He was also maintained on keppra for seizure prophylaxis and nadolol for history of a prolonged QTc thought secondary to cardiac toxicity from preparative regimens

He was also maintained on keppra for seizure prophylaxis and nadolol for history of a prolonged QTc thought secondary to cardiac toxicity from preparative regimens. and nephrotic syndrome (NS) [4]. We present a case of GVHD and proteinuria in a pediatric patient following stem cell transplant which highlights the potential role of T-cells in the pathogenesis of this condition. Case report Clinical history and initial laboratory results A 7-year-old boy with a past history of T-cell ALL presented ~?3 months after 5/6 matched allogeneic hematopoietic stem cell MRS 1754 transplant for evaluation of increasing serum creatinine levels. His initial presentation had included a high white count of 133,000/L with 71% blasts and mild anemia. Peripheral blood flow MRS 1754 cytometry showed T-cell ALL, which was confirmed by bone marrow biopsy. There was no initial testicular or CNS involvement. Induction chemotherapy included treatment with cytarabine, vincristine, daunorubicin, prednisone, and methotrexate. Risk assessment, performed after completion of induction therapy placed this patient in the high-risk group, as he had minimal residual disease (MRD). He CLG4B proceeded directly to consolidation therapy without waiting for count recovery and he received cranial radiotherapy during the delayed intensification phase of treatment. Maintenance chemotherapy was begun ~?7 months after initial presentation and was complicated by several episodes of fever and neutropenia, acute appendicitis requiring laparoscopic appendectomy and a bone marrow relapse of T-cell ALL. As per protocol, the patient began intensive reinduction chemotherapy for children with a bone marrow relapse after standard induction therapy. He received the following agents: vincristine, doxorubicin, prednisone, ifosfamide, etoposide, clofarabine, cytoxan, and nelarabine. After ~?1 month of therapy, preparations commenced for stem cell transplant. Total body irradiation was a component of his pre-transplant conditioning as was high-dose cytarabine and clofarabine. 14 months after initial presentation the patient received a 5/6 matched stem cell transplant. His post-transplant course was complicated by a central nervous system event that occurred ~?2 weeks after transplant which resembled HUS/TTP and resulted in deafness and paraplegia.He received GVHD prophylaxis with mycophenolate mofetil (MMF) and tacrolimus. Immediately following stem cell transplant the serum creatinine was normal at 0.7 mg/dL and rose gradually over a period of 6?C?8 weeks peaking at 2.5 mg/dL. Concurrently, the patient began to gain weight and develop significant total body edema. His blood pressure remained normal. He did not complain of any diarrhea or skin lesions though skin GVHD was MRS 1754 confirmed by biopsy and endoscopy showed grade 1 GVHD of the stomach ~?60 days after stem cell transplant; these resolved after treatment with several weeks of solumedrol. At the time of the initial nephrology evaluation, post-transplant medications included only physiologic hydrocortisone and mycafungin, a nephrotoxin. He was also maintained on keppra for seizure prophylaxis and nadolol for history of a prolonged QTc thought secondary to cardiac toxicity from preparative regimens. A renal ultrasound showed normal size kidneys with slightly increased echogenicity and mild pelvicalyceal dilatation. Liver function tests and electrolytes were normal apart from a low phosphorous of 2.7 mg/dL. A phosphate-wasting tubulopathy was confirmed by urine and serum studies, which showed a TRP of 53%. Complement levels were normal with C3 of 203 mg/dL and C4 of 42 mg/dL and an ANA screen was negative at ?1?:?40. Serial protein/creatinine ratios were 3.8?C?10.0 suggesting nephrotic range proteinuria, though the serum albumin was consistently 3.5 mg/dL. A 24-h urine collection showed 4.7 mg/kg creatinine, presumably so low due to muscle wasting and paralysis, and 14.6 mg/kg protein. There was no hematuria. As a result of this patients persistent and moderately impaired renal function as well as his proteinuria, a renal biopsy was pursued since there were some entities in the differential diagnosis which would potentially change management. Differential diagnosis at the time included progressing CKD, GVHD, bone marrow transplant-associated HUS/TTP, radiation nephritis, BK nephropathy, CMV nephritis, calcineurin inhibitor toxicity, and tubulointerstitial nephritis. Results Kidney biopsy The kidney biopsy showed two pieces of kidney cortex containing 25 glomeruli. The glomeruli had been hypercellular with mildly lobulated tufts and lymphocytes had been MRS 1754 present inside the glomerular capillary lumen. Many glomeruli demonstrated corrugated capillary cellar.

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