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Prepublished online as a Blood First Edition Paper on January 23, 2003; DOI 10.1182/blood-2002-07-2290.

Submitted July 29, 2002
Accepted January 9, 2003
Nephrotic syndrome with crescent formation and massive IgA deposition following allogeneic bone marrow transplantation for natural killer cell leukemia/lymphoma
Shinya Kimura*, Akiyo Horie, Yoshiyuki Hiki, Chie Yamamoto, Satoru Suzuki, Junya Kuroda, Masayo Deguchi, Gen-ichi Kato, Takahiro Karasuno, Akira Hiraoka, Toshikazu Yoshikawa, and Taira Maekawa
Department of Transfusion Medicine, Kyoto University Hospital, Kyoto, Japan
Department of Medicine, Daiko Medical Center, Nagoya, Japan
Department of Clinical and Laboratory Medicine, Fukui Medical University, Fukui, Japan
Department of Internal Medicine and Pathology, Kyoto Second Red Cross Hospital, Kyoto, Japan
Fifth Department of Internal Medicine, Osaka Medical Center for Cancer and Cardiovascular Diseases, Osaka, Japan
First Department of Internal Medicine, Kyoto Prefectural University of Medicine, Kyoto, Japan
* Corresponding author; email: shkimu{at}kuhp.kyoto-u.ac.jp.
We describe herein a case of nephrotic syndrome (NS) following allogeneic bone marrow transplantation (allo-BMT) for natural killer cell leukemia/lymphoma. Histological studies defined the diagnosis as crescentic glomerulonephritis with massive IgA deposition, which have never been reported in NS cases following allo-BMT. A majority of the massive infiltrated cells in the interstice were CD3(+)CD4(-)CD8(+) T cells derived from the donor. Mesangial deposition of Haemophilus parainfluenza outer membrane (OMHP) antigen and decreased glycosylation of the IgA1 hinge in the recipient's samples, with which we recently reported on the pathogenesis of IgA nephropathy and its association, were observed. Further, IgA antibody against OMHP titer of the donor serum was as high as other IgA nephropathy cases. These findings suggested that NS, crescentic glomerulonephritis, of this case was caused as one of the forms of chronic GVHD and IgA deposition was associated with Haemophilus parainfluenza and decreased glycosylation of the IgA1 hinge.

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