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Published on: September 18, 2011
Case Report: Autologous stem cell transplantation in a patient diagnosed with AL amyloidosis in Kazakhstan
Olga Kolesnikova1, Vadim Kemaykin1, Zhandos Burkitbayev2
1Center for Oncohematology and Bone Marrow Transplantation, National Research Oncology Center, Astana, Kazakhstan.
Introduction:
AL amyloidosis is a rare plasma cell disorder that causes progressive organ dysfunction due to extracellular deposition of misfolded immunoglobulin light chains. Reports from Central Asia are scarce, and there is limited documentation of successful integration of daratumumab-based therapy with autologous hematopoietic stem cell transplantation (auto-HSCT) in this region.
Case Presentation:
A 65-year-old woman presented with progressive left supraclavicular lymphadenopathy. Examination revealed bilateral cervical and supraclavicular lymphadenopathy with an ECOG performance status of 1. Laboratory testing showed elevated κ free light chains (50.96 mg/L; κ/λ ratio 12.0). Lymph node biopsy with Congo red staining revealed amyloid deposits with apple-green birefringence, and immunohistochemical protein typing confirmed AL amyloidosis type kappa.
Interventions And Outcomes:
The patient received four cycles of bortezomib-cyclophosphamide-dexamethasone without hematologic response, followed by two cycles of daratumumab-cyclophosphamide-bortezomib-dexamethasone, achieving a very good partial response (dFLC reduced from 46.7 to 11.9 mg/L) and renal recovery (GFR from 21 to 71 mL/min). She underwent high-dose melphalan conditioning and auto-HSCT, with neutrophil engraftment on day +19 and grade 3 mucositis as the main complication. At one-year follow-up, she remains in hematologic and organ remission on monthly daratumumab maintenance.
Conclusion:
This case was selected for reporting due to several distinctive clinical and organizational aspects. AL amyloidosis remains underdiagnosed in Central Asia, and experience with daratumumab-based regimens combined with autologous hematopoietic stem cell transplantation (auto-HSCT) is limited in this region. At our center, patients with suspected plasma cell dyscrasias frequently present with advanced or atypical disease manifestations, often leading to diagnostic delays. Compared with other AL amyloidosis cases treated at our institution, this patient demonstrated an uncommon presentation dominated by extensive extramedullary lymph node involvement, absence of overt cardiac manifestations, and prolonged diagnostic latency. Furthermore, the case illustrates the feasibility of integrating modern induction therapy with daratumumab, successful stem cell mobilization, and auto-HSCT in a resource-variable healthcare setting.
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