History Of ◽  
Plasma Cell Dyscrasias

Plasma cell dyscrasias are associated with a wide spectrum of renal lesions and clinical presentations. The most common is cast nephropathy, which clinically presents as severe acute kidney injury. It is usually seen in patients with multiple myeloma. In fact, the recent criteria of the International Myeloma Working Group from 2014 consider renal failure by light chain cast nephropathy as a myeloma defining event. Renal failure is a major cause of early mortality in patients with multiple myeloma, being second only to infection. Early diagnosis and treatment of multiple myeloma is the key to better outcomes in these patients. This chapter reviews, among other topics, the history of the terminology, mechanism of light chain cast formation and the clinical picture, precipitating factors, pathology, treatment, therapy, and future outlook for cast nephropathy.

scholarly journals Crystalglobulinemia in Multiple Myeloma: A Rare Case Report of Survival and Renal Recovery

2020 ◽  
Vol 7 ◽  
pp. 205435812092262
Author(s):  
Angela Chou ◽  
Christopher Long ◽  
Leon Vonthethoff ◽  
Shir-Jing Ho ◽  
Franziska Pettit ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Acute Kidney Injury ◽  
Light Chain ◽  
Monoclonal Gammopathy ◽  
Kidney Biopsy ◽  
Rare Complication ◽  
Kidney Injury ◽  
Endothelial Damage ◽  
Cast Nephropathy ◽  
Rare Case Report

Rationale: Crystalglobulinemia is a rare complication of monoclonal gammopathy wherein crystallized immunoglobulins deposit in various organs causing occlusive vasculopathy, endothelial damage, and thrombosis. It should be differentiated from light chain cast nephropathy without crystalline nephropathy through timely diagnosis with a kidney biopsy. Presenting concerns of the patient: We report a case of a 74-year-old female with polyarthralgia, chest pain, petechial rash, and acute kidney injury. Diagnoses: Kidney biopsy revealed eosinophilic casts in the tubular lumen and similar occlusive crystalline deposits within the glomerular vasculature and interlobular arteries. Bone marrow biopsy and serum electrophoresis confirmed immunoglobulin G (IgG) κ multiple myeloma. Interventions: Dialysis was initiated for severe oligoanuric acute kidney injury. The patient was treated with 5 sessions of plasmapheresis and 11 cycles of clone reduction chemotherapy with CyBorD (cyclophosphamide, bortezomib, and dexamethasone). Outcomes: This patient achieved excellent kidney recovery and is no longer dialysis dependent. Teaching points: Crystalglobulinemia should be suspected in patients with rapidly progressive acute kidney injury and monoclonal gammopathy. Timely investigation with kidney biopsy to differentiate this condition from light chain cast nephropathy and initiation of appropriate treatment can lead to remission of disease and excellent recovery of kidney function.

scholarly journals Multiple myeloma and light-chain amyloidosis: a rare presentation

Case reports ◽  
2018 ◽  
Vol 4 (2) ◽  
pp. 99-110
Author(s):  
Carmenza Liliana Sandoval ◽  
Bernarda Jinneth Acosta ◽  
Oscar Contreras ◽  
Jorge Vargas
Keyword(s):  
Multiple Myeloma ◽  
Light Chain ◽  
Rare Presentation ◽  
Light Chain Amyloidosis

Introducción. La amiloidosis sistémica primaria hace parte del espectro de neoplasias de células plasmáticas, donde las cadenas livianas de inmunoglobulina se depositan en múltiples órganos. El compromiso miopático con falla respiratoria y mieloma múltiple asociado es poco frecuente. Caso clínico. Se presenta el caso de un paciente con amiloidosis sistémica de cadenas livianas (AL) quien ingresó por miopatía con falla respiratoria e íleo adinámico, por lo que se llevó a la unidad de cuidados intensivos. Por histología se confirmó infiltración en piel y tracto digestivo y concomitantemente se presentó mieloma múltiple con lesiones óseas líticas y riñón de mieloma. El paciente tuvo buena respuesta al esquema CyBorD (ciclofosfamida, bortezomib, dexametasona), recuperó su función renal y tuvo disminución de las lesiones en piel. Sin embargo, el compromiso gastrointestinal y miopático fue difícil de manejar y se requirió soporte ventilatorio y nutrición parenteral. Discusión. El clínico puede pasar por alto esta patología, por lo que es probable llegar a fases avanzadas de la enfermedad. En la actualidad, con nuevos agentes de quimioterapia y trasplante autólogo, se puede aumentar la sobrevida de estos pacientes. Conclusión. La amiloidosis AL tiene un amplio espectro de manifestaciones y debe considerarse en los diagnósticos diferenciales a fin de hacer un diagnóstico precoz y hacerla una condición tratable.

scholarly journals Lambda Light Chain Myeloma with Oliguric Cast Nephropathy and Remission with Bortezomib, Doxorubicin and Dexamethasone

2012 ◽  
Vol 52 (188) ◽  
Author(s):  
R Hada ◽  
B Poudyal ◽  
A Sharma ◽  
R Khatri
Keyword(s):  
Multiple Myeloma ◽  
Acute Kidney Injury ◽  
Light Chain ◽  
Plasma Cells ◽  
Kidney Injury ◽  
Lambda Light Chain ◽  
Serum Free Light Chain ◽  
Cast Nephropathy ◽  
M Spike ◽  
Dependent State

Cast nephropathy is one of the major causes of renal failure in patients with multiple myeloma resulting from precipitation of free light chains inside the tubules. Timely diagnosis and treatment confers a better prognosis though around 10% of patients with cast nephropathy remain dialysis dependent in spite of treatment.We report the clinical course and outcome of a patient presenting with acute kidney injury and oliguria, preceded by acute gastroenteritis and intake of Chinese medications and dialysis dependent state for eight weeks. Kidney biopsy revealed cast nephropathy with lambda light chain restriction and severe tubular injury. Serum protein electrophoresis was normal with no “M spike” but serum free light chain ratio was altered with very high lambda and normal kappa light chain levels. Bone marrow biopsy showed >85% atypical plasma cells. Haemodialysis was continued and chemotherapy with bortezomib, doxorubicin and dexamethasone was started. Kidney function gradually improved with discontinuation of dialysis after 1 month and complete remission of acute kidney injury and myeloma in 4 months of chemotherapy.   Keywords: acute kidney injury; bortezomib; cast nephropathy; multiple myeloma.

scholarly journals LIGHT CHAIN MULTIPLE MYELOMA: A CASE REPORT

2020 ◽  
Vol 11 (10) ◽  
pp. 1-3
Author(s):  
Shamsudeen Moideen ◽  
Maheshwari V ◽  
Raghuveer Prabhu
Keyword(s):  
Multiple Myeloma ◽  
Light Chain ◽  
Rare Case ◽  
Bone Pain ◽  
Kidney Injury ◽  
Signs And Symptoms ◽  
Superior Efficacy ◽  
Urine And Serum ◽  
Ig G ◽  
Histopathology Examination

Light chain multiple myeloma (LCMM) initiates approximately 15 percentage of patients with multiple myeloma (MM). It has a lower prognosis when compared with the variant immunoglobulin (Ig) G or IgA. We report a rare case on Light chain multiple myeloma in 49-year-old male patient who presented with acute kidney injury, hypercalcemia. histopathology examination was found to be plasmacytoma, kappa restricted; and free kappa lamda chain in urine and serum was found to be greater. Bone pain and renal dysfunction were the most common prevalent initial signs and symptoms while extramedullary disease (EMD) was later acquired during disease. Bortezomib demonstrated superior efficacy over nonbortezomib in LCMM patients.

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