Venetoclax-induced vitiligo in a patient with chronic lymphocytic leukemia.

Venetoclax, a highly selective Bcl-2 inhibitor, is an orally bioavailable drug that has been approved as first-line therapy for chronic lymphocytic leukemia (CLL) in combination with obinutuzumab, as well as monotherapy in the setting of relapsed CLL. Although some of its life-threatening side effects are well known, including tumor lysis syndrome and cytopenias, others less known side effects include skin reactions. Skin rash is commonly reported in literature, which is often mild and not life-threatening. In this case report, the authors describe what is potentially the second case of venetoclax-induced vitiligo reported in literature. A 77-year-old man with CLL Rai stage II with cytogenetics showed 11 q23 deletion in 14% of cells, and 14q32 partial deletion in 9% of cells developed vitiligo in his extremities 2 years into treatment. A decision was made to continue venetoclax with close monitoring as the side effect was mild and not debilitating. The patient continued to do well. Although vitiligo is not associated with increased mortality risk, its development is associated with increased psychological stress. The mechanism by which vitiligo develops remains unclear. There may be an association between drug-induced vitiligo and improved cancer prognosis; however, larger studies need to be carried out to prove this hypothesis.

Testicular Plasmacytoma Masking as Epididymo-orchitis in a Known Multiple Myeloma Patient.

BACKGROUND/AIM: Extramedullary plasmacytoma (EMP) is defined as a localized plasma cell neoplasm that arises in tissues other than the bone. The most common sites of involvement of EMP are the upper airways followed by lymph nodes, gastrointestinal tract, thyroid gland, skin, brain, liver, and lungs. Testicular plasmacytoma has a very rare occurrence with about 70 cases reported in literature to date. CASE REPORT: We describe a 52-year-old-male with a diagnosis of multiple myeloma presenting with lytic lesions of the axial skeleton. He had lambda light chain restricted, R-ISS stage II with high risk cytogenetics as he tested positive for t(4;14). He underwent four cycles of cyclophosphamide, bortezomib and dexamethasone followed by auto-peripheral stem cell transplantation. He was kept on ixazomib, lenalidomide and dexamethasone maintenance therapy, but relapsed soon after and was diagnosed with plasmacytoma of the left lung. Therapy was switched to daratumumab, carfilzomib and dexamethasone and the patient received radiation of his left lung. He then developed left painless testicular mass which was treated with six weeks course of antibiotics. However due to persistence of concerning features on scrotal ultrasound post-treatment, the patient underwent radical orchiectomy with pathology coming back positive for plasma cells. CONCLUSION: The testes serve as a sanctuary site for hematological malignancies due to the presence of the testicular-blood barrier. Hence, it is imperative to keep a high index of suspicion for testicular plasmacytoma in the right clinical context when evaluating a patient with known multiple myeloma.

Polyserositis as a Unique Presentation of Isolated Myeloid Sarcoma: A Case Report.

BACKGROUND: Myeloid Sarcoma (MS) are tumors containing myeloid blasts occurring in a location other than the bone marrow, including lymph nodes, skin, and soft tissues. MS presenting as polyserositis however is very rare, with only a few cases in the literature. CASE REPORT: A 20-year-old male presented with cough, shortness of breath and was found to have left upper lobe consolidation, left pleural effusion, pericardial effusion, and a large anterior mediastinal mass. A transthoracic echocardiogram showed pericardial effusion with tamponade physiology. He underwent emergent pericardiocentesis and thoracentesis. The fluid studies showed flow cytometry findings consistent with MS/ acute myeloid leukemia (AML) phenotype. A bone marrow aspirate and biopsy were unremarkable and showed no immunophenotypic findings diagnostic of acute leukemia or a lymphoproliferative disorder. Cytogenetics was negative for AML abnormalities per FISH analysis. Videoassisted thoracoscopy surgery (VATS) with biopsy of the mediastinal mass, pericardium, and left upper lobe of the lung was consistent with MS. He was treated with induction cytarabine and idarubicin, and a follow up PET-CT scan showed complete remission. He is currently day 200 + post stem cell transplant with no evidence of disease recurrence. CONCLUSION: To the best of our knowledge, this is the first case of isolated myeloid sarcoma presenting as polyserositis, without prior leukemia/ bone marrow involvement. Hence, fluid studies should involve cytometry analysis and MS should be entertained as a differential for polyserositis, even without a history of prior leukemia. Timely diagnosis can expedite aggressive chemotherapy required for a potentially life-threatening disease.

Intrathecal Chemotherapy as a Potential Treatment for Steroid-refractory Immune Effector Cell-associated Neurotoxicity Syndrome.

BACKGROUND/AIM: Chimeric antigen receptor (CAR) T-cell therapy has revolutionized the treatment of various B-cell malignancies. However, it can cause serious adverse effects like immune effector cell-associated neurotoxicity syndrome (ICANS). ICANS is attributed to disruption of the blood-brain barrier due to inflammatory cytokines and increased levels of immune effector cells (IECs) in the cerebrospinal fluid (CSF). Corticosteroids and supportive management are the mainstays of ICANS treatment. However, no guidelines exist for the treatment of steroid-refractory ICANS. Some reports have shown favorable outcomes with no long-term complications in patients with steroid-refractory ICANS treated with intrathecal (IT) chemotherapy. CASE REPORT: We describe the outcomes of two patients with steroid-refractory ICANS treated with IT chemotherapy. Both patients had refractory large B-cell lymphoma and were not candidates for autologous transplant. They developed steroid-refractory ICANS after CAR T-cell infusion. IT chemotherapy with 12 mg methotrexate and 50 mg hydrocortisone resulted in prompt neurological improvement in both patients. One of them passed away due to multiple other comorbidities, and the other patient continues to do well without any complications. CONCLUSION: IT chemotherapy could be considered as a potential approach for the management of steroid-refractory ICANS based on our experience. Prospective studies are needed to validate this approach.

Predictors and Long-Term Outcomes for Diffuse Large B-Cell Lymphoma (DLBCL) Patients Undergoing Surgery Prior to Systemic Therapy: A Nationwide Analysis.

BACKGROUND: A minority of patients diagnosed with diffuse large B-cell lymphoma (DLBCL) undergo surgery before the initiation of systemic therapy. The aim of this study is to explore the characteristics of patients undergoing surgery prior to systemic therapy (surgfirst), the predictors for surgfirst, and the survival outcomes. METHODS: The National Cancer Database was queried for patients with DLBCL diagnosed between 2006 and 2015, and we performed a subgroup analysis of patients that received surgfirst. Time-to-initial therapy (TTI) was defined as the time in days (d) from diagnosis to systemic therapy. Overall survival was measured from the day of diagnosis in terms of months (m). RESULTS: Factors associated with lower likelihood of surgfirst were non-Hispanic Black race (p-value<0.005), rural location (p-value<0.005), treatment at academic center (p-value<0.005), Medicaid insurance (p-value=0.01), comorbidity score >=3 (p-value 0.007), year of diagnosis, advanced stages of disease, and presence of B-symptoms. The TTI of systemic therapy was delayed in the surgfirst group - 34 (IQR 22-52) days vs. 23 (IQR 13-38) days, p-value<0.005. The five-year overall survival was 62.7% (95% CI 62.1-63.2%) vs. 58.3% (95% CI 57.7-60.0%) - HR 0.87 (95% CI 0.85-0.89), p-value<0.005. The factors associated with higher mortality were advanced comorbidities, lower educational status, disease primarily located in the bone, brain, and spinal cord, advanced clinical stage, presence of B-symptoms, and advanced age. CONCLUSION: Despite the delay in systemic therapy, we could not identify a detrimental impact of surgfirst on survival. This needs to be confirmed in large-scale multicenter studies. We identified clinical and socioeconomic factors that affect treatment selection and survival.

Three Cases of Lenalidomide Therapy for Multiple Myeloma and Subsequent Development of Secondary B-ALL.

INTRODUCTION: Multiple myeloma (MM) is the second most common hematological malignancy, accounting for 1% of all cancers, with median age of diagnosis between 66-70 years. MM remains incurable despite advances in treatment over time. Lenalidomide is an important medication used in induction therapy for MM and is also used for maintenance therapy for standard risk patients. With its increasing use, data is emerging about its use being associated with increased risk of secondary primary malignancies (SPM), especially when used as maintenance therapy. CASE SERIES: In this case series, we describe three patients with refractory MM treated with lenalidomide maintenance who later developed sALL. All had a common presentation of pancytopenia. They developed cytopenias while being on lenalidomide which was refractory to lenalidomide cessation, prompting bone marrow biopsy. MANAGEMENT AND OUTCOME: Lenalidomide was subsequently stopped, and patients were treated for secondary B-ALL. However, all passed away either due to relapse of disease or complications arising from treatment. DISCUSSION: The mechanism of lenalidomide associated SPMs is not well understood however its incidence is well documented. At least 13 cases of ALL (predominantly B-cell ALL) following Immunomodulator imide drugs (IMiDs) have been reported in literature. An analysis of a larger cohort of patients is required to determine causality of lenalidomide with sALL. However, benefits of maintenance lenalidomide in patients with MM outweighs the risk of developing SPMs. Albeit persistent pancytopenia on lenalidomide therapy should be evaluated with bone marrow biopsy since it could be caused by secondary B -cell ALL.

A Case of Idiopathic Thrombocytopenic Purpura After Booster Dose of BNT162b2 (Pfizer-Biontech) COVID-19 Vaccine.

Vaccination is now considered the best measure in minimizing the morbidity and mortality from the Covid-19 pandemic. Almost all the vaccines are considered safe except for minor and occasional side effects. Some of the commonly reported complications from the COVID-19 vaccines are vaccine-induced thrombotic thrombocytopenia (VITT)/thrombosis with thrombocytopenia syndrome/vaccine-induced pro-thrombotic immune thrombocytopenia syndrome. In this case report, we present a case of a 75-year-old female who had an uncomplicated first and second vaccine dose but developed VITT after the booster dose of the vaccine. The patient was treated with dexamethasone and platelet transfusions. So far no such cases have been reported after the third (booster) dose of the Pfizer-Biontech vaccine. With this case report, we present the case of the patient and discuss the literature related to vaccine-induced thrombocytopenia.

An Internal Review of Rates of Palliative Medicine Referral for Patients With Advanced Pancreatic Cancer.

Background The American Society of Clinical Oncology recommends that patients with advanced cancer receive palliative care services in concurrence with active treatment. While the benefits of palliative care are clear, integration of palliative care can be challenging. We aim to review rates of palliative care consultation in patients with advanced pancreatic cancer at our institution, intending to improve these rates. Methods We retrospectively reviewed the electronic records of all patients with pancreatic cancer treated at Allegheny General Hospital diagnosed between 2009-2020. Summary statistics are presented as percentages for categorical data and median with interquartile range for quantitative data. Results Of the 171 patients reviewed, 121 completed all treatment and evaluation within our health network (Pittsburgh, United States). The median age was 63 years (IQR 40-91 years); 55 patients (45%) were male; the majority were white (107 patients, 88%). At the time of diagnosis, 28% of our patients had stage IV disease (34 patients), and 19.8% of patients who developed stage IV disease had palliative care referrals. Conclusions Palliative care is an integral part of usual care for advanced pancreatic cancer. Our analysis showed that palliative care is underutilized in our hospital. We aim to improve palliative care integration in our patients' care by adding a hard stop to electronic medical records to remind physicians to offer palliative care to our patients with pancreatic cancer and to arrange lecture series to emphasize the importance of palliative care in this setting.

Carcinoid Tumor: Advances in Treatment Options.

Small bowel neoplasms are rare, accounting for only 3%-6% of all gastrointestinal neoplasms. Carcinoid tumors represent a large portion of these (20%-30%), making them the second most common small bowel malignancy after adenocarcinoma. Gastrointestinal carcinoids constitute 70% of all neuroendocrine tumors, and out of those, 42% originate in the small bowel. They are predominantly seen in older patients around the age of 65 years. From 1973 to 2004, there has been more than a fourfold increase in the incidence of carcinoid tumors. This can be probably due to increased diagnostic accuracy rather than an actual increase in the number of new cases. The workup of a suspicious case of gastrointestinal bleeding consists of esophagogastroduodenoscopy and/or colonoscopy, and other imaging tests including video capsule endoscopy and balloon-assisted endoscopy. Management of the tumors is dependent on the size and location of the lesion. Treatment options include surgery, endoscopic removal of tumors, and various immunotherapy and chemotherapeutic agents.

The influence of operation theatre environment on patients' perceptions during awake procedures: a cross-sectional study.

OBJECTIVE: To determine the perceptions of patients regarding operation theatre conversation and environment during an awake procedure. METHODS: The descriptive, cross-sectional study was conducted from February to August 2017 in postoperative wards of Military Hospital, Rawalpindi, Pakistan, and comprised women undergoing gynaecological or obstetric procedures under regional anaesthesia. Closedended questionnaires were distributed via nonprobability convenient sampling. Operation theatre data was collected from the anaesthesia notes, and data was analysed using SPSS 22. RESULTS: There were 93 female subjects with a mean age of 28.23}5.42 years (range: 19-48 years). Overall, 90(96.8%) subjects regarded the conduct of operation theatre staff as cooperative and friendly with 64(68.8%) viewing the conversation among the staff as helpful in relieving anxiety. The thought of anaesthesia waning caused anxiety in 40(43%) subjects, but there was no difference in perceived anxiety between those counselled 61(65%) and not counselled by the anaesthetist. With regards to the environment, 41(44%) subjects said more measures were required to prevent patients from seeing the operating field. Besides, 30(32.2%) and 20(21%)subjects had reservations regarding the presence of male staff and medical students respectively. The opinion regarding medical professionals did not change for 60(64%) subjects after the procedure, and 37(39.8%) recommended that communication skills of medical professional needed improvement. CONCLUSIONS: The environment in the theatre can have an influence on the patient's anxiety levels. Counselling by surgeons, casual conversations in the theatre can help alleviate apprehensions of the patients.