Trasplante hepático en hemofilia A y enfermedad de von Willebrand tipo 3. Manejo perioperatorio y evolución postrasplante / Liver transplantation in hemophilia A and von Willebrand disease type 3: perioperative management and post-transplant outcome

Introducción: la infección por virus hepatitis C (VHC) ha sido causa de importante morbi-mortalidad en hemofilia, planteándose el trasplante hepático (TH) por cirrosis y/o carcinoma hepatocelular (CHC). Caso clínico: presentamos los casos con coagulopatías congénitas e infección por VHC sometidos a TH en nuestro centro: tres pacientes con hemofilia A y uno con enfermedad de von Willebrand (EvW) tipo 3. Evaluamos el curso de la coagulopatía, el manejo perioperatorio, el consumo de factor y componentes sanguíneos y la supervivencia postrasplante. El factor deficitario se comenzó a administrar en bolo iv directo una hora antes del inicio de la cirugía para alcanzar un nivel deseado de dicho factor de 100 UI/dl, mantenido hasta conseguir el control estable de la hemostasia. Los tres pacientes con hemofilia A curaron su coagulopatía postrasplante. El factor VIII (FVIII) fue 93 UI/dl a los once años, 59 UI/dl a los 13 meses y 109 UI/dl a los nueve meses postrasplante en cada uno de los casos. El consumo medio perioperatorio de concentrados de FVIII fue 175 UI/kg, infundido hasta 36 h postrasplante de media. El paciente con EvW tipo 3 consiguió atenuar el curso natural de su sintomatología hemorrágica sin que se detectaran niveles hemostáticos del antígeno del factor von Willebrand (FVW:Ag) postrasplante. Discusión: tras el trasplante hepático, se produce la curación de la hemofilia A y la mejoría del fenotipo hemorrágico en la EvW tipo 3

Introduction: infection with the hepatitis C virus (HCV) causes significant morbidity and mortality in patients with hemophilia. Finally, patients are considered for a liver transplantation (LT) due to cirrhosis and/or hepatocellular carcinoma (HCC). Case report: we report the cases of congenital coagulopathy and HCV infection that underwent LT in our institution. There were three patients with hemophilia A and one patient with von Willebrand disease (vWD) type 3. The coagulopathy outcome, perioperative management, factor and blood product usage and post-transplant survival were assessed. The deficient factor was initially administered in a direct bolus one hour before surgery with a target level of 100 IU/dl, which was sustained until stable hemostasis was reached. All three patients with hemophilia A were cured of their coagulopathy following transplantation. Factor VIII (FVIII) was 93 IU/dl at eleven years, 59 IU/dl at 13 months and 109 IU/dl at nine months post-transplant, in each case. The mean perioperative usage of FVIII concentrates was 175 IU/kg; concentrates were infused for an average of 36 hours post-transplant. The natural course of the bleeding symptoms of the patient with type-3 vWD was attenuated, with no detectable hemostatic levels of von Willebrand factor antigen (vWF:Ag) after transplantation. Discussion: after transplantation, hemophilia A cure and improved bleeding phenotype of type-3 vWD reduced morbidity and mortality. However, potential graft reinfection with HCV and relapsing HCC cast a shadow over these optimum results

Liver transplantation in hemophilia A and von Willebrand disease type 3: perioperative management and post-transplant outcome.

INTRODUCTION: infection with the hepatitis C virus (HCV) causes significant morbidity and mortality in patients with hemophilia. Finally, patients are considered for a liver transplantation (LT) due to cirrhosis and/or hepatocellular carcinoma (HCC). CASE REPORT: we report the cases of congenital coagulopathy and HCV infection that underwent LT in our institution. There were three patients with hemophilia A and one patient with von Willebrand disease (vWD) type 3. The coagulopathy outcome, perioperative management, factor and blood product usage and post-transplant survival were assessed. The deficient factor was initially administered in a direct bolus one hour before surgery with a target level of 100 IU/dl, which was sustained until stable hemostasis was reached. All three patients with hemophilia A were cured of their coagulopathy following transplantation. Factor VIII (FVIII) was 93 IU/dl at eleven years, 59 IU/dl at 13 months and 109 IU/dl at nine months post-transplant, in each case. The mean perioperative usage of FVIII concentrates was 175 IU/kg; concentrates were infused for an average of 36 hours post-transplant. The natural course of the bleeding symptoms of the patient with type-3 vWD was attenuated, with no detectable hemostatic levels of von Willebrand factor antigen (vWF:Ag) after transplantation. DISCUSSION: after transplantation, hemophilia A cure and improved bleeding phenotype of type-3 vWD reduced morbidity and mortality. However, potential graft reinfection with HCV and relapsing HCC cast a shadow over these optimum results.

Anticoagulation with rivaroxaban in a hematology unit: clinical profile, events and discontinuation rates in real-life patients with nonvalvular atrial fibrillation.

AIM: To assess the clinical profile and thromboembolic and bleeding events in patients with nonvalvular atrial fibrillation (AF) who were attended in a hematology unit. METHODS: Retrospective study of AF patients that started treatment with rivaroxaban between February 2012 and June 2016 in a hematology unit from a tertiary hospital in Spain. RESULTS: Overall, 243 patients (mean age 78.4 ± 10.1 years; 47.5% women, CHA2DS2-VASc 3.7 ± 1.5) were included. After a mean follow-up of 16.5 ± 12.7 months, rivaroxaban was discontinued in only 2.4% of patients. During the follow-up, seven (2.0 events/100 patient-years) patients had a thromboembolic event and six patients (1.7 events/100 patient-years) a major bleeding. CONCLUSION: Rivaroxaban was effective and safe among AF patients treated in a hematology unit, with very low discontinuation rates.

Dysuria and acute urinary retencion as initial presentation of primary vaginal lymphoma. Case report and bibliographic review.

OBJECTIVE: We report a case of primary vaginal lymphoma. The clinical presentation was an episode of dysuria and acute urinary retention. We performed a bibliographic review. METHODS: Thirty-six year-old patient who consulted in the urology clinic for hesitancy that triggered an episode of acute urinary retention. Physical examination revealed thickening of the vaginal wall. Biopsy was performed and diagnosis of diffuse large B-cell primary vaginal non-Hodgkin's lymphoma was obtained. RESULTS: Primary lymphomas of the female genital tract are rare. The third most frequent location is vagina. The most common manifestation is vaginal bleeding. Urinary symptoms are rarely the first sign. Diagnosis requires a biopsy. The first choice for treatment is Rituximab- CHOP immuno-chemotherapy. CONCLUSIONS: Vaginal lymphoma is a rare disease. Unfrequently, the first clinical manifestations are urinary tract symptoms, and even less acute urinary retention.

Disuria y retención aguda de orinacomo primera manifestación de un linfoma vaginal primario. Aportación de un caso y revisión de la literatura / Dysuria and Acute Urinary Retencion as Initial Presentation of Primary Vaginal Lymphoma. Case Report and Bibliographic Review

OBJETIVO: Aportamos un caso de un linfoma vaginal primario cuya primera manifestación fue un episo-dio de disuria y retención aguda de orina, con revisión bibliográfica. MÉTODOS: Paciente, 36 años. Consulta en urología por disuria inicial que desencadena un episodio de retención aguda de orina. La exploración muestra engrosamiento de la pared vaginal. Se biopsia obteniendo el diagnóstico de linfoma no Hodgkin difuso de células grandes B vaginal primario. RESULTADOS: Los linfomas primarios del tracto genital femenino son infrecuentes. La tercera localización en frecuencia es la vagina. La manifestación más frecuente es el sangrado vaginal. Rara vez son los síntomas urinarios el primer signo. Su diagnóstico es por anatomía patológica. El tratamiento de elección es la poliquimioterapia según el protocolo Rituximab-CHOP. CONCLUSIONES: El Linfoma vaginal es una patología infrecuente. Es raro que la primera manifestación sean síntomas urológicos, más todavía que se presente en forma de retención urinaria aguda

OBJECTIVE: We report a case of primary vaginal lymphoma. The clinical presentation was an episode of dysuria and acute urinary retention. We performed a bibliographic review. METHODS: Thirty-six year-old patient who consulted in the urology clinic for hesitancy that triggered an episode of acute urinary retention. Physical examination revealed thickening of the vaginal wall. Biopsy was performed and diagnosis of diffuse large B-cell primary vaginal non-Hodgkin's lymphoma was obtained. RESULTS: Primary lymphomas of the female genital tract are rare. The third most frequent location is vagina. The most common manifestation is vaginal bleeding. Urinary symptoms are rarely the first sign. Diagnosis requires a biopsy. The first choice for treatment is Rituximab- CHOP immuno-chemotherapy. CONCLUSIONS: Vaginal lymphoma is a rare disease. Unfrequently, the first clinical manifestations are urinary tract symptoms, and even less acute urinary retention

Síndrome de Job asociado a linfoma de Hodgkin / Job’s syndrome associated with Hodgkin’s lymphoma

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Effect of chemotherapy with alkylating agents on the yield of CD34+ cells in patients with multiple myeloma. Results of the Spanish Myeloma Group (GEM) Study.

BACKGROUND AND OBJECTIVES: Although alkylating agents are clearly beneficial in multiple myeloma (MM), their deleterious effect on bone marrow hematopoietic progenitor cells usually precludes their use as front-line therapy in patients scheduled to undergo autologous stem cell transplantation (ASCT). We analyzed the impact of first-line chemotherapy with alkylating agents on stem cell collection in MM patients. DESIGN AND METHODS: Seven hundred and eighty-nine patients included in the Spanish multicenter protocol GEM-2000 underwent mobilization therapy after four courses of alternating VBMCP/VBAD chemotherapy. RESULTS: The mobilization regimens consisted of standard or high-dose granulocyte colony-stimulating factor (G-CSF) in 551 (70%) patients, and chemotherapy and G-CSF in 206 (26%) patients. The CD34+ cell yield was lower than 4x10(6)/kg in 388 patients (49%), and equal or greater than 4x10(6)/kg in 401 patients (51%). Multivariate analysis indicated that advanced age (p<0.0001) and longer interval between diagnosis and mobilization (p=0.012) were the two variables associated with a lower CD34+ cell yield. Significant differences in CD34+ cell yield were not observed between the mobilization regimens. Of the 789 patients included in the protocol, 726 (92%) underwent the planned ASCT, whereas 25 (3%) patients did not because of the low number of CD34+ cells collected. Following ASCT, 0.5x10(9) neutrophils/L could be recovered after 11 days (median time; range, 5-71 days) and 20x10(9) platelets/L could be recovered after 12 days (median time; range, 6-69 days). INTERPRETATION AND CONCLUSIONS: A short-course of therapy with alkylating agents according to the GEM-2000 protocol was associated with an appropriate CD34+ cell collection, and allowed the planned ASCT to be performed in the majority of MM patients.