Anormalidades hematológicas en enfermedad hepática, fisiopatología y consideraciones terapéuticas / Hematologic disorders in liver failure: pathophysiology and therapeutic considerations

RESUMEN Las alteraciones en los recuentos celulares sanguíneos representan los hallazgos clínicos más notorios y recurrentes en pacientes que padecen enfermedad hepática, tanto aguda como crónica. Estos cambios constituyen un marcador importante de la disfunción hepática y, a menudo, desempeñan un papel crucial en la evaluación y manejo de estos pacientes. En conjunto con el alargamiento de las pruebas de coagulación, la trombocitopenia es la irregularidad más prevalente en estos individuos. Esta condición, así como las leucopenias, se le atribuye en gran medida al hiperesplenismo, una alteración en la que el bazo retiene y destruye las células sanguíneas, incluidas las plaquetas. Sin embargo, cuando el conteo plaquetario desciende por debajo de 10 x 103/µl, es fundamental considerar otras causas, como factores autoinmunitarios que pueden estar contribuyendo con la trombocitopenia. La anemia, definida como una disminución en el número de glóbulos rojos o en los niveles de hemoglobina, es otra característica constante que acompaña a la enfermedad hepática. Aunque en la mayoría de los casos la anemia es macrocítica, en algunas situaciones puede ser secundaria a eventos hemolíticos, como lo observado en el síndrome de Zieve. Esta diversidad en las manifestaciones de la anemia en pacientes hepáticos subraya la complejidad de las interacciones entre el hígado y los componentes sanguíneos. A pesar de los avances en la comprensión de las causas subyacentes de estas citopenias, las opciones del tratamiento siguen siendo limitadas. Generalmente, las opciones terapéuticas se enfocan en la administración de transfusiones de hemocomponentes para compensar las deficiencias en los recuentos celulares o en el uso de análogos de trombopoyetina (TPO) para estimular temporalmente la producción de las plaquetas en la medula ósea. No obstante, estos tratamientos tienden a abordar los síntomas más que las causas fundamentales de las alteraciones hematológicas en la enfermedad hepática. La persistencia y el empeoramiento de estas alteraciones pueden servir como indicadores tempranos de la progresión de la disfunción hepática. La relación intrincada entre el hígado y la homeostasis hematológica continúa siendo objeto de investigación, la compresión más profunda de estos mecanismos podría abrir potencialmente la puerta hacia enfoques terapéuticos más específicos y efectivos para abordar las citopenias en el contexto de la enfermedad hepática.

ABSTRACT Alterations in blood cell counts are the most prominent and recurrent clinical findings among patients suffering from both acute and chronic liver disease. These changes are an important marker of liver failure and often play a key role in the evaluation and management of these patients. Together with the prolongation of coagulation tests, thrombocytopenia is the most common disorder among these individuals. This condition, as well as leukopenia, is largely attributable to hypersplenism, a disorder in which the spleen retains and destroys blood cells, including platelets. However, when the platelet count drops below 10x103/µl, it is essential to consider other causes, such as autoimmune factors that may be contributing to the development of thrombocytopenia. Anemia, defined as a decrease in red blood cell count or hemoglobin levels, is another common characteristic of liver disease. Although in most cases macrocytic anemia occurs, in some situations it can be secondary to hemolytic events, as observed in Zieve's syndrome. This wide range of manifestations of anemia among liver patients highlights the complex interaction between liver and blood components. Despite advances in understanding the underlying causes of these cytopenias, treatment options remain limited. Therapeutic options generally focus on the transfusion of blood products to compensate for deficiencies in cell counts or on the use of thrombopoietin (TPO) analogues to temporarily stimulate platelet production in the bone marrow. However, these treatments tend to address the symptoms rather than the root causes of hematologic disorders in liver disease. The persistence and worsening of these disorders may serve as early indicators of the progression of liver failure. The complicated relationship between liver and hematological homeostasis remains the subject of research. A deeper understanding of these mechanisms could potentially open the door toward more targeted and effective therapeutic approaches to address cytopenias in the context of liver disease.

Efficacy of Partial Splenic Artery Embolization Versus Splenectomy for Hypersplenism Secondary to Liver Cirrhosis:a Meta-analysis / 医学研究杂志

Objective To systematically evaluate the efficacy of partial splenic artery embolization(PSE)and splenectomy in the treatment of secondary hypersplenism in liver cirrhosis.Methods PubMed,Cochrane Library,Embase,CNKI,Wan Fang were searched to collect randomized controlled trials and cohort studies about the efficacy of PSE versus splenectomy in the treatment of hyper-splenism secondary to liver cirrhosis from inception to October 30,2021.Two reviewers screened the literature,extracted data,and as-sessed the risk of bias of included studies.Meta-analysis was then conducted.Results A total of 14studies were included with 1092 patients.The results of the meta-analysis showed that there was no significant difference in postoperative leukocyte levels at 1 week,1 month,and 1 year after surgery between the PSE group and the splenectomy group.However,6months after surgery,the level of postop-erative leukocyte in the splenectomy group was significantly higher than that in the PSE group.For postoperative platelet counts,there was no significant difference at 1 month and 1 year after surgery between the two groups.However,1 week(MD=-65.46,95％CI:-116.39--14.52,P=0.01)and 6months(MD=-117.99,95％CI:-229.71--6.27,P=0.04)after surgery,the level of postoperative platelet in splenectomy group was significantly higher than that in PSE group.There was no significant difference in postoperative erythro-cyte levels at 1 week,1 month,and 1 year after surgery between the two groups.The level of postoperative natural killer cells in the PSE group was significantly higher than that in the splenectomy group at 1 month(MD=6.02,95％CI:4.27-7.77,P＜0.001)and 1 year(MD=3.53,95％CI:1.68-5.37,P=0.0002)after surgery.Compared with splenectomy group,PSE group exhibited less intraopera-tive bleeding(MD=-73.92,95％CI:-89.39--58.45,P＜0.001),less hospitalization costs(MD=-0.80,95％CI:-1.27--0.34,P=0.0008)and shorter length of stay(MD=-4.08,95％CI:-5.22--2.95,P＜0.001).Conclusion The current evi-dence shows that PSE has certain short-term and long-term effects on hypersplenism.Compared with splenectomy,it has less surgical trauma,less hospital stay and less cost,easy to control complications,and retains some immune function,which is worth spreading in the clinic.Limited by the quantity and quality of the included literature,more high-quality studies are needed to confirm the above conclu-sions.

Embolização de múltiplos aneurismas de artéria esplênica em paciente com hiperesplenismo por hipertensão portal: relato de caso / Embolization of multiple splenic artery aneurysms in a patient with hypersplenism due to portal hypertension: a case report

Resumo Os aneurismas de artéria esplênica são o terceiro tipo mais comum de aneurismas intra-abdominais, sendo considerados o tipo mais comum de aneurismas viscerais. A hipertensão portal é um fator de risco significativo para o seu desenvolvimento. Relatamos o caso de uma paciente do sexo feminino, branca, de 52 anos, com múltiplos aneurismas de artéria esplênica com hiperesplenismo por hipertensão portal e cirrose. Por meio da angiotomografia abdominal, foram identificados seis aneurismas esplênicos. Nesse contexto, optou-se por intervenção endovascular por meio da embolização com molas de destaque controlado e material embolizante Onyx™. Os três aneurismas maiores foram tratados. As angiografias de controle mostraram boa exclusão dos aneurismas. Portanto, a técnica endovascular foi uma boa opção devido às comorbidades e às discrasias sanguíneas apresentadas. Neste caso, o procedimento foi bem-sucedido. Não houve intercorrências imediatas ou complicações a longo prazo. A paciente evoluiu bem, seguindo em acompanhamento clínico.

Abstract Aneurysms of the splenic artery are the third most common type of intra-abdominal aneurysms and the most common type of visceral aneurysms. Portal hypertension is a significant risk factor for development of these aneurysms. We report the case of a white, female, 52-year-old patient with multiple splenic artery aneurysms and hypersplenism secondary to portal hypertension and cirrhosis. Abdominal angiotomography identified six splenic aneurysms. In this scenario, an endovascular intervention was scheduled to conduct embolization using controlled release coils and Onyx™ embolization agent. The three largest aneurysms were treated. Control angiographs showed good exclusion of the aneurysms. The endovascular technique therefore proved to be a good choice considering the patient's comorbidities and blood disorders. In this case, the procedure was successful. There were no immediate or long-term complications. The patient recovered well and is in clinical follow-up.

Normal platelet count and leukocytosis findings in chronic liver disease patient with portal hypertension: a rare case

Haematologic abnormalities are commonly encountered in chronic liver disease (CLD) due to hypersplenism occurred. Hypersplenism in CLD is a major cause of peripheral pancytopenia in patients with hepatic cirrhosis and portal hypertensive gastropathy and is characterized by splenomegaly. Peripheral pancytopenia is defined as a reduction in all three major constituents of the blood to below lower normal range, manifesting as anemia, leukopenia, and thrombocytopenia all occurring at the same time. We report an unusual case, a 44-year-old female patient, no splenomegaly, presented with severe anemia, leukocytosis and normal platelet which are rarely found in CLD.

Normal platelet count and leukocytosis findings in chronic liver disease patient with portal hypertension: a rare case

Haematologic abnormalities are commonly encountered in chronic liver disease (CLD) due to hypersplenism occurred. Hypersplenism in CLD is a major cause of peripheral pancytopenia in patients with hepatic cirrhosis and portal hypertensive gastropathy and is characterized by splenomegaly. Peripheral pancytopenia is defined as a reduction in all three major constituents of the blood to below lower normal range, manifesting as anemia, leukopenia, and thrombocytopenia all occurring at the same time. We report an unusual case, a 44-year-old female patient, no splenomegaly, presented with severe anemia, leukocytosis and normal platelet which are rarely found in CLD.

The mechanism of action of partial splenic artery embolization in treatment of liver cirrhosis and hypersplenism / 临床肝胆病杂志

Hypersplenism is a common complication caused by liver cirrhosis and portal hypertension, and at present, splenectomy and partial splenic artery embolization (PSE) are the main methods for the treatment of hypersplenism. Splenectomy has a marked effect in the treatment of hypersplenism and can significantly improve the clinical symptoms of patients with hypersplenism. Compared with splenectomy, PSE causes partial splenic parenchymal infarction and thus achieve similar clinical efficacy as partial splenectomy while preserving the spleen and its function. Although PSE is an effective method for the treatment of hypersplenism, there are few reports on the effect of PSE on liver fibrosis, immunity, and liver regeneration in China and globally. This article summarizes the common causes of hypersplenism, the mechanism of PSE in the treatment of hypersplenism, the therapeutic effect of different embolization methods and materials, and the effect of PSE on liver fibrosis, immunity, and liver regeneration, so as to provide a theoretical basis and new ideas for the clinical treatment of hypersplenism.

The pathological role of hypersplenism in the course of liver cirrhosis / 中华肝胆外科杂志

Hypersplenism is an important complication of cirrhotic portal hypertension, and splenectomy is an important means to treat hypersplenism in cirrhosis. It is realized that hypersplenism played a pathological role in the course of cirrhosis. This article analyzes and compares the changes in the condition of patients with cirrhosis between splenectomy with and without hyperfunction, and comprehensively discusses the pathological role and mechanism of hypersplenism in the course of cirrhosis, in order to strengthen the clinical prevention and treatment of hypersplenism in cirrhosis and to better improve the condition and prognosis of patients with cirrhosis.

A clinical study of endoscopic histoacryl injection for newly-developed esophagogastric varices in cirrhotic patients undergoing splenectomy combined with pericardial devascularization / 中华消化内镜杂志

Objective:To investigate the efficacy of endoscopic histoacryl injection in cirrhotic patients with newly-developed esophagogastric varices (EGV) who have previously undergone splenectomy combined with pericardial devascularization.Methods:From January 2015 to January 2020, 125 cirrhotic patients with EGV treated with endoscopic histoacryl injection at the Department of Gastroenterology, Jinling Hospital, Medical School of Nanjing University, were included in the retrospective analysis. There were 45 patients in the group of splenectomy combined with pericardial devascularization (splenectomy group for short) and 80 patients in the non-splenectomy group. The efficacy of endoscopic treatment, postoperative variceal improvement, rebleeding rate, and complications were analyzed between the two groups.Results:Endoscopic histoacryl injection was successfully completed in all 125 patients, and the median volume of histoacryl was 4.5 mL. The overall effective rate in splenectomy and non-splenectomy group was 80.0% (36/45) and 57.5% (46/80), respectively. The difference in the number of significantly effective, effective, and ineffective cases between the two groups was statistically significant (16, 20, 9 cases, and 20, 26, 34 cases, respectively, χ 2=6.469, P=0.039). Two and 14 patients developed rebleeding in the splenectomy group and non-splenectomy group, respectively; and the difference in the rebleeding rate between the two groups was statistically significant (4.4% VS 17.5%, Log-rank P=0.039). No patient died within 1 year in either group, and no serious complications such as ectopic embolism occurred. Conclusion:After splenectomy combined with pericardial devascularization in cirrhotic patients with EGV and hypersplenism, the application of histoacryl has better short-term efficacy and can significantly reduce the rebleeding rate compared with the non-splenectomy group.

Guías para el diagnóstico y seguimiento de niños y adolescentes portadores de hipertensión portal / Guidelines for diagnosis and follow-up of children and adolescents with portal hypertension

La hipertensión portal es un síndrome complejo producido por un aumento de la resistencia al flujo venoso esplácnico a nivel de la vena porta o sus ramas, con una circulación sistémica hiperdinámica caracterizada por vasodilatación periférica y aumento del gasto cardíaco. El sitio de obstrucción al flujo portal puede ser prehepático (hígado normal), intrahepático (como en la cirrosis) o posthepático (síndrome de BuddChiari). En los pacientes pediátricos, las causas prehepáticas e intrahepáticas se reparten en proporciones casi iguales (aproximadamente el 50 % cada una). La expresión clínica y el impacto individual son muy variados, pero en todos los casos expresan un deterioro en la salud de los pacientes y la necesidad de corregir el problema, tanto en sus consecuencias como, idealmente, en sus causas.

Portal hypertension is a complex syndrome caused by increased resistance to the splachnic venous flow at the portal vein level, with a hyperdynamic systemic circulation characterized by peripheral vasodilation and high cardiac output. Portal flow can be obstructed at prehepatic (¨normal liver¨), intrahepatic (as in cirrhosis), or post-hepatic level (as in Budd-Chiari syndrome). In pediatric patients, prehepatic and intrahepatic causes are almost equally distributed (nearly 50% each). Clinical presentation and individual impact are heterogeneous, but in each case, it is the expression of a worsening condition and the need to solve the problem, either by treating its consequences or (ideally) its causes.

Impact of intraoperative ligation of splenic artery on prognosis of liver transplantation on patients with severe hypersplenism / 中华肝胆外科杂志

Objective:To study the impact of simultaneous ligation of splenic artery on prognosis of patients with severe hypersplenism in liver transplantation.Methods:A retrospective analysis was performed on the clinical data of 206 patients who underwent liver transplantation in the Fifth Medical Center of PLA General Hospital from December 2016 to February 2019. There were 180 males and 26 females, aged (51.0±9.0) years old. Fifty-one patients underwent splenic artery ligation during liver transplantation and they were enrolled into the observation group, and 155 patients without splenic artery ligation were enrolled into the control group. The changes in white blood cells (WBC), platelets, alanine aminotransferase, total bilirubin and serum creatinine as well as the incidence of postoperative complications were compared between the two groups.Results:The platelet count of the observation group was significantly lower than those of the control group before operation and on days 1, 3, 7, 30 and 90 after operation, (all P<0.05). The WBC counts in the observation group were significantly lower than those in the control group before operation and on days 1 and 3 after operation (all P<0.05). However, there were no significant differences in the WBC counts between the two groups on days 5, 7, 30 and 90 after operation (all P>0.05). There were also no significant differences in alanine aminotransferase and total bilirubin indexes between the two groups after surgery (all P>0.05), but the serum creatinine levels in the observation group were significantly lower than those in the control group on days 3, 5, 7 and 30 after surgery (all P<0.05). There were no significant differences in the rates of infection, severe acute rejection, biliary tract complications, arterial/portal thrombosis and mental complications between the two groups (all P>0.05). The rate of renal replacement therapy for acute kidney injury in the observation group (9.8%, 5/55) was significantly higher than that in the control group (1.3%, 2/155) ( P<0.05). Conclusion:Ligation of splenic artery during liver transplantation was safe and it had a significant advantage in the early postoperative recovery of WBC count and creatinine without increasing the incidence of complications in patients with severe hypersplenism.

Clinical efficacy of Da Vinci robotic assisted vagus nerve-preserving splenectomy and azygo-portal disconnection / 中华消化外科杂志

Objective:To investigate the clinical efficacy of Da Vinci robotic assisted vagus nerve-preserving splenectomy and azygoportal disconnection.Methods:The retrospective and descriptive study was conducted. The clinicopathological data of 10 cirrhotic portal hypertension patients with esophagogastric variceal bleeding and hypersplenism who were admitted to Clinical Medical College of Yangzhou University from February to May 2021 were collected. There were 4 males and 6 females, aged from 43 to 64 years, with a median age of 55 years. All 10 patients underwent Da Vinci robotic assisted vagus nerve-preserving splenectomy and azygoportal discon-nection. Observation indicators: surgical situations, intraoperative autologous blood transfusion, conversion to open laparotomy, allogeneic blood transfusion, the operation time, volume of intra-operative blood loss, time to initial diet intake, time for out-of-bed activity, postoperative complica-tion, duration of postoperative hospital stay and follow-up. Follow-up was conducted using out-patient examination and telephone interview to detect recurrent gastrointestinal hemorrhage and gastric retention up to July 2021. Measurement data with normal distribution were represented as Mean± SD and measurement data with skewed distribution were represented as M(range). Count data were described as absolute numbers. Results:All 10 patients underwent Da Vinci robotic assisted nerve-preserving splenectomy and azygoportal disconnection successfully, with intraopera-tive autologous blood transfusion and without conversion to open laparotomy or allogeneic blood transfusion. The operation time, volume of intraoperative blood loss, time to initial diet intake and time for out-of-bed activity of 10 patients were (180±14)minutes, (111±28)mL, (1.5±0.5)days and (2.5±0.7)days, respectively. Of the 10 patients, 1 case underwent mild pancreatic leakage, 1 case underwent pneumonia, 2 cases underwent portal vein thrombosis and 3 cases underwent splenic vein thrombosis. Patients with postoperative complications was cured after conservative treatment. The duration of postoperative hospital stay of 10 patients was (8.9±0.9)days. All 10 patients were discharged without perioperative death and followed up for 1 to 4 months, with a median follow-up time of 3 months. There was no patient undergoing gastrointestinal hemorrhage and gastric reten-tion.Conclusion:Da Vinci robotic assisted vagus nerve-preserving splenectomy and azygoportal disconnection is safe and feasible for the treatment of cirrhotic portal hypertension patients with esophagogastric variceal bleeding and hypersplenism.

Non-selective versus highly selective partial splenic embolization in treating patients with hypersplenism secondary to liver cirrhosis: a comparative study / 中华肝胆外科杂志

Objective:To compare the clinical outcomes of treatment using non-selective versus highly selective partial splenic embolization in patients with hypersplenism secondary to liver cirrhosis.Methods:The clinical data of patients with hypersplenism secondary to hepatitis B cirrhosis who underwent splenic embolization at the Department of Hepatobiliary and Pancreatic Surgery of Leshan People's Hospital from July 2017 to July 2020 were analyzed retrospectively. Of 65 patients with hypersplenism, there were 42 males and 23 females, with age of (58.5±9.8) years. Twenty-six patients underwent splenic artery non-selective partial splenic embolization (the non-selective group) and 39 patients underwent partial splenic embolization using highly selective intubation (the highly selective group). The postoperative peripheral hematological indexes, liver function, operation-related complications and portal vein color Doppler ultrasonography were compared between the two groups.Results:The white blood cell count and platelet count of patients in the 2 groups were significantly higher than those before operation. The white blood cell count at 4, 12 and 24 weeks after operation and the platelet count at 12 and 24 weeks after operation in the highly selective group were significantly higher than those in the non-selective group ( P<0.05). Compared with the non-selective group, the total bilirubin, ICG-R15, portal vein diameter and portal vein blood flow in the highly-selective group significantly lower ( P<0.05). The incidences of 0/Ⅰ/Ⅱ/Ⅲ pain in the non-selected group was significantly higher when compared with that in the highly selected group (5/10/11/1 vs. 12/22/7/0), ( P<0.05). There were no significant differences in the incidences of postoperative complication between the two groups ( P>0.05). Conclusion:Compared with non-selective partial splenic embolization, highly selective partial splenic embolization gave more stable and lasting treatment outcomes in patients with hypersplenism caused by liver cirrhosis with better recovery of blood-related indicators, better improvement in postoperative liver function and relief of portal hypertension symptoms.

Research progress on the outcome of hypersplenism after liver transplantation for liver cirrhosis / 中华肝胆外科杂志

Hypersplenism is the most common splenic disease and usually refers to a clinical syndrome of increased splenic size and/or cytopenia due to various causes. Hypersplenism is most often secondary to cirrhotic hypertension. Liver transplantation can effectively relieve hypersplenism in patients with liver cirrhosis, but there are also some patients with persistent hypersplenism after liver transplantation or recurrence after remission. Other treatment modalities for postoperative intractable hypersplenism include splenectomy and partial splenic artery embolization. This article reviews the research progress of hypersplenism after liver transplantation for liver cirrhosis with hypersplenism.

O índice esplênico como preditor de sangramento e recidiva varicosa no seguimento tardio de paciente esquistossomóticos após tratamento endoscópico exclusivo / The splenic index as predictor of bleeding and variceal recurrence in the late follow-up of schistosomotic patients after exclusive endoscopic treatment

RESUMO - RACIONAL: O tratamento endoscópico das varizes esofágicas tem sido utilizado como principal intervenção em pacientes com hipertensão portal secundária à esquistossomose, mas com taxas significativas de recorrência de varizes esofágicas e ressangramento. Os resultados em longo prazo do tratamento endoscópico exclusivo são pouco estudados quanto à relação das dimensões esplênicas neste contexto. OBJETIVO: Avaliar, por meio da ultrassonografia, o índice esplênico e a dimensão longitudinal (craniocaudal) do baço como preditores de ressangramento e recorrência de varizes no seguimento tardio de pacientes esquistossomóticos não operados, após erradicação endoscópica das varizes esofágicas. MÉTODOS: Estudo observacional retrospectivo por meio da análise de prontuários de pacientes com diagnóstico de esquistossomose hepatoesplênica. A curva ROC foi usada para determinar o melhor ponto de corte para o índice esplênico médio como preditor de recorrência e sangramento. RESULTADOS: Foram analisados 54 pacientes, durante o período de 2002 a 2018. O tempo médio de seguimento foi de 8 anos. O índice esplênico provou ser um teste sensível em valores acima de 144 como preditor de ressangramento. Na análise da dimensão longitudinal, o valor acima de 20 cm apresentou teste estatisticamente significativo para recorrência de varizes e valor acima de 19 cm apresentou-se como teste muito sensível e estatisticamente significativo para ressangramento. CONCLUSÃO: A análise do índice esplênico e da dimensão craniocaudal, obtidos por ultrassonografia, podem predizer recorrência de varizes e ressangramento após erradicação endoscópica exclusiva.

ABSTRACT - BACKGROUND: Endoscopic treatment for esophageal variceal has been used as the main intervention in patients with portal hypertension secondary to schistosomiasis, but with significant rates of recurrence of esophageal variceal and rebleeding. The long-term results of exclusive endoscopic treatment are poorly studied as the relationship of the splenic dimensions in this context. AIM: The aim of this study was to identify, through ultrasonography, whether the splenic index and the longitudinal (craniocaudal) dimension of the spleen are the predictors of rebleeding and variceal recurrence in late follow-up of patients with nonoperated schistosomiasis, after endoscopic eradication of esophageal variceal. METHODS: This is a retrospective and observational study analyzing the medical records of patients diagnosed with hepatosplenic schistosomiasis. The receiver operating characteristic curve was used to determine the best cutoff point for the mean splenic index as a predictor of recurrence and bleeding. Results: A follow-up of 54 patients were analyzed during the period from 2002 to 2018. The mean follow-up time was 8 years. The splenic index with value >144 was proved to be a sensitive test for rebleeding. In the analysis of the longitudinal dimension, the spleen length of >20 cm showed a statistically significant test for recurrence of variceal and a length >19 cm presented as a very sensitive and statistically significant test for rebleeding. CONCLUSION: Splenic index and craniocaudal dimension analysis, obtained by ultrasonography, can predict recurrence of varicose veins and rebleeding after exclusive endoscopic treatment.

Comparison of the efficacy of PVA particles and Embosphere microspheres in the treatment of hypersplenism / 实用放射学杂志

Objective To compare and analyze the efficacy of PVA particles and Embosphere microspheres for partial splenic embolization (PSE)in the treatment of hypersplenism secondary to liver cirrhosis.Methods Sixty patients underwent PSE for treatment of hypersplenism secondary to liver cirrhosis were analyzed retrospectively.According to different PSE embolization materials,they were divided into two groups:30 patients with PVA particles embolization (group A),and other 30 patients with Embosphere microspheres embolization (group B).The changes of white blood cells (WBC),platelets (PLT),spleen thickness,portal vein diameter and postoperative pain and fever were compared on the 3rd day,the 1st week,the 3rd week,the 1st month,the 3rd month,and the 6th month after PSE and compared between the two groups.Results The WBC and PLT of the 2 groups after PSE were significantly higher than those before PSE (all P＜0.05 ).The spleen shrank after 2 months,and there was no significant difference in spleen thickness and portal vein diameter between the two groups at the same time (all P＞0.05).The postoperative pain in group A was significantly less than that in group B (P＜0.05).Conclusion Both PVA particles and Embosphere microspheres have good curative effect in the treatment of hypersplenism secondary to liver cirrhosis. The price of PVA particles is lower than that of Embosphere microspheres,and the postoperative pain degree is lighter than that of Embosphere microsphere,which suggests PVA particles with more advantages in clinical application.

The improvement of splenomegaly and hypersplenism after liver transplantation in children / 中华肝胆外科杂志

Objective@#To investigate the recovery of splenomegaly and hypersplenism after liver transplantation in children and explore the necessity of splenomegaly management before and during liver transplantation.@*Method@#The data of 22 children who were underwent liver transplantation with preoperative splenomegaly and hypersplenism who were not treated with splenomegaly before and during operation and with no recurrence of portal hypertension during postoperative follow-up in Department of Hepatobiliary Surgery, Children’s Hospital of Chongqing Medical University from December 2008 to January 2019 were collected. There were 13 male patients and 9 female patients with a median age of 6.5 months. The changes of erythrocyte, platelet, white blood cell and spleen length and thickness were analyzed by paired t-test before and at the 1st, 3rd and 6th months after surgery. Correlation analysis was performed on the change of spleen long diameter and thickness product and blood cell recovery.@*Results@#There were 21 cases of red blood cell decreased before surgery, with a mean value of(3.1±0.5)×1012/L, and(3.7±0.7)×1012/L, (4.6±0.6)×1012/L and (4.3±0.5)×1012/L at the 1st, 3rd and 6th months after operation, respectively. The differences between the preoperative and postoperative groups were statistically significant (P<0.05). There were 7 cases of thrombocytopenia before operation, with an average of (70.0±17.0)×109/L, and (191.0±129.0)×109/L, (156.0±79.0)×109/L and (167.0±63.0)×109/L at the 1st, 3rd and 6th months after operation, respectively. The differences between the first, third, and sixth months after surgery were significant compared with that before surgery (P<0.05). Leukocyte count decreased in 5 cases with an average value of (3.30±0.56)×109/L before surgery, (7.5±4.4)×109/L, (7.4±1.4)×109/L, (5.1± 2.5)×109/L at the 1st, 3rd and 6th months after operation, respectively. The differences between the two groups were significant (P<0.05). The average value of preoperative spleen length times thickness was (35.7±12.9) cm2 and at the 1st, 3rd, 6th month after surgery it was (26.2±8.1)cm2, (25.2±13.4)cm2, (27.2±7.4)cm2, respectively. The differences between the first, third months after surgery were significant compared with that before surgery (P<0.05). The white blood cell and platelet counts of children before and after surgery were correlated with spleen size negatively (correlation coefficients were -0.902, -0.933, respectively), and the differences were statistically significant (P<0.05).@*Conclusions@#In the early stage of liver transplantation, the size of spleen retracts to varying degrees, and hypersplenism can be alleviated continuously. Therefore, it is unnecessary to treat splenomegaly and hypersplenism before and during liver transplantation.

The research advances on treatment of secondary hypersplenism in the patients with cirrhosis and portal hypertension / 中华肝胆外科杂志

Hypersplenism is a common and serious complication in patients with cirrhosis and portal hypertension. Currently, the therapeutic options for severe hypersplenism mainly include total splenectomy, partial splenic embolization, local thermal ablation, splenic artery ligation, surgical shunt, and partial splenectomy. This article discussed the current progress in the relative mechanisms and invasive treatment of severe secondary hypersplenism in the patients with cirrhosis and portal hypertension.

The improvement of splenomegaly and hypersplenism after liver transplantation in children / 中华肝胆外科杂志

Objective To investigate the recovery of splenomegaly and hypersplenism after liver transplantation in children and explore the necessity of splenomegaly management before and during liver transplantation.Method The data of 22 children who were underwent liver transplantation with preopera-tive splenomegaly and hypersplenism who were not treated with splenomegaly before and during operation and with no recurrence of portal hypertension during postoperative follow-up in Department of Hepatobiliary Surgery,Children's Hospital of Chongqing Medical University from December 2008 to January 2019 were collected.There were 13 male patients and9 female patients with a median age of 6.5 months.The changes of erythrocyte,platelet,white blood cell and spleen length and thickness were analyzed by paired t-test before and at the 1st,3rd and 6th months after surgery.Correlation analysis was performed on the change of spleen long diameter and thickness product and blood cell recovery.Results There were 21 cases of red blood cell decreased before surgery,with a mean value of(3.1±0.5)X 1012/L,and(3.7±0.7)×1012/L,(4.6±0.6)×1012/L and(4.3±0.5)×1012/L at the 1st,3rd and 6th months after operation,respective-ly.The differences between the preoperative and postoperative groups were statistically significant(P＜0.05).There were 7 cases of thrombocytopenia before operation,with an average of(70.0±17.0)×109/L,and(191.0±129.0)×109/L,(156.0±79.0)×109/L and(167.0±63.0)×109/L at the 1st,3rd and 6th months after operation,respectively.The differences between the first,third,and sixth months after sur-gery were significant compared with that before surgery(P＜0.05).Leukocyte count decreased in 5 cases with an average value of(3.30±0.56)×109/L before surgery,(7.5±4.4)×109/L,(7.4±1.4)× 109/L,(5.1±2.5)×109/L at the 1st.3rd and 6th months after operation,respectively.The differences between the two groups were significant(P＜0.05).The average value of preoperative spleen length times thickness was(35.7±12.9)em2 and at the 1st,3rd.6th month after surgery it was(26.2±8.1)cm2,(25.2±13.4)cm2,(27.2±7.4)cm2,respectively.The differences between the first,third months after surgery were significant compared with that before surgery(P＜0.05).The white blood cell and platelet counts of children before and after surgery were correlated with spleen size negatively(correlation coefficients were-0.902,-0.933,respectively),and the differences were statistically significant(P＜0.05).Conclusions In the early stage of liver transplantation,the size of spleen retracts to varying degrees,and hypersplenism can be alleviated continuously.Therefore,it is unnecessary to treat splenomegaly and hyper-splenism before and during liver transplantation.

The research advances on treatment of secondary hypersplenism in the patients with cirrhosis and portal hypertension / 中华肝胆外科杂志

Hypersplenism is a common and serious complication in patients with cirrhosis and portal hypertension.Currently,the therapeutic options for severe hypersplenism mainly include total splenectomy,partial splenic embolization,local thermal ablation,splenic artery ligation,surgicat shunt,and partial sple-nectomy.This article discussed the current progress in the relative mechanisms and invasive treatment of severe secondary hypersplenism in the patients with cirrhosis and portal hypertension.

Gaucher's disease with bone destruction as main performance: A case report and literature review / 吉林大学学报(医学版)

Objective: To analyze the clinical characteristics of a patient with of Gaucher's disease with bone destruction as the main performance, and to provide the reference for its clinical diagnosis and treatment. Methods: The clinical data of a case of Gaucher's disease with bone destruction as the main performance was summarized. Combined with the related literature review, the clinical performance, complications, treatment plan, prognosis and the latest relevant research progress of the patients with Gaucher's disease were analyzed. Results: A male patient, 49 years old, was admitted to the hospital due to intermittent right thigh pain for two years, with family history of Gaucher's disease and his parents were consanguineous marriage. The patient was diagnosed as Gaucher's disease 7 years ago. Combined with the patient's history, clinical performance, family history and various auxiliary examination results, the patient was diagnosed as Gaucher's disease, right femur bone destruction, hypersplenism. The patient's right femur was extensively damaged, and the right thigh was severely painful. The patient could not afford the long-term enzyme replacement treatment and planned to have a lesion resection. Because hypersplenism and thrombocytopenia were contraindication for surgery, partial embolization of the splenic artery was performed twice (the first embolization volume was 25%, and the second embolization volume was 35%). Blood cell count was performed one week after spleen embolization, which was significantly improved: platelet count 87×109L-1, hemoglobin 91 g · L-1, and surgical treatment was planned. However, the pain was relieved and the patient gave up surgery. The patient was recommended to avoid injury, reduce weight-bearing, and prevent the pathological fractures; the patient was followed up regularly. Conclusion: The incidence of Gaucher's disease is increased in consanguineous marriage. A preliminary diagnosis was made based on the family history, clinical performance, and various auxiliary examinations, and further confirmed according to the detection of glucocerebrosidase activity. The patients with type I Gaucher's disease are eligible for enzyme replacement therapy. Without enzyme replacement therapy, the patients with chronic history will be concurrent bone destruction, hypersplenism, need symptomatic treatment such as surgery.