Heavy-chain deposition disease: a morphological, immunofluorescence and ultrastructural assessment

Rane, Swapnil; Rana, Seema; Mudrabettu, Chetan; Jha, Vivekanand; Joshi, Kusum

doi:10.1093/ckj/sfs062

Cited by 10 publications

(4 citation statements)

References 36 publications

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“…Confirmation of heavy chain deposition has to be done by immunostaining and immunoelectrophoresis of IgA levels. Heavy chain-associated myelomas have been reported previously and lead to end-stage renal disease in a slow process [8, 9]. The diagnosis is supported by the medium expression of LCN2 levels, which is a kidney injury marker as opposed to high expression representing kidney infection.…”

Section: Discussionmentioning

confidence: 99%

Case Report of a Rare Incidence of IgH Amplification Leading to Acute Kidney Injury in a Multiple Myeloma Patient

Thanikachalam

Srinivasalu

et al. 2021

Case Rep Oncol

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We present a case report of a 62-year-old male, treated for kappa light chain multiple myeloma with chemotherapy followed by autologous stem cell transplant (ASCT) in 2014. He has been in complete remission for 4 years. In 2018, he was evaluated for hypercreatinemia and acute kidney injury(AKI) with a suspicion of disease relapse; he underwent evaluation with bone marrow aspiration cytology which showed no evidence of relapse. However, careful cytogenetic analyses showed IgH amplification (14q32) which probably was the cause for AKI in the absence of any structural abnormality in the kidney. Heavy chain deposition leads to AKI in multiple myeloma, and its association with IgH amplification leading to AKI is reported here. Though heavy chain deposition leading to AKI is common, IgH amplification at chromosome level is the first case observed.

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Section: Discussionmentioning

confidence: 99%

Case Report of a Rare Incidence of IgH Amplification Leading to Acute Kidney Injury in a Multiple Myeloma Patient

Thanikachalam

Srinivasalu

et al. 2021

Case Rep Oncol

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“…HCDD is also a rare B cell proliferative disorder that has clinical characteristics similar to AL amyloidosis and LCDD. The deposits in HCDD are intact or truncated heavy chains that also do not stain positive with Congo red [44].…”

Section: Q15: Are There Cases Of Systemic Monoclonal Immunoglobulin D...mentioning

confidence: 99%

Light-Chain Amyloidosis: The Great Impostor

Stefani,

Kouvata,

Vassilopoulos

2023

Life

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Light-chain amyloidosis (AL) is a disease of protean manifestations due to a wide spectrum of organs that can be affected. The disorder is caused by the deposition of an extracellular amorphous material, the amyloid, which is produced by malignant plasma cells. The latter usually reside in the bone marrow; plasma cell infiltration is often low, in sharp contrast to what we observe in multiple myeloma. The disease may run below the physician’s radar for a while before clinical suspicion is raised and targeted tests are performed. In this short review, we try to answer most of the questions that a practicing physician may ask in a relative clinical setting. The text is formed as a series of reader-friendly questions that cover the subject of AL amyloidosis from history to current therapy.

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“…HCDD is a much rarer form of monoclonal Ig deposition disorder due to the synthesis of abnormal HC with C H 1 and V H region abnormalities, leading to their early secretion from plasma cells prior to conjugation with LC. Less than 50 cases have so far been reported in the literature, featuring isolated HC from IgA or IgD, mostly accumulated in the form of nodular glomerulosclerosis around mesangial deposits, similar to LCDD [27] (see below).…”

Section: Biology Of Immunoglobulin Lc and Significance Of MC Componentsmentioning

confidence: 99%

Monoclonal Gammopathies of Renal Significance: Renal Biopsy and Beyond

Menè¹,

Alexandris

Moioli

et al. 2020

Cancers

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Monoclonal Gammopathies of Renal Significance (MGRS) are a rather heterogeneous group of renal disorders caused by a circulating monoclonal (MC) immunoglobulin (Ig) component, often in the absence of multiple myeloma (MM) or another clinically relevant lymphoproliferative disorder. Nevertheless, substantial kidney damage could occur, despite the “benign” features of the bone-marrow biopsy. One example is renal amyloidosis, often linked to a small clone of plasma cells, without the invasive features of MM. However, patients with amyloidosis may present with a nephrotic syndrome and renal failure, eventually leading to end-stage kidney disease. At the same time, other organs, such as the heart and the liver, may be severely damaged by Ig light chains or amyloid deposits, occasionally resulting in fatal arrhythmias and/or organ failure. Acute kidney injury (AKI) may as well result from massive excretion of MC proteins, with deposition disease in glomeruli or renal tubules, not rarely obstructed by luminal aggregates, or “casts”. Proliferative glomerulonephritis with monoclonal Ig deposits is another, less frequent clinical presentation of an MGRS. The present review deals with the implications of MGRS for renal function and prognosis, and the potential of tools, such as the renal biopsy, for assessing clinical risk and guiding therapy of the underlying condition.

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Heavy-chain deposition disease: a morphological, immunofluorescence and ultrastructural assessment

Cited by 10 publications

References 36 publications

Case Report of a Rare Incidence of IgH Amplification Leading to Acute Kidney Injury in a Multiple Myeloma Patient

Case Report of a Rare Incidence of IgH Amplification Leading to Acute Kidney Injury in a Multiple Myeloma Patient

Light-Chain Amyloidosis: The Great Impostor

Monoclonal Gammopathies of Renal Significance: Renal Biopsy and Beyond

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