Mcwilliamsschroeder3913

Off-label drug use refers to drug use beyond the specifications authorized for marketing [J Med Case Rep. 2014 Dec;8(1)303]. Eltrombopag is a thrombopoietin receptor agonist that has been used in treating thrombocytopenia due to chronic liver disease (CLD) as an off-label medication. Treatment of thrombocytopenia in patients with CLD constitute a real dilemma as the options are limited and some of them are invasive. However, thrombopoietin receptor agonist has been increasingly used for this purpose. Here we report a 34-year-old woman who has been diagnosed with CLD due to autoimmune hepatitis 20 years ago. Her condition was complicated with portal vein thrombosis, chronic thrombocytopenia, and variceal hemorrhage, and she has been listed as a candidate for liver transplantation. Given her high risk of bleeding, we started her on low dose of eltrombopag (25 mg daily) in order to maintain a platelet level of ≥50 × 10/µL. However, 1 year after initiation of the therapy, she developed left lower limb deep vein thrombosis.We present a very rare case of pure erythroid leukemia arising in a young patient with sickle cell disease being treated with hydroxyurea for almost 5 years. Diagnosing and managing this rare condition has been a challenge and the majority of patients with pure erythroid leukemia have a very poor prognosis with survival in months despite treatment. This form of leukemia could be therapy related and in our case, hydroxyurea may have been responsible for the development of this aggressive condition.Castleman disease is a rare cause of retroperitoneal mass in children. Clinical presentation and laboratory findings are usually nonspecific. Imaging shows features of a hypervascular or soft tissue mass. We present a 12-year-old boy who complained of frequent colds, fatigue, and failure to gain weight for the past 4 years who was referred to our hospital. Anemia and hypergammaglobulinemia were revealed in laboratory tests. Imaging showed a well-delineated retroperitoneal soft tissue mass with intense homogenous enhancement at the midline below the aortic bifurcation. The histopathological features were consistent with mixed type unicentric Castleman disease. Surgical removal was curative. Our patient's hematological abnormalities resolved, and he gained 10 kg in the next 4 weeks after the operation and reached the 25-50th percentile for his age.An 80-year-old Japanese male was treated with chemotherapy consisting of cyclophosphamide, doxorubicin, vincristine, and prednisolone, for non-Hodgkin lymphoma. Nine months after the chemotherapy, he was diagnosed with acute myeloid leukemia (AML) (M4) with translocation 8p11 and 22q13. The patient bone marrow indicated a remarkable degree of sea-blue histiocytosis. His disease was aggressive, and he died of the disease. Sea-blue histiocytes are macrophages harboring blue vacuoles and granular deposition, which results from the phagocytosis of dead cells and the subsequent deposition of phospholipids. AML with the t(8; 22) (p11; q13) translocation is a rare subtype of AML, which is a rare translocation with a prevalence of less than 1.0% among all AML cases. EGFR inhibitor The oncogenesis of t(8; 22) (p11; q13) is caused by the fusion protein monocytic leukemia zinc finger protein (MOZ) and transcription factor p300. MOZ can be fused to various translocation targets including CBT, TIF2, and p300, corresponding to t(8; 16), inv(8), and t(8; 22), respectively. This subgroup of AML reveals the hallmarks of the disease, including monocytic arrest and erythro/hemophagocytosis by blasts. A substantial proportion of the AML M4/M5 subtype harboring MOZ as an aberrant fusion gene represents erythrophagocytosis. Although rare, t(8; 22) is very specific to the AML M4/M5 subtype and seems to represent sea-blue histiocytosis as one of the characteristic features of monocytic AML with macrophage activation. Thus, sea-blue histiocytes are considered to be one of hallmarks in monocytic AML with MOZ translocation.Pulmonary tumor thrombotic microangiopathy (PTTM) is characterized by tumor cell microemboli with occlusive fibrointimal remodeling in small pulmonary vessels. Platelet-derived growth factor (PDGF) has been implicated in the development of PTTM, and fibroblast growth factor (FGF) promotes PDGF signaling via PDGF receptor β. We here describe a cancer patient who presented with dyspnea of uncertain etiology and whose condition worsened rapidly. A 68-year-old man with hypopharyngeal squamous cell carcinoma (cT4aN2bM0, stage IVA) was treated with surgery followed by radiation. Two years later, a lung metastatic lesion was surgically removed on the basis of suspected primary lung cancer. The patient was thereafter monitored without chemotherapy. Two months later, he had third-degree burns and received conservative therapy including debridement and application of trafermin (FGF2) spray. Two weeks later, he was hospitalized with complaints of fever and dyspnea. Pneumonia and pulmonary embolism were ruled out by chest computed tomography with pulmonary arterio-graphy, whereas intravascular lymphoma was excluded by laboratory testing. Malignant cells were detected in his peripheral blood on hospital day 8, and their number increased gradually thereafter. His respiratory symptoms worsened, and the patient died on hospital day 10. We concluded that the cause of death was PTTM, with the clinical course suggesting a possible relation to trafermin. This suggestion was supported by the detection of FGF receptor 2 overexpression in the primary tumor by immunostaining.The survival of patients with head and neck squamous cancer with locoregional recurrence is short if salvage surgery or radiation cannot be performed. Systemic chemotherapy based on platinum salts and cetuximab produces only partial and transient responses. Immune checkpoint inhibitors (i.e., nivolumab) lead to a low complete response rate of only about 10%, but in some cases the effects can be long-lasting. Intratumoral chemotherapy (ITC) has been proposed for patients with local recurrence of head and neck squamous cell carcinoma with an objective response rate of 27-50%. However, it often leads to peritumoral tissue necrosis, and the duration of local control is limited. Here, we present 2 patients with head and neck squamous cell cancer whose local recurrences were refractory to intravenous chemotherapy and nivolumab. ITC using nonnecrotizing molecules, associated with nivolumab, led to complete stable local and distant response. ITC seems to trigger tumor resensitization to previously ineffective immunotherapy. This combination deserves an evaluation in the framework of a prospective trial.We here report on 2 cases of monostotic Paget's disease of bone, one in the ilium and the other in the skull, including quantitative values obtained with bone single photon emission computed tomography/computed tomography (SPECT/CT), which were useful to evaluate the response to bisphosphonate treatment. The quantitative parameters determined with those findings were decreased, with the maximum standardized uptake value (SUV), peak SUV, mean SUV, metabolic bone volume, and total bone uptake in case 1 and case 2 reduced by 48.8 and 60.3%, 46.6 and 58.8%, 24.3 and 60.5%, 87.0 and 11.8%, and 90.2 and 55.8%, respectively, while TRACP-5B and alkaline phosphatase (ALP) were also reduced by 39.5 and 88.6% and by 53.7 and 78.1%, respectively. Quantitative SPECT/CT parameter decreases were correlated with TRACP-5B and ALP, indicating the usefulness of this modality to examine treatment response.Small cell tumors arise from the neuroendocrine cell system and they are most frequently found in the lung (SCLC). Small cell tumor could occasionally arise in other body sites, such as the cervix, prostate, gastrointestinal tract, and very rarely from other sites. Metastatic SCLC patients present with metastatic disease in 80% of cases, and the metastases typically are reported in brain, liver, lung, and bone; they rarely could be found in the ovary. Differently, primitive small cell carcinoma of the ovary of pulmonary type is a rare and highly aggressive tumor arising from the ovarian cells; no suitable treatment strategy has been established yet. In this paper, we talk about a 72-year-old woman who presented with abdominal bleeding and a large mass in her pelvic region. A primary ovarian carcinoma was suspected, and she underwent hysterectomy with laparoscopic surgery and bilateral oophorectomy, lymph node resection, omentectomy, complementary appendix and sigmoid resection. The postoperative pathologic diagnosis was a differential diagnosis between small cell ovarian carcinoma of the pulmonary type and metastasis of SCLC.Subtotal and extended left colectomies with ileocecal junction preservation represent preferable alternatives in cases of massive involvement of the colon in the pathological process. However, these approaches might be challenging in terms of reconstructive steps. Antiperistaltic cecorectal anastomosis is one of the possible techniques. Still, this type of pouch formation is described mostly in slow-transit constipation surgical management. We report on a patient with synchronous colorectal cancer who underwent extended left colectomy. In the case of compromised vessel anatomy, it was decided to perform antiperistaltic cecorectal anastomosis. We present all clinical and intraoperative patient's data, determining the surgical tactics, and short-term postoperative results. An antiperistaltic cecorectal anastomosis can be considered in nonstandard clinical cases and variable anatomy of the patient.Uterine tumor resembling ovarian sex cord tumor (UTROSCT) is a rare indolent stromal neoplasm of unclear histogenesis with a distinct histopathological entity. Immunophenotypes of sex cord positivity are the most significant information to confirm the diagnosis. We present the case of a 61-year-old female with a polypoid mass in the uterus which was successfully removed by surgical resection as hysterectomy. The pathological diagnosis was UTROSCT, which was characterized microscopically by sex cord images and immunohistochemical features of calretinin, CD99, and WT1 positivity.Herein, we present the case of a patient who suffered from adult T-cell leukemia/lymphoma (ATLL) and hepatocellular carcinoma (HCC) after obtaining a sustained virological response following treatment with a direct-acting antiviral (DAA) at different points in time. The patient went into complete remission (CR) for ATLL. Unfortunately, subsequent relapse of ATLL was observed. This situation was overcome using chemotherapy with pegylated interferon alpha-2b. Human T lymphotropic virus type 1 Tax-specific cytotoxic T lymphocytes (CTLs) were recognized after obtaining second CR, and those CTLs have been maintained for many years. After 4 years from the second CR, chronic hepatitis type C was treated with a DAA, and sustained virological response was attained. However, the occurrence of HCC was detected. Surprisingly, the tumor disappeared spontaneously. Hepatitis virus type C-specific CTLs were also detected in the patient. T-cell receptor (TCR) V beta gene repertoire analyses revealed oligoclonal expansion of effector and memory CTLs. The number of CTLs expressing the TCR V beta 13.1 has increased over the years since HCC occurrence. The activation and maintenance of anticancer cellular immunity may have allowed the patient to obtain long-term survival and overcome two lethal neoplasms.