Barriers and facilitators to early initiation of palliative care as perceived by nurses working on pneumology and nephrology, a descriptive qualitative study.

AIMS AND OBJECTIVES: Initiation of palliative care early in the disease trajectory is beneficial for patients with a life-limiting disease. However, palliative care is still introduced rather late or not at all. Therefore, this study aims to explore barriers and facilitators to early initiation of palliative care as perceived by nurses working on pneumology and nephrology. METHODS: A descriptive interview-based study was conducted from a critical realist perspective until data sufficiency was reached. Fifteen nurses presented and discussed a patient for whom palliative care was initiated too late. Template analysis was conducted to develop themes and subthemes. RESULTS: Five key themes were extracted: (1) communication, (2) fear, (3) personal beliefs about life and death, (4) ambiguity in terminology and (5) workload and time pressure. Barriers related to poor interdisciplinary communication were therapeutic obstinance, hierarchy, unawareness of the patient's wishes and fear of saying something inappropriate. Other barriers were patients' religious beliefs which often hindered the use of sedatives or morphine and led to discomfort and time restraints. A palliative support team in hospital and advance care planning (ACP) were enablers for early palliative care. STUDY LIMITATIONS: This study started from a negative experience, leading to identification of a lot of barriers and only a few facilitators. The limited sample size and the restriction to two wards within one single hospital limit the diversity of perspectives and the generalisability of the findings. CONCLUSION: More attention is needed for ACP and interdisciplinary communication. Palliative care, including ACP, and interdisciplinary communication should be included in the basic curricula of all healthcare professional courses. Further research is needed to explore barriers and facilitators to early initiation of palliative care in other healthcare settings and patient populations. This is crucial in order to develop and implement sustainable interventions for specific groups of patients.

Complex Transfusion Management in a Sickle Cell Patient With Anti-Fy3 Alloimmunization: A Case Report.

Fy3 is a high-prevalence red blood cell antigen of the Duffy (Fy) blood group system. Anti-Fy3 antibodies are rare and solely arise in individuals with a Duffy null phenotype (Fy(a-b-)), which is a phenotype that mainly occurs in people of African descent. Clinically, anti-Fy3 antibodies can cause both acute and delayed hemolytic transfusion reactions in adults as well as hemolytic disease in fetuses and newborns. Here, we report the case of a 26-year-old male with sickle cell disease (SCD) and a history of anti-E alloantibodies, who was admitted to the hospital with a vaso-occlusive crisis (VOC) and associated low hemoglobin (Hb) level. For the latter he received one unit of antigen-matched and crossmatch-compatible packed red blood cells (pRBCs) without complications. Ten days later the patient was readmitted with a further VOC and associated low Hb level, again requiring a red cell transfusion. However, no crossmatch-compatible pRBCs could be identified. Laboratory testing demonstrated pan-reactivity with additional reference testing demonstrating the presence of anti-E, anti-Fy3 and anti-Jkb alloantibodies. This case highlights the diagnostic and therapeutic challenges associated with blood transfusion in SCD patients with rare alloimmunization profiles.

Combined Results of Two Cross-Sectional Surveys on the Participation in Clinical Trials and the e-Consent Procedure in the Landscape of Haematology.

Despite the motivation of oncology patients to take part in clinical trials, only a minority of them are enrolled in clinical trials. Implementation of new practical procedures can become a barrier that withholds patients from participating in clinical trials. Treating physicians are crucial in augmenting trial accrual. The drivers that promote physicians to allocate patients for clinical trials need further assessment. We conducted two separate cross-sectional surveys, addressing patients with a haematological disease in one survey and haematologists in another survey. The patient survey was filled out by 420 patients. Significant relationships between the willingness to participate in a trial and trial knowledge (p < 0.001) and between doctor-patient relationship and participation willingness (p = 0.007) were noted. Patients above 60 years were less willing to use an electronic consent procedure vs. patients younger than 60 (p < 0.001). The physician questionnaire was completed by 42 participants of whom most (83%) were active in and (94%) motivated for clinical trials. Apart from the patient benefit and scientific interest, prestige was an equal motivator closely followed by financial remunerations. First goal was not to harm the patient. Our study confirms the high willingness of patients for trial participation and the need to rethink the structure of trial organisation. The e-consent procedure is not the method preferred by most patients above 60 years old.

Disease Perception Is Correlated with Health-Related Quality of Life in Patients Suffering from Myelodysplastic Syndromes: Results of the Belgian Be-QUALMS Study.

Patients with myelodysplastic syndromes suffer from an impaired quality of life that is only partially explained by physical symptoms. In an observational study, we aimed to investigate the impact of current MDS treatments and the influence of disease perception on quality of life. Serial measurement of health-related quality of life was performed by 'the QUALMS', a validated MDS-specific patient reported outcome tool. Disease perception was evaluated by means of the Brief Illness Perception Questionnaire (B-IPQ). We prospectively collected data on 75 patients that started on a new treatment and could not demonstrate a significant change in QUALMS score or B-IPQ score during treatment. Six out of eight items evaluated in the B-IPQ correlated significantly with QUALMS score. In this small sample, no significant difference in QUALMS score was found between lower vs. higher risk MDS patients or other studied variables, e.g., targeted hemoglobin showed no correlation with QUALMS score. In daily practice attention must be paid to initial formation of disease perception as it correlates independently with health-related quality of life and does not change during treatment (clinicaltrials.gov identifier: NCT04053933).

Quality of Life in Low-Risk MDS: An Undervalued Endpoint.

The opinion I put forward in this paper is that attention must be paid to health-related quality of life as a study endpoint in lower-risk MDS patients. At the moment therapeutic options are limited in this population. New treatments are predominantly available in clinical studies. In announcing trial publications and during manuscript introductions, quality of life is widely valued as a treatment goal. However, data on health-related quality of life during phase III studies are not published in the original publications, thereby undermining the importance of quality of life as a study endpoint. What seems to be forgotten is that quality of life comprises more than a study endpoint. It is the highest good of lower-risk MDS patients and should also be acknowledged as a safety aspect. Current phase II trials with new medications do not collect data on health-related quality of life, a practice that I consider unethical. In this opinion I demonstrate the current attitude towards health-related quality of life in lower risk MDS patients among leading journals and trial sponsors with several recent examples. I also argue that health-related quality of life should be the main treatment goal in this population. In the event that we shift our focus towards health-related quality of life as the main treatment goal, new treatments could come to a field where gains in overall survival have been marginal over the years.

Lymphoma-like monoclonal B cell lymphocytosis in a patient population: biology, natural evolution, and differences from CLL-like clones.

High-count monoclonal B cell lymphocytosis (MBL) with a chronic lymphocytic leukemia (CLL) phenotype is a well-known entity, featuring 1-4% annual risk of progression towards CLL requiring treatment. Lymphoma-like MBL (L-MBL), on the other hand, remains poorly defined and data regarding outcome are lacking. We retrospectively evaluated 33 L-MBL cases within our hospital population and compared them to 95 subjects with CLL-like MBL (C-MBL). Diagnoses of L-MBL were based on asymptomatic B cell clones with Matutes score < 3, B cells < 5.0 × 103/µl, and negative computerized tomography scans. We found that median B cell counts were considerably lower compared to C-MBL (0.6 vs 2.3 × 103/µl) and remained stable over time. Based on immunophenotyping and immunogenetic profiling, most L-MBL clones did not correspond to known lymphoma entities. A strikingly high occurrence of paraproteinemia (48%), hypogammaglobulinemia (45%), and biclonality (21%) was seen; these incidences being significantly higher than in C-MBL (17, 21, and 5%, respectively). Unrelated monoclonal gammopathy of undetermined significance was a frequent feature, as the light chain type of 5/12 paraproteins detected was different from the clonal surface immunoglobulin. After 46-month median follow-up, 2/24 patients (8%) had progressed towards indolent lymphoma requiring no treatment. In contrast, 41% of C-MBL cases evolved to CLL and 17% required treatment. We conclude that clinical L-MBL is characterized by pronounced immune dysregulation and very slow or absent progression, clearly separating it from its CLL-like counterpart.

False homozygous HLA genotyping results due to copy number neutral loss of heterozygosity in acquired aplastic anaemia.

The aim of this case report is to draw attention on possible false human leucocyte antigen (HLA) genotyping in acquired aplastic anaemia prior to allogeneic haematopoietic stem cell transplantation. In acquired aplastic anaemia loss of heterozygosity (LOH) of chromosome 6p is known to occur in around 12%. We report false HLA genotyping results due to LOH and a coinciding steep rise in neutrophils following filgrastim stimulation in a patient with very severe aplastic anaemia. At diagnosis we obtained heterozygous results on peripheral blood. Failing to reach a partial response at 6 months with immune-suppressive therapy we repeated HLA genotyping, obtaining homozygous results. Repeated testing confirmed loss of HLA genotype heterozygosity. HLA genotyping on cells obtained by a buccal swab confirmed the previous HLA heterozygosity. A second course of filgrastim at the time of homozygous HLA genotyping resulted in a steep rise in neutrophils. Stopping filgrastim resulted in an equally steep drop.

A case report of immunosuppression-related Kaposi's sarcoma after autologous stem cell transplantation.

BACKGROUND: Kaposi's sarcoma (KS) is a tumor formed by angioproliferations driven by Human herpes virus 8 also known as Kaposi's sarcoma-associated herpes virus (KSHV). It is best known as an acquired immune deficiency syndrome (AIDS) defining illness that may be fatal. There are only a few reports of KS after hematopoietic cell transplantation (HCT). This is the first case describing the disappearance of KS with immune recovery after autologous HCT. CASE PRESENTATION: We present the case of a 61-year-old male heterosexual patient of Moroccan origin treated for primary mediastinal non-Hodgkin lymphoma. Because of refractory disease he received multiple lines of chemotherapy prior to autologous HCT. After the second course of low-dose bis-chloroethylnitrosourea, etoposide, cytarabine, melphalan (BEAM) the patient developed several round blue skin lesions. A biopsy was performed, showing many small vessels and positive immune histochemical staining for Human herpes virus 8 (HHV-8), confirming diagnosis of KS. Human immunodeficiency virus testing was negative and work-up showed that there were no visceral lesions. When KS are limited to the skin, prognosis is usually better. The extensive chemotherapy resulted in an important immunosuppression; on day 105 after autologous HCT CD4(+) count was 82/mm(3). Since KS were limited to the skin and attributed to severe immune suppression a watchful waiting strategy was adopted even though in the first months after autologous HCT new skin lesions appeared. With immune recovery (CD4(+) count > 200/mm(3)) 277 days after transplant, skin lesions faded. CONCLUSION: Kaposi's sarcoma remains a rare tumor that should be thought of in any patient whose immunity is down. If immune recovery is expected and disease is limited to the skin, a watchful waiting strategy can be more rewarding than intensive chemotherapy.