The Oncologic Safety of Sentinel Lymph Node Biopsy in Patients with Node-Positive Breast Cancer with Complete Response to Neoadjuvant Chemotherapy: A Single-Center Experience.

Objective: To evaluate the efficiency and safety of sentinel lymph node biopsy (SLNB) in patients with breast cancer with complete response to neoadjuvant chemotherapy (NAC). Methods: Ninety-two consecutive (T1-4 and N1-2) patients with breast cancer who had pathologic and/or clinical and radiologic axillary lymph node involvement were included. All patients received NAC. Patients with a clinical and radiologic complete response in the axilla after NAC underwent SLNB. Pathologic complete response (ypCR) was defined as the absence of residual invasive and in situ cancer, and near-complete response (ypNCR) represented in situ and/or ≤ 1 mm residual tumor in the breast and/or presence of malignant cell clusters (≤0.2 mm) and/or micrometastases (≤2.0 mm) in the axillary lymph nodes (ALN) (ypTis/T1mi, ypN0i+/pN1mi). Results: The mean age of the 92 patients was 49.6 ± 10.3 years and the mean follow-up was 34.0 ± 17.8 months. With respect to breast tumors, 23 (25.0%) patients had complete and 14 (15.2%) had a near-complete response to NAC. Complete response in ALN was obtained in 39 (42.4%) patients and near-complete in six (6.5%) patients. The overall survival of the 33 patients who achieved ypCR and ypNCR was 100% and the remaining 59 patients with partial or no response to NAC was 83.1% at a mean follow-up of 34 months (p=0.063). Conclusions: In this study, no event developed in cases with ypCR and ypNCR in the breast and axilla. The persistence of the same results in long-termfollow-ups may enable the use of ypNCR as a positive prognostic marker in addition to ypCR.

The effectiveness and safety of neoadjuvant pertuzumab and trastuzumab in women with locally advanced, inflammatory, or early-stage human epidermal growth factor receptor 2-positive breast cancer: Turkish Oncology Group study.

In our study, we aimed to evaluate the pathological response rates and side effect profile of adding pertuzumab to the treatment of HER2+ locally advanced, inflammatory, or early-stage breast cancer. This study was conducted by the Turkish Oncology Group (TOG) with data collected from 32 centers. Our study was multicentric, and a total of 364 patients were included. The median age of the patients was 49 years (18-85 years). Two hundred fifteen (60%) of the cases were hormone receptor/HER2+ positive(ER+ or PR+, or both), and 149 (40%) of them were HER2-rich (ER and PR negative). The number of complete responses was 124 (54%) in the docetaxel+trastuzumab+pertuzumab arm and 102 (45%) in the paclitaxel+trastuzumab+pertuzumab arm, and there was no difference between the groups in terms of complete response. In 226 (62%) patients with complete response, a significant correlation was found with DCIS, tumor focality, removed lymph node, and ER status P < 0.05. Anemia, nausea, vomiting, myalgia, alopecia, and mucosal inflammation were significantly higher in the docetaxel arm, P < 0.05. In our study, no statistical difference was found between the before-after echocardiography values. DCIS positivity in biopsy before neoadjuvant chemotherapy, tumor focality; the number of lymph nodes removed and ER status were found to be associated with pCR. In conclusion, we think that studies evaluating pCR-related clinicopathological variables and radiological imaging features will play a critical role in the development of nonsurgical treatment approaches.

The Importance of the Pathological Perspective in the Management of the Invasive Lobular Carcinoma.

Background: Invasive lobular carcinomas (ILC) account for 10-15% of all breast cancers and are the second most common histological form of breast cancer. They usually show a discohesive pattern of single cell infiltration, tend to be multifocal, and the tumor may not be accompanied by a stromal reaction. Because of these histological features, which are not common in other breast tumors, radiological detection of the tumor may be difficult, and its pathological evaluation in terms of size and spread is often problematic. The SSO-ASTRO guideline defines the negative surgical margin in breast-conserving surgeries as the absence of tumor detection on the ink. However, surgical margin assessment in invasive lobular carcinomas has not been much discussed from the pathological perspective. Methods: The study included 79 cases diagnosed with invasive lobular carcinoma by a Tru-cut biopsy where operated in our center between 2014 and 2021. Clinicopathological characteristics of the cases, results of an intraoperative frozen evaluation in cases that underwent conservative surgery, the necessity of re-excision and complementary mastectomy, and consistency in radiological and pathological response evaluation in cases receiving neoadjuvant treatment were questioned. Results: The tumor was multifocal in 37 (46.8%) cases and single tumor focus in 42 (53.2%) cases. When the entire patient population was evaluated, regardless of focality, mastectomy was performed in 27 patients (34.2%) and breast-conserving surgery (BCS) was performed in 52 patients (65.8%). Of the 52 patients who underwent BCS, 26 (50%) required an additional surgical procedure (cavity revision or completion mastectomy). There is a statistical relationship between tumor size and additional surgical intervention (p < 0.05). BCS was performed in 7 of 12 patients who were operated on after neoadjuvant treatment, but all of them were reoperated with the same or a second session and turned to mastectomy. Neoadjuvant treatment and the need for reoperation were statistically significant (p < 0.05). Additional surgical procedures were performed in 20 (44.4%) of 45 patients in BCS cases who did not receive neoadjuvant therapy. Conclusions: Diagnostic difficulties in the intraoperative frozen evaluation of invasive lobular carcinoma are due to the different histopathological patterns of the ILC. In our study, it was determined that large tumor size and neoadjuvant therapy increased the need for additional surgical procedures. It is thought that the pathological perspective is the determining factor in order to minimize the negative effects such as unsuccessful cosmesis, an additional surgical burden on the patient, and cost increase that may occur with additional surgical procedures; for this reason, new approaches should be discussed in the treatment planning of invasive lobular carcinoma cases.

To what extent estimated or measured GFR could predict subclinical graft fibrosis: a comparative prospective study with protocol biopsies.

Monitoring of allograft function entails methods more accurate than serum creatinine and creatinine-based GFR equations (eGFR). This prospective trial aimed at investigating the diagnostic accuracy of creatinine- and cystatin C-based eGFR with measured GFR (mGFR) and compared them with graft fibrosis detected by protocol biopsies (PBx). Forty-four kidney transplant recipients were enrolled. PBx were obtained postengraftment and at 6th and 12th months. GFR was measured by Tc-99m DTPA at 3th, 6th, and 12th months after transplantation. Significant correlation existed between eGFR and mGFR at 3, 6, and 12 months (P < 0.0001). Cystatin C-based Hoek and Larsson equations had the lowest bias and highest accuracy. The sum of interstitial fibrosis and tubular atrophy score increased from implantation to 6th and 12th months (0.52 ± 0.79, 0.84 ± 0.88, 1.50 ± 1.35). This was accompanied by reduction of mGFR from 54.1 ± 15.2 to 49.9 ± 15.2 and 46.8 ± 16.5 ml/min/1.73 m(2) , while serum creatinine, cystatin C, and eGFR remained stable. Neither creatinine- nor cystatin C-based GFR equations are reliable for detecting insidious graft fibrosis. In the first year after transplantation, mGFR, with its best proximity to histopathology, can be used to monitor allograft function and insidious graft fibrosis.

Comprehensive Immunohistochemical Analysis of Epithelial-Mesenchymal Transition Biomarkers in the Invasive Micropapillary Cancer of the Breast.

Background: Invasive micropapillary carcinoma (IMPC) of the breast is commonly associated with a poor prognosis due to its high incidence of lymphovascular invasion and lymph node metastasis (LNM). Our study is aimed at investigating the prognostic significance of the expressions of E-cadherin (E-cad), N-cadherin (N-cad), CD44s, and ß-catenin (ß-cat). In addition, it is aimed at deciphering the consistency of these markers between the IMPC, the invasive breast carcinoma, no-special type (IBC-NST), and LNM components in the same IMPC cases. Methods: Sixty-two IMPC cases with LNM from 1996 to 2018 were analyzed. Immunohistochemical staining was performed separately on the three regions for each patient. Statistical analyses included Kaplan-Meier, Cox regression, and McNemar's statistical tests. Results: Loss of CD44 expression in IMPC, IBC-NST, and LNM areas was associated with poor prognosis in overall survival (OS) (p = 0.010, p < 0.0005, p = 0.025). Loss of CD44 expression in the IBC-NST, gain of N-cad expression in the IMPC, and loss of ß-cat expression in the LNM areas were indicators of poor prognosis in disease-free survival (DFS) (p = 0.005, p = 0.041, p = 0.009). Conclusion: Our evaluation of this rare subtype, focusing on the expression of key epithelial-mesenchymal transition (EMT) molecules, revealed that it shares characteristics with the IBC-NST component within mixed tumors. Notably, contrary to expectations, a reduction in CD44 expression was found to adversely affect both OS and DFS. By conducting staining procedures simultaneously across three regions within the same patient, a novel approach has provided valuable insights into the mechanisms of EMT.

Splanchnic vein thrombosis following renal transplantation: a case report.

BACKGROUND: Recurrent episodes of venous thrombosis have been closely correlated with JAK2 V617F mutation. Upto date, JAK2 gene mutation has not been defined as a prothrombic risk factor in renal transplant recipients. Herein; we present a case of portosplenic vein thrombosis in a primary renal transplant recipient with JAK2 V617F mutation who had no history of prior venous thromboembolism or thrombophilia. CASE PRESENTATION: A 59 year old female caucasian patient with primary kidney transplant admitted with vague abdominal pain at left upper quadrant. Abdominal doppler ultrasound and magnetic resonance imaging angiography demonstrated splanchnic vein thrombosis (SVT). The final diagnosis was SVT due to MPD (essential thrombocytosis, ET) with JAK2 V617F mutation. After 3 months of treatment with warfarin (≥5 mg/day, to keep target INR values of 1.9-2.5), control MRI angiography and doppler USG demonstrated partial (>%50) resolution of thrombosis with recanalization of hepatopedal venous flow. The patient is still on the same treatment protocol without any complication. CONCLUSION: JAK2 V617F mutation analysis should be a routine procedure in the diagnosis and treatment of kidney transplant patients with thrombosis in uncommon sites.

Glomerular c4d staining can be an indicator of disease activity in lupus nephritis.

BACKGROUND: Abnormalities in complement activation and clearance of immune complexes by erythrocytes are the central pathogenic mechanisms in systemic lupus erythematosus (SLE). Serum C4d level, which is a degradation product of complement factor C4, was found to be a sensitive indicator of SLE activity. Our aim was to determine whether glomerular C4d staining could be a useful marker of disease activity in patients with lupus nephritis. METHODS: This retrospective study included all consecutive patients who underwent a renal biopsy at our center between January 2005 and December 2009. A total of 29 patients with IgA nephritis were enrolled, and renal biopsy specimens of 24 patients have been evaluated. We evaluated baseline age, sex, hypertension, serum creatinine level, glomerular filtration rate (GFR), urine protein, and glomerular C4d staining. The primary endpoint of this study was the onset of end-stage renal disease (ESRD) in the course of study. RESULTS: Fourteen (58%) patients were C4d+ and 10 (42%) patients C4d-. Urinary protein excretion was more elevated in C4d+ group (p = 0.0001). The renal biopsy showed that activity index score >12 was a higher proportion in C4d+ patients. The patients were followed up for 3.5 years. Four patients in the C4d+ group evolved to ESRD in the follow-up, but none of the patients in the C4d- group (p = 0.064). DISCUSSION: We found a relationship between glomerular C4d staining and activity of lupus nephritis. C4d staining may be a useful marker to predict the prognosis of lupus nephritis.

Prognostic value of NGAL staining in patients with IgA nephropathy.

BACKGROUND: Renal tubulointerstitial injury plays an important role in disease progression of IgAN. Neutrophil gelatinase-associated lipocalin (NGAL) is a stress protein released by tubular cells. NGAL is a promising biomarker of acute kidney injury. There is a growing literature suggesting that NGAL is also a marker of chronic kidney disease and severity. Our aim was to evaluate the prognostic value of NGAL staining in patients with IgAN. METHODS: This retrospective study included all consecutive patients who underwent a renal biopsy at our center between January 2005 and December 2009. Forty-five patients with IgA nephritis were enrolled, and renal biopsy specimens of 29 patients were evaluated. We evaluated baseline age, sex, hypertension, serum creatinine, glomerular filtration rate (GFR), urine protein, NGAL staining, glomerulosclerosis, interstitial fibrosis, and extracapillary proliferation. The primary endpoint of this study was doubling of baseline serum creatinine and/or the onset of ESRD in the course of the study. At the end of the follow-up, patients whose estimated GFR (eGFR) was ≤15 mL/min/1.73 m(2) and/or baseline serum creatinine doubled, were defined as the progressor group. RESULTS: Nineteen patients (65.5%) were NGAL positive and 10 patients (34.5%) were NGAL negative. Female gender and hypertension were associated with NGAL-positive staining. Urinary protein excretion and serum creatinine levels were more elevated in the NGAL-positive group, but the difference was not significant. We found NGAL-positive staining in major proportion in the progressor group (88.9%) than the non-progressor group (55%) (p = 0.076). CONCLUSION: NGAL staining can be a new histological marker in IgAN progression.

Massive bone marrow involvement in an end stage renal failure case with erythropoietin-resistant anemia and primary hyperoxaluria.

Primary hyperoxaluria is a rare autosomal recessive disorder. Type 1 PH is the most common form and develops due to a defect in a liver specific enzyme the alanine aminotransferase enzyme. As a result of the enzyme deficiency, there is an overproduction of oxalate and excessive urinary excretion. Recurrent urolithiasis and nephrocalcinosis are the most important findings of the disorder and often at the beginning end-stage renal disease develops. This report presents a case backed up by literature of a patient with end stage renal failure and erythropoietin-resistant anaemia whose bone marrow biopsy showed crystal deposition which received delayed diagnosis of oxalosis.

Gastrointestinal Tract Metastases of Invasive Lobular Carcinoma of the Breast: An Immunohistochemical Survey Algorithm.

Invasive lobular carcinoma (ILC) accounts for almost 15% of all breast carcinomas. The potential of ILC to metastasize to the gastointestinal system is significantly greater than that of invasive ductal carcinoma. Gastric metastasis occurred in the ninth year of the follow-up in a patient who was operated on the right breast due to ILC. The patient was investigated for simultaneous masses in the stomach and colon, and a random mass was found in her right breast.

Clinicopathologic features of single bone metastasis in breast cancer.

ABSTRACT: The most common site for metastasis in patients with breast cancer is the bone. In this case series, we investigated patients whose surgical and medical treatment for primary breast cancer was conducted at our center and first disease recurrence was limited to only 1 bone.We analyzed 910 breast cancer patients, 863 had no metastasis and 47 cases had a single bone metastasis ≥ 6 months after their first diagnosis. Demographic, epidemiological, histopathological and intrinsic tumor subtype differences between the non-metastatic group and the group with solitary bone metastases and their statistical significance were examined. Among established breast cancer risk factors, we studied twenty-nine variables.Three variables (Type of tumor surgery, TNM Stage III tumors and mixed type (invasive ductalcarsinoma + invasive lobular carcinoma) histology) were significant in multivariate logistic regression analysis. Accordingly, the risk of developing single bone metastasis was approximately 15 times higher in patients who underwent mastectomy and 4.8 and 2.8 times higher in those with TNM Stage III tumors and with mixed type (invasive ductal carcinoma + invasive lobular carcinoma) histology, respectively.In conclusion, the risk of developing single bone metastasis is likely in non-metastatic patients with Stage III tumors and possibly in mixed type tumors. Knowing this risk, especially in patients with mixed type tumors, may be instrumental in taking measures with different adjuvant therapies in future studies. Among these, treatment modalities such as prolonged hormone therapy and addition of bisphosphonates to the adjuvant treatments of stage III and mixed breast cancer patients may be considered.

Breast cancer patients with isolated bone metastases and oligometastatic bone disease show different survival outcomes.

In this study, we planned to investigate the clinical course of patients with breast cancer with oligometastatic bone disease (OMBD). The patients were grouped according to the characteristics and the sites of metastases. Group I included 928 patients without metastasis. Group II, the OMBD group, included 68 patients. Group III, the widespread metastasis group, comprised 185 patients with multiple bone metastases and/or solid organ metastases. The mean overall survival of the groups was 16.7 ± 0.3 years in group 1, and 7.8 ± 0.8 and 5.9 ± 0.4 years in groups 2 and 3, respectively (p < 0.001 for the comparison of all three groups together; p < 0.001 for group 1 vs. 2 and 3) and (p = 0.037 for group 2 vs. group 3). In the subgroup survival analysis of patients in group 2 (OMBD), the mean and median survival was 5.5 ± 0.8 and 4.0 ± 0.8 years vs. 9.2 ± 0.98 and 9.0 ± 1.05 years in patients with more than one bone metastasis and single bone metastasis, respectively (p = 0.019). OMBD seems to be a different disease than breast cancer with isolated bone metastases. The high risk of developing OMBD, especially following locoregional recurrence, increases the importance of locoregional therapy in large T and N stage tumors.

Neuroendocrine Differentiated Breast Cancer Cases: A Retrospective Analysis and Literature Review.

Objectives: Neuroendocrine breast carcinoma (NEBC) is a rare subgroup of breast cancer, which makes up 2-5% of all invasive breast cancers. The aim of this retrospective analysis is to present and analyze our own data of primary NEBCs. Methods: We retrospectively analyzed clinical, pathological, and radiological characteristics of 36 patients diagnosed with neuroendocrine differentiated breast cancer between 2008 and 2019 compared to that of 925 patients with invasive ductal carcinoma (IDC/NOS) along with a literature review. Results: In this study, 36 patients with neuroendocrine differentiated breast carcinoma and 961 patients with (IDC/NOS), as the comparison group, were identified between 2008 and 2019. In NEBC patients, seven were premenopausal and 29 postmenopausal. Patients whose ultrasound (USG), magnetic resonance, and mammographic (MMG) images available in our hospital, high-density masses were detected in the MMG with irregular (77%), microlobulated (80%) and spiculated margins (63%), unaccompanied by asymmetry and structural distortion. Calcifications were less common than invasive breast cancer, present only in four patients (17%). When NEBC were compared to ductal carcinomas (n=925), NEBC were more often human epidermal growth factor receptor 2 negative (p=0.039), estrogen receptor positive (p=0.05), progesterone receptor positive (0.03), and the NEBC patients were older (p=0.02). Age, grade, metastatic status, lymph node number, and molecular type were identified as prognostic factors that significantly affect survival in both groups (p<0.05). Conclusion: NEBC is a subtype that is both histopathologically and radiologically distinct from other breast cancer subtypes, and neuroendocrine differentiation may be an important predictive marker in the future.

Progression of graft fibrosis under mammalian target of rapamycin inhibitor-based regimen.

AIM: Nephrotoxic potential of mammalian target of rapamycin inhibitors (mTORi) is different from calcineurin inhibitors (CNI). The aim of this study is to investigate the interstitial fibrosis (ci) and tubular atrophy (ct) progression from the baseline to first year under a mTORi-based, CNI-free regimen. METHODS: Thirty-five kidney transplant recipients who had to have adequate baseline and first year protocol biopsy were enrolled. Exclusion criteria were: the replacement of CNI at any time; acute deterioration in allograft functions; and serum creatinine level above 3 mg/dL at 12 months. Banff criteria were used for histopathological classification. Progression was defined as delta ci + ct ≥ 2 (difference between 12th month and baseline). RESULTS: Mean age of patients and donors were 34 ± 11 and 49 ± 10 years. Twelve patients had delayed graft function (DGF). The maintenance regimen consisted of sirolimus (n = 24) and everolimus (n = 11) with mycophenolate mofetil and steroids. Incidence of acute rejection was 25.7%. At baseline, the incidence of nil and mild fibrosis were 80% and 20%, respectively. At 12 months, 17.1% of patients had moderate, 40% had mild and 42.9% had nil fibrosis. Histological progression from baseline to first year was present in 34% of patients. In multivariate analysis the presence of DGF (P = 0.018) and deceased donor type (P = 0.011) were the most important predictors for fibrosis progression. CONCLUSION: Progression of graft fibrosis may be seen in one-third of patients under a mTORi-based regimen particularly manifested in deceased donor recipients with subsequent DGF.

A rare cause of small bowel transection: metastatic lung cancer.

OBJECTIVE: To present a case of small bowel perforation due to metastatic lung cancer. CASE PRESENTATION AND INTERVENTION: A 78-year-old male patient, who had been diagnosed with lung cancer 3 months earlier, presented to our clinic with acute abdominal pain. He underwent emergency laparotomy. At surgery, there was a circumferential lesion encompassing the ileum with complete transection. There was no obvious macroscopic appearance of metastatic disease. The involved bowel segment was resected and an ileostomy was performed. Postoperative pathologic analysis confirmed metastatic squamous cell carcinoma metastasis to the ileum, arising from the patient's lung cancer. CONCLUSION: This case report showed that small bowel metastases should always be considered in the differential diagnosis of lung cancer patients presenting with acute abdominal pain.

Comparison of ePTFE graft repair with primary suturing in an experimental duodenal perforation model in rats.

BACKGROUND: In this experimental study, we treated peptic ulcer perforation in the first portion of the duodenum using an ePTFE (expanded polytetrafluoroethylene) soft tissue graft and compared results with primary suturing. METHODS: Wistar Albino rats were randomized into two groups. A duodenal defect of 3 mm was created in the first portion of the duodenum in all 24 rats. Macroscopic evaluation was done to determine presence of intraabdominal leakage, peritonitis, intraabdominal adhesions, and the level of adhesion, if present. The adhesion severity scoring system was used to score severity of adhesions. Histology section score criteria were used for scoring in microscopic evaluation. RESULTS: There was no statistically significant difference between the two groups in the adhesion severity scoring according to grades. However, when overall scoring was considered, a statistically significant difference was determined between the two groups (p=0.045). According to microscopic examination, there was no statistically significant difference between the two groups when classified into none to minimal cell accumulation, thin immature granulations and moderately thick granulation (p=0.089, p=0.178, p=0.755); however, there was a statistically significant difference between the two groups in the thick vascular granulation class (p=0.005). CONCLUSION: Use of the ePTFE graft method is easy, and results are comparable to those achieved with primary suturing. However, our results need further confirmation with larger series in animals and also with human studies.

The renal effect of replacement fluids in controlled severe hemorrhagic shock: an experimental study.

BACKGROUND: This experimental study examined the effects of resuscitation with Ringer's lactate (RL), 6% hydroxyethyl starch (130/0.4-HES), and the combination of RL and HES on renal function in hemorrhagic shock (HS). METHODS: Twenty-four male New Zealand white rabbits weighing 2198-3435 g were divided at random into four groups. HS was constituted by maintaining the mean arterial blood pressure at 30 mmHg and blood lactate at >4 mM/L. Subsequently, Group 1 (control) was not resuscitated, while the study rabbits' resuscitation was initiated with RL (Group 2), HES (Group 3), or the combination of RL and HES (Group 4). RESULTS: In all groups, the serum creatinine and blood urea nitrogen (BUN) levels were observed to be within normal limits, while the lactate dehydrogenase and alpha-1 microglobulin levels statistically significantly increased when time points were compared with beginning values (p<0.05). Furthermore, cystatin-C levels were observed to be increased after the HS (p<0.05), but returned to the normal level after resuscitation in all the study groups. Interleukin (IL)-6 and tumor necrosis factor (TNF)-alpha levels were increased in all the rabbits after HS (p<0.05), and there were no significant differences among the study groups after resuscitation (p>0.05). There were no differences in the histological imaging between the groups (p>0.05). CONCLUSION: The 6% HES (130/0.4) did not have any harmful effects on the kidney when it was used alone or in combination with crystalloid for resuscitation of HS in rabbits.

Impact of Subclinical Rejection on Kidney Graft Function.

BACKGROUND: In kidney transplant recipients with borderline infiltration, protocol biopsy results demonstrated the relationship with chronic injury. The purpose of this study was to evaluate the effect of subclinical rejection (SCR) on 6-month protocol biopsy results in long-term renal function in renal transplant recipients with stable graft function. MATERIAL AND METHODS: Transplant protocol biopsies performed in 45 patients with stable renal function were included in this study at 6 months. Biopsy specimens were evaluated for SCR. Study groups were divided into patients with and without SCR. Renal functions were compared with pathologic evaluation. The effect of immunosuppressive regimens on renal function were evaluated in patients with SCR RESULT: The median age of patients was 32 years (range, 18-64 years). The median follow-up was 56 months (range, 24-84 months). According to the 6-month protocol biopsy results, 20 of 45 patients (44.4%) met SCR criteria based on Banff 07 parameters. There was not a statistically significant difference in renal function with SCR. CONCLUSION: The presence of SCR on the 6-month protocol biopsy results in renal transplant recipients with a stable graft function does not cause deterioration in the long-term graft function.

An adult case of atypical hemolytic uremic syndrome presented with posterior reversible encephalopathy syndrome: Successful response to late-onset eculizumab treatment.

Atypical hemolytic uremic syndrome is a rare and progressive disease caused by uncontrolled alternative complement activation. Dysregulation of the complement activation results in thrombotic microangiopathy and multiorgan damage. A 29-yearold woman who was admitted with complaints of vomiting and headache was detected to have acute renal failure with microangiopathic hemolytic anemia (MAHA). After the diagnosis of atypical hemolytic uremic syndrome (aHUS), she was treated with plasma exchange (PE) and hemodialysis (HD). She has experienced hypertensionrelated posterior reversible encephalopathy syndrome (PRES) at the second plasma exchange. She was initiated on eculizumab therapy because of no response to PE on the 34th days. Her renal functions progressively improved with eculizumab treatment. Dependence on dialysis was over by the 4th month. Dialysis free-serum Creatinine level was 2.2 mg/dL [glomerular filtration rate (e-GFR): 30 mL/min/1.73 m2] after 24 months. Neurological involvement (PRES, etc.) is the most common extrarenal complication and a major cause of mortality and morbidity from aHUS. More importantly, we showed that renal recovery may be obtained following late-onset eculizumab treatment in patient with aHUS after a long dependence on hemodialysis.