Chronic Graft-Versus-Host Disease-Related Membranous Nephropathy: Report of 3 Cases.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 5274-5274
Author(s):  
Kanger Zhu ◽  
Wei-jian Zhu ◽  
Tao Zhang

Abstract Objective: To investigate the clinical manifestation, pathological features, management and prognosis of chronic graft-versus-host disease (cGVHD)-related membranous nephropathy following allogeneic hematopoietic stem cell transplantation (allo-HSCT). Methods: Clinical and pathological data from 3 patients with nephrotic syndrome following allo-HSCT were retrospectively analyzed. Results: cGVHD-related membranous nephropathy occurred in 3 out of 150 patients (2%) undergoing allo-HSCT in our BMT unit. 3 patients, with two male and one female, median age of 15 years, and median interval of 11 months between diagnosis and post-transplantation, typically presented with full nephrotic syndrome, including heavy proteinuria (median 8.1 g/24h), edema, hypoalbuminemia (median 29.6 g/L), and hypercholesterolemia (median 11.7 mmolL). A major finding of renal biopsy that was consistent with membranous nephropathy in early stage was diffusely thickened glomerular basement membranes (GBMs) with deposits of IgG along it. All patients showed evidence of cGVHD. Two patients responded well to steroid, with gradual improvement of clinical symptoms and disappearance of proteinuria, and are still alive without evidence of relapse. But the other one died of disseminated hemorrhagic varicella one month following immunosuppressive therapy. Conclusion: These findings suggest that the kidney may be the target organ of alloimmunity, and membranous nephropathy may be one of clinical manifestations of cGVHD, which is response to steroid, resulting in disappearance of proteinuria. It is necessary for transplant physicians to keep in mind the possibility of cGVHD-related membranous nephropathy when a case of nephritic syndrome is encountered.

Author(s):  
N. V. Sidorova ◽  
K. I. Kirgizov ◽  
A. S. Slinin ◽  
E. A. Pristanskova ◽  
V. V. Konstantinova ◽  
...  

The choice of the optimal donor in the absence of an HLA-compatible relative, as well as the analysis of the risks of hematopoietic stem cell transplantation (HSCT), is extremely important, especially in patients with non-cancerous diseases. The article analyzes 99 allogeneic HSCTs from unrelated donors in the bone marrow transplantation department of the Russian Children’s Clinical Hospital. The analysis included patients with acquired and congenital forms of non-malignant diseases. The choice of an optimal unrelated donor in the absence of a compatible relative donor, as well as an analysis of the risks of treatment, requires studying the factors that influence the outcome of treatment in this group of patients. It was shown that the level of 2-year overall survival (OS) was 74 % (standard deviation ± 4.7 %). At the same time, clinical manifestations of the acute graft versus host disease of grade I–IV were recorded in 67 % (n = 66) of patients, and severe forms of grade III–IV in 13 % (n = 13) of children. Chronic graft versus host disease (chGVHD) was observed in 29 % (n = 29) patients. When studying the factors associated with the donor, it was found that the differences in the HLA system have a negative effect on the incidence of chGVHD; in a (9/10) HLA-incompatible donor, it was 29 % higher (p = 0.019). Increasing the age of the donor for every 10 years consistently reduces the OS by 9–11 % (p = 0.117), however, the OS with a donor over 46 years old was 100 % (n = 7). No effect on the agents with respect to the following factors with respect to the recipient was found: by sex, blood group, serostatus for cytomegalovirus (CMV). It was noted that the combination of CMV-positive serostatus of the donor and the negative status of the recipient increases the risk of transplant rejection up to 50 % in comparison with other variants of CMV serostatus (p = 0.001). In general, the possibility of performing HSCT from an unrelated donor for patients with non-malignant diseases and possible ways of selecting the optimal donor was noted. Conflict of interest. The authors declare no conflict of interest.Funding. The study was performed without external funding.


2019 ◽  
Vol 4 (1-2) ◽  
pp. 37-40
Author(s):  
Parikshit Padhi ◽  
Timothy Muchayi ◽  
Evan Teske ◽  
Michael Kuperman ◽  
Fidel Barrantes

Chronic graft-versus-host disease is a potentially life-threatening immunological complication which can occur any time after hematopoietic stem cell transplant. Many organ systems can be affected; however, kidneys are normally not and development of nephrotic syndrome is particularly rare. In our report, a 64 year old who was diagnosed with acute myelogenous leukemia developed acute kidney injury and nephrotic syndrome secondary to minimal change disease. The patient responded well to treatment with steroids and rituximab therapy. Our goal is to communicate that nephrotic syndrome should be suspected in patients with significant hypoalbuminemia after graft-versus-host disease and withdrawing immunosuppression.


Blood ◽  
2008 ◽  
Vol 111 (8) ◽  
pp. 4403-4412 ◽  
Author(s):  
Tsukasa Hori ◽  
Yasuyoshi Naishiro ◽  
Hitoshi Sohma ◽  
Nobuhiro Suzuki ◽  
Naoki Hatakeyama ◽  
...  

Abstract Although graft-versus-host disease (GVHD) is a life-threatening complication of hematopoietic stem-cell transplantation (HSCT), its current diagnosis depends mainly on clinical manifestations and invasive biopsies. Specific biomarkers for GVHD would facilitate early and accurate recognition of this grave condition. Using proteomics, we screened for plasma proteins specific for GVHD in a mouse model. One peak with 8972-Da molecular mass (m/z) retained a discriminatory value in 2 diagnostic groups (GVHD and normal controls) with increased expression in the disease and decreased expression during cyclosporin A treatment, and was barely detectable in syngeneic transplantation. Purification and mass analysis identified this molecule as CCL8, a member of a large chemokine family. In human samples, the serum concentration of CCL8 correlated closely with GVHD severity. All non-GVHD samples contained less than 48 pg/mL (mean ± SE: 22.5 ± 5.5 pg/mL, range: 12.6-48.0 pg/mL, n = 7). In sharp contrast, CCL8 was highly up-regulated in GVHD sera ranging from 52.0 to 333.6 pg/mL (mean ± SE: 165.0 ± 39.8 pg/mL, n = 7). Strikingly, 2 patients with severe fatal GVHD had extremely high levels of CCL8 (333.6 and 290.4 pg/mL. CCL8 is a promising specific serum marker for the early and accurate diagnosis of GVHD.


Author(s):  
E. B. Machneva ◽  
V. Yu. Panarina ◽  
T. Z. Aliev ◽  
D. V. Shevtsov ◽  
A. M. Suleymanova ◽  
...  

Allogeneic hematopoietic stem cell transplantation (HSCT) is a potentially curative treatment for different spectrum of diseases. This type of treatment is constantly improving, but HSCT remains a risky procedure with various possible complications, the main is – chronic “graft versus host” disease (cGVHD). сGVHD is immune disregulation, and characterized by a variety of clinical manifestations that reflect the multiple underlying pathophysiology mechanisms. The study of cGVHD has now made great progress, but there’s still a lot of questions. General characteristics, risk-factors of development, clinical manifestations, pathogenesis of cGVHD will be discussed in this article. Clinical case presented in this article explains usage of basic and novel agents for cGVHD treatment, prevention criterions for treatment of cGVHD in children.


2021 ◽  
Vol 12 ◽  
Author(s):  
Mingming Li ◽  
Yue Zhang ◽  
Yujia Guan ◽  
Zunwei Zhang ◽  
Hanbing Dong ◽  
...  

As an adverse immune phenomenon, graft-versus-host disease often occurs after allogeneic hematopoietic stem cell transplantation. The incidence of acute and chronic graft-versus-host disease is about 40–60% and the mortality rate can reach 15%, which is a potentially fatal disease. There are rare GvHD cases involving the central nervous system. We reported a rare case of diffuse white matter changes after haploid bone marrow transplantation, summarizing its clinical manifestations and diagnosis and treatment in conjunction with the literature.


Blood ◽  
2015 ◽  
Vol 125 (4) ◽  
pp. 606-615 ◽  
Author(s):  
Mary E. D. Flowers ◽  
Paul J. Martin

Abstract Chronic graft-versus-host disease (GVHD) remains a common and potentially life-threatening complication of allogeneic hematopoietic stem cell transplantation (HCT). The 2-year cumulative incidence of chronic GVHD requiring systemic treatment is ∼30% to 40% by National Institutes of Health criteria. The risk of chronic GVHD is higher and the duration of treatment is longer after HCT with mobilized blood cells than with marrow cells. Clinical manifestations can impair activities of daily living and often linger for years. Hematology and oncology specialists who refer patients to centers for HCT are often subsequently involved in the management of chronic GVHD when patients return to their care after HCT. Treatment of these patients can be optimized under shared care arrangements that enable referring physicians to manage long-term administration of immunosuppressive medications and supportive care with guidance from transplant center experts. Keys to successful collaborative management include early recognition in making the diagnosis of chronic GVHD, comprehensive evaluation at the onset and periodically during the course of the disease, prompt institution of systemic and topical treatment, appropriate monitoring of the response, calibration of treatment intensity over time in order to avoid overtreatment or undertreatment, and the use of supportive care to prevent complications and disability.


Cancers ◽  
2021 ◽  
Vol 13 (4) ◽  
pp. 613
Author(s):  
Nidhi Sharma ◽  
Qiuhong Zhao ◽  
Bin Ni ◽  
Patrick Elder ◽  
Marcin Puto ◽  
...  

Acute graft versus host disease (aGVHD) remains a leading cause of morbidity and mortality in allogeneic hematopoietic stem cell transplant (allo-HSCT). Tacrolimus (TAC), a calcineurin inhibitor that prevents T-cell activation, is commonly used as a GVHD prophylaxis. However, there is variability in the serum concentrations of TAC, and little is known on the impact of early TAC levels on aGVHD. We retrospectively analyzed 673 consecutive patients undergoing allo-HSCT at the Ohio State University between 2002 and 2016. Week 1 TAC was associated with a lower risk of aGVHD II–IV at TAC level ≥10.15 ng/mL (p = 0.03) compared to the lowest quartile. The cumulative incidence of relapse at 1, 3 and 5 years was 33%, 38% and 41%, respectively. TAC levels at week 2, ≥11.55 ng/mL, were associated with an increased risk of relapse (p = 0.01) compared to the lowest quartile. Subset analysis with acute myeloid leukemia and myelodysplastic syndrome patients showed significantly reduced aGVHD with TAC level ≥10.15 ng/mL at week 1 and a higher risk of relapse associated with week 2 TAC level ≥11.55 ng/mL (p = 0.02). Hence, achieving ≥10 ng/mL during the first week of HCT may mitigate the risk of aGVHD. However, levels (>11 ng/mL) beyond the first week may be associated with suppressed graft versus tumor effect and higher relapse.


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