Successful Outcome in Patients with Fanconi Anemia Undergoing T Cell-Replete Mismatched Related Donor Hematopoietic Cell Transplantation Using Reduced-Dose Cyclophosphamide Post-Transplantation.

Allogeneic hematopoietic cell transplantation (HCT) has been shown to restore normal hematopoiesis in patients with Fanconi anemia (FA), with excellent results in matched related donor HCT. Outcomes of alternative donor HCT are less favorable, however. In patients without FA, several reports have documented stable engraftment and/or a low risk of graft-versus-host disease (GVHD) using unmanipulated HLA-mismatched related donors and post-HCT cyclophosphamide (PT-CY) for GVHD prophylaxis. Data on the use of this approach in patients with FA are scarce, and thus we launched a study of HLA-mismatched related donor HCT in these patient. Here we report our findings in 19 patients. The conditioning was fludarabine 30 mg/m2/day for 5 days, antithymocyte globulin 5 mg/kg/day for 4 days, and total body irradiation (total dose, 200 cGy). GVHD prophylaxis was cyclosporine and mycophenolate and reduced doses of PT-CY, 25 mg/kg, on days +3 and +5. All patients exhibited absolute neutrophil count recovery. Grade III-IV acute GVHD occurred in 3 patients, and chronic GVHD occurred in 1 patient. At a mean follow-up of 38.3 ± 5.8 months, the 5-year probability of overall survival for our patients was 89.2% ± 7.2%. The regimen was well tolerated; hemorrhagic cystitis occurred in 7 patients, and severe mucositis occurred in 5 patients. There were 2 deaths; the primary cause of death was severe GVHD in 1 patient and leukemia recurrence in the other. We conclude that in patients with FA lacking a matched related donor, the use of mismatched related HCT with low-dose PT-CY is a viable option; it is well tolerated, with a high rate of engraftment and an acceptable incidence of GVHD.

Esophageal Schwannoma: An Important Differential Diagnosis for Esophageal Subepithelial Lesions.

Esophageal schwannoma is a rare tumor and is classified as one of the esophageal subepithelial lesions (SELs). Endoscopic ultrasound (EUS) evaluation is the gold standard for evaluating subepithelial lesions. Differentiation through EUS-guided fine needle aspiration is sometimes important to exclude lesions with malignant potential. Immunohistochemistry differentiates schwannoma from other subepithelial lesions. Strong and diffuse positivity for S100 is characteristic. The decision for conservative management versus endoscopic or thoracoscopic intervention is made based on the tumor size, location, and symptoms.

A Rare Case of Bacteremic Community-Acquired Pneumonia Due to Pasteurella Multocida Presenting With Hemoptysis.

Pasteurella multocida (PM) is a gram-negative bacterium known to cause soft tissue infections, especially after animal bites, with some human infections occurring after animal exposure, usually via inhalation of contaminated secretions. PM pneumonia mainly affects those who are immunocompromised and in individuals with comorbidities. The spectrum of pulmonary disease due to PM is wide, ranging from pneumonia to empyema. The clinical features are indistinguishable from other pathogens, however, hemoptysis seldom occurs as a consequence of PM infection. We present a case of PM pneumonia in an immunocompetent host who had a chief complaint of hemoptysis, making this the sixth documented case to ever-present with hemoptysis.

A Case of Esophageal Plasmablastic Lymphoma With a Literature Review.

Plasmablastic lymphoma is a rare B-cell lymphoma that is mainly associated with immunocompromised patients, such as those affected with human-immunodeficiency virus (HIV) or those who have received solid organ transplants; however, it has also been documented in immunocompetent patients. Moreover, there is also a strong association with Epstein-Barr virus (EBV). Although mainly found in the oral cavity, a few cases were documented to affect the gastrointestinal tract, and of those, only three cases were found to involve the esophagus, and we present the fourth case in a 48-year-old HIV-positive male.

Primary Combined Hepatocellular-Cholangiocarcinoma: A Case of Underdiagnosed Primary Liver Cancer.

Combined hepatocellular-cholangiocarcinoma (CHC) is a rare primary tumor of the liver. Histologically, it comprises components of both hepatocellular carcinoma (HCC) and cholangiocarcinoma (CC) but is associated with a worse prognosis. International guidelines regarding its management are scarce, with surgical management (major hepatectomy) being the treatment of choice. In this report, we present a challenging case of a 73-year-old male with primary CHC who was not a surgical candidate but underwent hepatic artery radioembolization instead.

An Early Presentation of Buried Bumper Syndrome.

Percutaneous endoscopic gastrostomy (PEG) is a well-established and successful method of nutritional delivery. Complications, although rare, are divided into early or late. Buried bumper syndrome (BBS) is usually a late complication of PEG tube insertion and can cause many issues such as pressure necrosis, peritonitis, and septic shock. Endoscopic evaluation is the definitive diagnosis, and treatment depends on each patient and the degree of depth of disc migration. We present to you a case of buried bumper syndrome in a 66-year-old female that was initially thought to be complicated with peritonitis, and surprisingly occurring only one week after initial PEG tube placement.

Primary Leiomyosarcoma of the Mesentery: A Case Report With Review of Literature.

Mesenteric leiomyosarcoma (LMS) is a rare gastrointestinal mesenchymal tumor. It was often misdiagnosed as a gastrointestinal stromal tumor (GIST) until the introduction of immunohistochemistry staining (IHC) in 1998. Currently, a positive IHC staining for smooth muscle markers represents the main diagnostic modality. Herein, we present a case of Mesenteric LMS in a 68-year-old woman, who presented with nausea, vomiting, and abdominal pain and was found to have a right-sided mesenteric tumor encasing the right ureter, with right hydroureteronephrosis. The patient underwent surgical removal of the tumor and IHC stains were compatible with LMS. She achieved remission until she developed recurrence 12 months after initial diagnosis and subsequently expired due to postoperative complications when re-resection was attempted. Typically, mesenteric LMS carries a poor prognosis with a propensity for hematogenous metastasis. In the absence of a standardized protocol for therapy, early surgical resection is the only known curative modality but with a high risk of recurrence.