Evans' Syndrome: From Diagnosis to Treatment Audia, Sylvain; Grienay, Natacha; Mounier, Morgane ...
Journal of clinical medicine,
11/2020, Letnik:
9, Številka:
12
Journal Article
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Odprti dostop
Evans' syndrome (ES) is defined as the concomitant or sequential association of warm auto-immune haemolytic anaemia (AIHA) with immune thrombocytopenia (ITP), and less frequently autoimmune ...neutropenia. ES is a rare situation that represents up to 7% of AIHA and around 2% of ITP. When AIHA and ITP occurred concomitantly, the diagnosis procedure must rule out differential diagnoses such as thrombotic microangiopathies, anaemia due to bleedings complicating ITP, vitamin deficiencies, myelodysplastic syndromes, paroxysmal nocturnal haemoglobinuria, or specific conditions like HELLP when occurring during pregnancy. As for isolated auto-immune cytopenia (AIC), the determination of the primary or secondary nature of ES is important. Indeed, the association of ES with other diseases such as haematological malignancies, systemic lupus erythematosus, infections, or primary immune deficiencies can interfere with its management or alter its prognosis. Due to the rarity of the disease, the treatment of ES is mostly extrapolated from what is recommended for isolated AIC and mostly relies on corticosteroids, rituximab, splenectomy, and supportive therapies. The place for thrombopoietin receptor agonists, erythropoietin, immunosuppressants, haematopoietic cell transplantation, and thromboprophylaxis is also discussed in this review. Despite continuous progress in the management of AIC and a gradual increase in ES survival, the mortality due to ES remains higher than the ones of isolated AIC, supporting the need for an improvement in ES management.
To describe Health-Related Quality of Life (HRQoL) and to identify the association between sociodemographic, clinical and psychosocial factors, and self-reported HRQoL among NHL survivors.
The data ...of the cancer registry specialized in hematological malignancies in Côte d'Or (France) were used to identify all patients diagnosed with follicular lymphoma (FL) and diffuse large B-cell lymphoma (DLBCL) from 2010 to 2017. Patients were invited to complete SF-12 and other questionnaires.
The HRQoL of NHL survivors was poorer than that of the French general population (
< 0.05) in vitality (48 vs. 56), general health (56 vs. 63), role physical scores (60 vs. 70), role emotional scores (64 vs. 72) and the Mental Component Scale (45 vs. 49). The mean difference in physical functioning decreased per unit increase in age (β = -1.1 (0.3);
< 0.001). Men had better vitality than women (β = 12.4 (6.1);
0.04) and the high education level was associated with greater role emotional scores (β = 14.1 (5.4);
0.01). Symptoms of anxiety and depression were associated with poorer HRQoL. The satisfaction of social support was associated with significantly greater scores on mental health (β = 17.3 (5.1);
= 0.001) and social functioning (β = 15.7 (7.8);
0.04). Socioeconomic deprivation was associated with poorer general health (β = -12.8 (5.2);
0.01).
From 3 to 11 years post-diagnosis, the main factors found to be associated with poor HRQoL of NHL survivors were age, sex, presence of anxiety, depression and economic problems. These findings suggest the need for supportive care to improve HRQOL and the consideration of these problems when developing care plans for NHL survivors.
Background: No robust data assesses the risk of all-cause death and cardiovascular (CV) events in multiple myeloma (MM) patients. Patients and Methods: From 1 January to 31 December 2013, 3,381,472 ...adults were hospitalised (for any reason) in French hospitals. We identified 15,774 patients diagnosed with known MM at baseline. The outcome analysis (all-cause death, CV death, myocardial infarction (MI), ischaemic stroke, or hospitalization for bleedings) was performed with follow-ups starting at the time of the last event. For each MM patient, a propensity score-matched patient without MM was selected. Results: The mean follow-up in the propensity-score-matched population was 3.7 ± 2.3 years. Matched patients with MM had a higher risk of all-death (yearly rate 20.02 vs. 11.39%) than patients without MM. No difference was observed between the MM group and no-MM group for CV death (yearly rate 2.00 vs. 2.02%). The incidence rate of MI and stroke was lower in the MM group: 0.86 vs. 0.97%/y and 0.85 vs. 1.10%/y, respectively. In contrast, MM patients had a higher incidence rate of rehospitalization for major bleeding (3.61 vs. 2.24%/y) and intracranial bleeding (1.03 vs. 0.84%/y). Conclusions: From a large nationwide database, we demonstrated that MM patients do not have a higher risk of CV death or even a lower risk of both MI and ischaemic stroke. Conversely, MM patients had a higher risk of both major and intracranial bleedings, highlighting the key issue of thromboprophylaxis in these patients.
Epidemiological data on myeloid malignancies are very rare in the literature due to a lack of registration by cancer registries until 2000. The Registry of Hematologic Malignancies of the Côte d'Or ...Department in France has, however, steadfastly registered data on cases occurring in the Department since 1980, resulting, to date, in a database of over 5,000 cases classified according to the ICD-O-3 classification, following the most recent World Health Organization classification criteria.
Twenty-five years of data on myeloid malignancies, including acute myeloid leukemia, myeloproliferative neoplasms, myelodysplastic syndromes and myelodysplastic/myeloproliferative syndromes were analyzed. World population standardized incidence rates were calculated as were as observed and relative survival.
Incidence rates per 100,000 inhabitants/year were 2.5 for acute myeloid leukemia, 1.3 for myelodysplastic syndromes, 3.2 for myeloproliferative neoplasms and 0.6 for myelodysplastic/myeloproliferative syndromes. It was found that the incidence rate of myelodysplastic syndromes increased significantly over the period. The median overall survival is 8.9 months for patients with acute myeloid leukemia, 33.8 months for patients with myelodysplastic syndromes, 91.7 months for those with myeloproliferative neoplasms and 26.6 months for patients with myelodysplastic/myeloproliferative syndromes. Observed and relative 20-year survival rates are, respectively, 12% and 13% in acute myeloid leukemia, 2% and 6% in myelodysplastic syndromes and 20% and 34% in myeloproliferative neoplasms.
These population-based data on myeloid malignancies are the first data collected over such a long period and provide interesting information for clinicians and public health authorities, particularly given the paucity of other long-term, population-based data from cancer registries.
Objectives: The UMACOACH Lymphoma is a multidisciplinary monitoring program for patients initiating a first highly haematotoxic treatment for Hodgkin or non-Hodgkin lymphoma. Patient follow-up is ...based on consultation with a pharmacist and planed phone calls by nurses supervised by a clinical haematologist. Our objective was to assess effectiveness and cost of the UMACOACH Lymphoma Program (ULP) and to investigate patient satisfaction and quality of life (QoL). Methods: This French monocentric case-control study included all patients enrolled in the ULP over a one-year period (cases) matched with retrospective patients receiving usual care (controls). Numbers of adverse events (AEs), re-hospitalisations, average relative dose intensity (ARDI), treatment response and survival were compared between the two groups. Among cases, patient satisfaction and QoL using the EORTC-QLQC30 questionnaire before and after treatment were evaluated. Results: Seventy-eight cases were matched to 78 controls. Twenty-six percent grade 3−4 AEs were observed in cases versus 38% in controls (p = 0.001). There were 76 and 88 re-hospitalisations in the case and control groups, respectively (p = 0.217). ARDI > 85% was observed in 92% and 82% of cases and controls, respectively (p = 0.138). No differences were observed in terms of treatment responses and survival. Estimated cost savings were of EUR 81,782 in favour of the case group. An improvement of 5.1 points was observed in the total QoL score before and after treatment in cases. Conclusions: A nurse−pharmacist−haematologist collaboration seems to be promising to reduce grade 3−4 AEs in HL and NHL patients receiving highly haematotoxic chemotherapy regimens. Cost savings from hospitalisation being avoided were also shown.
Background
The excess mortality observed in Acute Myeloblastic Leukaemia (AML) patients, partly attributed to unequal access to curative treatments, could be linked to care pathways.
Methods
We ...included 1039 AML incident cases diagnosed between 2012–2016 from the 3 French blood cancer registries (3,625,400 inhabitants). We describe patients according to age, the medical entry unit and access to the specialised haematology unit (SHU) during follow‐up. Multivariate logistic regression model was done to determine the association between covariables and access to SHU. A total of 713 patients (69%) had access to SHU during care.
Results
The most common care pathway concerned referral from the general practitioner to SHU, n = 459(44%). The univariate analysis observed a downward trend for the most deprived patients. Patients who consulted in SHU were younger (66 years vs. 83, p < 0.001), and 92% had access to cytogenetic analysis (vs. 54%, p < 0.001). They also had less poor prognosis AML‐subtypes (AML‐MRC, t‐AML/MDS and AML‐NOS) (38% vs. 69%); 77% with de novo AML (vs. 67%, p < 0.003), more favourable cytogenetic prognostic status (23% vs. 6%, p < 0.001), less comorbidities (no comorbidity = 55% vs. 34%, p < 0.001) and treatments proposed were curative 68% (vs. 5.3%, p < 0.001). Factors limiting access to SHU were age over 80 years (OR, 0.14; 95% CI, 0.04–0.38), severe comorbidities (OR, 0.39; 95% CI, 0.21–0.69), emergency unit referral (OR, 0.28; 95% CI, 0.18–0.44) and non‐SHU referral (OR, 0.12; 95% CI, 0.07–0.18). Consultation in an academic hospital increased access to SHU by 8.87 times (95% CI, 5.64–14.2).
Conclusion
The high proportion of access to cytogenetic testing and curative treatment among patients admitted to SHU, and the importance of early treatment in AML underlines the importance of access to SHU for both diagnosis and treatment.
In this study, we show for the first time that well‐known clinical and biological prognostic factors limit the access of AML patients to a specialised haematology unit, whereas this consultation strongly influences the access to cytogenetic analyses and curative treatments.
Since 2001, the World Health Organization classification of tumours of haematopoietic and lymphoid tissues and the International Classification of Diseases for Oncology (third edition) have improved ...data collection for lymphoma subtypes in most European cancer registries and allowed reporting on the major non-Hodgkin lymphoma subtypes. Treatment of non-Hodgkin lymphoma has changed profoundly, benefiting patients with follicular lymphoma or diffuse large B-cell lymphoma. We aimed to compare dynamics of cancer mortality in patients with follicular lymphoma or diffuse large B-cell lymphoma in five large European areas using data for survival from the largest number of collaborative European population-based cancer registries (EUROCARE).
We considered follicular lymphoma and diffuse large B-cell lymphoma cases in patients aged older than 15 years diagnosed between Jan 1, 1996, and Dec 31, 2004, and recorded in 43 cancer registries in five areas: Scotland and Wales, and northern, central, eastern, and southern Europe. We excluded cases incidentally diagnosed at autopsy or known from death certificates only. The vital status could be updated on Dec 31, 2008, in all registries but the French ones (Dec 31, 2007). We obtained changes in net survival with the Pohar-Perme estimator and excess mortality rate with a flexible parametric model according to age and year of diagnosis.
We identified 13,988 follicular lymphoma and 25,320 diffuse large B-cell lymphoma cases. We noted improvements in 5-year net survival for all ages between the 1999-2001 and 2002-04 periods for both cancers (except for follicular lymphoma in Scotland and Wales and diffuse large B-cell lymphoma in eastern Europe). For follicular lymphoma, 5-year net survival in northern Europe was 64% (95% CI 58-71) in 1999-2001 versus 75% (69-80) for 2002-04, for Scotland and Wales, it was 71% (66-76) versus 68% (64-72), for central Europe, it was 64% (61-67) versus 72% (70-75), for southern Europe, it was 67% (63-70) versus 73% (70-76), and for eastern Europe, it was 50% (43-57) versus 61% (54-69). For diffuse large B-cell lymphoma, 5-year net survival in northern Europe was 41% (35-49) versus 58% (54-62), in Scotland and Wales, it was 44% (41-48) versus 52% (49-54), in central Europe, it was 46% (44-47) versus 50% (48-51), in southern Europe, it was 44% (42-47) versus 50% (48-52), and in eastern Europe, it was 47% (41-54) versus 46% (43-50). We noted the largest area disparity during the 2002-04 period between eastern and northern Europe. We noted a significant effect of the year of diagnosis on the excess mortality rate for all ages in all areas, except for diffuse large B-cell lymphoma in eastern Europe. The excess mortality rate was not constant during the follow-up period: we noted a high rate early for both lymphomas, except for follicular lymphoma in northern Europe.
Although survival for follicular lymphoma and diffuse large B-cell lymphoma is improving, the results from this study should foster the search for more and better means of improvement of access to adequate care than that at present, as there remains variation in survival between European regions. Study of the dynamics of the excess mortality rate seems to be a useful clinical indicator to help the practitioner's choice of optimum management of patients.
Compagnia di San Paolo, Fondazione Cariplo Italy, Italian Ministry of Health, European Commission, Registre des Hémopathies Malignes de Côte d'Or, and French Agence Nationale de la Recherche.
1 Laboratoire dHématologie, Centre Hospitalier Universitaire (CHU) de Dijon, Dijon
2 Registre des hémopathies malignes de Côte dOr, EA Université de Bourgogne, Dijon
3 INSERM U892, Centre de ...Recherche en Cancérologie Nantes/Angers, Nantes
4 Service dHématologie Clinique, CHU de Dijon, Dijon and
5 Laboratoire dHématologie, Centre Hopitalier Universitaire, Nantes, France
Correspondence: François Girodon, Laboratoire dHématologie, Hôpital du Bocage, CHU de Dijon, Dijon, France. E-mail: francois.girodon{at}chu-dijon
ABSTRACT
We analyzed the effect of hydroxyurea on the JAK2V617F allelic ratio (% JAK2 V617F), measured in purified blood granulocytes, of patients with polycythemia vera and essential thrombocythemia. Thirty-six patients were examined sequentially prior to and after start of hydroxy-urea therapy (8 polycythemia vera, 17 essential thrombocythemia), or while remaining untreated (2 polycythemia vera, 9 essential thrombocythemia). Hydroxyurea therapy (median duration: 15 months) reduced the % JAK2 V617F by >30% in 13/25 patients (4 polycythemia vera, 9 essential thrombocythemia). For 3 patients, JAK2 V617F remained undetectable for 3–27 months. In addition, a single time point study of two large cohorts of patients, examined either at the time of diagnosis (99 polycythemia vera, 178 essential thrombocythemia) or while receiving hydroxyurea (36 polycythemia vera, 98 essential thrombocythemia; median length of therapy: 32 months), confirmed reduction of % JAK2 V617F in the hydroxyurea-treated group (24% vs. 33% JAK2 V617F at diagnosis, p <0.01). Prospective studies are needed to determine the prognostic value of reduced JAK2 V617F allele burden under cytoreductive therapy.
Key words: JAK2V617F, hydroxyurea, myeloproliferative neoplasm, polycythemia vera, essential thrombocythemia, allele-specific real time quantitative PCR.
With improvements in acute myeloid leukemia (AML) diagnosis and treatment, more patients are surviving for longer periods. A French population of 9453 AML patients aged ≥15 years diagnosed from 1995 ...to 2015 was studied to quantify the proportion cured (P), time to cure (TTC) and median survival of patients who are not cured (MedS). Net survival (NS) was estimated using a flexible model adjusted for age and sex in sixteen AML subtypes. When cure assumption was acceptable, the flexible cure model was used to estimate P, TTC and MedS for the uncured patients. The 5-year NS varied from 68% to 9% in men and from 77% to 11% in women in acute promyelocytic leukemia (AML-APL) and in therapy-related AML (t-AML), respectively. Major age-differenced survival was observed for patients with a diagnosis of AML with recurrent cytogenetic abnormalities. A poorer survival in younger patients was found in t-AML and AML with minimal differentiation. An atypical survival profile was found for acute myelomonocytic leukemia and AML without maturation in both sexes and for AML not otherwise specified (only for men) according to age, with a better prognosis for middle-aged compared to younger patients. Sex disparity regarding survival was observed in younger patients with t-AML diagnosed at 25 years of age (+28% at 5 years in men compared to women) and in AML with minimal differentiation (+23% at 5 years in women compared to men). All AML subtypes included an age group for which the assumption of cure was acceptable, although P varied from 90% in younger women with AML-APL to 3% in older men with acute monoblastic and monocytic leukemia. Increased P was associated with shorter TTC. A sizeable proportion of AML patients do not achieve cure, and MedS for these did not exceed 23 months. We identify AML subsets where cure assumption is negative, thus pointing to priority areas for future research efforts.
1 Laboratoire dHématologie, Centre Hospitalier Universitaire (CHU) de Dijon
2 Registre des hémopathies malignes de Côte dOr, EA 4184 Université de Bourgogne, Dijon
3 Laboratoire de Cytologie ...Clinique et Cytogénétique CHU de Nîmes
4 Service dHématologie Clinique, CHU de Dijon, France
Correspondence: François Girodon, Laboratoire dHématologie, Hôpital du Bocage, CHU de Dijon, Dijon, France. E-mail: francois.girodon{at}chu-dijon.fr
ABSTRACT
To observe the effect of the new World Health Organization (WHO) criteria on the incidence of myeloproliferative neoplasms, we performed a retrospective study of a population-based registry in the Côte dOr area, France, from 1980 to 2007. A total of 524 myeloproliferative neoplasms were registered for the 1980–2007 period, including 135 polycythemia vera, 308 essential thrombocythemia and 81 idiopathic myelofibroses. No change in the incidence of either polycythemia vera or idiopathic myelofibrosis was observed for the 2005–2007 period, compared to 1980–2004. On the contrary, a pronounced increase in the incidence of essential thrombocythemia was noted after 2005, mainly due to the use of JAK2 mutation screening and a lower threshold of platelet count. Our study confirms the relevance of the new WHO diagnostic criteria in allowing earlier diagnosis of essential thrombocythemia.
Key words: JAK2 mutation, myeloproliferative neoplasm, polycythemia vera, essential thrombocythemia, incidence.
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