11 January 2019 : Case report
A Case of T-Cell Large Granular Lymphocytic Leukemia and Renal Immunoglobulin Heavy Chain Amyloidosis
Challenging differential diagnosis, Diagnostic / therapeutic accidents, Rare disease, Educational Purpose (only if useful for a systematic review or synthesis)Julie Fu123ABCDEF*, Lisa X. Lee14E, Ping Zhou4BCD, Teresa Fogaren1AE, Cindy Varga124E, Raymond L. Comenzo124ABCDEFG
Am J Case Rep 2019; 20:43-47
BACKGROUND: 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.
Keywords: Amyloid, Antigens, CD98 Heavy Chain, Leukemia, Large Granular Lymphocytic, Amyloidosis, Bortezomib, Cyclophosphamide, Dexamethasone, Drug Therapy, Combination, Fatigue, Glucocorticoids, Immunoglobulin Heavy Chains, Immunosuppressive Agents, Kidney Diseases, Middle Aged, Proteinuria
09 Jun 2023 : Case reportVenous Thoracic Outlet Syndrome with an Upper Extremity Deep Vein Thrombosis Caused by a Dislocated Clavicl...
Am J Case Rep In Press; DOI: 10.12659/AJCR.939250
08 Jun 2023 : Case reportChylothorax: A Tangled Road to Definitive Diagnosis of Non-Hodgkin Lymphoma
Am J Case Rep In Press; DOI: 10.12659/AJCR.939098
06 Jun 2023 : Case reportA Classical Case of Cesarean Scar Endometriosis in a 35-Year-Old Woman Presenting with Cyclical Abdominal P...
Am J Case Rep In Press; DOI: 10.12659/AJCR.940200
05 Jun 2023 : Case reportPeri-Hilar Cystic Lymphangioma Mimicking a Biliary Cystic Lesion Causing Biliary Obstruction: A Case Report...
Am J Case Rep In Press; DOI: 10.12659/AJCR.939421
Most Viewed Current Articles
06 Dec 2021 : Case reportLipedema Can Be Treated Non-Surgically: A Report of 5 Cases
Am J Case Rep 2021; 22:e934406
13 Jul 2022 : Case reportWhistling Scrotum: An Unusual Presentation of Pneumomediastinum in the Setting of an Open Scrotal Wound
Am J Case Rep 2022; 23:e936441
07 Dec 2021 : Case reportEdwardsiella tarda: A Classic Presentation of a Rare Fatal Infection, with Possible New Background Risk Fac...
Am J Case Rep 2021; 22:e934347
23 Feb 2022 : Case reportPenile Necrosis Associated with Local Intravenous Injection of Cocaine
Am J Case Rep 2022; 23:e935250