Kidney Week

Abstract: TH-PO0280

Beyond Congo Red: Diagnosing ALECT2 and AL Amyloidosis in Three Challenging Cases at a Single Center over 24 Months

Session Information

• Glomerular Diseases: Cell Biology
  October 22, 2026 | Location: Exhibit Hall A, Convention Center
  Abstract Time: 10:00 AM - 12:00 PM

Category: Glomerular Diseases

• 1401 Glomerular Diseases: Mechanisms, including Podocyte Biology

Authors

• Siddiqi, Mahwash, Penn State Health Milton S Hershey Medical Center, Hershey, Pennsylvania, United States

Mahwash Siddiqi, MD

• Tahir, Maria, Penn State Health Milton S Hershey Medical Center, Hershey, Pennsylvania, United States

Maria Tahir, MD

• Raza, Muhammad, Penn State Health Milton S Hershey Medical Center, Hershey, Pennsylvania, United States

Muhammad Raza, MD

• Portela-Colon, Rafael, Penn State Health Milton S Hershey Medical Center, Hershey, Pennsylvania, United States

Rafael Portela-Colon, MD, FASN

• Abendroth, Catherine, Penn State Health Milton S Hershey Medical Center, Hershey, Pennsylvania, United States

Catherine Abendroth, MD

• Farooq, Umar, Penn State Health Milton S Hershey Medical Center, Hershey, Pennsylvania, United States

Umar Farooq, MD, MS, MBA, FASN

• Miller, Ronald P., Penn State Health Milton S Hershey Medical Center, Hershey, Pennsylvania, United States

Ronald P. Miller, MD

• Ghahramani, Nasrollah, Penn State Health Milton S Hershey Medical Center, Hershey, Pennsylvania, United States

Nasrollah Ghahramani, MD, FASN

• Verma, Navin, Penn State Health Milton S Hershey Medical Center, Hershey, Pennsylvania, United States

Navin Verma, MD

Group or Team Name

• Division of Nephrology

Introduction

Renal amyloidosis is a rare cause of chronic kidney disease, with subtypes that differ substantially in clinical course and management. We present three cases: one ALECT2 (leukocyte chemotactic factor 2) and two AL amyloidosis, illustrating the diagnostic challenges and contrasting outcomes.

Case Description

Case 1: A 77-year-old Egyptian male with nephrotic-range proteinuria (UPCR 3.68 g/g) and preserved renal function (eGFR 86, creatinine 0.92, albumin 4.2). Electrophoresis was unremarkable. Kidney biopsy showed Congo red-positive amyloid with diffuse mesangial expansion; immunofluorescence was negative for monoclonal light chains. Mass spectrometry confirmed ALECT2 amyloidosis. Managed with Angiotensin Receptor Blocker and SGLT2 inhibitor.

Case 2: A 64-year-old White female presented with heart failure with reduced ejection fraction and progressive CKD (creatinine rising from 1.03 to 2.7 mg/dL). Kidney biopsy demonstrated Congo red-positive amyloid deposits with 3+ lambda and 2+ IgG staining on immunofluorescence, confirming AL amyloidosis. Chemotherapy was initiated but suspended due to renal decompensation requiring dialysis. This case represents the most aggressive presentation in our series, with rapid, renal and cardiac involvement.

Case 3: A 57-year-old White female with multiple myeloma presented with nephrotic syndrome (UPCR 23, albumin 1.8, cholesterol 505). Serum protein electrophoresis showed IgG lambda monoclonal protein; serum-free lambda chains were 266 mg/L (ratio 0.10). Kidney biopsy confirmed Congo red-positive amyloid with 4+ lambda staining; bone marrow confirmed Congo red-positive plasma cells, establishing AL amyloidosis with multiple myeloma. She achieved a complete hematologic response with chemotherapy and proceeded to autologous stem cell transplantation (SCT). Her renal function improved with a new baseline.

Discussion

These cases illustrate the heterogeneity of renal amyloidosis. ALECT2 (Case 1) is indolent, required mass spectrometry, and only supportive care exists. AL amyloidosis (Cases 2 and 3) varied widely — from dialysis-dependent multi-organ failure precluding transplant, to complete remission and successful autologous SCT. All cases required testing beyond routine Congo red staining for definitive subtyping.