A Unique Case of a Patient With Pancreatic Cancer Developing Leptomeningeal Metastases While on Gemcitabine and Nab-Paclitaxel.

Brain metastases and leptomeningeal disease are rare with pancreatic cancer. Leptomeningeal disease is a catastrophic complication to have as patients deteriorate rapidly. Patients can present with symptoms of cranial nerve neuropathies, headache, nausea, and focal neurological deficits. We present a patient with metastatic pancreatic cancer who was treated initially with FOLFIRINOX (5-fluorouracil, leucovorin, irinotecan, and oxaliplatin) which resulted in marked clinical and radiologic improvement. However, he started to develop severe peripheral neuropathy and was switched to maintenance gemcitabine and nab-paclitaxel. On this regimen, his systemic disease was well controlled but he developed leptomeningeal carcinomatosis. To our knowledge, this is the first case of leptomeningeal metastases developing in a patient with pancreatic adenocarcinoma while on treatment with gemcitabine and nab-paclitaxel after cessation of FOLFIRINOX. We should maintain high clinical suspicion for leptomeningeal disease in pancreatic cancer, especially when systemic disease is well controlled, as the chemotherapeutic agents may not be crossing the blood-brain barrier effectively contributing to high morbidity and mortality.

Co-occurrence of High-Risk Myelodysplastic Syndrome With a Complex Karyotype/TP53 Mutation and IgG Lambda Monoclonal Gammopathy of Undetermined Significance.

Myelodysplastic syndrome (MDS) represents a group of hematologic disorders marked by abnormal cellular development in the bone marrow, while monoclonal gammopathy of undetermined significance (MGUS) involves abnormal plasma cells without symptomatic manifestations. This paper presents a compelling case of a 74-year-old Hispanic female diagnosed with a rare combination of high-risk MDS characterized by a complex karyotype and TP53 mutation, alongside IgG lambda MGUS. The patient's clinical presentation included a spectrum of symptoms such as body aches, rash, fever, dyspnea, and bloody watery diarrhea. Initial diagnostic evaluations yielded no significant findings, but subsequent investigations revealed abnormalities in both bone marrow and peripheral blood, indicative of coexisting MDS and MGUS. Chromosomal analysis further confirmed the presence of a complex karyotype with multiple aberrations, notably including 5q deletion. This case underscores the rarity of simultaneous high-risk MDS and MGUS, particularly with the additional complexity of a TP53 mutation and complex karyotype. It underscores the imperative for continued research efforts to elucidate the underlying mechanisms and optimal management strategies for such intricate cases. Moreover, it highlights the therapeutic challenges posed by concurrent MDS and plasma cell disorders, advocating for more aggressive interventions such as stem cell transplantation as potential avenues for improved patient outcomes.

A Nearly Missed Case of Mitral Bioprosthetic Paravalvular Leak (PVL)-Related Waring Blender Syndrome Treated With Transcatheter Mitral PVL Closure.

A 72-year-old woman with recently diagnosed non-small cell lung cancer, who underwent cardiac bypass and bioprosthetic mitral valve replacement presented to our cancer center with lightheadedness, severe fatigue, and shortness of breath. Initial blood tests showed mild hemolytic anemia. The patient also complained of occasional bright red bleeding per rectum. Esophagogastroduodenoscopy and colonoscopy did not reveal an acute source of bleeding. An initial transesophageal echocardiogram did not show significant valvular or paravalvular abnormalities. Meanwhile, the patient's hemolytic anemia worsened. She received eight units of packed red blood cell transfusions. Schematic workup for hemolytic anemia revealed negative Coomb's test, positive urine hemosiderin, normal ADAMTS13 activity, and absent splenomegaly. A relook of the patient's transesophageal echocardiogram (TEE) showed a small paravalvular leak of the bioprosthetic mitral valve. The patient was referred to a tertiary center, and repair of the perivalvular leak with glue resolved her hemolytic anemia, subsequently improving the lab values, symptoms, and quality of life. This case highlights the schematic workup of hemolytic anemia and also the importance of recognizing the association between hemolytic anemia and valvular abnormalities.

Skull Metastasis With Orbital Invasion as a Primary Manifestation of Hepatocellular Carcinoma.

Hepatocellular carcinoma (HCC) is a common worldwide cancer with a poor prognosis despite treatment advancements. Patients typically exhibit signs and symptoms pertaining to the liver. Extrahepatic metastasis of HCC is documented to be as low as 5% of cases. Bone metastasis ranks third following lungs and regional lymph nodes. The typical locations for bone metastasis include the vertebral column, pelvis, femora, and ribs, with skull metastasis, being reported in less than 1.6% of cases. Herein, we describe a case of HCC presenting with skull metastases and orbital invasion as the initial manifestation.

Exploring the impact of therapeutic advances in HIV-related mortality in the United States.

Objectives: Mortality from HIV has significantly declined with the introduction of highly active antiretroviral therapy (HAART). This study sought to examine the longitudinal trends in mortality from HIV-related diseases by race, sex, geographical region, and over time as HAART trends changed. Methods: We queried the Centers for Disease Control and Prevention's Wide-Ranging Online Data for Epidemiologic Research database and performed serial cross-sectional analyses of national death certificate data for all-cause mortality with comorbid HIV from 1999 to 2020. HIV diseases (International Classification of Diseases, Tenth Revision codes B20-B24, O98.7, R75) were listed as the contributing cause of death. We calculated the age-adjusted mortality ratio (AAMR) per 1,000,000 individuals and determined mortality trends using the Joinpoint Regression Program. Subgroup analyses were performed by sex, race, region, and organ system. The study period was further stratified into three groups when specific drug regimens were more prevalent. Results: In the 22-year study period, 251,759 all-cause mortalities with comorbid HIV were identified. The leading cause of death was infectious disease (84.0%, N = 211,438). Men recorded a higher AAMR than women (4.66 vs 1.65, P < 0.01). African American individuals had the highest AAMR (13.46) compared to White, American Indian, and Asian individuals (1.70 vs 1.65 vs 0.47). The South region had the highest AAMR (4.32) and urban areas had a higher AAMR compared to rural areas (1.77 vs 0.88). Conclusions: More than 80% of deaths occurred because of infectious disease over the last 2 decades with a decreasing trend over time when stratified by race, sex, and geographical region. Despite advances in HAART, mortality disparities persist which emphasizes the need for targeted interventions in these populations.

A Rare Case of Acute Myeloid Leukemia Initially Presenting With Fever of Unknown Origin and Rapidly Progressing Pericardial Effusion.

This case report highlights a patient who had persistent fevers for weeks and rapidly progressing pericardial effusion following a positive test for coronavirus disease 2019 (COVID-19) two weeks before presentation to the hospital. The initial thought was that her fever was of infectious etiology, but relevant investigations led to the diagnosis of acute myeloid leukemia (AML). AML, which is characterized by clonal expansion of immature "blast cells" in the peripheral blood and bone marrow resulting in ineffective erythropoiesis and bone marrow failure, is the most prevalent form of leukemia. It is the most aggressive form of leukemia, which has varying prognoses determined by the subtypes. This report explores the association between AML, fever of unknown origin, and pericardial effusion, shedding light on a notable clinical aspect. Fever in AML may be attributed to underlying inflammatory processes, cytokine dysregulation, or bone marrow failure. Recognition of fever as a potential indicator of AML contributes to enhanced clinical vigilance. Pericardial effusions and cardiac tamponade, although rare, can be a presenting feature of AML, and can present side by side with fever of unknown origin as seen in this case report.

Long-Lasting SARS-CoV-2 Infection With Post-COVID-19 Chronic Interstitial Pneumonia in a Patient With Chronic Lymphocytic Leukemia Treated Successfully With Intravenous Immunoglobulin.

Post-coronavirus disease 2019 (post-COVID-19) condition is a post-acute syndrome characterized by non-specific symptoms that remain for at least two months and typically appear three months after the start of the acute phase. Individuals with chronic lymphocytic leukemia (CLL) are considered to be at high risk of contracting COVID-19. It is also becoming increasingly evident that the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccine response is frequently lacking or insufficient. We present a 77-year-old male patient with CLL who had multiple hospitalizations for the management of pneumonia related to persistent COVID-19 infection due to hypogammaglobulinemia. He was subsequently treated with intravenous immunoglobulin (IVIG). This case emphasizes the importance of the early detection of hypogammaglobulinemia in patients with CLL and long COVID because of the potential therapeutic benefit of IVIG therapy. We also provide a literature review on COVID-19 infection in CLL patients, focusing mainly on the subset population of patients with hypergammaglobulinemia.

Irritable bowel syndrome (IBS) among Lebanese adults: unidentified IBS and associated factors.

BACKGROUND: Irritable bowel syndrome (IBS) is one of the most frequent functional gastrointestinal disorders, but the condition is still underdiagnosed. The high of rate of unidentified IBS by patients can be related to different factors. The aim of this study is to assess the rate of unidentified IBS among Lebanese adults and investigate the role of socio-demographic factors, anxiety, depression, insomnia and eating attitudes on IBS diagnosis. METHODS: A cross-sectional study was conducted among Lebanese adults older than 18 years between June 2022 and December 2022, using a self-reporting questionnaire distributed via social media. RESULTS: A total of 425 participants was enrolled in the study with around 184 (46.8%) having a possible unidentified IBS. Higher psychological distress (aOR = 1.07) and insomnia severity (aOR = 1.08) were significantly associated with higher odds of having possible unidentified IBS whereas a higher household crowding index (aOR = 0.67) was significantly associated with lower odds of having possible IBS. The correlation of eating attitudes with cigarette smoking (aOR = 1.33; p = .025; 95% CI 1.04; 1.70) and insomnia severity with cigarette smoking (aOR = .89; p = .023; 95% CI .80; .98) were significantly associated with the presence of possible IBS. In nonsmokers, higher psychological distress (aOR = 1.07) and insomnia severity (aOR = 1.10) were significantly associated with higher odds of having possible IBS. In smokers, higher BMI (aOR = .78) was significantly associated with lower odds of having possible IBS, whereas higher eating attitudes scores (more inappropriate eating) (aOR = 1.40) were significantly associated with higher odds of having possible IBS. CONCLUSION: The study highlighted the implication of raising awareness about IBS among the Lebanese population to promote early diagnosis and minimize the rate of unidentified IBS by patients. Initiation of appropriate treatment plans, tailored symptomatic management approach, and diet programs should be highly encouraged.

BRAF Inhibitors in BRAF-Mutated Colorectal Cancer: A Systematic Review.

Colorectal cancer (CRC) is the second-leading cause of cancer-related deaths globally. BRAF mutation is present in about 10% of CRC patients and is associated with a poor response to chemotherapy. These patients have a relatively poor prognosis. This review aims to assess the efficacy and safety of BRAF inhibitors in BRAF-mutated CRC patients. A literature search was performed on PubMed and Embase, and clinical trials relevant to BRAF inhibitors in CRC were included. Data were extracted for efficacy and safety variables. Two randomized clinical trials (n = 765) and eight non-randomized trials (n = 281) were included based on inclusion criteria. In RCTs, an overall response was reported in 23% of the patients treated with BRAF inhibitor-based regimens compared to 2.5% with control regimens. The hazard ratio of overall survival was also significantly better with triplet encorafenib therapy at 0.52 (95% CI = 0.39-0.70). In single-arm trials, ORR was 17% and 34% in two-drug and three-drug regimens, respectively. BRAF inhibitor-based regimens were safe and effective in the treatment of BRAF-mutated CRC. Large-scale randomized trials are needed to find a suitable population for each regimen. PROSPERO registration No. CRD42023471627.

Epstein-Barr Virus-Positive Primary Diffuse Large B-cell Lymphoma of the Colon in a Patient With Human Immunodeficiency Virus.

Colorectal lymphomas of primary origin are rare neoplasms accounting for 3% of all lymphomas involving the gastrointestinal (GI) tract, and of that 3%, 0.1-0.5% involve the colorectal region. Among the types of non-Hodgkin's lymphomas involving the GI tract, diffuse large B-cell lymphoma (DLBCL) is the most common type. While extranodal involvement of DLBCL in the GI is common, DLBCL of the colon with Epstein-Barr virus positivity is a rare entity with only a few cases reported in the literature. Here, we present a rare case of a 53-year-old female with human immunodeficiency virus (HIV) who presented with generalized abdominal pain, weight loss, night sweats, and fevers. A computed tomography scan of the abdomen and pelvis showed a mass on the right side of the colon with associated retroperitoneal and right inguinal lymphadenopathy. She later underwent a colonoscopy with a biopsy. Histopathology showed DLBCL of the ascending colon and chemotherapy was initiated.

Lung Cancer Presenting as Upper Extremity Musculoskeletal Pain: A Case Report.

Adenocarcinoma is a tragically common iteration of lung cancer. Risk factors included primary or secondary exposure to tobacco smoke, family history of the disease, and occupationally related hazards, among others. Metastasis to various distant organs may present quite late and in unusual ways, providing a challenge to healthcare providers. A combination of imaging, biopsy, and histochemical analysis can be used to clinch the diagnosis and guide management. Effective treatment relies on a prompt diagnosis, from surgery to radiation and chemotherapy. Our case illustrates how an advanced metastatic lung cancer clinically manifested as something as seemingly benign as shoulder pain.

Hyperhemolytic Crisis Following Transfusion in Sickle Cell Disease With Acute Hepatic Crisis: A Case Report.

Hyperhemolysis syndrome (HHS) is a catastrophic unpredictable consequence of blood transfusion in sickle cell disease. It leads to further drop in hemoglobin via immune mechanisms complicating a hospital course and prolonging length of stay. Although sickle cell patients receive multiple transfusions throughout their disease course, this condition remains underreported by health care professionals or misinterpreted for other sickle cell crises. We present a similar case highlighting the importance of early recognition of HHS and judicious blood transfusion in sickle cell disease patients to avoid such a complication.

Splenic Marginal Zone Lymphoma With Histological Transformation to Hodgkin's Lymphoma.

Marginal zone lymphoma (MZL) is a rare, slow-growing/indolent B cell lymphoid neoplasm accounting for 10.5% to 11.8% of all B cell lymphomas. MZL originates from the mature B lymphocytes, which are usually present in the marginal zone of the lymphoid follicle. Histological transformation (HT) is defined as sheets of large cells arising in an indolent lymphoma with morphological and immunophenotypic changes suggestive of a high-grade lymphoma such as Hodgkin's lymphoma, diffuse large B cell lymphoma (DLBCL), or Burkitt lymphoma. The median time of transformation ranges from one year to 15 years following the initial diagnosis of MZL. Studies reported that the deletion of TP53 and 7q and mutations in NOTCH2 are commonly associated with HT in MZL. This case report outlines the rare happening of an MZL transformation into a nodular subtype of Hodgkin's lymphoma in a 56-year-old female, which prompted further investigations and a different therapeutic approach. By reporting this case, we emphasize that HT changes the natural history and significantly affects the overall survival of patients with MZL. Hence, it is necessary to get a core needle or excisional biopsy whenever there is a clinical suspicion of HT in MZL for early diagnosis and a better therapeutic approach.

A Rare Case of Extra-Medullary Portacaval Plasmacytoma in a Patient With Relapsed Multiple Myeloma.

Multiple myeloma (Kahler disease) is a monoclonal plasma cell immunoproliferative neoplasm originating within the bone marrow that involves the production of monoclonal immunoglobulins, mostly IgG and IgA. Extramedullary plasmacytoma (EMP) is a subset of plasma cell neoplasms that can develop in patients at the time of diagnosis with multiple myeloma, or relapse of the disease. Symptoms related to plasmacytomas depend on the primary location. Here in, we present a rare case of extramedullary plasmacytoma involving the portacaval space in an 83-year-old African American female with relapsed multiple myeloma. She was treated successfully with radiation therapy with complete resolution of the mass. In this case report, we aim to discuss the clinical features along with diagnostic methods and treatment for extramedullary plasmacytomas with emphasis on utilizing a multidisciplinary approach in managing these rare cases.

A Case of Chronic Myelomonocytic Leukemia Unmasked After Receiving J&J COVID-19 Vaccine.

Chronic myelomonocytic leukemia (CMML) is a clonal hematopoietic stem cell disease that comes under the overlap syndrome (myelodysplastic and myeloproliferative disorders). CMML is characterized by peripheral blood monocytosis and bone marrow dysplasia. The pathogenesis of CMML is poorly understood. Although cytogenetic and molecular abnormalities are common, they are not diagnostic. Herein, we present a rare case of CMML after receiving the J&J COVID-19 vaccine with the rare association of limited scleroderma. Based on the Surveillance, Epidemiology, and End Result (SEER) cancer statistics review 2014-2018, the five-year age-adjusted incidence rate of CMML in both sexes is 0.5/100,000, with greater incidence in males (0.7/100,000) compared to females (0.3/100,000). We emphasize the fact that, based on the previous studies reported, the association of scleroderma with CMML is very rare. Our patient had concomitant CMML and scleroderma, which were unmasked after the patient received the COVID-19 vaccine. Our case suggests the possibility of developing CMML after receiving the J&J COVID vaccine. Immunization has always been a life-saving intervention in history. As the world is foreseeing getting the COVID-19 vaccine, it is essential to report all the possible adverse events for safety monitoring. Physicians should be aware of this unusual complication of the vaccine, and more cases are needed to confirm the association between them.

A Rare Case of Solitary Plasmacytoma Arising From the Sternum.

Solitary plasmacytoma (SP) is characterized by an accumulation of neoplastic monoclonal plasma cells in a localized fashion, without evidence of multiple myeloma. It makes up <5% of all plasma cell neoplasms and is typically found in regions like the pelvis, ribs, vertebra, and spine. SP is classified into extramedullary plasmacytoma (EMP), which primarily affects soft tissues, and solitary bone plasmacytoma (SBP), which primarily affects the pelvis, ribs, vertebrae, and spine. We report a case of a 66-year-old man with sternal plasmacytoma presenting as chest pain. He was treated with radiation therapy. Here, we aim to describe the clinical features, diagnostic methods, treatment, and potential outcome in a patient with SBP.

A Rare Case of Aggressive Metastatic Laryngeal Chondrosarcoma Presenting as Bilateral Necrotizing Pneumonia.

Laryngeal chondrosarcomas are rare tumors that account for only 0.2% of malignant tumors of the larynx. Approximately 80% of these tumors are low grade and well differentiated and are associated with a good long-term prognosis. Herein, we report a case of a 77-year-old male presenting with acute hypoxic respiratory failure that required intubation and mechanical ventilation. Chest CT showed multiple pulmonary nodules and cavities. He then required a tracheostomy, and a soft tissue mass in the subglottic mass was discovered. A laryngoscopy-guided excisional biopsy of the mass was performed. Histopathological examination confirmed the diagnosis of laryngeal chondrosarcoma. Clinicians should consider metastatic laryngeal chondrosarcoma as a differential diagnosis for lung cavities. Overall, we believe this to be the first case of aggressive laryngeal chondrosarcoma with mediastinal and pulmonary metastasis mimicking necrotizing pneumonia.

Peritumoral Immunoglobulin M Lambda Light Chain Amyloidosis in a Patient With Advanced Follicular Lymphoma.

Peritumoral light chain (AL) amyloidosis secondary to lymphoid malignancies is a rare but well-described entity. Peritumoral deposition of amyloid without systemic amyloidosis has been described in mucosa-associated lymphoid tissue (MALT) lymphomas; however, there are no reported cases of follicular lymphoma with localized peritumoral AL amyloidosis without systemic involvement of amyloidosis. We present a rare case of a patient with advanced follicular lymphoma with peritumoral lymph node IgM lambda light chain amyloidosis without an underlying monoclonal gammopathy or plasma cell dyscrasia.

Extra-Medullary Hematopoiesis in Sickle Cell Disease Presenting as a Right Adrenal Mass.

Extramedullary hematopoiesis can occur during normal fetal development or as a compensatory mechanism in chronic anemia when the primary sites of hematopoiesis fail. When it occurs, it is mostly seen in sites such as the liver, spleen, and lymph nodes. Extramedullary hematopoiesis is seen in patients with abnormal morphology of red blood cells like thalassemia and abnormal red blood cell shape like hereditary spherocytosis. The incidence of extramedullary hematopoiesis in sickle cell disease is very rare. We report a case of focal adrenal extramedullary hematopoiesis in a 21-year-old patient with sickle cell disease who presented with pain in the right thorax. A retroperitoneal mass was seen in the MRI of the abdomen of the patient suggestive of extramedullary hematopoiesis. Our case highlights the importance of physician awareness of this rare pathology for proper diagnosis and management.