A Case of Long-term Survival of 36 Months in the Setting of Extensive-disease Small-cell Lung Cancer.

Small-cell lung cancer (SCLC) is an extremely aggressive disease characterized by early regional spread and distant metastases. Patients with extensive-disease (ED) SCLC have a median survival rate of 8-11 months. Despite high response rates to initial therapy, relapses are frequent. Systemic therapy after the first-line failure remains vital in the treatment paradigm of SCLC. The National Comprehensive Cancer Network (NCCN) guidelines dictate that previously administered first-line chemotherapy can be used in relapses that occur after six months from the completion of initial therapy. For relapses within six months of initial therapy, sequential treatment with single agents is recommended. In this report, we discuss the case of a long-term SCLC survivor with an ED. The patient underwent several lines of chemotherapy and prophylactic cranial irradiation (PCI) and survived for 36 months.

B-cell Prolymphocytic Leukemia: Case Report and Challenges on a Diagnostic and Therapeutic Forefront.

B-cell prolymphocytic leukemia (B-PLL) is a rare malignancy of mature B-cells with characteristic morphologic, immunophenotypic, cytogenetic, and molecular features characterized by late onset (median age 69 years), an aggressive clinical course, refractoriness to chemotherapy, and median survival of around three years. Treatment is influenced by the presence or absence of specific high-risk genetic mutations like 17P/TP53 deletion, the presence of which translates into poor prognosis. Patients without 17P deletion, who are <70 years, without significant co-morbidities, are initially treated with a combination chemotherapy regimen used for chronic lymphocytic leukemia (CLL) such as fludarabine, cyclophosphamide, and rituximab. On the other hand, patients with a 17P deletion, age >70 years, with multiple co-morbidities, receive ibrutinib or alemtuzumab as the initial therapy. Relapsed or refractory cases are managed with BCL-2 signaling inhibitors like venetoclax. We discuss the case of an 84-year-old male with B-PLL (positive TP53 mutation), resistant to ibrutinib therapy, with extremely high white blood cell (WBC) counts, thus creating a dilemma regarding the best treatment in the second-line setting.

Cor Triatriatum Dexter as an Incidental Finding: Role of Two-Dimensional Transthoracic Echocardiography.

Cor triatriatum dexter (CTD) is a rare congenital cardiac anomaly in which a membranous structure divides the right atrium (RA) into two chambers. Persistence of the right valve of the sinus venosus, which usually regresses as a part of normal embryological development, is responsible for membranous partition. There is a high incidence of right-sided congenital abnormalities of the heart associated with this condition. Clinical manifestations vary depending on the degree of partitioning or septation of the RA. We present a case of CTD discovered as an incidental finding during transthoracic echocardiography and further discuss the role of two-dimensional echocardiography as a noninvasive diagnostic tool.

Hemophagocytic Lymphohistiocytosis: Management and Special Consideration in Human Immunodeficiency Virus Positive Patients with Immune Reconstitution Syndrome.

The human body is capable of reacting to multiple aggressors by developing an inflammatory response with the secretion of inflammatory cytokines. The worrisome clinical manifestations occur when this inflammatory response is disproportionate. Hemophagocytic lymphohistiocytosis (HLH) is a rare and severe condition characterized by an overwhelming inflammatory response that may result in end-organ damage and might be fatal. Correspondingly, immune reconstitution inflammatory syndrome (IRIS) is another well-known disorder, seen commonly in human immunodeficiency virus (HIV)-infected patients after the commencement of highly active antiretroviral therapy (HAART). Both entities share a similar clinical presentation and a dismal prognosis. Due to widespread clinical manifestations and laboratory abnormalities, diagnosis is often missed at the time of presentation. There is little consensus on the treatment of secondary HLH, which is usually handled on a case-by-case basis. Rapid curbing of the widespread inflammatory response is the main goal of treatment. To the best of our knowledge, there is scarce literature available on the coexistence of HLH and IRIS; therefore, medical management in the co-occurrence of these two conditions needs to be further investigated.

CD-20 Negative Plasmablastic Lymphoma Lurking in the Shadow of a Leiomyoma - Diagnosis and Management.

CD20-negative diffuse large B-cell lymphoma (DLBCL) is a rare entity and constitutes 1-2% of all DLBCLs. Major subtypes include plasmablastic lymphomas (PBLs), primary effusion lymphomas, anaplastic kinase positive large B-cell lymphomas, and large B-cell lymphomas arising in human herpesvirus 8 (HHV8)-associated multicentric Castleman disease. Amongst the known subtypes, PBL is the most common and presents as an aggressive extranodal disease with high resistance to routine chemotherapy regimens, thereby posing a therapeutic challenge. Though more commonly seen in HIV-positive patients, PBL cases have also been reported in HIV negative patients. We report a unique case of PBL with pelvic organ involvement in an HIV/Epstein-Barr virus-negative patient. The neoplastic cells were found to be positive for CD79a, MUM1, BCL6, and PAX5, with a Ki-67 proliferation index of 92%. Our case met the criteria for the plasmablastic variant, and remission was obtained with etoposide, vincristine, and doxorubicin with bolus doses of cyclophosphamide and oral prednisone (EPOCH) therapy. This case report aims to highlight the challenges related to the diagnosis and treatment of CD20-negative DLBCL, with special emphasis on the PBL subtype and to provide an insight into some of the upcoming, less conventional treatment modalities.