Literature DB >> 35790695

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

Fu-Shiuan Whitney Lee1, Chih-Ying Lee1,2, Giun-Yi Hung1,2, Min-Hua Tseng3, Hsin-Hui Wang1, Hsiu-Ju Yen4,5.   

Abstract

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.

Entities:  

Keywords:  6-mercaptopurine; Atypical hemolytic uremic syndrome; Complement-mediated hemolytic uremic syndrome; Lymphoblastic lymphoma; Microangiopathic hemolytic anemia

Year:  2022        PMID: 35790695     DOI: 10.1007/s12185-022-03409-6

Source DB:  PubMed          Journal:  Int J Hematol        ISSN: 0925-5710            Impact factor:   2.319


  1 in total

Review 1.  Atypical hemolytic uremic syndrome (aHUS): essential aspects of an accurate diagnosis.

Authors:  Jeffrey Laurence; Hermann Haller; Pier Mannuccio Mannucci; Masaomi Nangaku; Manuel Praga; Santiago Rodriguez de Cordoba
Journal:  Clin Adv Hematol Oncol       Date:  2016-11
  1 in total

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