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Following extended colon resections, it may not always be possible to perform colorectal anastomosis. The Deloyers procedure, which involves the transposition of the right colon, has been identified as a viable solution. This report aims to discuss the circumstances under which the Deloyers procedure was performed, as well as to evaluate the early and late postoperative outcomes, by reviewing cases conducted between 2010 and 2023. In a 22-year-old female patient who suffered major organ and tissue loss (with injuries to the sigmoid colon, descending colon, transverse colon, and mesentery) due to a firearm injury, the Deloyers procedure was applied during restorative surgery following initial damage control surgery. The procedure involved mobilizing the cecum and right colon, performing a cranio-caudal rotation over the ileocolic artery pedicle, followed by an appendectomy, and creating a colorectal anastomosis using circular staplers. There were no complications during the postoperative follow-ups. By the 14th postoperative day, the patient was discharged and experienced bowel movements four times a day, managed with 2.5 mg of diphenoxylate hydrochloride and 0.025 mg of atropine sulfate. At the 6-month follow-up, the frequency of bowel movements had decreased to twice daily without the need for medical treatment. Given the functional outcomes in patients after extended left colectomies, the Deloyers procedure, with its low associated morbidity, stands out as a viable option.
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Neoplasias Colorrectales , Armas de Fuego , Heridas por Arma de Fuego , Femenino , Humanos , Adulto Joven , Adulto , Anastomosis QuirúrgicaRESUMEN
Proper blood collection and timely analysis are vital steps for reliable results. This study aims to compare potassium(K), calcium(Ca), and phosphorus(P) concentrations in serum separator tube (SST), lithium heparin tube without gel (LiH), and lithium heparin tube with a barrier (Barricor)tubes in essential thrombocytosis(ET) patients. Additionally, we assessed short-term stability of these analytes at room temperature. K, Ca and P concentrations of blood taken from 40 ET patients into SST, LiH and Barricor tubes were measured at 0, 2, 4 and 8 h. We calculated the percentage difference and defined the maximum permissible difference (MPD) using the Biological Variation Database. Intertube comparisons were conducted using Passing-Bablok regression and Bland-Altman analysis. Comparing SST to LiH, the percentage difference values for all tests exceeded the MPD. When comparing Barricor to LiH, K and Ca tests were above MPD, except for P. At the 8th hour, LiH showed clinically significant changes in all three electrolytes. Barricor exhibited stability for K, Ca, and P for up to 8 h, with only Ca levels borderline higher than the MPD. Our study reveals clinically significant alterations in K, Ca, and P concentrations in SST compared to LiH tubes, and in K and Ca concentrations in Barricor compared to LiH tubes. While K, Ca and P concentrations were stable for up to 4 h at room temperature in all tube types tested, significant changes were observed in all electrolytes at 8 h in the LiH tube.
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Potasio , Trombocitosis , Humanos , Calcio , Fósforo , Litio , Heparina , Electrólitos , Recolección de Muestras de Sangre/métodosRESUMEN
OBJECTIVE: To identify predictive factors associated with the occurrence of postoperative pancreatic fistula (POPF) following pancreatoduodenectomy (PD) in an increasingly geriatric population. STUDY DESIGN: Observational study. Place and Duration of the Study: Department of General Surgery, Inonu University, Malatya, Turkey, from January 2010 to April 2022. METHODOLOGY: Demographic and clinicopathological data of 74 geriatric patients who underwent PD for periampullary tumours in the clinic at Inonu University were retrieved from the patient database. POPF was defined and categorised based on the guidelines established by the International Study Group for Pancreatic Surgery (ISGPS). The patients were stratified into two cohorts of POPF and no POPF. Univariate and multivariate analyses were conducted to compare variables between the two groups. RESULTS: The median age of the patient population was 72 (65-92) years, and 51 (68.9%) individuals were male. Among the 74 patients, 35 (47.3%) experienced POPF. In the multivariate analysis, hypertension (HT, p=0.012), Wirsung diameter <3.5 mm (p<0.01), and pancreaticojejunostomy (PJ, p=0.022) emerged as independent risk factors for POPF. CONCLUSION: In the context of geriatric patients undergoing PD, HT, intraoperative wirsung diameter <3.5 mm, and PJ were identified as independent risk factors for POPF. These findings can guide the adoption of safer techniques in preoperative and intraoperative evaluations, as well as in postoperative follow-ups of patients presenting with these risk factors. KEY WORDS: Elderly, Whipple procedure, Anastomotic leakage, Predictive factor, Postoperative pancreatic fistula, Pancreatoduodenectomy.
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Neoplasias Duodenales , Fístula Pancreática , Humanos , Masculino , Anciano , Anciano de 80 o más Años , Femenino , Fístula Pancreática/epidemiología , Fístula Pancreática/etiología , Pancreaticoduodenectomía/efectos adversos , Páncreas , Pancreatoyeyunostomía/efectos adversos , Factores de Riesgo , Neoplasias Duodenales/cirugía , Complicaciones Posoperatorias/etiología , Estudios RetrospectivosRESUMEN
BACKGROUND: Modified Graham omentopexy is the most commonly used operative technique in the repair of peptic ulcer perfo-ration (PUP); however, there is little data on falciformopexy in the literature. The aim is to investigate the feasibility of falciformopexy in the repair of PUP, comparing with modified Graham omentopexy. METHODS: Data of 471 patients who were operated for PUP were retrospectively analyzed. Patients' demographics, pre-operative basic laboratory findings, American Society of Anesthesiologists (ASA) status, operative findings, and post-operative complications were recorded. The patients were classified into two groups modified Graham omentopexy and falciformopexy, and then compared with each other in terms of clinical characteristics, operative findings, and post-operative complications. RESULTS: Modified Graham omentopexy and falciformopexy were performed in 425 (90.2%) and 46 (9.8%) patients, respectively. The two groups were similar in terms of basic patient characteristics and pre-operative laboratory findings (P>0.05). ASA physical status was significantly different between the groups (P=0.001). No statistically significant difference was found between the groups in terms of complications, except for an anastomotic leak. Anastomotic leak was observed more frequently in patients who underwent falciformopexy than in patients with modified Graham omentopexy (P=0.017). CONCLUSION: Although falciformopexy technique has a higher rate of leak compared to the modified Graham omentopexy method, it should be kept in mind as an alternative method for repair of PUP, especially in cases where omentopexy cannot be applied for various reasons such as the presence of unavailable or unsuitable omentum.
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Laparoscopía , Úlcera Péptica Perforada , Humanos , Fuga Anastomótica/etiología , Úlcera Péptica Perforada/cirugía , Estudios Retrospectivos , Estudios de Factibilidad , Complicaciones Posoperatorias/cirugía , Laparoscopía/efectos adversosRESUMEN
Introduction: COVID-19 pandemic has led to an increased rate of intensive care unit (ICU) stays. Intermediate care units (IMCUs) are a useful resource for the management of patients with severe COVID-19 that do not require ICU admission. In this research, we aimed to determine survival outcomes and parameters predicting mortality in patients who have been admitted to IMCU. Materials and Methods: Patients who were admitted to IMCU between April 2019 and January 2021 were analyzed retrospectively. Sociodemographics, clinical characteristics, and blood parameters on admission were compared between the patients who died in IMCU and the others. Blood parameters at discharge were compared between survived and deceased individuals. Survival analysis was performed via Kaplan-Meier analysis. Blood parameters predicting mortality were determined by univariate and multivariate Cox regression analysis. Results: A total of 140 patients were included within the scope of this study. The median age was 72.5 years, and 77 (55%) of them were male and 63 (45%) of them were female. A total of 37 (26.4%) patients deceased in IMCU, and 40 patients (28.5%) were transferred to ICU. Higher platelet count (HR 3.454; 95% CI 1.383-8.625; p=0.008), procalcitonin levels (HR 3.083; 95% CI 1.158-8.206; p=0.024), and lower oxygen saturation (HR 4.121; 95% CI 2.018-8.414; p < 0.001) were associated with an increased risk of mortality in IMCU. At discharge from IMCU, higher procalcitonin levels (HR 2.809; 95% CI 1.216-6.487; p=0.016), lower platelet count (HR 2.269; 95% CI 1.012-5.085; p=0.047), and noninvasive mechanic ventilation requirement (HR 2.363; 95% CI 1.201-4.651; p=0.013) were associated with an increased risk of mortality. Median OS was found as 41 days. The overall survival rate was found 40% while the IMCU survival rate was 73.6%. Conclusions: IMCU seems to have a positive effect on survival in patients with severe COVID-19 infection. Close monitoring of these parameters and early intervention may improve survival rates and outcomes.
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COVID-19 , Unidades de Cuidados Intensivos , Anciano , Femenino , Humanos , Masculino , Pandemias , Polipéptido alfa Relacionado con Calcitonina , Estudios Retrospectivos , Instituciones de Cuidados IntermediosRESUMEN
BACKGROUND: Sigmoid volvulus is a pathology that can be mortal because it is frequently encountered in elderly patients. In case of bowel gangrene, mortality and morbidity increase further. We planned a retrospective study, in which the effectiveness of the model was evaluated by creating a model that aims to predict whether intestinal gangrene is present in patients with sigmoid volvulus only by blood tests and thus to quickly guide treatment methods. METHODS: In addition to demographic data such as age and gender, laboratory values such as white blood cell, C-reactive protein (CRP), lactate dehydrogenase (LDH), potassium, and colonoscopic findings and whether there was gangrene in the colon during the operation were evaluated retrospectively. In the analysis of the data, independent risk factors were determined by univariate and multivariate logistic regression analyzes as well as Mann-Whitney U and Chi-square tests. Receiver operating characteristic (ROC) analysis was performed for statistically significant continuous numerical data, and cutoff values were determined and Malatya Volvulus Gangrene Model (MVGM) was created. The effectiveness of the created model was again evaluated by ROC analysis. RESULTS: Of the 74 patients included in the study, 59 (79.7%) were male. The median age of the population was 74 (19-88), and gangrene was detected in 21 (28.37%) patients at surgery. In univariate analyzes, leukocytes <4000/mm3 and >12000/mm3 (OR: 10.737; CI 95%: 2.797-41.211, p=0.001), CRP ≥0.71 mg/dl (OR: 8.107 CI 95%: 2.520-26.082, p<0.0001), potassium ≥3.85 mmoL/L (OR: 3.889; 95% CI): 1.333-11.345, p=0.013), and LDH ≥288 U/L (OR: 3.889; CI 95%: 1.333-11.345, p=0.013), whereas, in multivariate analyzes, only CRP ≥0.71 mg/dL (OR: 3.965; CI 95%: 1.071-15.462, p=0.047) was found to be an independent risk factor for bowel gangrene. The strength of MVGM was AUC 0.836 (0.737-0.936). In addition, it was observed that the probability of bowel gangrene increased approximately 10 times if MVGM was ≥7 (OR: 9.846; 95% CI: 3.016-32.145, p<0.0001). CONCLUSION: Besides being non-invasive compared to the colonoscopic procedure, MVGM is a useful method for detecting bowel gangrene. In addition, it will guide the clinician in taking the patients with intestinal loop gangrene to emergency surgery without wasting time in the treatment steps, as well as avoiding complications that may occur during colonoscopy. In this way, we think that morbidity and mortality rates can be reduced.
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Vólvulo Intestinal , Isquemia Mesentérica , Humanos , Masculino , Anciano , Femenino , Vólvulo Intestinal/complicaciones , Vólvulo Intestinal/diagnóstico , Vólvulo Intestinal/cirugía , Estudios Retrospectivos , Gangrena/cirugía , Gangrena/complicaciones , Colonoscopía/efectos adversos , Colon/patologíaRESUMEN
This study aimed to establish a strong regression model by revealing the preoperative predictive factors for sentinel lymph node (SLN) positivity in patients with early stage breast cancer (ESBC). In total, 445 patients who underwent SLN dissection for ESBC were included. All data that may be potential predictors of SLN positivity were retrospectively analyzed. Tumor sizeâ >2 cm, human epidermal growth factor receptor 2 (HER2)â +â status, lymphovascular invasion (LVI), palpable tumor, microcalcifications, multifocality or multicentricity, and axillary ultrasonographic findings were defined as independent predictors of SLN involvement. The area under the receiver operating characteristic (ROC) curve (AUC) values were 0.797, 0.808, and 0.870 for the Memorial Sloan-Kettering Cancer Center (MSKCC) nomogram, MD Anderson Cancer Center (MDACC) nomogram, and our regression model, respectively (Pâ <â .001). The recent model for predicting SLN status in ESBC was found to be stronger than existing nomograms. Parameters not included in current nomograms, such as palpable tumors, microcalcifications, and axillary ultrasonographic findings, are likely to make this model more meaningful.
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Neoplasias de la Mama , Ganglio Linfático Centinela , Humanos , Femenino , Neoplasias de la Mama/cirugía , Estudios RetrospectivosRESUMEN
BACKGROUND: Considerable progress has been made in the treatment of multiple myeloma (MM) patients with the development of various new agents that increased survival rates over the past fifteen years. Cereblon (CRBN) plays an important role in mediating the antitumor effects of immunomodulatory drugs (IMiDs) among these new agents. The aim of our study is to investigate immunohistochemically (IHC) cereblon protein expression status in MM. METHODS: Immunohistochemically, CRBN expression and its relationship with various prognostic factors were evaluated in bone marrow biopsies of 96 patients with MM in a single centre. RESULTS: Cytoplasmic and nuclear CRBN expression was detected in all neoplastic cells. While a complete or partial response to treatment was obtained in 45 patients, the disease was stable in 13 and progressive in 17 patients. Survival was longer in those treated with IMiD-containing regimens (p = 0.044). Both the survival rate (p = 0.013) and the survival time were significantly increased (p = 0.023) in those who received the treatment protocol containing protease inhibitors. A significant relationship was found between the treatment protocol and treatment response in the chi-squared analysis (p = 0.008). Although the longest survival time - though not statistically significant - was detected in the group treated with protease inhibitors (log rank, p = 0.217). The survival analysis revealed the presence of a relationship between IgG and IgA positivity and survival. CONCLUSIONS: In this study, the survival time of the patients who received treatment regimens containing protease inhibitors and IMiD was longer, independent of the presence of strong nuclear CRBN expression. The survival rate was significantly higher in those who used IMiD and protease inhibitors in combination. Since the survival rate was found to be increased in IgG positive cases and we thought that evaluation of immunoglobulin tissue expression in MM cases can provide prognostic prediction.
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Mieloma Múltiple , Proteínas Adaptadoras Transductoras de Señales , Humanos , Inmunoglobulina A , Inmunoglobulina G/metabolismo , Mieloma Múltiple/diagnóstico , Mieloma Múltiple/tratamiento farmacológico , Mieloma Múltiple/metabolismo , Péptido Hidrolasas/metabolismo , Péptido Hidrolasas/uso terapéutico , Pronóstico , Inhibidores de Proteasas/uso terapéutico , Talidomida/uso terapéutico , Ubiquitina-Proteína Ligasas/metabolismo , Ubiquitina-Proteína Ligasas/uso terapéuticoRESUMEN
Objectives: This study aimed to evaluate seasonal effects on the mechanisms of burn injuries in patients requiring hospitalization. Material and Methods: A retrospective evaluation was made using the information of 419 hospitalized burns patients, including demographic data, degree and percentage of burn injury, cause and mechanism of burn injury, morbidity and mortality. Burn mechanisms were grouped as thermal burns (flame, boiling liquid, contact), chemical burns and electrical burns. When calculating the percentage of body surface area burned, the rule of nines was applied. Seasonal classification was made appropriate to the northern hemisphere. Results: According to the seasons, the most burns were seen in spring months (n= 130, 31.0%). In the examination of the mechanism of burn injury, the most common type of injury was boiling liquid in 159 patients followed by flame injury in 146 patients. There was an increase in electrical and chemical burns in spring and summer. A statistically significant difference was determined between the types of burns according to the seasons (p= 0.024). The burn injury occurred as a result of a workplace accident in 82 cases, the majority of which were in autumn, and summer, and the difference in the seasons was determined to be statistically significant (p= 0.045). There was a statistically significant increase in the exposure of individuals aged >65 years to boiling liquid burns in winter and summer months (p= 0.014). Conclusion: The results of this study showed a seasonal effect on the types of burn injuries. A higher rate of thermal burns was expected to be found in winter, but this was not the case in patients with indications for hospitalization, as chemical and electrical burns in workplace accidents were seen more frequently in warmer seasons of spring and summer. In this context, burns units should be prepared for patient profiles to vary according to the season.
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BACKGROUND: Many predictive factors and scoring systems associated with Fournier's gangrene have been proposed, including comorbidities, vital signs, biochemical and hematological parameters, and demographic characteristics of the patient. The aim of this study was to determine the strengths of the scoring systems that have been formed by revealing these factors from a wider perspective and in a larger patient population. METHODS: The patient population included 144 patients, 21 of whom died. Age, biochemical and hematological parameters, Uludag Fournier's Gangrene Severity Index (UFGSI), Fournier's Gangrene Severity Index (FGSI), and Age-Adjusted Charlson Comorbidity Index (ACCI) scores were analyzed using the Mann Whitney U-test due to their non-parametric distribution. Categorical data such as comorbidities, gender, need for positive inotropes, diversion ostomy status, and UFGSI grading status was analyzed with the Chi-square test, and independent risk factors were determined from the significant data emerging from univariate and multivariate logistic regression analysis. Their strengths were compared using the logistic regression model (Fournier's Gangrene Mortality Prediction Model: FGMPM) created through ROC analysis of the FGSI, UFGSI, and ACCI scores. RESULTS: The results of the statistical analyses showed that albumin (p<0.001) and need for positive inotropic support (p<0.001) were independent risk factors for mortality and ROC analysis revealed that the created FGMPM regression model (AUC: 0.995) was a stronger model than the FGSI (AUC: 0.874), UFGSI (0.893), and ACCI (0.788) scoring systems. CONCLUSION: The results of this study revealed that albumin and the need for positive inotropic support are independent risk factors for mortality. It is thought that the determination of these two parameters can be used to predict mortality more practically than the parameters used in the UFGSI and FGSI.
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Gangrena de Fournier , Albúminas , Gangrena de Fournier/diagnóstico , Humanos , Masculino , Curva ROC , Estudios Retrospectivos , Índice de Severidad de la EnfermedadRESUMEN
AIMS: JAK2V617F (JAK2), calreticulin (CALR) and MPL515L/K (MPL) mutations are important in essential thrombocythemia (ET) and may be associated with various clinical consequences of the disease. This study aimed to compare the clinical and haematological parameters of ET patients regarding the mentioned mutations and the role of plateletcrit (PCT). METHODS: Seventy patients who were diagnosed with ET between 2005 and 2017 in a single centre were included in this descriptive study. The initial symptoms and clinical findings were retrieved from the electronic database. JAK2 gene V617F mutations, MPL gene exon 10 mutations and CALR gene exon 9 DNA sequence analyses were performed. Forty-one healthy volunteers were included to perform ROC curve analysis for interpreting PCT value. RESULTS: The distributions of patients according to the mutations were as follows: Thirty-seven (52.9%) patients were JAK2-positive, 15 (21.4%) were CALR-positive, 2 (2.8%) patients were positive for both CALR and JAK2, and 1 (1.4%) was only MPL-positive. Fifteen (21.4%) patients were triple-negative. The ET patients with JAK2 mutation showed a higher level of haemoglobin at the time of diagnosis. The ET patients with CALR mutation presented with higher platelet and LDH levels (P = .002 and P = .001, respectively). The PCT level was higher in the CALR-positive group when compared to the others (P = .026). A sensitivity value of 97.6% and specificity value of 98.6% were determined regarding PCT% at a cut-off value of 0.37 in ET patients. In CALR-positive patients, the sensitivity and specificity values were 100% for the PCT at a cut-off value of 0.42%. CONCLUSION: We determined that the platelet count and blood LDH level was high in the ET patient group with CALR mutation. Besides, we found that the blood haemoglobin level was higher in the ET patient group with JAK2 mutation. Additionally, the PCT level was higher in the CALR group when compared to the other patient groups.
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Calreticulina , Trombocitemia Esencial , Calreticulina/genética , Calreticulina/metabolismo , Pruebas Hematológicas , Humanos , Janus Quinasa 2/genética , Janus Quinasa 2/metabolismo , Mutación/genética , Trombocitemia Esencial/genéticaRESUMEN
Antinuclear antibodies (ANA) facilitate the diagnosis and evaluation of patients in many systemic autoimmune conditions. Besides, ANA may also be detected in chronic infectious diseases. Although a number of investigations associated with autoantibody positivity in patients with chronic hepatitis C were reported, autoantibody positivity in patients with chronic hepatitis B remain rarely addressed in the literature. The aim of this study was to evaluate the antinuclear antibody (ANA), antimitocondrial antibody (AMA), anti-smooth muscle antibodies (ASMA) and anti-liver-kidney microsomal antigen (LKM) antibodies in chronic hepatitis B patients. Serum samples were obtained from adult patients with chronic hepatitis B diagnosis according to "European Association for the Study of the Liver (EASL)" criteria. Samples were taken from 47 patients (22 female, 25 male) with treatment-naive, histologically-proven chronic hepatitis B. Cases co-infected with HCV and/or HIV or that also had systemic autoimmune diseases were excluded. As a control group, 30 healthy blood donors were included in the study. Autoantibodies, including ANA, AMA, ASMA and LKM were detected with indirect immunofluorescence (IIF) method (Euroimmune, Lubeck, Germany) and evaluated by fluorescence microscope (Eurostar III plus, Germany). Positive results were graded into 4 levels ( "+", "++","+++" and "++++") from weak to strong Positive samples were studied with a immunoblotting method (ANA Profile 3, Euroimmun, AG) for the detection of extractable nuclear antigen (ENA). The positive results were detected in 8 (17%) of the HBV patients while all the samples were negative in the control group. The difference between the groups was significant (p< 0.05). Among the 47 serum samples tested, none of the patients were positive for AMA, ASMA, LKM. ANA was present in eight of the serum samples in which six were female and two were male patients. Among the IIF patterns of ANA positivity, one mixed pattern (homogeneous and nucleolar) and one cytoplasmic anti-golgi antibody pattern were detected. Positivity grade was ''++''. Other positive patterns were nucleolar (two patients), granular (two patients), ribozomal (one patient) and homogeneous (one patient) and positivity grade was ''+''. ENA was detected in three samples. Two of them was granular pattern positive samples. SS-A was borderline (±) in one and SS-B was borderline (±) in one of the samples. In the mixed pattern positive sample, histon was detected as ''+''. Autoantibody positivity between the patient and control groups were statistically significant (p< 0.05). The difference between autoantibody positivity and gender/age was not statistically significant. In conclusion, autoimmune manifestations may be detected in patients with chronic hepatitis B. Low level titer of antibodies such as ANA, AMA, ASMA or LKM may be present in such patients. An increased frequency of these autoantibodies may be associated with non-autoimmune conditions such as chronic viral infection even in treatment-naive patients.
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Anticuerpos Antinucleares/sangre , Hepatitis B Crónica/sangre , Adulto , Autoanticuerpos/sangre , Femenino , Humanos , MasculinoAsunto(s)
Leucemia Mielógena Crónica BCR-ABL Positiva/psicología , Calidad de Vida , Depresión/complicaciones , Depresión/diagnóstico , Depresión/psicología , Femenino , Humanos , Leucemia Mielógena Crónica BCR-ABL Positiva/complicaciones , Leucemia Mielógena Crónica BCR-ABL Positiva/tratamiento farmacológico , Leucemia Mielógena Crónica BCR-ABL Positiva/genética , Masculino , Persona de Mediana Edad , Encuestas y CuestionariosRESUMEN
We report a 63-year-old man with a history of chronic lymphocytic leukemia (CLL) who presented with asymmetrical Raynaud's phenomenon of sudden onset which progressed to acral gangrene rapidly in a week. These symptoms began approximately one week after the fourth cycle of fludarabine and cyclophosphamide chemotherapy and were accompanied by pain, numbness, and cyanosis in the fingers of his right hand except the first finger. Fludarabine may play a role in acral vascular syndrome. The treatment with fludarabine in patients with evolving digital ischemia should be carried out with caution.
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Enfermedades Pancreáticas/cirugía , Medición de Riesgo/métodos , Femenino , Humanos , MasculinoRESUMEN
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, progressive, and life-threatening hematopoietic stem cell disorder characterized by complement-mediated intravascular hemolysis and a prothrombotic state. Patients with PNH might have slightly increased risk of infections due to complement-associated defects subsequent to CD59 deficiency. Here, we report a rare case of a 65-year-old male patient with necrotic ulcers on both legs, where the recognition of pancytopenia and microthrombi led to the diagnosis of PNH based on FLAER (FLuorescent AERolysin) flow cytometric analysis. He was subsequently started on eculizumab therapy, with starting and maintenance doses set as per drug labelling. Progression of the patient's leg ulcers during follow-up, with fulminant tissue destruction, purulent discharge, and necrotic patches, led to a later diagnosis of necrotizing fasciitis due to Pseudomonas aeruginosa and Klebsiella pneumonia infection. Courses of broad-spectrum antibiotics, surgical debridement, and superficial skin grafting were applied with successful effect during ongoing eculizumab therapy. This case highlights the point that it is important to maintain treatment of underlying disorders such as PNH in the presence of life-threatening infections like NF.
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Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired disease caused by clonal expansion of one or more hematopoietic stem cell (HSC) lines due to a somatic mutation of the phosphatidylinositol glycan anchor (PIG-A) gene located on Xp22.1. PNH incidence is 1.5-2 cases per million of the population per year. PNH can affect multiple systems in the body and requires multidisciplinary clinical management. Patients can manifest with severe pancytopenia, life-threatening thrombosis affecting the hepatic, abdominal, cerebral, and subdermal veins, and high requirements for blood transfusion due to haemolytic anemia. PNH can also be associated with bone marrow failure. Advances in diagnostic techniques and a targeted therapeutic approach for PNH have emerged in the last two decades. Eculizumab, a promising humanized monoclonal antibody against C5, is the first approved therapy for PNH.
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OBJECTIVE: Immune thrombocytopenia (ITP) is an immune-mediated disease characterized by transient or persistent decrease of the platelet count to less than 100x109/L. Although it is included in a benign disease group, bleeding complications may be mortal. With a better understanding of the pathophysiology of the disease, thrombopoietin receptor agonists, which came into use in recent years, seem to be an effective option in the treatment of resistant cases. This study aimed to retrospectively assess the efficacy, long-term safety, and tolerability of eltrombopag in Turkish patients with chronic ITP in the Aegean region of Turkey. MATERIALS AND METHODS: Retrospective data of 40 patients with refractory ITP who were treated with eltrombopag in the Aegean region were examined and evaluated. RESULTS: The total rate of response was 87%, and the median duration of response defined as the number of the platelets being over 50x109/L was 19.5 (interquartile range: 5-60) days. In one patient, venous sinus thrombosis was observed with no other additional risk factors due to or related to thrombosis. Another patient with complete response and irregular follow-up for 12 months was lost due to sudden death as the result of probable acute myocardial infarction. CONCLUSION: Although the responses to eltrombopag were satisfactory, patients need to be monitored closely for overshooting platelet counts as well as thromboembolic events.