{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,24]],"date-time":"2026-09-24T22:24:11Z","timestamp":1790288651890,"version":"4.1.0"},"reference-count":19,"publisher":"MDPI AG","issue":"10","license":[{"start":{"date-parts":[[2023,10,4]],"date-time":"2023-10-04T00:00:00Z","timestamp":1696377600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Medicines"],"abstract":"<jats:p>Background: Monoclonal immunoglobulin deposition disease (MIDD) includes three entities: light chain deposition disease (LCDD), heavy chain deposition disease (HCDD) and light and heavy chain deposition disease (LHCDD). The renal presentation can manifest with varying degrees of proteinuria and\/or nephrotic syndrome, microhematuria, and often leads to end-stage renal disease. Given the rarity of LHCDD, therapeutic approaches for this condition remain inconclusive, as clinical trials are limited. Case presentation: We report two male patients with underlying monoclonal gammopathy of renal significance (MGRS) associated with LHCDD lesions. Both cases had non-nephrotic proteinuria, moderately impaired renal function, and normal levels of C3 and C4. Light microscopy of the renal biopsies in both patients did not show lesions of nodular glomerulosclerosis. Immunofluorescence showed a staining pattern with interrupted linear IgA-\u03ba in patient #1 and IgA-\u03bb in patient #2 only along the glomerular basement membrane (GBM). Electron microscopy of patient #1 revealed electrodense deposits in the subendothelial and mesangial areas only along the GBM. Discussion: In this case series, we discuss the clinical, analytical, and histopathological findings of two rare cases of LHCDD. Both patients exhibited IgA monoclonality and were diagnosed with monoclonal gammopathy of undetermined significance (MGUS) by the hematology department at the time of renal biopsy. Treatment with steroids and cytotoxic agents targeting the clone cells responsible for the deposition disease resulted in a favorable renal and hematologic response.<\/jats:p>","DOI":"10.3390\/medicines10100055","type":"journal-article","created":{"date-parts":[[2023,10,4]],"date-time":"2023-10-04T11:58:57Z","timestamp":1696420737000},"page":"55","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Monoclonal Gammopathy of Renal Significance with Deposits of Infrequent Morphology: Two Case Reports of Light and Heavy Chain Deposition Disease with Atypical Presentation and Literature Review"],"prefix":"10.3390","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9346-2279","authenticated-orcid":false,"given":"Jos\u00e9 C.","family":"De La Flor","sequence":"first","affiliation":[{"name":"Department of Nephrology, Hospital Central de la Defensa, 28046 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maribel","family":"Monroy-Condori","sequence":"additional","affiliation":[{"name":"Section of Nephrology and Hypertension, Hospital General Universitario Nuestra Se\u00f1ora del Prado, 45600 Talavera de la Reina, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jacqueline","family":"Apaza-Chavez","sequence":"additional","affiliation":[{"name":"Department of Nephrology, Hospital Fuensanta, 28027 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Iv\u00e1n","family":"Arenas-Moncaleano","sequence":"additional","affiliation":[{"name":"Section of Nephrology and Hypertension, Hospital General Universitario Nuestra Se\u00f1ora del Prado, 45600 Talavera de la Reina, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5090-8488","authenticated-orcid":false,"given":"Francisco","family":"D\u00edaz","sequence":"additional","affiliation":[{"name":"Department of Anatomic Pathology, Hospital Gregorio Mara\u00f1\u00f3n, 28007 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xavier E.","family":"Guerra-Torres","sequence":"additional","affiliation":[{"name":"Section of Nephrology and Hypertension, Hospital General Universitario Nuestra Se\u00f1ora del Prado, 45600 Talavera de la Reina, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jorge L.","family":"Morales-Montoya","sequence":"additional","affiliation":[{"name":"Section of Nephrology and Hypertension, Hospital General Universitario Nuestra Se\u00f1ora del Prado, 45600 Talavera de la Reina, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana","family":"Lerma-Verdejo","sequence":"additional","affiliation":[{"name":"Department of Hematology, Hospital General Nuestra Se\u00f1ora del Prado, 45600 Talavera de la Reina, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Edna","family":"Sandoval","sequence":"additional","affiliation":[{"name":"Department of Hematology, Hospital Central de la Defensa, 28046 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8982-8442","authenticated-orcid":false,"given":"Daniel","family":"Villa","sequence":"additional","affiliation":[{"name":"Department of Nephrology, Hospital Cl\u00ednica Universiad Navarra, 31009 Pamplona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Coca-Mihaela","family":"Vieru","sequence":"additional","affiliation":[{"name":"Department of Anatomic Pathology, Hospital Gregorio Mara\u00f1\u00f3n, 28007 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,10,4]]},"reference":[{"key":"ref_1","first-page":"243","article-title":"Monoclonal gammopathy of renal significance (MGRS): Histopathologic classification, diagnostic workup, and therapeutic options","volume":"77","author":"Amaador","year":"2019","journal-title":"Neth. 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