Metastatic yolk sac tumor masquerading as multifocal hepatocellular carcinoma in a young adult: A case report.

Primary yolk sac tumor of the liver is very rare and can present as multifocal liver lesions. Multifocal nature may mimic other diagnoses such as hepatocellular carcinoma. Early recognition and therapeutic intervention are important as the prognosis of metastatic yolk sac tumors is poor. We present a case of a young adolescent who presented with bleeding per rectum abdominal pain and multiple liver lesions.

Tyrosine Kinase Inhibitors in pediatric chronic myeloid leukemia: a focused review of clinical trials.

Tyrosine Kinase Inhibitors (TKIs) is revolutionizing the management of pediatric Chronic Myeloid Leukemia (CML), offering alternatives to Allogeneic Hematopoietic Stem Cell Transplantation (AHSCT). We conducted a comprehensive review of 16 Randomized Controlled Trials (RCTs) encompassing 887 pediatric CML patients treated with TKIs including Imatinib, Dasatinib, and Nilotinib. The median patient age ranged from 6.5 to 14 years, with a median white blood cell count of 234 x 10^9/uL, median hemoglobin level of 9.05 g/dL, and median platelet count of 431.5 x 10^9/µL. Imatinib seems to be predominant first line TKI, with the most extensive safety and efficacy data. BCR::ABL response rates below 10% ranged from 60% to 78%, CCyR at 24 months ranged from 62% to 94%, and PFS showed variability from 56.8% to 100%, albeit with differing analysis timepoints. The Safety profile of TKIs was consistent with the known safety profile in adults. With the availability of three TKIs as first line options, multiple factors should be considered when selecting first line TKI, including drug formulation, administration, comorbidities, and financial issues. Careful monitoring of adverse events, especially in growing children, should be considered in long term follow-up clinical trials.

Patient with clinical celiac disease mimicking triple-negative essential thrombocythemia.

Platelets are acute-phase reactants, which can be elevated due to a secondary cause or less commonly because of a primary mechanism. Primary disorders include hematological conditions such as myelodysplastic syndrome, acute myeloid leukemia, chronic myeloid leukemia, polycythemia vera, and essential thrombocythemia (ET). Most ET patients have a mutation in the genes regulating thrombopoiesis, JAK2, CALR, or MPL genes. But 10%-15% of ET patients are triple-negative, where patients have no detectable mutation. We report a young patient with no significant past medical history evaluated for persistent thrombocytosis. She was initially diagnosed as triple-negative ET based on a bone marrow biopsy. She had positive antibodies for celiac disease, and the diagnosis was confirmed by a small bowel biopsy, which is confirmatory for diagnosing celiac disease in adults. We recommend screening triple-negative ET patients for celiac disease before going to more expensive tests.

Dasatinib-Induced Colitis with Rectal Sparing in a Patient with Chronic Myeloid Leukemia (Chronic Phase) on Dasatinib as an Upfront Therapy: Case Report.

Dasatinib is a BCR-ABL tyrosine kinase inhibitor which was approved in 2006 for the treatment of adults diagnosed with Philadelphia chromosome-positive (Ph+) chronic myeloid leukemia (CML) in chronic phase (CP) and accelerated (myeloid or lymphoid blast) phase and CML with resistance or intolerance to prior therapy including imatinib and in adults with Ph+ acute lymphoblastic leukemia. Common adverse reactions (>15%) in patients diagnosed with CP-CML include myelosuppression, fluid retention, and diarrhea. We report a 34-year-old Filipino female patient who received dasatinib as upfront therapy for the treatment of CP-CML who experienced chronic diarrhea for 2 months, which progressed to colitis.

Lurbinectedin-Induced Tumor Lysis Syndrome in Small Cell Neuroendocrine Cancer of the Cecum: A First-Ever Case Report.

BACKGROUND Lurbinectedin (Lurbi) was first approved in June 2020 for metastatic small cell lung cancer (SCLC) patients with progression following platinum-based chemotherapy. Extrapulmonary small cell neuroendocrine cancers (SCNECs) are treated with regimens used for SCLCs. Tumor lysis syndrome (TLS) in solid SCLCs and SCNECs following Lurbi use has not been reported in the literature so far. CASE REPORT We report a case of Lurbi-induced TLS in a patient with metastatic SCNEC of the cecum following a single intravenous dose of Lurbi 3.2 mg/m2. She presented to the hospital with abdominal pain, anuria, and grade 4 TLS. She required emergent hemodialysis due to acute renal failure. Our patient had a high Ki-67 proliferation index (95%), harbored a huge disease burden, and had bilateral renal metastasis, thus making her more susceptible to develop TLS. CONCLUSIONS Although data regarding the occurrence of TLS due to Lurbi in solid tumors are scarce, it remains a potential complication of Lurbi in neuroendocrine tumors with high proliferation index and large tumor burden.

Ocular Toxicity of Belantamab Mafodotin, an Oncological Perspective of Management in Relapsed and Refractory Multiple Myeloma.

Belantamab mafodotin (belamaf), an antibody-drug conjugate approved for the treatment of relapsed and refractory multiple myeloma (RRMM), is an anti B-cell maturation antigen (BCMA) agent. DREAMM-1, a first in-human trial of belamaf, reported several ocular toxicities requiring dose adjustments, dose delays and treatment discontinuations. In DREAMM-1, 53% of patients in part-1 and 63% of patients in part-2 had ocular toxicity. Similarly, 73% of patients in DREAMM-2 had keratopathy (71% in 2.5 mg/kg versus 75% in 3.4 mg/kg) with the most common symptoms being blurred vision and dry eyes. Ocular toxicity of belamaf is attributed to microtubule-disrupting monomethylauristatin-F (MMAF), a cytotoxic payload of the drug that causes an off-target damage to the corneal epithelial cells. Ocular adverse events (AEs) of belamaf are more frequent at higher doses compared with lower doses. Higher belamaf dose, history of dry eyes and soluble BCMA are associated with increased risk of corneal toxicity. Absence of ocular symptoms does not exclude the possibility of belamaf-induced ocular toxicity, so patients need slit lamp and Snellen visual acuity testing to detect microcytic-like epithelial changes and visual decline. Corticosteroid eyes drops for 4-7 days prior to belamaf dose do not prevent ocular AEs and may cause steroid-related AEs instead. Keratopathy and Visual Acuity scale (KVA) is recommended to document the severity of belamaf-induced ocular toxicity and make treatment adjustments. Management of toxicity includes dosage modifications, treatment interruption or discontinuations and preservative-free artificial tears along with close ophthalmology and hematology-oncology follow-ups.

Evaluation of Hepatitis B Reactivation Among Patients With Chronic Myeloid Leukemia Treated With Tyrosine Kinase Inhibitors.

Hepatitis B reactivation (HBVr) in cancer patients is a well-established complication due to chemotherapy-induced immunosuppression. Studies have reported HBVr associated with immunosuppressive medications, such as rituximab, methotrexate, and high dose steroids. There are different risks for different types of chemotherapy with rituximab carrying one of the highest risks for hepatitis B reactivation. Tyrosine kinase inhibitors (TKIs) are the standard of care in patients with chronic myeloid leukemia (CML). The risk of HBVr in chronic myeloid leukemia has been reported in many studies, but to this date, there are no clear guidelines or recommendations regarding screening and monitoring of HBV in CML patients receiving TKIs. We conducted this review to identify the risk of HBVr in patients with CML who are treated with tyrosine kinase inhibitors. We recommend testing for HBV status in patients who are to be treated with TKIs and to consider giving prophylaxis in those who are positive for HBsAg at baseline. More studies are needed to assess the risk of reactivation in patients with Hepatitis B core antibody positive receiving TKIs. Currently, monitoring such patients for reactivation may be the best strategy.

Advances in maintenance strategy in newly diagnosed multiple myeloma patients eligible for autologous transplantation.

INTRODUCTION: Multiple myeloma (MM) lacks curative therapy. Therefore, researchers continue to conduct studies in an effort to improve progression-free survival (PFS) and overall survival (OS). Maintenance therapy (MT) after autologous stem cell transplant (ASCT) was extensively studied in the last decade and now considered a standard approach. AREAS COVERED: This review evaluated the evidence and updates on various maintenance agents in newly diagnosed multiple myeloma (NDMM) after ASCT. Articles were searched on PubMed and Embase that were published in last 10 years. Both clinical trials and observational studies were evaluated. EXPERT OPINION: Maintenance strategy after ASCT has consistent PFS benefit but lacks conclusive OS improvement. Lenalidomide is superior to thalidomide given reduced neurotoxicity. OS advantage is controversial for both due to inconsistent evidence. Lenalidomide may confer a PFS advantage even at lower doses due to toxicity with higher doses. Bortezomib-based maintenance has some evidence for OS benefit in high-risk MM (HRMM) and renal dysfunction. Ixazomib has preliminary promising results. Two or three drug combinations for MT are potentially safe and more effective, particularly in HRMM although data on this subject is still evolving. Efficacy of various MT regimens in terms of minimal residual disease status needs to be further investigated.

Trends and outcomes of fungal infections in hospitalized patients of inflammatory bowel disease: a nationwide analysis.

BACKGROUND: Immunosuppressive therapy is being increasingly used in the management of inflammatory bowel disease (IBD) which comprises of ulcerative colitis (UC) and Crohn's disease (CD). Patients on immunosuppressive therapy are at increased risk of developing opportunistic fungal infections. We conducted this analysis to describe the epidemiology of opportunistic fungal infections in this cohort. METHODS: We analyzed the National Inpatient Sample (NIS) database for all subjects with discharge diagnosis of IBD (UC and Crohn's disease) & Fungal infections (Histoplasmosis, Pneumocystosis, Cryptococcosis, Aspergillosis, Blastomycosis, candidiasis, Coccidioidomycosis) as primary or secondary diagnosis via ICD 9 codes during the period from 2002-2014. RESULTS: In UC, the incidence of all fungal infections was more in age above 50 (except for pneumoconiosis) male gender (except Candidiasis) and in Caucasians. In CD, the incidence was more in age above 50 (except Pneumocystosis, Blastomycosis & Coccidioidomycosis), female gender (except Histoplasmosis, Pneumocystosis & Cryptococcosis) and in Caucasians. Histoplasmosis and Blastomycosis were more prevalent in Midwest, Cryptococcosis and Candidiasis in South, Coccidioidomycosis in west in both UC and CD. Age above 50, south region, HIV, Congestive heart failure, underlying malignancies, diabetes mellitus with complications, chronic pulmonary disease, anemia, rheumatoid arthritis, collagen vascular disease, pulmonary circulation disorders, weight loss were significant predictors of fungal infections in IBD. The yearly trend showed a consistent small rise in incidence, and the mortality dropped till 2006 to peak again in 2008 with a subsequent decline. CONCLUSIONS: Our study is the first one to describe the basic demographics features and characteristics of opportunistic fungal infections in hospitalized patients with IBD. The yearly incidence of fungal infections did not show a significant rise. The mortality increased between 2006-2008 and a significant difference remains between IBD patients with and without fungal infections. One explanation of rise in mortality but a consistent incidence could be due to the use of biologics that did not increase but compromised the ability of IBD patients to fight opportunistic fungal infections.

Jejunal Varices Bleeding in a Patient with Extensive Portomesenteric Thrombosis Secondary to Factor V Leiden Mutation: A Management Dilemma.

Ectopic varices are portosystemic collaterals that occur away from the gastroesophageal junction and account for 1-5% of all variceal bleeding. Its occurrence in the jejunum is rare. Most common cause of ectopic jejunal varices is portal hypertension especially in those patients who have undergone prior abdominal surgery. Portomesenteric thrombosis is a rare cause of ectopic jejunal varices. Ectopic varices are rare cause of obscure GI bleeding and hence should be always suspected in patients with history of portal hypertension who present with GI bleeding and have negative upper and lower GI endoscopies. Management of patients with ectopic varices is often very challenging and requires multidisciplinary approach. Therapeutic options include endoscopic therapy, interventional radiologic procedures, surgically creating shunting, or surgical resection. We present the case of a 52-year-old patient who was on anticoagulation for extensive portomesenteric thrombosis secondary to factor V Leiden heterozygous mutation and presented with melena and symptomatic anemia. Investigations showed bleeding jejunal varices as the cause of anemia. We discuss the therapeutic options and dilemma in the management of such cases.

Portomesenteric Thrombosis Secondary to Acute Cholecystitis: A Case Report.

Portomesenteric venous thrombosis (PMVT) is an uncommon clinical problem. Common risk factors include intra-abdominal infections, abdominal surgeries, malignancy, cirrhosis, and inherited thrombophilia. Early recognition and treatment of PMVT are important to avoid serious complications like mesenteric ischemia and infarction. Acute cholecystitis is a clinical condition encountered daily but rarely may be complicated by development of portomesenteric venous thrombosis. Only few cases have been reported of superior mesenteric vein thrombosis secondary to cholecystitis. We report a case of a forty-one-year-old male patient who developed partial portal and superior mesenteric vein thrombosis after mild acute cholecystitis for which surgery had been deferred. Patient had no other identifiable risk factors for thrombosis. Patient was successfully treated with 6 months of anticoagulation with warfarin and complete recanalization of portomesenteric veins was achieved at the end of treatment.

Medical thoracoscopy for exudative pleural effusion: an eight-year experience from a country with a young population.

BACKGROUND: With the exception of areas with high prevalence of tuberculosis, medical thoracoscopy is becoming the diagnostic modality of choice for exudative pleural effusions. The aims of this study were to determine the diagnostic yield and safety of medical thoracoscopy for exudative pleural effusions and ascertain the etiology of such effusions in Qatar. METHODS: This is a retrospective-descriptive study of 407 patients who underwent diagnostic medical thoracoscopy for exudative pleural effusions from January, 2008 till December, 2015 at the only tertiary referral center performing this procedure in Qatar. RESULTS: Tuberculosis was the most common etiology of exudative pleural effusions in Qatar accounting for 84.5% of all causes. Around 85% of patients were young males (mean age of 33 ± 12.1 years). The diagnostic yield of medical thoracoscopy for tuberculous pleural effusion was 91.4%. Malignant pleural effusions accounted for 5.2% of cases. Minor bleeding occurred in 1.2% of cases with no procedure-related mortality observed. CONCLUSION: Medical thoracoscopy is a very safe procedure. Tuberculous pleuritis is by far the most common etiology of exudative pleural effusions in Qatar. Closed needle biopsy is a worth consideration as an initial safe, easy and low-cost diagnostic modality for exudative pleural effusions in this country.