A prognostic scoring model for survival after locoregional therapy in de novo stage IV breast cancer.

BACKGROUND: The role of locoregional treatment (LRT) remains controversial in de novo stage IV breast cancer (BC). We sought to analyze the role of LRT and prognostic factors of overall survival (OS) in de novo stage IV BC patients treated with LRT utilizing the National Cancer Data Base (NCDB). The objective of the current study is to create and internally validate a prognostic scoring model to predict the long-term OS for de novo stage IV BC patients treated with LRT. METHODS: We included de novo stage IV BC patients reported to NCDB between 2004 and 2015. Patients were divided into LRT and no-LRT subsets. We randomized LRT subset to training and validation cohorts. In the training cohort, a seventeen-point prognostic scoring system was developed based on the hazard ratios calculated using Cox-proportional method. We stratified both training and validation cohorts into two "groups" [group 1 (0-7 points) and group 2 (7-17 points)]. Kaplan-Meier method and log-rank test were used to compare OS between the two groups. Our prognostic score was validated internally by comparing the OS between the respective groups in both the training and validation cohorts. RESULTS: Among 67,978 patients, LRT subset (21,200) had better median OS as compared to that of no-LRT (45 vs. 24 months; p < 0.0001). The group 1 and group 2 in the training cohort showed a significant difference in the 3-year OS (p < 0.0001) (68 vs. 26%). On internal validation, comparable OS was seen between the respective groups in each cohort (p = 0.77). CONCLUSIONS: Our prognostic scoring system will help oncologists to predict the prognosis in de novo stage IV BC patients treated with LRT. Although firm treatment-related conclusions cannot be made due to the retrospective nature of the study, LRT appears to be associated with a better OS in specific subgroups.

Primary clear cell sarcoma in the lung apex with spinal metastasis: a fatal outcome.

Clear cell sarcoma (CCS) is a rare tumour that manifests in tendons and aponeuroses of the distal extremities and comprises roughly 1% of all soft tissue cancers. Only two cases of a primary CCS arising in the lung have been reported in the literature, and neither were located in the lung apex. We report a case of a primary CCS located in the lung apex that originally presented as ptosis, as well as arm pain and weakness. Distant metastasis was discovered on diagnosis, which led to irreversible paraplegia and death of the patient.

Upper Limb Phlegmasia Cerulea Dolens Secondary to Heparin-induced Thrombocytopenia Leading to Gangrene.

We present a case of a dialysis-dependent end-stage renal disease patient who originally presented with sepsis and later developed heparin-induced thrombocytopenia-related upper extremity deep venous thrombosis that rapidly progressed to phlegmasia. Argatroban, a direct thrombin inhibitor, was initiated without delay. Argatroban restored the venous patency completely but did not reverse his two gangrenous fingers. The patient finally underwent digital amputation. The management of this uncommon, but life-threatening, situation of upper limb phlegmasia cerulea dolens secondary to heparin-induced thrombocytopenia leading to gangrene is discussed in this case report.

Priapism in a patient with sickle cell trait using marijuana.

A 22-year-old man with a history of multiple episodes of priapism presented to the emergency room with an erection lasting more than 48 h after conservative management failed at home. He had no known family history of sickle cell disease or trait. He was haemodynamically stable. Physical examination revealed an enlarged, tender penis. Laboratory data revealed a positive sickle solubility test. Haemoglobin electrophoresis revealed sickle cell trait and urine drug screen was positive for cannabinoids. Initial management was attempted with intracavernosal phenylephrine without any success. The patient underwent a limited El-Ghorab procedure on the right corpora cavernosa but the priapism did not resolve adequately. Two days later, the patient had to undergo a bilateral El-Ghorab procedure and achieved complete resolution of the priapism.

Acute necrotising pancreatitis: a late and fatal complication of pancreaticoduodenal arterial embolisation.

A 70-year-old man was diagnosed with a massive bleeding duodenal ulcer which was refractory to emergency endoscopic management. Angiogram of the coeliac and superior mesenteric arteries revealed bleeding from the superior and inferior pancreaticoduodenal arteries. Transcatheter arterial embolisation of superior and inferior pancreaticoduodenal arteries along with the gastroduodenal artery was performed. Two weeks later he developed severe necrotising pancreatitis of the pancreatic head probably due to ischaemia, which was managed conservatively. Three months later the patient experienced another episode of pancreatitis which progressed into multiorgan dysfunction and the patient passed away.

Benign and Malignant Hematological Manifestations of Chronic Hepatitis C Virus Infection.

Chronic hepatitis C virus (HCV) infection, that affects 3% of world's population, is associated with several hematological manifestations mainly benign cytopenias, coagulopathy and lymphoproliferative diseases. Immune or non-immune-mediated thrombocytopenia is a major challenge in chronic HCV infected patients especially in the setting of an advanced liver disease, with average prevalence of nearly 24%. Although several treatment modalities such as steroids, intravenous immunoglobulin, splenectomy and immunosuppresants have been tried with some success, their efficacy is not impressive and can result in an increase in viral load or other thrombotic complications. Even though a recent phase 2 study has shown promising role of a platelet growth factor, eltrombopag, in boosting platelets counts prior to antiviral treatment, its use in pre-operative setting had unexpected complications. Unlike thrombocytopenia, anemia and neutropenia are more frequently seen in treated patients and are often the result of antiviral therapy. HCV infection also pre-disposes to lymphoproliferative diseases, mainly non-Hodkings lymphomas, likely as a result of chronic antigenic stimulation and mutation of several genes involved in carcinogenesis. Understanding of the role of HCV infection in these conditions has therapeutic implications. Whereas antiviral therapy has shown therapeutic role in HCV-associated indolent lymphomas, monitoring of hepatic function and viral load is important in the management of diffuse large B-cell lymphoma in HCV-infected patients. Although our knowledge about the HCV infection and hematological manifestations has substantially grown in last few decades, further studies are important to advance our therapeutic approach.

Therapy-related myeloid neoplasms after autologous hematopoietic stem cell transplantation in lymphoma patients.

Lymphoma patients treated with autologous transplantation (ASCT) live an increasingly long life with the recent advancement in therapeutic modalities. This has resulted in an increase in the incidence of therapy-related myeloid neoplasms (t-MN), which is one of the leading causes of non-relapse mortality. Several observational studies have linked the development of t-MN after ASCT with the intensity and frequency of chemotherapy, particularly alkylating agents, use of total body irradiation (TBI), and peripheral blood progenitor cells. In addition, role of genetic factors is increasingly being identified. It is postulated that the use of chemotherapy prior to ASCT results in DNA damage of progenitor cells, mitochondrial dysfunction, and altered gene expression related to DNA repair, metabolism as well as hematopoietic regulation. Cytogenetic studies have shown the presence of abnormalities in the peripheral blood progenitor cells prior to ASCT. It is, therefore, likely that the reinfusion of peripheral blood progenitor cells, proliferative stress on infused progenitor cells during hematopoietic regeneration and associated telomere shortening ultimately result in clonal hematopoiesis and blastic transformation. Cytopenias, myelodysplasia, or cytogenetic abnormalities are common and can be transient after ASCT; therefore, only when present together, they do confirm the diagnosis of t-MN. Attempts to reduce the occurrence of t-MN should be directed toward minimizing the exposure to the identified risk factors. Although the median survival is few months to less than a year, studies have shown the promising role of allogeneic transplantation in select young t-MN patients without high-risk cytogenetics. In this review we will explain the recent findings in the field of t-MN in lymphoma patients that have implications for identifying the molecular and genetic mechanisms of leukemogenesis and discuss potential strategies to reduce the risk of t-MN in this patient population.