A Case Report of Chronic Myelogenous Leukemia Presenting as Blastic Crisis with a T-Cell Acute Lymphoblastic Leukemia Phenotype: Awareness of a Rare Entity.

Chronic myelogenous leukemia at blast crisis with a T-cell phenotype (T-ALL CML-BC) at diagnosis, without any prior history of CML is extremely rare. After the introduction of tyrosine kinase inhibitors (TKIs), CML patients have a median survival comparable to general population and accelerated/blast crisis are rarely encountered. Most CML patients (80%) transform into acute myeloid leukemia and the rest into B-ALL. Anecdotal cases of Ph+ T-ALL, either de novo or in the context of CML-BC have been reported. Left shift in the blood, the presence of splenomegaly/extramedullary infiltration and the occurrence of BCR::ABL1 rearrangement in both the blastic population, as well as in the myeloid cell compartment are key points in differentiating de novo Ph+ T-ALL from T-ALL CML-BC. The latter is a rare entity, characterized by extramedullary disease, p210 transcript and clonal evolution. Lack of preceding CML does not rule out the diagnosis of T-ALL CML-BC. Prompt TKI treatment with ALL-directed therapy followed by allogeneic stem cell transplantation may offer long-term survival in this otherwise poor prognosis entity. In this paper, we describe a patient with T-ALL CML-BC at presentation, still alive 51 months after diagnosis and we offer a review of the literature on this rare subject. All clinical and laboratory features are provided in order to distinguish de novo Ph+ T-ALL from T-ALL CML-BC, underscoring the prognostic and therapeutic significance of such a differentiation.

Jejunal Diverticulitis Mimicking Small Bowel Perforation: Case Report and Review of the Literature.

Jejunal diverticulitis is a rare entity with a higher prevalence among patients between 60 and 70 years. Jejunal diverticula are most often considered an incidental finding, but, they can have complications such as diverticulitis, perforation, abscess, generalized peritonitis, fistula, obstruction and bleeding.Setting the diagnosis still remains challenging. Physicians should be aware of their existence and the clinical suspicion should be raised, especially in the setting of acute abdominal pain where jejunal diverticulitis should be included in the differential diagnosis. A small amount of free air adjacent to the small bowel can be confusing and easily misdiagnosed as small bowel perforation, but, it can actually be found as a result of the inflammation itself without macroperforation or complications.This fact can change the therapeutic strategy to less aggressive, conservative treatments. We present a case of a patient coming to the emergency department with acute abdominal pain, signs of peritonitis, a small amount of extraluminal air, and jejunal diverticulitis without perforation was diagnosed on laparotomy, and a review of the current literature.

Squamous cell carcinoma arising from chronic sacrococcygeal pilonidal disease: a case report.

BACKGROUND: Sacrococcygeal pilonidal disease is a chronic, well-recognized entity, characterized by the recurrent formation of an abscess or draining sinus over the sacrococcygeal area. It is one of the most common surgical problems. Rarely, chronic inflammation and recurrent disease leads to malignant transformation, most commonly to squamous cell carcinoma (SCC). CASE PRESENTATION: We describe an extremely unusual case of SCC developing in a 60-year-old patient with a chronic pilonidal sinus complicated by an anal fistula. After wide surgical excision of the pilonidal sinus and fistulas and because of the poor healing process 6 months later, colonoscopy and a percutaneous fistulography were performed, revealing an anal canal-pilonidal fistula. Patient was treated with a more radical surgical resection with a prophylactic loop colostomy, but healing was not accelerated. Multiple biopsies were then taken from the surgical site at the time, which revealed the development of SCC. CT and MRI imaging techniques revealed SCC partial invasion of the coccyx and sacrum. As a result, aggressive surgical approach was decided. Histological examination revealed moderately to poorly differentiated SCC, and the patient was treated with adjuvant radiation therapy postoperatively. Nine months later, recurrence was found in the sacrum and para-aorta lymph nodes and the patient died shortly after. We discuss the clinical features, pathogenesis, treatment options, and prognosis of this rare malignant transformation. CONCLUSIONS: The development of SCC in chronic pilonidal disease is a rare but serious complication. Symptoms are usually attributed to the sacrococcygeal pilonidal disease (SPD), and diagnosis is often made late by histological examination of biopsies. Malignant transformation should be suspected in chronic SPD with recurrent episodes of inflammation, repeated purulent discharge, poor healing, and chronic complex fistulas.

Is Routine Post-operative Biological Laboratory Assessment Necessary After Sleeve Gastrectomy?

BACKGROUND: Incidence of post-operative complications after sleeve gastrectomy (SG) is low. However, the early identification of these complications remains crucial. Here, we report the impact of routine laboratory monitoring for the early diagnosis of complications after SG. MATERIAL AND METHODS: From January 2018 to December 2019, all consecutive patients who underwent primary SG (n = 457) were included. This was a comparative study of patients undergoing primary SG. Patients were divided into two groups: one group with routine laboratory monitoring performed at postoperative day (POD) 1 and 3 (LAB group) and another group without routine laboratory monitoring (control group). The study's primary endpoint was the overall impact of routine laboratory monitoring. The secondary endpoints were evaluation of patients with complications. RESULTS: The population in the two groups were similar in term of demographic and intra-operative data. There was a statistical difference between the two groups in term of length of stay (5.7 days in the LAB group and 3.5 days in the control group (p < 0.001)). There were 19 complications (6.0%) in the LAB group and 5 complications in the control group (3.5%) (p = 0.25). A cut-off C-reactive protein level of 46.3 mg/l was found to be significant (p = 0.006). In the LAB group, 9 patients (2.9%) required readmission vs. three patients (2.0%) in the control group (p = 0.62). CONCLUSION: The interest of routine laboratory monitoring after SG seems limited. Routine laboratory monitoring alone is not associated with earlier diagnosis of complications. This routine monitoring is associated with an increase of stay in hospital.

Endoscopic Administration of Combined Autologous Mesenchymal Stem Cells and Platelet-Rich Plasma for the Treatment of Gastric Staple Line Leaks After Sleeve Gastrectomy.

BACKGROUND: Gastric staple line leak treatment after laparoscopic sleeve gastrectomy (LSG) remains challenging. Regenerative medicine is gaining place in the accelerated treatment of damaged tissues. This study presents the first series of gastric leak treatment after LSG using endoscopic intragastric administration of combined autologous mesenchymal stem cells (MSC) and platelet-rich plasma (PRP). METHODS: MSC-PRP harvesting and endoscopic administration techniques are described in detail. Data were prospectively gathered and analyzed. Primary endpoints were morbidity/mortality rates and fistula closure time. RESULTS: Twelve patients (9 women, 3 men) were included. Median age was 41.5 years, median weight 105.5 kg and median BMI 38.9 kg/m2. Median time to gastric staple line leak detection was 10 days post-LSG. Median time between re-laparoscopy and MSC-PRP administration was 5 days. MSC-PRP endoscopic administration was successfully performed and tolerated by all patients, with median procedure duration of 27 min and minimal blood loss. Four postoperative complications were noted: two patients with increased tibial pain at tibial puncture site, one with tibial hematoma, and one with epigastric pain/dysphagia. Median length of hospital stay was 1 day. Gastric leak healing occurred after a median of 14 days, only two patients requiring a second MSC-PRP endoscopic injection. Median follow-up was 19 months, all patients being in good health at last contact. CONCLUSION: Endoscopic administration of combined autologous MSC-PRP seems to be a good option for treatment of gastric leaks after sleeve gastrectomy. It is a challenging procedure that should be performed in specialized bariatric centers by expert bariatric surgeons and endoscopists after meticulous patient selection.

High alloreactivity of low-dose prophylactic donor lymphocyte infusion in patients with acute leukemia undergoing allogeneic hematopoietic cell transplantation with an alemtuzumab-containing conditioning regimen.

The value of prophylactic donor lymphocyte infusion (pDLI) is unclear and differs among diseases and transplantation protocols. Experience with this approach in patients with acute leukemia undergoing hematopoietic cell transplantation (HCT) with an alemtuzumab-incorporating conditioning protocol is lacking. We conducted a single-center prospective study to investigate the applicability and efficacy of prophylactic donor lymphocyte infusion (pDLI) in patients with leukemia undergoing HCT with a low-dose alemtuzumab-containing conditioning regimen. Inclusion criteria were high-risk acute myelogenous leukemia, acute lymphoblastic leukemia, or increasing mixed chimerism. All patients included were tapered off of immunotherapy. Exclusion criteria were a history of ≥ grade II or active graft-versus-host disease (GVHD). Of the 56 consecutive patients who underwent HCT with an alemtuzumab-containing regimen, 15 patients (8 with acute myelogenous leukemia and 7 with acute lymphoblastic leukemia) met the study inclusion criteria and received prophylactic DLI (total of 45 infusions) from 7 sibling donors and 8 unrelated donors. The first infusion was given at a median of 162 days posttransplantation. The median number of DLIs was 3, and the median cumulative CD3(+) cell dose was 2 × 10(6)cells/kg. Six of the 8 patients (75%) who received pDLI while in mixed chimerism converted to stable, complete donor chimerism. Some 47% of DLI recipients developed GVHD (4 acute GVHD and 3 with chronic GVHD) after a median cumulative dose of 2 × 10(6) CD3(+) cells/kg. After a median follow-up of 575 days, 11 (73%) pDLI recipients were alive. All 4 deaths were due to GVHD-related causes. None of the patients who received pDLIs relapsed. Patients with leukemia who received low-dose pDLI after conditioning with alemtuzumab are at low risk for relapse; however, this approach is associated with a relatively high incidence of severe GVHD.

Laparoscopic Roux-en-Y fistulojejunostomy as a salvage procedure in patients with chronic gastric leak after sleeve gastrectomy.

BACKGROUND: The most common postoperative complication of laparoscopic sleeve gastrectomy (LSG) is staple-line leak. Even if its rate following LSG has been recently reduced, management of chronic leaks remains challenging. OBJECTIVE: To present a series of patients treated with laparoscopic Roux-en-Y fistulojejunostomy (LRYFJ) for chronic gastric leak (>12 wk) post-LSG. SETTING: University hospitals; specialized bariatric surgery units. METHODS: Data were prospectively gathered and retrospectively analyzed. Parameters of interest were patient characteristics, perioperative data, and postoperative outcomes. Hemodynamically unstable patients and/or presentations of signs of severe sepsis were excluded. Surgical technique was standardized. RESULTS: Fourteen patients underwent LRYFJ for chronic gastric leak (12 women, 2 men). The mean age was 49.2 years and the mean weight was 88.7 kg with a mean body mass index of 31.1 kg/m2. All procedures were successfully performed by laparoscopy except 1 (7.1%) converted to open surgery. The mean operative time was 198 minutes, with a mean estimated blood loss of 135.7 mL and 2 patients necessitating transfusion (14.2%). Mortality was null. Five postoperative complications were noted (35.7%): 2 leaks of the fistulojejunostomy treated by antibiotherapy and endoscopic drainage; 1 perianastomotic hematoma treated by relaparoscopy and antibiotherapy; and 1 pleural effusion and 1 hematemesis both medically treated. The mean length of hospital stay was 14 days. The mean follow-up was 40 months, with all patients being in good health at last contact. CONCLUSIONS: LRYFJ seems to be a good salvage option in selected patients for the treatment of chronic gastric leaks after LSG. However, it is a challenging procedure and should be performed in experienced bariatric centers by expert bariatric surgeons.

Routine Early Computed Tomography Scanner After Laparoscopic Sleeve Gastrectomy in High-Risk Severely Obese Patients Is Effective for Bleeding or Hematoma Diagnosis but not for Staple-Line Leak Detection: a Prospective Study.

PURPOSE: Laparoscopic sleeve gastrectomy (LSG) is the most frequently performed bariatric procedure worldwide. Postoperative staple-line leak and intraabdominal hemorrhage can increase associated morbidity and mortality. The value of routine early computed tomography (CT) scanner examination in the early diagnosis of complications in high-risk severely obese patients undergoing LSG is studied. METHODS: This was a prospective, non-randomized study including all patients undergoing LSG in our department from 2014 to 2020. Patients presenting at least one potential risk factor for postoperative gastric leak and bleeding (as defined by the current literature) were included. Primary endpoint was the efficacy of postoperative day (POD) 2 CT-scanner examination in diagnosing these complications. RESULTS: One thousand fifty-one high-risk patients were included. Median age was 44 years. Early postoperative surgical complications occurred in 48 patients (4.5%): 25 (2.3%) intraabdominal hemorrhage and 23 (2.2%) staple-line leak. Early CT-scanner detected intraabdominal bleeding or hematoma in 22/25 patients, with 95.6% sensitivity (Youden's index = 0.95), while specificity was 100%, positive predictive value (PPV) 100%, and negative predictive value (NPV) 99.9%. Sensitivity of early postoperative CT-scanner was 43.4% (10/23 patients; Youden's index = 0.43) for staple-line leak detection, with specificity of 100%, PPV 100%, and NPV 98.7%. CONCLUSION: POD 2 CT-scanner in high-risk severely obese patients undergoing LSG is an excellent tool for early diagnosis of intraabdominal hemorrhage, but sensitivity remains low for staple-line leak detection. Close postoperative clinical follow-up of these patients is essential and any suspicion of postoperative surgical complication should motivate the performance of a CT-scanner.

Spontaneous pneumomediastinum and COVID-19 pneumonia: Report of three cases with emphasis on CT imaging.

Spontaneous pneumomediastinum is a rare complication of coronavirus disease 2019. The published literature consists mainly of case reports and small case series. There are still many questions regarding the pathogenesis, the prognostic significance and the implications on patient management. In our hospital, 3 coronavirus disease 2019 patients developed spontaneous pneumomediastinum: 1 on admission at the emergency department and the other 2 during hospitalization. In this study we describe their clinical course and computed tomography (CT) findings. All of them had severe disease according to the total severity score on admission CT. The management of pneumomediastinum was conservative and follow-up CT showed resolution in all patients. As the correlation between extension of parenchymal lung lesions and development of pneumomediastinum is still under investigation, we highlight the importance of reporting the severity score on chest CT in order to obtain more comparable results between different studies. Furthermore, in this tragic circumstance we also had the opportunity to familiarize ourselves with the otherwise uncommon occurrence of air along the bronchovascular sheaths (Macklin effect) and evaluate the ability of CT to detect it.

Hypoparathyroidism: is it that easy to treat?

Hypoparathyroidism is a relatively rare endocrine disease characterised by either null or inappropriately low secretion of parathyroid hormone (PTH) for serum calcium levels. The other main laboratory findings include hypocalcaemia, inappropriately normal or high urine calcium excretion and hyperphosphataemia with low urine phosphate excretion. The management of hypoparathyroidism should be tailored to each individual case, which makes it a demanding undertaking in everyday clinical practice. In this review, we sought to focus on the diagnostic approach of hypoparathyroidism and the therapeutic challenges of the disease from a clinical perspective. Conventional treatment with vitamin D analogues and calcium salts is no longer the only available treatment, since replacement treatment with PTH(1-84) has recently been approved for the disease. However, the optimal treatment schedule is yet to be defined.

Asymptomatic migration of ventral mesh for incisional hernia into the small intestine: A case report.

Even though mesh migration is a rare complication, it must be considered in the differential diagnosis when investigating abdominal pain and digestive complications in patients with history of abdominal operations.

Abdominal pain caused by a giant liver hydatid cyst and the management of the residual cavity in a tertiary public hospital in Greece.

Diagnosis and treatment of liver hydatid cysts may be challenging. Many surgical techniques have been proposed for the treatment of liver hydatid cysts, but the problem of the residual cavity still remains controversial and challenging, especially in giant liver hydatid cysts which are rare entities that have not been widely described in the literature so far. Capitonnage, external tube drainage and omentoplasty are the most commonly used techniques. Herein, we report the case of a 70-year-old man with a mild upper quadrant pain that proved to have a giant liver hydatid cyst, 21*14 cm2, occupying the entire right lobe of the liver. We describe a successful surgical approach with cyst unroofing and careful evacuation of the multiple daughter cysts by aspiration, and the effective management of the residual cavity by the combination of all three aforementioned techniques.

Giant colonic diverticulum-a rare cause of acute abdomen.

Giant colonic diverticulum (GCD), defined as diverticulum larger than 4 cm, is a rare entity. It is generally a manifestation of colonic diverticular disease and can have dramatic complications such as perforation, abscess, volvulus, infarction and adenocarcinoma. This report documents the case of a 63-year-old man coming to the Emergency Department with acute abdomen due to a perforation of a GCD. In the plain abdominal X-ray the 'Balloon-sign' was revealed, computed tomography scan and Hartmann's procedure were performed. Acute abdomen can occur as a manifestation of a complication of a GCD, and this report highlights the fact that GCD should be considered for patients with a high risk of diverticular disease and abdominal pain.

Laparoscopic Resection of Desmoid Type Fibromatosis of the Splenic Hilum Mimicking a Greater Curvature Gastric GIST.

Desmoid tumor, recently renamed as desmoid type fibromatosis, is an uncommon neoplasm. The mesentery is the usual origin of intra-abdominal desmoid-type fibromatosis and despite the fact there is no metastatic potential, it can infiltrate adjacent organs. There etiology remains unknown, preoperative diagnosis is difficult and differential diagnosis includes among other gastrointestinal stromal tumor (GIST). In resectable tumors, the mainstay of treatment is surgical resection with at least clean microscopic margins. We present a case of a 51-year lady who presented with a 6.2 x 4.5 x 3.3 cm neoplasm in the anatomic area of the greater gastric curvature, the splenic hilum and the tail of the pancreas that was diagnosed as GIST on the CT scan. The patient was submitted to laparoscopic excision of the tumor and histopathological examination revealed desmoid type fibromatosis of the splenic hilum infiltrating the spleen, pancreatic tale and greater gastric curvature. According to the authors' knowledge, this is the first reported case of a sporadic splenic desmoid tumor, which has been treated successfully by laparoscopic en block resection.