Rituximab (RTX) has become a therapeutic option for inducing remission of anti-neutrophil cytoplasmic autoantibody-associated vasculitis (AAV). However, the optimum dosage of RTX to induce remission of AAV and reduce adverse events, such as infection, remains unclear. We herein report an elderly and renally impaired patient with alveolar hemorrhaging due to refractory AAV who was successfully treated with single infusion of RTX. Single infusion of RTX may be a therapeutic option in refractory AAV patients who are vulnerable to infections.
Although the renal toxicity of Deferasirox, an oral iron chelator, has been reported to be mild, there have been reports of acute interstitial nephritis or Fanconi syndrome due to this agent. Thin basement membrane disease (TBMD) is a hereditary disease characterized primarily by hematuria, with gross hematuria also observed in about 7% of cases. We herein report a case of TBMD that presented with acute kidney injury and gross hematuria during treatment with Deferasirox. The patient was a 63-year-old man who had been diagnosed with myelodysplastic syndrome 6 years ago. He had started taking Deferasirox at 125 mg due to post-transfusion iron overload 6 months ago. Deferasirox was then increased to 1000 mg three months ago. When the serum creatinine level increased, Deferasirox was reduced to 500 mg three weeks before hospitalization. Although the serum creatinine level decreased once, he developed a fever and macroscopic hematuria one week before hospitalization. The serum creatinine level increased again, and Deferasirox was stopped four days before hospitalization. He was admitted for the evaluation of acute kidney injury and gross hematuria. Treatment with temporary hemodialysis was required, and a kidney biopsy was performed on the eighth day of admission. Although there was no major abnormality in the glomeruli, the leakage of red blood cells into the Bowman's space was observed. Erythrocyte cast formation was observed in the tubular lumen, which was associated with acute tubular necrosis. The results of an electron microscopic study were compatible with TBMD. Although Deferasirox is known to be nephrotoxic, gross hematuria is relatively rare. When we encounter a case of acute kidney injury with gross hematuria during treatment with Deferasirox, TBMD should be considered as a possible cause of gross hematuria.
There is no specific treatment for recurrent Henoch-Schönlein purpura nephritis (HSPN) in a transplanted kidney. We herein report a case of a kidney transplant recipient with recurrent HSPN that was successfully treated with steroid pulse therapy and epipharyngeal abrasive therapy (EAT). A 39-year-old Japanese man developed HSPN 4 years ago and had to start hemodialysis after 2 months despite receiving steroid pulse therapy followed by oral prednisolone, plasma exchange therapy, and cyclophosphamide pulse therapy. He had undergone tonsillectomy 3 years earlier in the hopes of achieving a better outcome of a planned kidney transplantation and received a living-donor kidney transplantation from his mother 1 year earlier. Although there were no abnormalities in the renal function or urinalysis 2 months after transplantation, a routine kidney allograft biopsy revealed evidence of mesangial proliferation and cellular crescent formation. Mesangial deposition for IgA and C3 was noted, and he was diagnosed with recurrent HSPN histologically. Since the renal function and urinalysis findings deteriorated 5 months after transplantation, 2 courses of steroid pulse therapy were performed but were ineffective. EAT using 0.5% zinc chloride solution once per day was combined with the third course of steroid pulse therapy, as there were signs of chronic epipharyngitis. His renal function recovered 3 months after daily EAT and has been stable for 1.5 years since transplantation. Daily EAT continued for >3 months might be a suitable strategy for treating recurrent HSPN in cases of kidney transplantation.
Bis-dioxopiperazines have been termed catalytic inhibitors to distinguish them from topoisomerase poisons that induce DNA double-strand breaks (DSBs). However, it has been reported that ICRF-193 acts as a poison in cells containing mutated genes related to checkpoint mechanisms. We also showed previously that 20-O-ingenolEZ acts as a catalytic inhibitor to inhibit the ATPase activity of topoisomerase IIα, inducing the G2 arrest of mouse mammary tumor (MMT) cells. In this study, we observed the effects of 20-O-ingenolEZ on cells containing a mutation in the RecQ helicase gene. 20-O-IngenolEZ completely inhibited the proliferation of BLM-/-cells in BLM-/- and WRN-/-DT40 cells and wild-type DT40 cells. This inhibition induced the phosphorylation of H2AX in response to agents that introduce topoisomerase II-mediated DSBs. Following DNA damage, the induction of apoptosis in the BLM-/-cells by 20-O-ingenolEZ showed the characteristics of a topoisomerase II poison.
Renal failure due to the infiltration of chronic lymphocytic leukemia (CLL) cells into the tubulointerstitial area of the kidney is uncommon. Furthermore, granulomatous interstitial nephritis (GIN) is a rare histological diagnosis in patients undergoing a renal biopsy. We herein report a case of GIN due to the diffuse infiltration of CLL cells in a patient who developed progressive renal failure. The patient was a 55-year-old man who had been diagnosed with CLL 4 years earlier and who had been followed up without treatment. Although his serum creatinine level had remained normal for three and a half years, it started to increase in the six months prior to his presentation. A urinalysis showed mild proteinuria without any hematuria at the time of presentation. A renal biopsy revealed the diffuse infiltration of CLL cells into the tubulointerstitial area with non-caseating epithelioid cell granulomas. Despite cyclophosphamide treatment, his renal function did not improve, and he ultimately required maintenance hemodialysis. When progressive renal failure is combined with CLL, GIN due to the direct infiltration of CLL cells should be considered as a differential diagnosis.
A 66-year-old man, who had been diagnosed with deep venous thrombosis (DVT), and who was treated with a vitamin K antagonist (VKA) and who had undergone the implantation of an inferior vena cava filter, was admitted due to an exacerbation of DVT. VKA was administered again; however, the patient's DVT worsened. Further examinations revealed colon cancer, which led to a diagnosis of Trousseau's syndrome. The regression of the thrombi was confirmed after the administration of heparin and the resection of the tumors. Trousseau's syndrome should always be kept in mind when patients present with refractory venous thrombosis. The administration of heparin, and cancer control are necessary for the effective treatment of thrombosis in such cases.
Tubulointerstitial nephritis (TIN) is an inflammatory disorder in renal tubules and interstitium without the involvement of glomerular lesions.1 TIN is often induced by drugs and infections, but idiopathic or systemic disease−related forms have also been observed.2
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