Hammondbynum2405

Hypereosinophilia (HE) is rare but often secondary to a nonhematologic disease such as allergic disorders and parasitic infections. HE can also be associated with hematologic malignancies and be the result of a clonal proliferation or reactive to another hematologic condition. Association of HE with acute lymphoblastic leukemia (ALL) is rare in children. We reported a case of a teenager presented with HE secondary to B-ALL who experienced severe cardiac complications with severe absolute eosinophil count. We compared his clinical evolution with other published cases and we reported 2 mutations linked to B-ALL never described before in this context.

Mutations in IKZF1, which encodes Ikaros family zinc finger 1 (IKAROS) transcription factor, are associated with recurrent infections, cytopenia, autoimmune diseases, and hematologic malignancies. Diverse clinical phenotypes resulting from IKZF1 mutations include pulmonary fungal infections, cytopenia, autoimmune hemolytic anemia (AIHA), and malignancies. In this study, we aimed to assess the DNA-binding ability and pericentromeric (PC) localization of a variant of IKZF discovered in a patient.

DNA-binding ability of a pathogenic IKZF variant was tested using electrophoretic mobility shift assay and PC localization of the variant was assessed by immunofluorescent microscopy in NIH3T3 cells.

Clinical features of a 3-month-old male infant who underwent hematopoietic stem cell transplantation because of an IKZF1 mutation-associated common variable immunodeficiency, AIHA, and pancytopenia are described. DNA studies revealed a heterozygous missense variant (IKZF1 NM_006060 c.427C>T; p.R143W). Cotransfection studies revealed that mutant R143W has a partial dominant-negative effect over PC targeting and DNA binding.

IKZF1 mutation must be kept in mind if neonatal AIHA, common variable immunodeficiency, and pancytopenia are observed.

IKZF1 mutation must be kept in mind if neonatal AIHA, common variable immunodeficiency, and pancytopenia are observed.Rasburicase is a recombinant urate oxidase enzyme indicated for tumor lysis syndrome, a potential life-threatening oncologic emergency that occurs most commonly during initial chemotherapy for hematological malignancies. As a result of the defects in the physiological antioxidant pathway, erythrocytes of patients with glucose-6-phosphate dehydrogenase deficiency are not protected against the oxidizing stress exerted by hydrogen peroxide generated with the administration of rasburicase. The authors report a 14-year-old patient, diagnosed with T-cell acute lymphoblastic leukemia, who developed methemoglobinemia and hemolytic anemia with low oxygen saturation after starting steroids, hyperhydratation, and rasburicase administration. The complications resolved with supportive therapy only.Between 2014 and 2020, 31 patients with severe aplastic anemia (SAA) underwent full match allogeneic hematopoietic stem cell transplantation at our center. Of the 31 patients with SAA, 19 had acquired aplastic anemia, 2 had Diamond Blackfan anemia and 10 had Fanconi anemia. Donors were either matched sibling (n=29), related donors (n=2), or unrelated donors (n=3). Peripheral blood stem cells were the graft source in all the cases except 1. Fludarabine-based reduced intensity conditioning was used in all except for patients with a diagnosis of Diamond Blackfan anemia. All patients except 1 achieved hematological recovery in the form of neutrophil engraftment at 13 days (range, 9 to 17), whereas platelet engraftment occurred at 14 days (range, 10 to 18). Graft versus host disease (GvHD) prophylaxis consisted of cyclosporine and methotrexate ±antithymocyte globulin (horse/rabbit). Acute GvHD developed in 12.9% patients, whereas no patients developed chronic GvHD till the time of last follow-up. The 2-year overall survival for the entire cohort was 93.21±4.6%. In patients with SAA, allogeneic stem cell transplant using fludarabine-based conditioning regimens are very well tolerated and have excellent outcomes in a full match setting.Adrenocortical carcinoma (ACC) is a rare, aggressive malignancy of the adrenal cortex. This study characterizes a single-institution cohort of children treated for ACC, and explores the relationship between clinical outcomes of ACC and germline TP53 mutation status. We performed a retrospective chart review of 23 consecutive pediatric patients with ACC treated at The Hospital for Sick Children, Toronto, Canada, between 1977 and 2017. Clinical, biochemical, radiologic, pathologic, and genetic data were collected for each patient. ACC diagnosis followed a bimodal age distribution of 0 to 6 (n=17) and 12+ (n=6) years, with a femalemale ratio of 3.61. Ten of 20 patients tested for germline TP53 status carried a pathogenic (9) or likely pathogenic (1) variant, including all but 1 male patient. Only 3 patients died of ACC-related causes, each 5 months post-diagnosis. When treated with resection and combination chemotherapy, carriers of germline TP53 mutations may respond more favorably than their wild-type counterparts. In addition, the survival of patients reported in our cohort with high-stage ACC was appreciably greater than previously described (100.0% for stage II, 50.0% for stage III, and 42.9% for stage IV), favoring aggressive intervention in these patient populations.

Countertransference in forensic inpatient settings has received little empirical attention despite frequent emotional reactions in staff members, such as anger, disgust, or fear. In this exploratory study, we investigated countertransference in two forensic medium-secure units for patients with psychotic disorders.

We measured countertransference using the Therapist Response Questionnaire and measured staff personality using the Ten-Item Personality Inventory. Our design allowed all staff members to participate anonymously.

One hundred thirty-four Therapist Response Questionnaire forms, along with data on patient and staff characteristics, were collected. Staff characteristics such as profession, experience, and personality were associated with different countertransference reactions. Psychologists and psychiatrists tended to report more countertransference feelings than nursing staff. Patient and staff variables (such as patient having committed violent offenses or a diagnosis of personality disorder and staff experience or gender) were associated with more negative countertransference feelings and subscale scores as well as less positive countertransference feelings such as parental, protective, and satisfying countertransference feelings. Some patient and staff variables (such as patient cooperativeness, staff personality trait agreeableness) had the inverse effect on countertransference feelings.

We discussed several conceptual problems inherent to measuring countertransference (in forensic inpatient settings) and the clinical implications of our findings.

We discussed several conceptual problems inherent to measuring countertransference (in forensic inpatient settings) and the clinical implications of our findings.

Despite the significant advances in EGFR-mutant nonsmall cell lung cancer (NSCLC), some challenges remain. One of the permanent and inevitable issues is the emergence of acquired resistance. Therefore, blocking the activation of EGFR pathway and overcoming drug resistance with novel agents are still in high demand. Here, we review the development of novel drugs in EGFR-mutant, advanced NSCLC, including targeting EGFR exon 20 insertion (EGFR20ins), and novel role of epidermal growth factor receptor, tyrosine kinase inhibitor (EGFR-TKIs) in early-stage NSCLC.

EGFR-TKIs as adjuvant therapy or neoadjuvant therapy in patients with early-stage NSCLC with EGFR-sensitizing mutations have shown promising efficacy. The resistance mechanisms of third-generation EGFR-TKIs can be divided into two types EGFR dependent and EGFR independent. Several clinical trials have demonstrated that the addition of MET inhibitors to EGFR-TKIs was an effective option for patients who had acquired resistance to EGFR-TKIs caused by hepatocyte growth factor receptor gene (MET) amplification or overexpression. Novel compounds that selectively and potently inhibit EGFR20ins are being investigated in phase III studies.

A better characterization and understanding of resistance mechanisms to first-line osimertinib and adjuvant osimertinib is helpful to guide further treatment.

A better characterization and understanding of resistance mechanisms to first-line osimertinib and adjuvant osimertinib is helpful to guide further treatment.

We aimed to assess the frequency of ICU-acquired bloodstream infections in coronavirus disease 2019 patients.

Retrospective observational study.

The emergency expansion of an ICU from eight general beds to 30 coronavirus disease 2019 beds.

Patients with coronavirus disease 2019 admitted to the ICU of Luigi Sacco Hospital (Milan, Italy) for greater than or equal to 48 hours between February 21, 2020, and April 30, 2020.

None.

The frequency of bloodstream infections per 1,000 days of ICU stay was calculated in 89 coronavirus disease 2019 patients, and the cumulative probability of bloodstream infection was estimated using death and ICU discharge as competing events. Sixty patients (67.4%) experienced at least one of the 93 recorded episodes of bloodstream infection, a frequency of 87 per 1,000 days of ICU stay (95% CI, 67-112).The patients who experienced a bloodstream infection had a higher Sequential Organ Failure Assessment score upon ICU admission (9.5; interquartile range, 8-12 vs 8, interquartoccus-related bloodstream infection) after ICU admission. This may have been due to enteric involvement in patients with severe coronavirus disease 2019 and/or limitations in controlling the patient-to-patient transmission of infectious agents in extremely challenging circumstances.

The starting point of neoadjuvant therapy was to use preoperative chemotherapy in order to provide surgeons and radiotherapists with the possibility of local treatment in patients with locally advanced, primary inoperable or inflammatory disease. Since then, this treatment approach has dramatically evolved and is now a standard of care in patients with high-risk early breast cancer.

The role of surgery after neoadjuvant therapy is to remove residual disease in the breast and or in the lymph nodes and to provide further treatment possibilities according to pathohistologic findings at surgery.

Innovative medical treatments are now being used for neoadjuvant treatment in order to reduce the extent of locoregional surgery in the breast and the axilla and also to adjust further medical treatment after neoadjuvant therapy and surgery.

Innovative medical treatments are now being used for neoadjuvant treatment in order to reduce the extent of locoregional surgery in the breast and the axilla and also to adjust further medical treatment after neoadjuvant therapy and surgery.

Spinal anaesthesia-induced hypotension is frequently reported in patients undergoing caesarean section. Mechanistically, sympathetic blockade reduces the systemic vascular resistance and the left ventricular preload, causing hypotension, which is augmented by aortocaval compression. The corrected blood flow time (FTc) is affected by the preload and is inversely related to the afterload.

We hypothesised that the preanaesthetic carotid artery FTc could predict hypotension after induction in patients undergoing a caesarean section with spinal anaesthesia.

A prospective observational study.

A tertiary referral centre in South Korea from September 2018 to November 2019.

Thirty-eight parturients scheduled for elective caesarean section under spinal anaesthesia.

Using carotid ultrasonography, FTc was measured twice prior to inducing spinal anaesthesia. FTc was calculated using both Bazett's (B) and Wodey's (W) formulae. Hypotension was defined as an SBP decrease to less than 80 mmHg, or less than 75% of baseline, or if symptoms consistent with hypotension occurred from the time of injection of the spinal anaesthetic until delivery.