Journal of Pathology and Translational Medicine

Case Study

A case of monoclonal gammopathy of renal significance presenting as atypical amyloidosis with IgA lambda paraproteinemia: Chankyung Kim, John Brealey, Anjelo Jobert, James Nolan; J Pathol Transl Med. 2020;54(6):504-507. Published online November 9, 2020; DOI: https://doi.org/10.4132/jptm.2020.09.18

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Monoclonal gammopathy of renal significance is defined as any B cell or plasma cell clonal lymphoproliferation which neither causes tumor complications nor meets any current hematological criteria for specific therapy, with one or more kidney lesions related to the produced monoclonal immunoglobulin, such as amyloidosis. A 50-year-old male presented with heavy proteinuria and blood tests showing IgA and Lambda paraproteinemia. Light microscopy showed mesangial eosinophilic ground substance extending into the capillary loops, and positive staining within the glomeruli and vessel walls for amyloid P immunohistochemistry was also noted. Immunofluorescence showed positive staining for IgA and Lambda in the mesangia and capillary loops. Electron microscopy exhibited organized fibrils measuring 4–5 nm in diameter in the mesangia, glomerular basement membranes and vessel walls. We interpreted the overall findings as atypical renal amyloidosis with IgA and Lambda deposition on immunofluorescence. Further amyloid typing using laser microdissection-liquid chromatography and mass spectrometry will be useful.

Citations

Citations to this article as recorded by

AB-Amy: machine learning aided amyloidogenic risk prediction of therapeutic antibody light chains
Yuwei Zhou, Ziru Huang, Yushu Gou, Siqi Liu, Wei Yang, Hongyu Zhang, Anthony Mackitz Dzisoo, Jian Huang
Antibody Therapeutics.2023; 6(3): 147. CrossRef

Case Report

Localized Primary Thymic Amyloidosis Presenting as a Mediastinal Mass: A Case Report.: Sang Yun Ha, Jae Jun Lee, Heejung Park, Joungho Han, Hong Kwan Kim, Kyung Soo Lee; Korean J Pathol. 2011;45:S41-S44.; DOI: https://doi.org/10.4132/KoreanJPathol.2011.45.S1.S41

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Abstract PDF

We herein describe a case of a 55-year-old healthy woman with localized primary thymic amyloidosis presented as a mediastinal mass, found incidentally by chest radiography. Computed tomography revealed a 4.1 cm soft tissue lesion with nodular calcification in the left anterior mediastinum. The resected specimen was a well-defined lobulating mass with calcification. Microscopically, the mass was consisted of amorphous eosinophilc hyalinized substances involving the thymus and intrathymic lymph nodes. These eosinophilic substances showed apple-green bi-refringence under polarized light after staining with Congo red. In immunohistochemical study, they were positive for kappa and lambda light chains and negative for amyloid A. There was no evidence of systemic amyloidosis in clinical investigations. A final diagnosis of localized primary thymic amyloidosis was made.

Citations

Citations to this article as recorded by

EBUS-TBNA diagnosis of localised amyloidosis presenting as mediastinal lymphadenopathy
Avnee Kumar, Barathi Sivasailam, Ellen Marciniak, Janaki Deepak
BMJ Case Reports.2018; 11(1): e226619. CrossRef
Ocular Myasthenia Gravis Associated With Thymic Amyloidosis
Kristin O. Chapman, Debra M. Beneck, Marc J. Dinkin
Journal of Neuro-Ophthalmology.2016; 36(1): 50. CrossRef
Localized Thymic Amyloidosis Presenting with Myasthenia Gravis: Case Report
Seung-Myoung Son, Yong-Moon Lee, Si Wook Kim, Ok-Jun Lee
Journal of Korean Medical Science.2014; 29(1): 145. CrossRef

Original Article

Structural-Functional Relationships in Renal Amyloidosis.: Myeong Cherl Kook, Hyun Soon Lee; Korean J Pathol. 1997;31(11):1190-1199.

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Abstract: The pathogenetic mechanism of renal dysfunction in renal amyloidosis is poorly understood. To evaluate the morphologic parameters which are correlated with renal function in this disorder, we have examined renal biopsies from 14 patients with renal amyloidosis by morphometry. Of the 14 patients, 8 were male and 6 were female. They were between 41 and 70 years of age. The serum concentration of albumin and creatinine were 2.1+/-0.7 mg/dl and 1.1+/-0.5 mg/dl, respectively. The 24-hour excretion of urinary protein was 7.9+/-5.2 g. Creatinine clearance was 62+/-23 ml/min/1.73m2. The mean glomerular volume (MGV) was (2.2+/-1.3) 10(6) micrometer3. The surface density of peripheral glomerular basement membrane [Sv (PGBM/glom)] was 0.049+/-0.027 (micrometer3/micrometer3). Volume density of mesangium [Vv (mes/glom)] was 0.31+/-0.14 (micrometer3/micrometer3) and volume density of glomerular amyloid deposition [Vv (amyl/glom)] was 0.21+/-0.14 (micrometer3/micrometer3). The volume density of cortical interstitium [Vv (int/cortex)] was 0.14+/-0.09 (micrometer3/micrometer3). The serum creatinine concentration was significantly correlated with Vv (int/cortex) (r=+0.66, p<0.05). MGV was correlated with Vv (mes/glom) (r=+0.75, p<0.01) and Vv (amyl/glom) (r= +0.68, p<0.05) but showed negative correlation with Sv (PGBM/glom) (r=-0.79, p<0.01). Sv (PGBM/glom) showed negative correlation with Vv (mes/glom) (r=-0.77, p<0.01) and with Vv (amyl/glom) (r=-0.87, p<0.01). Positive correlation was observed between Vv (mes/glom) and Vv (amyl/glom) (r=+0.95, p<0.01). These results suggest that the decreased renal function in patients with amyloidosis is related to interstitial fibrosis rather than glomerular lesions. In addition, glomerular hypertrophy in these patients is related to amyloid deposition in the mesangium and peripheral glomerular basement membrane.

Case Reports

Fine Needle Aspiration Cytology of Primary Pulmonary Amyloidosis: A Case Report .: Hyuni Cho, Seung Yeon Ha, Young Ha Oh, Seong Hwan Jeong; Korean J Cytopathol. 2000;11(2):99-102.

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Abstract PDF: Pulmonary amyloid deposition generally occurs with concurrent primary systemic amyloidosis. Localized forms of pulmonary amyloidosis are rare and appear most frequently as an incidental finding on chest radiographs. We present a case of nodular pulmonary amyloid tumor suggested by fine needle aspiration cytology (FNAC) and confirmed by histologic examination with the polarizing light microscopy. A 41-year-old woman presented with ill-defined nodules in the middle and lower lobes of both lungs. FNAC of the nodules revealed waxy, acellular amorphous fragments. Thoracotomy for diagnosis may be avoided by FNAC diagnosis of this unusual lesion.

Ureteral Washing Cytology of Localized Ureteral Amyloidosis: A Case Report.: Won Ae Lee, Jeong Hae Kie, Yoon Mi Jeen, Mi Kyung Lee; Korean J Cytopathol. 2002;13(1):38-41.

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Abstract PDF: Localized amyloidosis of the ureter is rare and clinically confused with neoplasm. We describe a case of localized amyloidosis of the ureter in which the presence of amyloid was detected in ureteral washing cytology. A 75-year-old female presented with gross hematuria. Abdominal CT and retrograde pyelography revealed hydronephrosis and hydroureter on the left side with abrupt narrowing of the distal ureter. Ureteral washing cytology yielded a hypocellular smear with many irregular clumps of amorphous, extracellar, waxy material. Biopsy sections demonstrated submucosal deposits of eosinophilic amorphous material which gave characteristic apple green birefringence with Congo-red stain under the polarized light. Familarity with the cytologic features of amyloid is helpful for preoperative diagnosis and proper treatment.

Localized Amyloidosis of the Ureter: A Report of Two Cases.: Ho Jung Lee, Dong Eun Song, Jong Eun Joo, Won Mi Lee, Dong Hoon Kim, Eun Kyung Kim, Jae Y Ro; Korean J Pathol. 2004;38(3):184-187.

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Abstract PDF: We report on two cases of localized amyloidosis involving the ureter. The patients were a 64-year-old woman with right upper quadrant pain (case 1) and a 36-year-old woman suffering from left flank pain and intermittent gross hematuria (case 2). An intravenous pyelography of case 1 revealed multiple filling defects in the entire right ureter, whereas retrograde pyelography in case 2 showed diffuse narrowing in the mid and lower portions of the left ureter. Localized amyloidosis of the ureter was diagnosed in the two cases, and both had amyloid deposit in the renal pelvis and the urinary bladder in case 1, and in the contralateral ureter and the renal pelvis in case 2. Right nephroureterectomy was performed in case 1, but a segmental resection of the ureter with preservation of the kidney was administered in case 2. These two cases demonstrate that ureteral amyloidosis can be associated with amyloid deposition in the renal pelvis and the urinary bladder. Although ureteral amyloidosis is a rare occurrence, it should be considered in the differential diagnosis of ureteral obstruction to avoid unnecessary radical surgery.

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