BACKGROUND: Minimal-change nephrotic syndrome has recently been attributed to an immature, dysfunctional T-cell population. CASE-DIAGNOSIS/TREATMENT: A woman, now 23 years old, developed nephrotic syndrome when she was 6 years old. Despite treatment with steroids and immunosuppressants such as cyclosporine, mizoribine, mycophenolate mofetil, and tacrolimus, the patient relapsed 14 times. At the age of 19 years, she developed chronic myelogenous leukemia, against which imatinib achieved cytogenetic remission. The patient received an allogeneic bone marrow graft transplantation from an unrelated marrow bank donor, with an uncomplicated recovery and molecular genetic remission. Immunosuppressants were withdrawn within 6 months. The patient is now without drug treatment. Complete remission of nephrotic syndrome has also been maintained for over 4 years without any drug administration. CONCLUSIONS: The patient's course supports suggestions that immunological dysfunction in nephrosis is associated with abnormality of immature, relatively unclassified T cells (CD34(+)) representing hematopoietic stem cells, as opposed to mature T cells (CD34(-)).
BACKGROUND: Minimal-change nephrotic syndrome has recently been attributed to an immature, dysfunctional T-cell population. CASE-DIAGNOSIS/TREATMENT: A woman, now 23 years old, developed nephrotic syndrome when she was 6 years old. Despite treatment with steroids and immunosuppressants such as cyclosporine, mizoribine, mycophenolate mofetil, and tacrolimus, the patient relapsed 14 times. At the age of 19 years, she developed chronic myelogenous leukemia, against which imatinib achieved cytogenetic remission. The patient received an allogeneic bone marrow graft transplantation from an unrelated marrow bank donor, with an uncomplicated recovery and molecular genetic remission. Immunosuppressants were withdrawn within 6 months. The patient is now without drug treatment. Complete remission of nephrotic syndrome has also been maintained for over 4 years without any drug administration. CONCLUSIONS: The patient's course supports suggestions that immunological dysfunction in nephrosis is associated with abnormality of immature, relatively unclassified T cells (CD34(+)) representing hematopoietic stem cells, as opposed to mature T cells (CD34(-)).
Authors: K Tenbrock; A Schubert; L Stapenhorst; M J Kemper; J Gellermann; K Timmermann; D E Müller-Wiefel; U Querfeld; B Hoppe; D Michalk Journal: Clin Sci (Lond) Date: 2002-05 Impact factor: 6.124
Authors: V Audard; F Larousserie; P Grimbert; M Abtahi; J-J Sotto; A Delmer; F Boue; D Nochy; N Brousse; R Delarue; P Remy; P Ronco; D Sahali; P Lang; O Hermine Journal: Kidney Int Date: 2006-05-03 Impact factor: 10.612
Authors: Hee Gyung Kang; Heewon Seo; Jae Hyun Lim; Jong Il Kim; Kyoung Hee Han; Hye Won Park; Ja Wook Koo; Kee Hyuck Kim; Ju Han Kim; Hae Il Cheong; Il-Soo Ha Journal: J Int Med Res Date: 2017-01-01 Impact factor: 1.671