Cure of relapsing nephrosis by an allogeneic marrow graft for chronic myelogenous leukemia

• Published in: Pediatric nephrology (Berlin, West) Vol. 28; no. 6; pp. 975 - 978
• Main Authors: Sugimoto, Keisuke, Sakata, Naoki, Fujita, Shinsuke, Miyazawa, Tomoki, Nishi, Hitomi, Takemura, Tsukasa, Okada, Mitsuru
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer-Verlag 01.06.2013; Springer; Springer Nature B.V
• Subjects: Adult; Antigens; Bone marrow; Bone Marrow Transplantation; Brief Report; Care and treatment; Development and progression; Drug dosages; Female; Genetic aspects; Hematopoietic stem cells; Humans; Immunology; Immunosuppressive agents; Kidney diseases; Leukemia; Leukemia, Myelogenous, Chronic, BCR-ABL Positive - therapy; Lymphocytes; Medicine; Medicine & Public Health; Nephrology; Nephrotic syndrome; Nephrotic Syndrome - immunology; Nephrotic Syndrome - therapy; Patients; Pediatrics; Phosphatase; Physiological aspects; Recurrence; Remission (Medicine); Stem cell transplantation; Steroids; T cells; Transplantation; Transplantation, Homologous; Urology; Young Adult; Young adults; Japan; Bone marrow graft transplantation; T-cell; Minimal-change nephrotic syndrome; Stem cell; Proteinuria
• ISSN: 0931-041X; 1432-198X
• DOI: 10.1007/s00467-013-2433-8

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 Immunosuppressants were withdrawn within 6 months.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 − ).