Outcomes of Burkitt lymphoma with central nervous system involvement: evidence from a large multicenter cohort study.

Central nervous system (CNS) involvement in Burkitt lymphoma (BL) poses a major therapeutic challenge, and the relative ability of contemporary regimens to treat CNS involvement remains uncertain. We described prognostic significance of CNS involvement and incidence of CNS recurrence/progression after contemporary immunochemotherapy using real-world clinicopathologic data on adults with BL diagnosed between 2009 and 2018 across 30 US institutions. We examined associations between baseline CNS involvement, patient characteristics, complete response (CR) rates, and survival. We also examined risk factors for CNS recurrence. Nineteen percent (120/641) of patients (age 18-88 years) had CNS involvement. It was independently associated with HIV infection, poor performance status, involvement of ≥2 extranodal sites, or bone marrow involvement. First-line regimen selection was unaffected by CNS involvement (P=0.93). Patients with CNS disease had significantly lower rates of CR (59% versus 77% without; P<0.001), worse 3-year progression-free survival (adjusted hazard ratio [aHR], 1.53, 95% confidence interval [CI], 1.14-2.06, P=0.004) and overall survival (aHR, 1.62, 95%CI, 1.18-2.22, P=0.003). The 3-year cumulative incidence of CNS recurrence was 6% (95%CI, 4-8%). It was significantly lower among patients receiving other regimens (CODOX-M/IVAC, 4%, or hyperCVAD/MA, 3%) compared with DA-EPOCH-R (13%; adjusted sub-HR, 4.38, 95%CI, 2.16-8.87, P<0.001). Baseline CNS involvement in BL is relatively common and portends inferior prognosis independent of first-line regimen selection. In real-world practice, regimens with highly CNS-penetrant intravenous systemic agents were associated with a lower risk of CNS recurrence. This finding may be influenced by observed suboptimal adherence to the strict CNS staging and intrathecal therapy procedures incorporated in DA-EPOCH-R.

Impact of Psychological Distress on Treatment Timeliness in Oncology Patients at a Safety-Net Hospital.

BACKGROUND: Psychological distress is common in patients with cancer. Distress can affect patients' engagement with treatment. We examined the relationship between psychological distress and treatment timeliness in a sample of adult oncology patients at a safety-net hospital. METHODS: A retrospective review was conducted of all patients screened for distress at a first outpatient oncology visit between March 1, 2014, and December 31, 2015 (n=500). The analytic sample (n=96) included patients with a new cancer diagnosis and a curative-intent treatment plan for lymphoma (stage I-IV), solid tumor malignancy (stage I-III), or head and neck cancer (stage I-IVb). Distress was measured using the Hospital Anxiety and Depression Scale. Using Poisson regression, we determined the effects of depression and anxiety on treatment timeliness. Patient age, sex, race/ethnicity, insurance type, cancer site, and cancer stage were included as covariates. RESULTS: Mean patient age was 54 years. The median treatment initiation interval was 28 days. Clinically significant anxiety was present in 34% of the sample, and clinically significant depression in 15%. Greater symptom severity in both anxiety and depression were associated with a longer treatment initiation interval after controlling for demographics and disease factors. The average days to treatment (DTT) was 4 days longer for patients with elevated anxiety scores and for those with elevated depression scores compared with those without. Overall survival was not associated with anxiety, depression, or DTT. CONCLUSIONS: In this safety-net patient sample, greater psychological distress was associated with slower time to treatment. As of writing, this is a new finding in the literature, and as such, replication studies utilizing diverse samples and distress measurement tools are needed.

Bevacizumab-induced atypical hemolytic uremic syndrome and treatment with eculizumab.

Bevacizumab (Avastin) is a recombinant humanized monoclonal antibody used for the management of various solid malignancies including colorectal, lung, brain, renal, and ovarian cancers as well as age-related macular degeneration of the eye. It is a vascular endothelial growth factor inhibitor which exhibits its action by blocking the growth of blood vessels in cancerous tissue. Common side effects include hypertension, fatigue, headaches, and increased risk of infections. Atypical hemolytic uremic syndrome is a serious side effect associated with bevacizumab due to its anti-angiogenic effect. It encompasses the clinical triad of thrombocytopenia, microangiopathic hemolytic anemia, and acute renal failure, without any association with Shiga toxins. Eculizumab is a terminal complement inhibitor used in the treatment of atypical hemolytic uremic syndrome. Herein, we present three cases of bevacizumab-induced atypical hemolytic syndrome treated successfully with eculizumab.

The Paleolithic Diet.

The promotion of healthy diets is likely one of the most cost-effective strategies for preventing a wide range of disorders, including cardiovascular disease, obesity, hypertension, and type 2 diabetes. The majority of present non-communicable chronic diseases are attributed to civilization, an abundance of food, and a lack of physical activity. According to the purported lifestyles of early humans, the paleolithic diet (PD) encourages the intake of wild animal and plant foods. In recent decades, the paleolithic diet has grown in popularity, particularly among younger populations and those with cardiometabolic syndrome and other chronic diseases. Due to the nutrition profile of the paleolithic diet, historical studies have postulated that hunter-gatherers (HGs) have slender physiques and were physically fit and devoid of chronic ailments such as cardiovascular diseases (CVDs). This review highlights the composition/constituent of the paleolithic diet along with an emphasis on current studies and evidence on the effectiveness of the paleolithic diet in mitigating chronic diseases.

Treadmill Exercise Stress Test-Induced Takotsubo Cardiomyopathy: A Case Report and Review of Literature.

Noninvasive stress testing is routinely indicated and preferable in the diagnosis of coronary artery disease. We present the case of a patient who developed Takotsubo syndrome/cardiomyopathy (TTS) as a result of an exercise stress echocardiography, as well as a literature review of comparable cases. An abnormal stress test necessitated coronary angiography, which revealed nonobstructive coronaries with apical left ventricular ballooning and a decreased ejection fraction (EF), both of which are concerning for TTS. The patient was medically managed with metoprolol and lisinopril, with improvement in the EF on the follow-up echocardiogram.

A Case of Compartment Syndrome Due to Out-of-Hospital Intraosseous Misplacement During Cardiopulmonary Resuscitation.

Resuscitation relies heavily on gaining access to the circulatory system. During cardiopulmonary resuscitation (CPR), the biggest, most readily accessible vein that does not impede resuscitation is desired. Intraosseous (IO) access is designated for life-threatening emergencies and is a relatively safe procedure with fewer complications. We describe an intriguing and uncommon consequence of out-of-hospital IO placement: compartment syndrome resulting from the displacement of the IO needle by emergency medical services (EMS) workers in a diabetic woman with hypoglycemia. A few hours later, the patient had swelling, discomfort, and loss of motor and sensory sensations at the IO site, necessitating further examinations. The IO needle had traversed both the anterior and posterior cortices of the tibia and was located in the soft tissues along the posterior portion of the tibia as shown by imaging of the afflicted area. Immediate decompression fasciotomy was performed to preserve the patient's limb.

An Interesting Case of Serratia Endocarditis in a Patient With Chronic Myeloid Leukemia.

Serratia is a rare cause of infective endocarditis (IE) and usually occurs in patients with underlying risk factors, such as intravenous (IV) drug use, and human immunodeficiency virus patients. Gram-negative bacteria endocarditis is associated with high mortality when it involves the left side of the heart and often requires surgical intervention in addition to medical treatment. Although most gram-negative endocarditis cases are hospital-acquired, community cases have also been reported. Here, we present a case of Serratia endocarditis in an individual who was later diagnosed with chronic myeloid leukemia (CML) during the same hospitalization. The patient was treated with IV meropenem and started on targeted therapy for CML. CML is presumed to have likely predisposed the patient to bacteremia and IE.

Infective Endocarditis as a Complication of Crohn's Disease on Immunotherapy.

A patient with a history of Crohn's disease on infliximab presented to the hospital with sepsis and a new heart murmur. He was found to have native aortic valve infective endocarditis from a rare species of group D Streptococcusin his blood. The patient was also noted to be in an acute flare of Crohn's disease. The hospital course was complicated by florid heart failure from acute aortic insufficiency. He eventually improved after source control and appropriate antibiotic therapy. S. pasteuranis bacteremia and endocarditis are attributable to the patient's immunocompromised state as a result of infliximab treatment. While S. pasteuranis is infrequently grown in blood cultures, it is commonly found in normal gut flora. We hypothesize that it gained access to the bloodstream through the epithelium in the terminal ileum, which was inflamed due to an acute flare of Crohn's disease.

Treatment of Metastatic Carcinoma of an Unknown Primary to the Heart with Checkpoint Inhibitor Therapy.

INTRODUCTION: Secondary cardiac malignancies are much more common than primary cancers. Metastatic cancers to the heart from other solid organs are well known and carry a poor prognosis. CASE PRESENTATION: An 83-year-old Hispanic veteran presented with breathlessness, cough, and weight loss. Imaging studies showed extensive pericardial effusion and multiple pericardial/epicardial masses. Biopsy showed poorly differentiated metastatic carcinoma with no definitive origin, based on lab, imaging, and extensive immunohistochemistry (IHC) studies. Gene panel testing showed microsatellite stable status. Programmed cell death 1 ligand (PD-L1) IHC was 100% in the tumor cells. He was started on pembrolizumab and had a complete resolution of symptoms. Subsequent imaging studies showed improvement in the multiple pericardial/epicardial masses. DISCUSSION: Immune checkpoint inhibitor (ICI) therapy has changed the treatment landscape of many malignancies since the approval of the first ICI, ipilimumab, for melanoma in 2011. To the best of our knowledge, this is the first reported case of successful treatment by ICI therapy in undifferentiated metastatic carcinoma of an unknown primary source to the heart.

Outcomes of Specialty Palliative Care Interventions for Patients With Hematologic Malignancies: A Systematic Review.

CONTEXT: The outcomes of specialty palliative care (PC) interventions for patients with hematologic malignancies (HMs) is under-investigated. OBJECTIVES: We performed a systematic review to evaluate the effect of PC interventions on patient- and caregiver- reported outcomes and healthcare utilization among adults with HMs (leukemia, myeloma, and lymphoma). METHODS: From database inception through September 10, 2020, we systematically searched PubMed, CINAHL, Embase, Scopus, Web of Science, and Cochrane Reviews using terms representing HMs and PC. Eligible studies investigated adults aged 18 years and older, were published in the English language, and contained original, quantitative, or qualitative data related to patient- and/or caregiver-centered outcomes and healthcare utilization. RESULTS: We screened 5345 studies;16 met inclusion criteria and found that specialty PC led to improved symptom management, decreased likelihood of inpatient death, decreased healthcare utilization, decreased cost of healthcare, and improved caregiver-reported outcomes. Patients with HM have a high need for PC which, though increasing over time, is often provided late in the clinical disease course. CONCLUSIONS: Specialty PC interventions improve healthcare outcomes for patients with HMs and should be implemented early and often. There remains a need for additional studies investigating PC use exclusively in patients with HMs.

Malignant Gastrointestinal Neuroectodermal Tumor (GNET) with Prolonged Disease-Free Survival after Platinum-Based Chemotherapy.

Malignant gastrointestinal neuroectodermal tumor (GNET) is a rare disease with a handful of cases described in literature. GNET has only become a well-known/widely accepted entity in the recent years, but it is still not listed in the database of rare diseases. Due to the rarity of disease, there are no guidelines on standard therapeutic approaches in the adjuvant or metastatic setting. Here, we describe a unique case of GNET with a 7-year disease-free survival following adjuvant cisplatin and etoposide chemotherapy. This is the longest disease-free survival that has ever been described in literature and may support using this combination in a larger cohort of patients in the context of a global clinical trial. We will also review the histopathologic features of GNET and potential therapeutic options in the metastatic setting.

Hodgkin's lymphoma with HLH and complete remission with brentuximab-based therapy.

A 63-year-old man presented to the hospital with generalised weakness, fatigue and a 22 kg weight loss 4 months after being diagnosed with sarcoidosis on a mediastinal lymph node biopsy, with minimal improvement in symptoms on prednisone and methotrexate therapy. On arrival, he was found to have a haemoglobin of 57 g/L and platelet count of 82×109/L. Further work-up revealed six of eight diagnostic criteria for haemophagocytic lymphohistiocytosis (HLH): fever >38.9°C, splenomegaly, cytopaenia, hypertriglyceridaemia, haemophagocytosis and elevated ferritin >31 000 ng/mL. He was also found to have Epstein-Barr viraemia with greater than 17 000 copies. Bone marrow biopsy showed the presence of haemophagocytic histiocytes and evidence of classic Hodgkin's lymphoma. He was started on HLH-94 protocol. Later treatment was switched to lymphoma-directed therapy and he finished six cycles of A+AVD (brentuximab vedotin, doxorubicin, vinblastine and dacarbazine) with end-of-treatment positron emission tomography/CT and bone marrow negative for lymphoma.

Genetics of Hypercoagulable and Hypocoagulable States.

Hemostasis is the normal process of blood coagulation in vivo to stop pathologic bleeding. Virchow triad includes venous stasis, hypercoagulability, and vascular injury. Natural anticoagulants include protein C, protein S, and antithrombin. Factor V Leiden is the most common inherited thrombophilia, followed by prothrombin gene mutation. All inherited thrombophilias are passed down in an autosomal dominant fashion. Patients harboring the antiphospholipid antibodies have an increased risk for thrombosis. von Willebrand disease is the most common inherited bleeding disorder; the pattern of inheritance is autosomal. Hemophilia A and B are the only hereditary bleeding disorders inherited in a sex-linked recessive pattern.

Paclitaxel Induced MDS and AML: A Case Report and Literature Review.

Therapy related acute myelogenous leukemia (AML) and myelodysplastic syndromes (MDS) have been classically linked to alkylating agents and topoisomerase inhibitors. They constitute about 1% of all AMLs. There is less evidence on association of taxanes (paclitaxel and docetaxel) with these myeloid neoplasms. We present a case of paclitaxel therapy related acute myelogenous leukemia after treatment of endometrial cancer with a regimen containing paclitaxel and carboplatin. A 63-year-old female underwent surgery followed by a total of 6 cycles of chemotherapy with carboplatin and paclitaxel. Six months after last cycle of chemotherapy, she was diagnosed with myelodysplastic syndrome with refractory anemia and excess blasts. Six weeks later, she had worsening anemia and thrombocytopenia which prompted a bone marrow biopsy which revealed acute myelomonocytic leukemia. A thorough literature review revealed 12 other case reports where taxanes have been implicated in the development of therapy related myeloid neoplasm. Based on the timeline of events in our patient, paclitaxel is the likely culprit in the pathogenesis of this myeloid neoplasm. This rare but significantly grave adverse effect should be kept in consideration when deciding on treatment options for gynecological malignancies.

A Case of De Novo CD5+ Disseminated Intravascular Large B-Cell Lymphoma Presenting as Multiorgan Failure.

Intravascular large B-cell lymphoma is an extremely rare extranodal lymphoma that proliferates in the lumen of the blood vessels while sparing the organ parenchyma. It usually presents with CNS and skin involvement. A 65-year-old Caucasian female presented with fevers and chills of 3-4 months' duration. Bone marrow biopsy done 3 months prior showed no significant myelodysplasia or lymphoid aggregates. The patient later died due to multiorgan failure. A bone marrow biopsy showed 20-30% CD5+ B cells consistent with infiltrative large B-cell lymphoma. An autopsy performed revealed diffuse intravascular invasion by lymphoma cells. Multiorgan involvement by intravascular B-cell lymphoma is very rare. Based on our literature review and to the best of our knowledge, there are only 5 case reports describing the presentation of this lymphoma with multiorgan failure. The immunophenotypic studies performed revealed that our patient had de novo CD5+ intravascular large B-cell lymphoma which is known to be aggressive with very poor prognosis. Although it is an extremely rare lymphoma, it should be considered as a potential cause of multiorgan failure when no other cause has been identified. A prompt tissue diagnosis and high-dose chemotherapy followed by ASCT can sometimes achieve remission.