Management of acquired hemophilia A: results from the Spanish registry.

The Spanish Acquired Hemophilia A (AHA) Registry is intended to update the status of AHA in Spain. One hundred and fifty-four patients were included and retrospectively followed for a median of 12 months. Patients were predominantly male (56.3%), with median age at diagnosis of 74 years. AHA was more frequently idiopathic (44.1%) and autoimmune disorder-associated (31.7%). Thirty-four percent of patients were on antithrombotic therapy at diagnosis. Hemostatic treatment was used in 70% of patients. Recombinant activated factor VII was more frequently infused (60.3% vs 20.6% activated prothrombin complex concentrate). Only 1 patient did not achieve control of hemorrhage. Complete remission (CR) was achieved by 84.2% of cases after immunosuppressive therapy. Steroids alone were less efficient than the other strategies (68.2% vs 87.2%, P = .049), whereas no differences existed among these (steroids/cyclophosphamide, 88.5%, vs steroids/calcineurin inhibitors, 81.2%, vs rituximab-based regimens, 87.5%). Female sex and high inhibitor levels influenced CR negatively. Thirty-six deaths (23.8%) were reported. Main causes of death were infection (15 patients, 9.9%) and hemorrhage (5 patients, 3.3%). All hemorrhage-related and half the infection-related deaths occurred within 2 months of diagnosis. Prior antithrombotic therapy was inversely associated with survival, irrespective of age. Median age of nonsurvivors was significantly higher (79 vs 73 years in survivors). Patients dying of infection were older than the other nonsurvivors (85 vs 78 years). In summary, fatal infection in the first months is common in our series. Antithrombotic therapy is associated with mortality. Particular care should be taken to avoid misdiagnosis.

Patient General Condition at Diagnosis: A Systematic Evaluation for Adults Diagnosed with Hematologic Malignancies.

Several societies have published recommendations for evaluating older adults with cancer in standard conditions. It is vital to assure a proper systematic patient condition evaluation, not only in the oldest (geriatric assessment) but in all adult patients. We have investigated the feasibility of a systematic evaluation of the general condition of all patients diagnosed with hematologic malignancies, and the degree of acceptance by the clinical team, in a prospective cohort of 182 consecutive adults, by using the ECOG performance status scale (ECOG, age 18 and over, 18+), Lee Index for Older Adults (LEE, 50+), Geriatric Assessment in Hematology (GAH, 65+), and the Comprehensive Geriatric Assessment (CGA, 75+). Clinical team acceptance was analyzed with a visual analogue scale, and the objective feasibility was calculated as the proportion of patients that could be finally evaluated with each tool. Acceptance was high, but the objective feasibility was progressively lower as the complexity of the different tools increased (ECOG 100%, LEE 99.4%, GAH 93.2%, and CGA 67.9%). LEE and GAH categories showed a weak concordance (Cohen's Kappa 0.24) that was slight between LEE and CGA (Kappa 0.18). Unexpectedly, we found no significant association between the GAH and CGA categories (p = 0.16). We confirm that a systematic evaluation of all adult patients diagnosed with hematologic malignancies is feasible in daily practice by using an age-adapted approach. Direct comparisons among the different predictive tools in regard to patients' tolerance to treatments of different intensities must be a priority research subject in the coming years.

Tratamiento de la hemofilia adquirida en el paciente anciano. Estudio de 10 casos / Treatment of acquired hemophilia in the elderly. Study of 10 cases

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Estudio de incidencia de las neoplasias hematopoyéticas en Castilla y León / Incidence of haematological neoplasms in Castilla y León, Spain

Fundamento y objetivo: Conocer la incidencia de las neoplasias hematopoyéticas (NH) en Castilla y León, una región de 2,5 millones de habitantes, y su distribución en función de la edad, el sexo y el subtipo histológico. Pacientes y método: Se ha analizado el perfil epidemiológico en función de las variables descritas de las 10.943 NH diagnosticadas durante un período de 10 años, comparándolo con el de otros estudios. Resultados: La incidencia ajustada por edad alcanzó 29,4 casos/105 habitantes-año, con ciertas variaciones geográficas. La edad media fue de 67,3 años, con un punto de inflexión entre la sexta y séptima décadas de la vida, a partir del cual se produjo un aumento muy importante de la incidencia. A medida que avanzaba la edad, ocurrieron de forma paralela otros 2 hechos relevantes: una disminución de la incidencia de los procesos linfoides y el aumento de la de las neoplasias de bajo grado de agresividad. Los procesos linfoides de bajo grado representaron la mitad de los casos del registro, mostraron una mayor preferencia por el sexo masculino y alcanzaron la moda antes que el resto de las NH. La incidencia de neoplasias mieloides (9,5) fue superior a la descrita en otros registros europeos, especialmente en los países del sur de Europa, contrariamente a lo observado con las neoplasias linfoides (20,0). Conclusiones: Se observó una mayor incidencia de neoplasias mieloides y menor de linfoides de lo esperado. El punto de inflexión de incidencia se situó entre la sexta y séptima décadas de la vida, con predominio del sexo masculino, que se reduce con el aumento de la edad. La mayor incidencia de NH se observó en la zona donde se concentra una mayor densidad de industrias potencialmente contaminantes (AU)

Background and objective: We aimed to assess the incidence of haematological neoplasms (HNs) in Castilla y León (2,5 million inhabitants) and its distribution by age, gender and histological type. Patients and method: The epidemiological profile based on the described variables of the 10,943 HNs diagnosed during a 10-years period was analyzed, compared with other studies. Results: The overall age-adjusted incidence was 29.4 cases/105 inhabitants-year, with some geographical differences. The mean age was 67.3 years, with a turning point between the 6th-7th decades of life from which there was a very significant increase of incidence. Two relevant facts where simultaneous with advancing age: decreased lymphoid neoplasms incidence and increased low degree neoplasms incidence. Lymphoid low degree neoplasms accounted for half of the registered processes, showed the greatest preference for male and reached the mode before the rest of neoplasms. Myeloid neoplasms incidence (9.5) was higher than that reported in other European registries, specially compared to southern European countries, opposite to lymphoid neoplasms incidence (20.0). Conclusions: A higher myeloid neoplasms incidence and lower lymphoid one than expected was observed. The turning point of incidence is between the 6th-7th decades of life, with a preference for male that decreases with age. There is an increased incidence of HNs in the area where a higher density of potentially polluting facilities is concentrated (AU)

[Incidence of haematological neoplasms in Castilla y León, Spain]. / Estudio de incidencia de las neoplasias hematopoyéticas en Castilla y León.

BACKGROUND AND OBJECTIVE: We aimed to assess the incidence of haematological neoplasms (HNs) in Castilla y León (2,5 million inhabitants) and its distribution by age, gender and histological type. PATIENTS AND METHOD: The epidemiological profile based on the described variables of the 10,943 HNs diagnosed during a 10-years period was analyzed, compared with other studies. RESULTS: The overall age-adjusted incidence was 29.4 cases/10(5) inhabitants-year, with some geographical differences. The mean age was 67.3 years, with a turning point between the 6th-7th decades of life from which there was a very significant increase of incidence. Two relevant facts where simultaneous with advancing age: decreased lymphoid neoplasms incidence and increased low degree neoplasms incidence. Lymphoid low degree neoplasms accounted for half of the registered processes, showed the greatest preference for male and reached the mode before the rest of neoplasms. Myeloid neoplasms incidence (9.5) was higher than that reported in other European registries, specially compared to southern European countries, opposite to lymphoid neoplasms incidence (20.0). CONCLUSIONS: A higher myeloid neoplasms incidence and lower lymphoid one than expected was observed. The turning point of incidence is between the 6th-7th decades of life, with a preference for male that decreases with age. There is an increased incidence of HNs in the area where a higher density of potentially polluting facilities is concentrated.

High incidence of acute leukemia in the proximity of some industrial facilities in El Bierzo, northwestern Spain.

OBJECTIVES: To estimate the incidence of acute leukemia (AL) in El Bierzo (BZ) and to carry out a cross-association analysis in order to suggest some etiological clues. MATERIALS AND METHODS: We registered all new AL cases diagnosed 2000-2005. Annual standardized incidence rate (SIR) was calculated by the direct method. A cross-association analysis was performed by non-parametric methods and we checked the potential interaction between putative etiological factors by calculating Chi-square-for-trend. RESULTS: SIR was 5.1 cases per 100 000, surpassing the Spanish, European and world average figures and heterogeneous throughout the region. We detected a negative correlation between acute myeloblastic leukemia (AML) SIR in every municipality and both the air distance to the nearest thermoelectric power plant (TPP) (Rho = -0.409; p = 0.01) and to the point of maximum density of the high-power lines (HPL) network (Rho = -0.329; p = 0.04). Accordingly, SIR was higher in the municipalities situated < 7.5 km away from TPP (9.58 vs. 1.72; p = 0.004) or < 10 km away from HPL (3.90 vs. 3.19; p = 0.045). A positive relation between both factors was observed (Chi-square-for-trend = 9.209; p = 0.006). CONCLUSIONS: SIR of AL in BZ is higher than the Spanish average and that of most countries in the world. Residing near TPP or HPL confers a higher risk of AML, with synergistic effect between both factors.