[CAR-T CELL THERAPY FOR B-CELL ACUTE LYMPHOBLASTIC LEUKEMIA WITH A LARGE OVARIAN EXTRAMEDULLARY MASS].

INTRODUCTION: Acute lymphoblastic leukemia (ALL) is the most common malignancy in childhood, arising from the bone marrow. ALL may present at diagnosis or relapse at extramedullary sites. In recent years, autologous T-cells engineered to express a chimeric-antigen receptor (CAR-T cells) have emerged as novel immune-based therapies for relapsed and refractory ALL, targeting the B-cell surface antigen CD19. Here we present a patient with relapsed ALL involving the ovaries, treated with CD19 CAR-T cells. Toxicity included cytokine-release syndrome and neurotoxicity. CAR-T cell therapy led to a complete remission, with evidence of CAR-T cell trafficking to disease sites including the bone marrow and ovaries.

Delayed diagnosis and treatment of children with cancer during the COVID-19 pandemic.

BACKGROUND: COVID-19, the novel coronavirus has caused a global pandemic affecting millions of people around the world. Although children, including children with cancer, have been found to be affected less commonly and less severely than adults, indirect effects of the pandemic on the diagnosis and treatment of children with cancer have been less described. METHODS: A survey was performed in the four largest tertiary pediatric hematology-oncology medical centers in Israel. Clinical and laboratory data were collected from the medical files of patients diagnosed or treated with cancer during April-October 2020. RESULTS: Seventeen patients are described, who had a significant delay in diagnosis or treatment of cancer. These represent approximately 10% of all pediatric cancer diagnosed during the study period in these centers. A main cause of delay was fear of exposure to COVID-19 (fears felt by the patient, parent, physician, or decision-makers at the institution; or the implementation of national guidelines). Delays also resulted from co-infection with COVID-19 and the attribution of the oncologic symptoms to the infection. In addition, treatment was delayed of patients already diagnosed with cancer, due to COVID-19 infection detected in the patient, a family member, or a bone marrow donor. CONCLUSION: Fear from the COVID-19 pandemic may result in delayed diagnosis and treatment of children with cancer, which may carry a risk to dismal prognosis. It is crucial that pediatricians and patients alike remember that other diseases still prevail and must be thought of and treated in a timely fashion.

Pearson disease in an infant presenting with severe hypoplastic anemia, normal pancreatic function, and progressive liver failure.

Pearson disease is a rare, usually fatal, mitochondrial disorder affecting primarily the bone marrow and the exocrine pancreas. We report a previously healthy 10-week-old girl who presented with profound macrocytic anemia followed by pancytopenia, synthetic liver dysfunction with liver steatosis, and metabolic acidosis with high lactate levels. She had no pancreatic involvement. Multiple cytoplasmic vacuoles in myelocytes and monocytes were seen upon microscopic evaluation of the bone marrow. Genetic analysis of the mitochondrial genome revealed a 5 kbp deletion, thus establishing the diagnosis of Pearson disease.

[Relatives' satisfaction with the care of elderly patients in skilled nursing wards].

BACKGROUND: Skilled Nursing Wards (SNW) in Israel are purposely equipped and staffed for treatment of patients that, in addition to functional dependence, suffer from active diseases which require close medical supervision. Patients' surveys on satisfaction with health care are increasingly accepted as an element of quality assessment. However, given the high rate of communication disorders present in these patients, this method is not feasible for use in SNWs. OBJECTIVE: We initiated a survey of patients' relatives--based on a questionnaire specifically designed to reflect their satisfaction with the level of care provided. METHODS: Respondents were asked to rank their level of satisfaction with medical, nursing and structural aspects of the care in the SNW on a 1-5 Likert Scale. RESULTS: Relatives of 147 SNW residents answered the questionnaire. The overall level of satisfaction was high, all components being ranked at 4.2 or above. Four point fifty-five was the average rating given to the medical component of the care, 4.39 to the nursing aspect and 4.32 to the structural section. Five points were awarded to the medical component by 60% of the participants, to the nursing element by 55% of respondents and to the structural section by 40% of respondents. No differences were observed between the rankings of frequent and less frequent visitors. All respondents were highly cooperative and expressed their appreciation of the initiative to perform a satisfaction survey. CONCLUSION: The results show that the families of the skilled nursing patients that participated in this study are satisfied with the health care provided to their relatives. Such surveys contribute to the quality of care and should be considered for routine/periodical use in long-term care settings.

Satisfaction with the care of institutionalized psychogeriatric patients, as reflected by a survey of their relatives.

The patients' satisfaction is an important aspect of quality of care assessment in long-term care institutions. However, many psychogeriatric patients are institutionalized at an advanced stage of their disease, when they are unable to communicate properly and cannot be questioned about their satisfaction with the care received in these psychogeriatric settings. Therefore we initiated a survey of patients' relatives based on a questionnaire purposely designed, to evaluate their satisfaction with the medical, nursing and structural components of the institutional care. The relatives of 101 patients, representing 79% of the patients in four psychogeriatric wards, responded to the questionnaire. The level of satisfaction with the care was high, as reflected by the fact that over 75% of the respondents rated the overall question about satisfaction with the medical, nursing, and structural components with the maximum of five points. The high correlation between the summative and the overall satisfaction supports and even strengthens this view. Another finding was the reluctance of most relatives to be involved in the personal care of the patients. Our study concluded that the relatives do appreciate the efforts of the staff and understand the constraints imposed by the nature of the patients' condition. Moreover, both families and staff welcomed this study and mentioned its contribution in improving communication.

Urethral masturbation and sexual disinhibition in dementia: a case report.

Urethral masturbation and sexual disinhibition as manifestations of behavioral and psychological symptoms of dementia (BPSD) are described in a 90-year-old patient who repeatedly self-inserted foreign bodies into his urethra. A diagnosis was made of late onset sexual disinhibition and hypersexuality in a patient with Dementia of the Alzheimer Type. Significant reduction of his sexual behavior was achieved with low doses of haloperidol. Similar symptoms are noted in Pick's disease, other fronto-temporal lesions, mania and following a seizure or treatment of Parkinson's disease, and have been described as Kluver-Busy-type. Clinicians should consider this diagnosis when investigating dysuria, cystitis, haematuria and urinary tract infections even in the very old.