Recurrent microangiopathic hemolysis after recovery from complement-mediated hemolytic uremia syndrome during chemotherapy for a CFH-mutated patient with T-lymphoblastic lymphoma.

Autor: Lee FW; Department of Pediatrics, Taipei Veterans General Hospital, and National Yang Ming Chiao Tung University School of Medicine, Peitou District, NO. 201, Shih-Pai Rd Sec 2, Taipei, 112, Taiwan, ROC., Lee CY; Department of Pediatrics, Taipei Veterans General Hospital, and National Yang Ming Chiao Tung University School of Medicine, Peitou District, NO. 201, Shih-Pai Rd Sec 2, Taipei, 112, Taiwan, ROC.; Department of Life Science, National Taiwan Normal University, Taipei, Taiwan, ROC., Hung GY; Department of Pediatrics, Taipei Veterans General Hospital, and National Yang Ming Chiao Tung University School of Medicine, Peitou District, NO. 201, Shih-Pai Rd Sec 2, Taipei, 112, Taiwan, ROC.; Department of Life Science, National Taiwan Normal University, Taipei, Taiwan, ROC., Tseng MH; Division of Nephrology, Department of Pediatrics, Chang Gung Memorial Hospital, Taoyuan, Taiwan, ROC., Wang HH; Department of Pediatrics, Taipei Veterans General Hospital, and National Yang Ming Chiao Tung University School of Medicine, Peitou District, NO. 201, Shih-Pai Rd Sec 2, Taipei, 112, Taiwan, ROC., Yen HJ; Department of Pediatrics, Taipei Veterans General Hospital, and National Yang Ming Chiao Tung University School of Medicine, Peitou District, NO. 201, Shih-Pai Rd Sec 2, Taipei, 112, Taiwan, ROC. hjyen2@vghtpe.gov.tw.; Department of Life Science, National Taiwan Normal University, Taipei, Taiwan, ROC. hjyen2@vghtpe.gov.tw.
Jazyk: angličtina
Zdroj: International journal of hematology [Int J Hematol] 2022 Nov; Vol. 116 (5), pp. 812-818. Date of Electronic Publication: 2022 Jul 05.
DOI: 10.1007/s12185-022-03409-6
Abstrakt: Complement-mediated hemolytic uremic syndrome (CM-HUS) following chemotherapy for pediatric acute lymphoid neoplasms has rarely been reported. We report the case of an 8-year-old boy with T-lymphoblastic lymphoma (T-LBL) who developed CM-HUS with complement factor H (CFH) mutations (S1191L, V1197A) during induction therapy. Safe administration of chemotherapy after CM-HUS recovery was challenging. By closely monitoring hemolytic and renal parameters during the 2-year treatment period, we observed four episodes of microangiopathic hemolytic anemia (MAHA) with hypocomplementemia and low haptoglobin but no renal dysfunction or thrombocytopenia. Here, we describe the MAHA and CM-HUS episodes in the hopes of elucidating the complex pathophysiology of disorders associated with CFH mutation.
(© 2022. Japanese Society of Hematology.)
Databáze: MEDLINE
Externí odkaz: