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1.
A spectrum of clinicopathological features of nephropathy associated with POEMS syndrome. 总被引:11,自引:2,他引:9
Y Nakamoto H Imai T Yasuda H Wakui A B Miura 《Nephrology, dialysis, transplantation》1999,14(10):2370-2378
BACKGROUND: In POEMS syndrome, substantial involvement of the kidney can occur and is reflected by proteinuria, haematuria, renal dysfunction, and renal failure requiring dialysis therapy. The mechanism by which renal dysfunction is induced and progresses to end-stage renal disease remains obscure. A pathogenic role of cytokines and growth factors has recently been implicated. METHODS: We reviewed cases of 52 Japanese patients with confirmed renal pathology who were reported in the literature, and personally analysed renal tissues from 22 subjects including nine patients of our own. Interleukin-6 (IL-6) and vascular endothelial growth factor (VEGF) were measured in our cases. RESULTS: Despite relatively mild renal symptomatology, about half of the cases had azotaemia with creatinine levels above 1.5 mg/dl and the BUN/creatinine ratio markedly raised by volume contraction or wasting. One-tenth of patients were placed on haemodialysis because of advanced or end-stage renal disease. Bilateral and unilateral contracted kidneys were found in four and two cases respectively. Pathological analyses disclosed two major changes: glomerular alterations and endarteritis-like lesions of renal small arteries. The former included glomerular enlargement, cellular proliferation, mesangiolysis and marked swelling of endothelial-mesangial cells. This structural disorganization led to a reduction in renal function to some degree by impairing the glomerular circulation. Vasculopathy of the small artery probably resulted in progressive renal damage and ultimately to kidney contraction. Serum IL-6 was elevated in about 40% of cases. IL-6 levels were found to be high in the ascites of three patients who were examined. In different studies, an increased level of VEGF was found in the peripheral blood (75-100%; overall 92.3%), but no apparent correlation with glomerular alterations was observed. CONCLUSION: POEMS nephropathy can be one cause of end-stage renal disease with variable intrarenal pathological changes of a microangiopathic nature which have differential influences on renal function. A pathogenic role for VEGF in POEMS syndrome appears to be likely, but its causal relation to the nephropathy awaits further investigation. 相似文献
2.
Glomeruloid hemangioma. A distinctive cutaneous lesion of multicentric Castleman's disease associated with POEMS syndrome 总被引:2,自引:0,他引:2
J K Chan C D Fletcher G A Hicklin J Rosai 《The American journal of surgical pathology》1990,14(11):1036-1046
A histologically distinctive cutaneous hemangioma occurring in two patients with biopsy-proven multicentric Castleman's disease associated with POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes) syndrome are reported. The lesions were multiple, and appeared as red to purple papules over the trunk and proximal limbs. Microscopically, ecstatic dermal vascular spaces were seen filled with aggregates of capillaries, resulting in structures reminiscent of renal glomeruli. Interspersed between the blood-filled capillary loops were plump "stromal" cells possessing clear vacuoles and periodic acid-schiff-positive eosinophilic globules. These cells had the immunohistochemical profile of endothelial cells (positivity for factor VIII-related antigen, and negativity for leukocyte common antigen, macrophage marker, and muscle-specific actin), and probably represented immature elements that had accumulated immunoglobulins and other proteinaceous material from the circulation. Because vascular lesions may appear before the full-blown POEMS syndrome develops, we suggest careful evaluation and follow-up of all patients presenting with glomeruloid hemangioma or cherry-type capillary hemangioma with focal glomeruloid features for potential development of this syndrome. 相似文献
3.
O Lambotte A Dürrbach M Ammor V Paradis R Djeffal D Machover B Charpentier 《Clinical nephrology》2001,55(6):482-486
Monoclonal immunoglobulin (Ig) deposition diseases are characterized by deposition in tissues of excessive amounts of the Ig, compromising organ functions. Light chain deposition disease (LCDD) and AL amyloidosis are the commonest [Buxbaum 1992]. LCDD is usually characterized by rapidly progressive renal failure with glomerular and tubular deposits of Ig fragments mostly composed by kappa light chain. Monoclonal Ig production can also be observed associated with various symptoms, that, taken together, have been described as the Crow-Fukase syndrome or POEMS syndrome. It associates polyneuropathy, organomegaly, endocrinopathy, monoclonal Ig, and skin changes. In POEMS syndrome, renal abnormalities are rare and are reported as a moderate renal insufficiency with mild proteinuria or acute functional renal insufficiency leading in some cases to end-stage renal failure [Fukatsu et al. 1991]. Although a monoclonal Ig is produced, no Ig deposit disease had been described in POEMS syndrome except a case of AL amyloidosis [Toyokuni et al. 1992]. Here, to our knowledge, we report the first case of an LCDD associated with a POEMS syndrome. Although an autologous bone marrow graft was realized, the monoclonal component reappeared and was responsible for end-stage renal disease, cachexia and death. 相似文献
4.
Renal involvement in POEMS syndrome 总被引:3,自引:0,他引:3
S Mizuiri K Mitsuo K Sakai I Hayashi T Suzuki Y Nagai T Ohara A Hasegawa S Kawamura 《Nephron》1991,59(1):153-156
We describe a patient with POEMS syndrome whose renal biopsy specimen also showed nephropathy. Immunoelectron microscopy of the renal biopsy revealed the localization of immunoglobulin (IgA and lambda light chain) in the subendothelial space of the glomerular capillaries, a previously unrecognized finding. We conclude that the renal pathology in POEMS syndrome is unusual and distinct from microangiopathic glomerular involvement or idiopathic mesangiocapillary glomerulonephritis. 相似文献
5.
This report describes a patient presenting with recurrent acute renal failure occurring in the course of POEMS syndrome, a
multisystem disease associated with plasma cell dyscrasia. Several combined immunosuppression therapies failed to resolve
recurrent acute renal failure; autologous peripheral blood stem cell transplantation was therefore applied. A renal biopsy
was performed on each of four occasions when he developed renal dysfunction. The renal biopsy showed typical renal histology
of POEMS, membranoproliferative glomerulonephritis-like lesions and narrowing of vessel lumina of various sizes caused by
endothelial injury, which progressed to glomerulosclerosis and vessel occlusion. Recurrent acute renal failure might be caused
by ischemia due to arterial occlusion. Serum levels of vascular epithelial growth factor (VEGF), which is considered to be
a causative factor of endothelial lesions in POEMS syndrome, were not elevated throughout the course of this case. 相似文献
6.
Vascular endothelial growth factor (VEGF121) protects rats from renal infarction in thrombotic microangiopathy 总被引:4,自引:0,他引:4
Suga S Kim YG Joly A Puchacz E Kang DH Jefferson JA Abraham JA Hughes J Johnson RJ Schreiner GF 《Kidney international》2001,60(4):1297-1308
BACKGROUND: Renal thrombotic microangiopathy, typified by the hemolytic uremic syndrome, is associated with endothelial cell injury in which the presence of cortical necrosis, extensive glomerular involvement, and arterial occlusive lesions correlates with a poor clinical outcome. We hypothesized that the endothelial survival factor vascular endothelial growth factor (VEGF) may provide protection. METHOD: Severe, necrotizing, thrombotic microangiopathy was induced in rats by the renal artery perfusion of antiglomerular endothelial antibody, followed by the administration of VEGF or vehicle, and renal injury was evaluated. RESULTS: Control rats developed severe glomerular and tubulointerstitial injury with extensive renal necrosis. The administration of VEGF significantly reduced the necrosis, preserved the glomerular endothelium and arterioles, and reduced the number of apoptotic cells in glomeruli (at 4 hours) and in the tubulointerstitium (at 4 days). The prosurvival effect of VEGF for endothelium may relate in part to the ability of VEGF to protect endothelial cells from factor-induced apoptosis, as demonstrated for tumor necrosis factor-alpha (TNF-alpha), which was shown to be up-regulated through the course of this model of renal microangiopathy. Endothelial nitric oxide synthase expression was preserved in VEGF-treated rats compared with its marked decrease in the surviving glomeruli and interstitium of the antibody-treated rats that did not receive VEGF. CONCLUSIONS: VEGF protects against renal necrosis in this model of thrombotic microangiopathy. This protection may be mediated by maintaining endothelial nitric oxide production and/or preventing endothelial cell death. 相似文献
7.
Renal and thymic pathology in thymoma-associated nephropathy: report of 21 cases and review of the literature. 总被引:5,自引:0,他引:5
Alexandre Karras Vincent de Montpreville Fadi Fakhouri Jean-Pierre Grünfeld Philippe Lesavre 《Nephrology, dialysis, transplantation》2005,20(6):1075-1082
BACKGROUND: Acquired thymic disease (malignant thymoma or thymic hyperplasia) is associated with various autoimmune diseases, such as myasthenia gravis (MG), pure red-cell aplasia (PRCA), pemphigus vulgaris or systemic lupus erythematosus (SLE). Renal disease has rarely been observed in association with thymoma. METHODS: This retrospective, multicentric study collected data on patients with thymic disease and biopsy-proven renal involvement. RESULTS: Twenty-one patients were studied (age: 49+/-14 years; male/female ratio: 8/13). Thymic pathology revealed mostly high-grade malignant thymoma (B2 and AB type); two cases were associated with non-malignant thymic hyperplasia. MG was found in nine out of 21 cases, SLE in three, PRCA in three and pemphigus in two. In 47% of these cases, nephropathy occurred after curative treatment of thymoma (108+/-83 months; range: 8-180 months), mainly based on surgical thymectomy associated with radiotherapy. Clinical and laboratory findings included nephrotic syndrome (75%), renal failure (50%), frequent presence of antinuclear antibodies and hypogammaglobulinaemia. Renal pathology showed minimal change disease in 14 patients and focal segmental glomerulosclerosis (FSGS) in one. Membranous nephropathy was observed in four cases, ANCA-associated glomerulonephritis in two and thrombotic microangiopathy in one. Most patients with minimal change disease or FSGS (11/13) were steroid-sensitive. Despite good response to steroids, 38% of patients died from thymoma and 17% developed end-stage renal failure. CONCLUSIONS: Glomerulopathy can be associated with thymoma or thymic hyperplasia. The present series shows that minimal change disease is the most frequent thymoma-associated glomerular lesion and that it may occur several years after thymectomy. 相似文献
8.
The aim of this study was to identify cases of post-renal transplant thrombotic microangiopathy in a single transplant center over a period of five years. In a retrospective study, we reviewed the renal biopsy specimens of 57 renal transplant recipients with allograft dysfunction. The presence of fibrin thrombi within the glomerular capillaries or arterioles was used to define thrombotic microangiopathy. Systemic thrombotic microangiopathy was justified with the presence of thrombocytopenia and evidence of microangiopathic hemolysis. Patients with the biopsy findings compatible with thrombotic microangiopathy but without any systemic findings were categorized as having localized thrombotic microangiopathy. Four out of 57 patients had systemic thrombotic microangiopathy, while two had localized disease. The characteristics of each patient are discussed. Post-transplant thrombotic microangiopathy constitutes 10.5% of cases of early renal allograft dysfunction. A high index of suspicion is needed for diagnosing this entity as a potential cause of post-kidney transplant allograft dysfunction. Further studies with a greater number of patients may be required to highlight the risk factors for post-renal transplant thrombotic microangiopathy. 相似文献
9.
BACKGROUND: IgA nephropathy is the most common glomerulonephritis in the world. Thrombotic microangiopathy occurs in a number of clinical settings, including but not limited to thrombotic thrombocytopenic purpura/hemolytic uremic syndrome, malignant hypertension, anti-phospholipid antibody syndrome and radiation nephropathy. Renovascular complications, such as thrombotic microangiopathy, in the setting of IgA nephropathy may be overlooked and their significance as a concomitant histologic finding is unclear. METHODS: We conducted a clinicopathologic study to understand the possible relationship between IgA nephropathy and a concurrent thrombotic microangiopathy injury process. We identified 10 patients with an established diagnosis of IgA nephropathy and concurrent findings of thrombotic microangiopathy based on their renal biopsies. RESULTS: Six patients presented with malignant hypertension, while three others had severe hypertension (> or = 100 mmHg, diastolic). Five patients had nephrotic-range proteinuria. Seven patients had occasional arteriolar thrombi identified by light microscopy and prominent glomerular subendothelial space widening by electron microscopy, while three patients demonstrated only ultrastructural features of thrombotic microangiopathy. Other possible etiologic causes of thrombotic microangiopathy were not identified with the available clinical information. CONCLUSION: Our study suggests that a thrombotic microangiopathy injury, when present, is usually found in advanced stages of IgA nephropathy and can be associated with severe proteinuria. Although other possible causes of thrombotic microangiopathy, such as anti-phospholipid antibody syndrome, were excluded in only two patients, the thrombotic microangiopathy injury process may be a cause or a consequence of the severe hypertension encountered in most of the patients which, in turn, may be a consequence of the disease progression of IgA nephropathy. 相似文献
10.
A case of POEMS syndrome showing elevated serum interleukin 6 and abnormal expression of interleukin 6 in the kidney. 总被引:6,自引:0,他引:6
A 46-year-old female presented with POEMS syndrome. Hemodialysis was initiated to control severe anasarca and declining renal function. Corticosteroids were effective in treating renal insufficiency and other symptoms. Serum interleukin 6 (IL-6) was elevated before the corticosteroid therapy but returned to the normal level under the therapy. Immunostaining for the kidney tissue obtained by a renal biopsy showed a diffuse distribution of IL-6 in the glomeruli; thus, in contrast to normal, IL-6 was detected not only in mesangial cells but also in endothelial cells. IL-6 was also distributed in capillaries in the interstitium. While these results suggest a pathogenic role of IL-6 in POEMS syndrome, other factors may be necessary for the full expression of symptoms. Furthermore, it is suggested that chronically stimulated glomerular endothelial cells are capable of producing IL-6. 相似文献
11.
Tiana Jespersen Nizamic Yihung Huang Muna Alnimri Mingyu Cheng Ling-Xin Chen Kuang-Yu Jen 《Transplantation proceedings》2021,53(4):1211-1214
Coronavirus disease 2019 (COVID-19) is associated with high morbidity and mortality worldwide in both the general population and kidney transplant recipients. Acute kidney injury is a known complication of COVID-19 and appears to most commonly manifest as acute tubular injury on renal biopsy. Coagulopathy associated with COVID-19 is a known but poorly understood complication that has been reported to cause thrombotic microangiopathy on rare occasions in native kidneys of patients with COVID-19. Here, we report the first case of biopsy-proven thrombotic microangiopathy in a kidney transplant recipient with COVID-19 who developed acute pancreatitis and clinical features of microangiopathic hemolytic anemia. The patient recovered with supportive care alone. 相似文献
12.
Chan GS Lam MF Au WY Chim S Tse KC Lo SH Fung SH Lai KN Chan KW 《Nephrology (Carlton, Vic.)》2008,13(4):322-330
Aim: The ever‐growing number and increasing survival of haematopoietic stem cell transplantation (HSCT) allow better recognition of its associated renal injuries. We aimed to study the clinicopathologic features of renal biopsies after HSCT by reviewing 13 percutaneous renal biopsies in our institute (Queen Mary Hospital). Methods: A retrospective clinicopathologic study of all renal biopsies archived to the Department of Pathology, Queen Mary Hospital during the period January 1999 to December 2006 was performed. Biopsies from patients with HSCT were selected. Clinical data on presentation and follow up were retrieved from hospital records and physicians. Results: In the 8‐year period, a total of 2233 native renal biopsies were archived. Thirteen renal biopsies were selected from 12 patients with HSCT (11 allogeneic, one autologous). All but one patient were male. The age at renal biopsy ranged from 7 to 63 years (median: 32 years). The median interval of renal biopsy after HSCT was 24 months (range 1–134 months). Evidence of graft‐versus‐host disease was found in nine patients. The most common presentation was significant proteinuria (10 cases) and renal impairment (eight cases). The predominant histological changes were membranous glomerulonephritis (n = 4) and thrombotic microangiopathy (n = 4). One case of focal segmental glomerulosclerosis, IgA nephropathy, minimal change disease, acute tubular necrosis and hypertensive nephrosclerosis were also recorded. Four of our patients died at 0–11 months after renal biopsy. Of the remaining eight patients with a mean follow up of 43.6 months (range, 10–98 months), chronic renal impairment were found in three (37.5%) patients and significant proteinuria also persisted in three. One patient had cytogenetic evidence of relapse of underlying haematological malignancy after HSCT. Conclusion: Among the various renal lesions after HSCT, membranous glomerulonephritis and thrombotic microangiopathy were the most common. Mechanisms of renal injury varied from graft‐versus‐host disease‐associated immune complex deposition to non‐immune complex injury on endothelial cells, glomerular epithelial cells and tubular epithelium. Pathologists and clinicians should attend to the histological and temporal heterogeneity of renal injury when managing patients after HSCT. 相似文献
13.
Renal thrombotic microangiopathy in patients with systemic lupus erythematosus and the antiphospholipid syndrome. 总被引:7,自引:0,他引:7
M D Hughson T Nadasdy G A McCarty C Sholer K W Min F Silva 《American journal of kidney diseases》1992,20(2):150-158
Current studies indicate that a thrombotic microangiopathy (TMA) identifies patients with systemic lupus erythematosus (SLE) who are at high risk of progressing to end-stage renal disease. We have observed two patients with SLE and one patient with a primary antiphospholipid syndrome (APS) who developed acute renal insufficiency with thrombocytopenia. Renal biopsies showed a TMA characterized by thrombi or by cellular and mucoid intimal hyperplasia of small arteries and arterioles. No arterial or arteriolar immune-complex deposits were detected by immunofluorescent or electron microscopy. Biopsies from one SLE patient and the APS patient showed no immune-complex glomerular disease. Both had serum antiphospholipid antibodies (aPL). aPL were not detected in the serum of the other SLE patient who had an active lupus nephritis. Acute renal failure and thrombocytopenia resolved in each case following treatment by plasmapheresis or prednisone and heparin. None of the patients were initially treated with cytotoxic drugs. As more knowledge is gained, the accurate identification of renal vascular lesions in SLE or related diseases could influence renal prognosis and choice of therapy. The cases reported here provide further evidence that a TMA can cause acute renal failure independent of lupus nephritis. TMA should be distinguished from other forms of renal vascular disease, particularly a noninflammatory lupus microangiopathy, which is probably mediated by subendothelial immune-complex deposits. The absence of immunoglobulin deposits in vessels involved by a TMA indicates that microvascular thrombosis is promoted by mechanisms other than those usually attributed to immune-complex disease. Phospholipid reactive antibodies may be pathogenetic in some cases. 相似文献
14.
The glomerular injury of preeclampsia 总被引:3,自引:0,他引:3
Stillman IE Karumanchi SA 《Journal of the American Society of Nephrology : JASN》2007,18(8):2281-2284
Preeclampsia is a pregnancy-specific disorder that complicates approximately 5% of all pregnancies, making it perhaps the most common glomerular disease in the world. It is characterized by new-onset hypertension and proteinuria, in association with a characteristic glomerular lesion, endotheliosis. "Glomerular endotheliosis" represents a specific variant of thrombotic microangiopathy that is characterized by glomerular endothelial swelling with loss of endothelial fenestrae and occlusion of the capillary lumens. Associated thrombosis is unusual. Recent evidence suggests that this unusual glomerular lesion is mediated by a soluble vascular endothelial growth factor receptor that deprives glomerular endothelial cells of the vascular endothelial growth factor that they require, leading to cellular injury and disruption of the filtration apparatus with subsequent proteinuria. This review summarizes the histologic changes and the pathogenesis of the glomerular lesions of preeclampsia. 相似文献
15.
Viral infections in renal allograft recipients constitute an important cause of renal graft dysfunction. They have shown an increasing incidence coinciding with more potent immunosuppression regimens. Cytomegalovirus (CMV) is well-known cause of a tubulointerstitial nephritis rich in plasma cells with cytopathic changes in tubular epithelial and endothelial cells. However, involvement of glomeruli and larger arteries in the absence of tubulointerstitial disease is rare. In this study, we demonstrated the spectrum of renal disease caused by CMV among renal allograft recipients. Retrospective analysis of 2900 renal allograft biopsies performed over a 10-year period revealed 10 cases of CMV infection, with half of them (5/10) detected in 2010-2011. Although tubulointerstitial nephritis due to CMV was the most common lesion (7/10), we noted an increased incidence of CMV glomerulopathy with (1/10) or without (3/10) coexisting tubulointerstitial CMV disease. Isolated glomerular involvement was characterized by a relative lack of inflammation in any of the compartments along with the presence of cytopathic changes in the glomerular endothelial cells and podocytes. Another patient had CMV-induced thrombotic microangiopathy. The coexistent diseases were calcineurin inhibitor toxicity (n = 1), antibody-mediated rejection (n = 1), cellular rejection (n = 2), and invasive fungal infection (n = 1). In conclusion, there is a wide spectrum of CMV-induced lesions. CMV glomerulopathy is characterized by cytopathic changes in glomerular endothelial cells and podocytes with a lack of significant inflammation. In contrast, CMV-induced arteriopathy can present as thrombotic microangiopathy. Coinfection with other pathogens like invasive fungi can lead to graft failure. 相似文献
16.
Manuel Mochon Bruce A. Kaiser J. P. de Chadarevian Martin S. Polinsky H. J. Baluarte 《Pediatric nephrology (Berlin, Germany)》1992,6(6):550-552
A white girl with a history of atypical hemolyticuremic syndrome (HUS) and persistent microangiopathic anemia, and thrombocytopenia for 2 months after the initial presentation at age 7 months, received her first cadaveric renal transplant at age 3 years. During the first 2.5 days post transplant, she developed progressive thrombocytopenia and anemia followed by tonic-clonic seizures and loss of consciousness, secondary to a diffuse cerebral infarction of the left hemisphere. Renal histology showed evidence of glomerular microthrombi and microangiopathy. A large cerebral infarct, previously described in patients during their initial presentation with HUS, presented in our patient as part of the recurrence of the disease post renal transplantation. 相似文献
17.
An 82-year-old female developed hemolytic uremic syndrome (HUS) after a prodromal illness of bloody diarrhea. No specific enteric pathogen was isolated. A renal biopsy performed 5 days after the onset of azotemia revealed typical thrombotic microangiopathy. By electron microscopy, massive annular-tubular deposits admixed with fibrillar fibrin were demonstrated in glomerular capillaries. Immunofluorescent staining of the intracapillary material was positive for IgG, IgM, C3, C1q and fibrin-related antigens. No evidence of plasma cell dyscrasia, cryoglobulinemia or systemic lupus erythematosus was found, and the patient recovered renal function uneventfully in 2 months. Organized immune deposits appear to have played a role in the pathogenesis of HUS in this patient. 相似文献
18.
Katja Keller Christoph Daniel Harald Sch?cklmann Karl-Hans Endlich Dontscho Kerjaschki Richard J Johnson Christian Hugo 《Nephrology, dialysis, transplantation》2006,21(10):2724-2735
BACKGROUND: Everolimus is a potent immunosuppressant used in renal transplant therapy, but its effects on renal endothelial cell regeneration after injury are unknown. The effects of an everolimus therapy were investigated in a model of renal thrombotic microangiopathy (TMA) with specific endothelial cell (EC) injury in the rat in vivo as well as in glomerular ECs in vitro. METHODS: During the early regenerative phase (day 3) of the renal microvascular injury model in vivo, everolimus inhibited glomerular EC proliferation by up to 60% compared with vehicle-treated rats, whereas apoptosis was not different in these groups. This decreased EC proliferation was associated with an enhanced deposition of fibrin in everolimus treated animals on day 3. In cultured glomerular endothelial cells, everolimus effectively and dose dependently inhibited cellular proliferation. This anti-proliferative effect was associated with a reduced phosphorylation of the p70S6 kinase and reduction of the pro-angiogenic factor VEGF in glomeruli in vivo and in cultured podocytes in vitro. RESULTS: Despite the prolonged EC repair and in contrast to the anti-Thy1 nephritis model, everolimus therapy did not disturb the long-term repair reaction in this thrombotic microangiopathy model. CONCLUSION: Everolimus is anti-proliferative for glomerular EC in vitro and in vivo and does not seem to have detrimental long-term effects in experimental renal TMA, when only the glomerular endothelium, but not the mesangium is severely injured. 相似文献
19.
Schärer K Krmar R Querfeld U Ruder H Waldherr R Schaefer F 《Pediatric nephrology (Berlin, Germany)》1999,13(9):816-823
We studied the long-term outcome of 64 children with biopsy-proven Schönlein-Henoch purpura (SHP) nephritis over 1–23 years of follow-up. Overall renal survival 10 years after onset was 73%. Multivariate logistic regression analysis identified initial renal insufficiency (P=0.004), nephrotic syndrome (P=0.037), and the severity of histological alterations, as defined by the proportion of glomerular crescents (P=0.051), as significant independent predictors of progressive renal failure. Four patients followed for more than 19 years showed glomerular damage after transient recovery. Eight children with crescentic nephritis associated with a rapidly progressive course and/or persistent nephrotic syndrome were treated by at least seven sessions of plasma exchange (PE) within 16 weeks of onset of purpura. During treatment serum creatinine levels dropped in each patient from a mean of 2.3 to 1.1 mg/dl, followed by a rebound increase. Repeated courses of PE in 5 patients produced comparable responses. Four patients undergoing PE reached end-stage renal disease at 1.2.–3.7 years after onset, whilst 3 finally were in preterminal renal failure (creatinine 3.2–6.1 mg/dl after 7–13.5 years), and 1 patient reached a normal glomerular filtration rate. Our experience suggests that initial renal insufficiency is the best single predictor of the further clinical course in children with SHP nephritis. Early PE appears to delay the progression in some patients with severe, rapidly progressive forms of the disease. 相似文献
20.
Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a multisystem autoinflammatory disease due to an underlying plasma cell disorder that lacks a standard treatment strategy because of its rarity. We report a case of relapsed POEMS syndrome successfully treated with a second ambulatory autologous hematopoietic-cell transplantation (AHCT) after a daratumumab desensitization protocol performed during the coronavirus disease (COVID-19) pandemic in a patient with coexisting human immunodeficiency virus (HIV), hepatitis B virus (HBV) and syphilis infections. He is a 37-year old Latin-American male who had been treated with radiation, CyBorD regimen, AHCT and bortezomib therapy before being referred to our service. It was decided to administer daratumumab therapy and subsequently perform the transplant. Placement of central venous access, fluid infusion, conditioning regimen with melphalan and previously cryopreserved autograft infusion were carried out in an outpatient basis. Following second AHCT, the patient demonstrated clinical, VEGF, hematological response and remains SARS-CoV-2 infection-free and in POEMS remission with excellent quality-of-life at last follow up (6 months). We evidenced that thanks to an outpatient transplant program, the best therapeutic modalities can be offered to patients with hematologic malignancies in the context of present or future pandemics. Finally, high-complexity patients with HIV infection should have access to the same treatment strategies as non–infected patients. A second AHCT in the outpatient setting is feasible, safe and highly effective to treat patients with relapsed POEMS syndrome. 相似文献