A case of primary (AL) amyloidosis with predominantly vascular amyloid deposition in the kidney

Murakami, Yoichi; Hattori, Soken; Sugiyama, Fumiko; Yoshikawa, Kazuyuki; Sugiura, T.; Matsushima, Hideki

doi:10.1007/s13730-014-0157-7

Cited by 6 publications

(7 citation statements)

References 23 publications

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“…Due to the several causes of proteinuria in the elderly, renal biopsy should be performed in all cases of urinary abnormalities despite the finding of positive Congo red staining in another tissue. The extent and distribution of renal amyloid deposition might also be important when comparing the outcomes of these patients [ 22 ].…”

Section: Discussionmentioning

confidence: 99%

Daratumumab Monotherapy in Severe Patients with AL Amyloidosis and Biopsy-Proven Renal Involvement: A Real Life Experience

Roccatello

Fenoglio

Naretto

et al. 2020

JCM

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Objectives: This paper aims to describe the clinical experience with Daratumumab (DARA), a first-in-class anti-CD38 human monoclonal IgG1κ antibody monotherapy, in severe patients with AL and biopsy-proven renal involvement. Immunoglobulin light chain (AL) amyloidosis with multi-organ involvement is characterized by short survival. Novel powerful drugs are expanding the therapeutic options. Current treatment of AL amyloidosis, which has been adopted from multiple myeloma (MM), is based on chemotherapy targeting the underlying plasma cell clone. DARA is effective in treating MM. The clinical activity and toxicity profile of DARA as a single agent in the treatment of AL amyloidosis is currently under evaluation. Patients and Methods: DARA was administered in a series of patients with severe AL amyloidosis and biopsy-proven renal involvement. Five patients(mean age 64.2 years) were treated. One patient was refractory and one intolerant to conventional bortezomib-based therapy, two were treated with DARA for relapsing disease, and one was treated front-line. Results: Data showed that DARA monotherapy resulted in good clinical results, with the disappearance of M-proteins in four out of five patients and with serum free light chains (sFLC) ratio normalization in three out of four and a remarkable amelioration in the remaining patient. The four patients with still preserved renal function at baseline also showed serum creatinine stabilization or improvement and a decrease in proteinuria. These data were paralleled by the reduction of the N-terminal prohormone of brain natriuretic peptide (NT pro-BNP)values. Conclusions: Our data show that monotherapy with DARA had significant clinical efficacy in pretreated/naïve patients with severe AL amyloidosis and biopsy-proven renal involvement.

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Section: Discussionmentioning

confidence: 99%

Daratumumab Monotherapy in Severe Patients with AL Amyloidosis and Biopsy-Proven Renal Involvement: A Real Life Experience

Roccatello

Fenoglio

Naretto

et al. 2020

JCM

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“…Much less commonly, amyloid fibrils deposit exclusively in the renal vasculature (Tab. II) or tubulointerstitium and causes minimal or no proteinuria (2,(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22). This presents a diagnostic challenge.…”

Section: Discussionmentioning

confidence: 99%

Severe Orthostatic Hypotension Complicating Multiple Myeloma

Sury

Mutter

Moeckel

et al. 2017

Journal of Onco-Nephrology

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greater renal dysfunction and shorter median survival than patients who had widespread amyloid deposition (2). Cases of isolated vascular amyloidosis have been described in the literature, with a variety of presenting symptoms, including typical angina (3), headaches and visual changes consistent with giant cell arteritis (4), and various dermatologic conditions (5, 6). Approximately 15%-30% of patients with multiple myeloma have concomitant amyloidosis. Most with multiple myeloma and acute kidney injury (AKI) have light chain cast nephropathy, which often presents as non-albumin proteinuria composed of monoclonal immunoglobulins and/or light chains. Vascular amyloidosis, which generally presents with absent or lower grade proteinuria than the typically nephrotic-range seen with glomerular amyloidosis, may be under-recognized and may delay diagnosis and treatment. Though the clinical features of isolated vascular amyloidosis versus diffuse amyloidosis of the kidney have been described in the literature (2), the unique difficulties of renal replacement therapy (RRT) in these patients have not been well described. Here, we present a case of isolated vascular amyloidosis in a patient with multiple myeloma, marked by severe orthostatic hypotension and difficult to manage hemodynamic instability during RRT.

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“…Amyloidoza jest chorobą występującą rzadko; częstość zachorowań w krajach wysoko rozwiniętych, w tym w Polsce, szacuje się na 5-13 nowych przypadków/mln mieszkańców/rok [2,8,[12][13][14][15], a chorobowość -na około 20/mln [12]. Najczęściej amyloidozę rozpoznaje się u osób w wieku 60-79 lat i jest ona przyczyną 0,58/1000 zgonów [15].…”

Section: Typy Amyloidozy I Ich Rozpowszechnienieunclassified

“…Amyloidozę stwierdza się w 2,5-6,5% bioptatów gruboigłowych nerek u osób dorosłych [14,21,31,32]. Ryzyko rozpoznania amyloidozy w bioptacie nerki zwiększa się z wiekiem pacjenta i wynosi 3,7% wśród osób w wieku 18-64 i aż 13,9% wśród osób w 65. roku życia i starszych [32].…”

Section: Amyloidoza Nerekunclassified

“…W przeszłości Wojciech Wołyniec i wsp., Amyloidoza nerek dominującymi przyczynami amyloidozy AA były przewlekłe infekcje: zapalenia kości, rozstrzenia oskrzeli, gruźlica, nadal częste w Afryce i Azji. W Turcji, a także wśród Żydów sefardyjskich, Arabów, Ormian, Greków i Włochów najczęstszą przyczyną (21-64% przypadków) amyloidozy AA jest rodzinna gorączka śródziemnomorska (FMF, familial mediterranean fever) [14,38,47]. Mniej częste przyczyny amyloidozy AA to inne genetycznie uwarunkowane zespoły gorączek nawrotowych: zespół gorączki nawrotowej zależny od receptora dla czynnika martwicy nowotworów (TNF) (TRAPS, tumor necrosis factor receptor-associated periodic syndrome), zespół hiperimmunoglobulinemii D (HIDS, hyperimmunoglobulin D syndrome), kriopirynozależny zespół gorączek nawrotowych (CAPS, cryopyrin-associated periodic syndrome), obejmujący rodzinny autozaplany zespól reakcji na zimno i zespół Muckle'a-Wellsa [48].…”

Section: Amyloidoza Aaunclassified

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Amyloidoza nerek

et al. 2018

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Kidney amyloid deposition is one of the most common and most severe manifestations of systemic amyloidosis. Since kidney involvement occurs in almost each type of amyloidosis, proteinuria and glomerular filtration rate decrease are often among the first symptoms of this disease. Kidney involvement is, beside heart failure, a major prognostic factor. The most typical presentation is isolated nephrotic syndrome, especially in two most common types of the disease: AA and AL amyloidoses. The less common manifestation is chronic renal failure, which typically occurs in the acquired types of amyloidosis (ALECT2, AApoAIV), but also in several hereditary genetic forms. In

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A case of primary (AL) amyloidosis with predominantly vascular amyloid deposition in the kidney

Cited by 6 publications

References 23 publications

Daratumumab Monotherapy in Severe Patients with AL Amyloidosis and Biopsy-Proven Renal Involvement: A Real Life Experience

Daratumumab Monotherapy in Severe Patients with AL Amyloidosis and Biopsy-Proven Renal Involvement: A Real Life Experience

Severe Orthostatic Hypotension Complicating Multiple Myeloma

Amyloidoza nerek

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