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Objective: To investigate the clinical manifestations, pathology, diagnosis, treatment, and pathogenesis of late-onset nephrotic syndrome (NS) after allogenic hematopoietic stem cell transplantation (allo-HSCT).
Methods: NS post-HSCT was investigated in 167 patients with hematopoietic malignancies who survived more than 3 months after allo-HSCT. The clinical manifestations, pathology, diagnosis, and treatment were investigated in a retrospective study. The association of the onset of NS post-HSCT with sex, age, transplant type, conditioning regiments, human leucocyte antigen (HLA) matching, donor-recipient relationship, acute graft versus host disease (aGVHD), chronic graft versus host disease (cGVHD), and cytomegalovirus infection were analyzed.
Results: Five patients (2.99%) were diagnosed as with NS post-HSCT, 4 with membranous glomerulonephritis (MGN) and 1 with minimal change disease (MCD). Immunohistochemistry of glomerular lesions revealed that the immunoglobulin (Ig) of immune complex deposition included IgG in 3 patients, IgM in 1 patient, and co-existence of IgG/IgM in 1 patient. Serum ANA was positive in 2 patients and serum IgG and IgM were in high levels in 2 patients. Glucocorticoid combined with cyclophosphamide (CTX) was fundamentally effective treatment for NS post-HSCT. 1 patient got complete response, 3 got partial response, and 1 was stable after the treatment. Binary logistic regression showed that the he onset of NS post-HSCT was not significantly associated with sex, age, transplant type, conditioning regimen, HLA matching, donor-recipient relation, aGVHD, cGVHD and CMV infection.
Conclusion: The predominant pathological type of NS post-HSCT is MGN, followed by MCD. The pathogenesis of NS post-HSCT may be related to the abnormality of humoral immunity. Glucocorticoid combined with CTX is an effective treatment.
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