A Case of T-Cell Large Granular Lymphocytic Leukemia and Renal Immunoglobulin Heavy Chain Amyloidosis

Abstract

Patient: Female, 57Final Diagnosis: Renal heavy chain amyloidosisSymptoms: Fatigue • proteinuriaMedication: —Clinical Procedure: Chemotherapy, consideration of autologous stem cell transplantSpecialty: HematologyObjective:Rare diseaseBackground:T-cell large granular lymphocytic leukemia (T-LGL) is a rare hematological malignancy that currently has no standard therapy. Immunoglobulin heavy chain amyloidosis (AH) involving the kidney is a rare condition and the pathology, diagnosis, clinical characteristics, and prognosis are becoming understood. This report is of a rare case of T-LGL associated with renal AH and discusses the approach to management.Case Report:A 57-year-old woman presented with symptoms of fatigue and she had proteinuria. A diagnosis of T-LGL associated with renal AH was made, which is an association that has not been previously reported in the literature. Given the dysregulation of her immune function due to her underlying T-LGL and her comorbidities, treatment options were limited. She was clinically stable and was initially observed. After one year, her symptoms of fatigue became worse, and her proteinuria increased. Treatment was initiated with the triple drug combination of bortezomib, cyclophosphamide, and dexamethasone (CyBorD) with consideration for future hematopoietic stem cell transplantation (HSCT). Her clinical condition improved, with a reduction in proteinuria.Conclusions:A rare case of T-LGL and renal AH is presented. Currently, there is no standard therapy for T-LGL and AH amyloidosis, and the approach, in this case, was to manage the patient initially with CyBorD triple chemotherapy.

Case Report: Endocapillary Glomerulopathy Associated With Large Granular T Lymphocyte Leukemia

“…Abnormal B-cell activities in LGLL patients could also induce kidney impairment. Zhang M et al reported crescentic glomerulonephritis induced by anti-GBM disease in a patient of T-LGLL ( 9 ). There is also a case report of AH renal amyloidosis type (γ1) with T-LGLL ( 10 ).…”

How We Manage Systemic Immunoglobulin Heavy Chain Amyloidosis (AH Amyloidosis) and Immunoglobulin Heavy-and-Light-Chain Amyloidosis (AH/AL Amyloidosis)

“…This case was published previously before HSCT. 4 Table 1 compares the patients' baseline laboratory characteristics.…”

“…In AH, the amyloid fibrils originate from fragments of the Ig heavy chain, whereas in Ig heavy-and-light-chain amyloidosis (AH/AL amyloidosis), both Ig heavy chains and LC contribute to the amyloid fibrils. [2][3][4] AH/AL amyloidosis is the rarest form of Igrelated systemic amyloidosis. 2,3 Renal involvement characterized by proteinuria with or without renal insufficiency is common in AL, AH, and AH/AL amyloidosis.…”

Spectrum of Kidney Disorders Associated with T-Cell Immunoclones

“…Cytopenia (autoimmune hemolytic anemia and neutropenia) and rheumatoid arthritis are the most frequent immune disorders associated with LGL, but a wide panel of autoimmune diseases were also observed in patients with LGL, including hematological (red cell aplasia, pernicious anemia, thrombocytopenia), neurological (myasthenia gravis, polyradiculoneuropathy), or rheumatic (Sjögren's syndrome, lupus) disorders [1,2]. To date, only seven cases of renal disease related to LGL have been reported, including renal infiltration by the LGL or heterogeneous glomerulopathies (focal and segmental glomerulosclerosis, vasculitis with anti-glomerular basement membrane antibodies, heavy-chain amyloidosis, glomerulonephritis with endocapillary proliferation) [6][7][8][9][10][11][12]. Recently, we reported two cases of inflammatory renal fibrosis associated with a small-sized T-cell clone (i.e., a clonal population not associated with lymphocytosis and thus requiring dedicated immunophenotyping to be identified) [13].…”