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1.
BMJ Case Rep ; 14(2)2021 Feb 08.
Artigo em Inglês | MEDLINE | ID: mdl-33558379

RESUMO

Rapidly progressive ascites is a frequent clinical manifestation of advanced abdominal malignancies or portal hypertension due to liver diseases. We report a case of 61-year-old man who presented with rapidly progressive ascites. The presence of ascites, generalised lymphadenopathy, osteosclerosis on imaging and hepatosplenomegaly initially pointed towards the diagnosis of advanced high-grade lymphoma or accelerated myeloid neoplasm. Lymph node biopsy revealed infiltration by CD45, cKIT and CD30; tryptase and toluidine blue-positive mast cells (MCs). Bone marrow examination revealed infiltration by MCs and next generation sequencing revealed the pathognomic exon 17 D 816V KIT mutation. The patient was started on weekly pegylated interferon with significant symptom relief. Systemic mastocytosis should be considered as a differential diagnosis in a clinical case of ascites of unknown aetiology even in the absence of typical skin manifestations.


Assuntos
Ascite/etiologia , Linfadenopatia/etiologia , Mastocitose Sistêmica/diagnóstico , Osteosclerose/etiologia , Biópsia , Exame de Medula Óssea , Diagnóstico Diferencial , Hepatomegalia/etiologia , Humanos , Interferon-alfa/uso terapêutico , Linfonodos/patologia , Masculino , Mastocitose Sistêmica/complicações , Mastocitose Sistêmica/tratamento farmacológico , Pessoa de Meia-Idade , Polietilenoglicóis/uso terapêutico , Proteínas Recombinantes/uso terapêutico , Esplenomegalia/etiologia , Tomografia Computadorizada por Raios X
2.
Medicine (Baltimore) ; 100(1): e24081, 2021 Jan 08.
Artigo em Inglês | MEDLINE | ID: mdl-33429772

RESUMO

BACKGROUND: Liver cirrhosis is a common clinical chronic progressive disease. Due to the obstruction of blood flow after cirrhosis, it leads to long-term congestion of splenic sinus, hyperplasia of fibrous tissue and proliferation of splenic myeloid cells, resulting in hepatocirrhosis and splenomegaly. At present, western medicine still uses splenectomy and interventional therapy are the main treatment, but the adverse reactions are more and the curative effect is not good. Many clinical trials have proved that Traditional Chinese medicine has a great therapeutic effect on Hepatocirrhosis with splenomegaly, which can effectively delay the development of the disease and improve the survival rate of patients. This systematic review aims to evaluate the efficacy and safety of Traditional Chinese medicine in the treatment of hepatocirrhosis with splenomegaly. METHODS: The databases of Pubmed, CENTRAL (The Cochrane Central Register of Controlled Trials), China National Knowledge Infrastructure (CNKI), Wanfang Data Knowledge Service Platform (WANFANG Data), Weipu Information Chinese Periodical Service Platform (VIP), and China Biomedical Literature Service System (SinoMed) will be searched online to collect randomized controlled trials related to the treatment of hepatocirrhosis with splenomegaly with Traditional Chinese medicine The time is limited from the construction of the library to November 2020. We will use the criteria provided by Cochrane 5.1.0 for quality assessment and risk assessment of the included studies, and use the Revman 5.3 and Stata 13.0 software so as to systematically review the effectiveness of Traditional Chinese medicine for hepatocirrhosis with splenomegaly. ETHICS AND DISSEMINATION: This systematic review will evaluate the efficacy and safety of Traditional Chinese medicine for hepatocirrhosis with splenomegaly. Because all data used in this systematic review and meta-analysis have been published, this review does not require ethical approval. In addition, all data will be analyzed anonymously during the review process. RESULTS: In this study, we will evaluate the efficacy of Traditional Chinese medicine in the treatment of cirrhosis with splenomegaly. CONCLUSION: The conclusion of this study will be evidence to ensure the efficacy of Traditional Chinese medicine© in the treatment of cirrhosis with splenomegaly and provide guidance for its treatment. TRIAL REGISTRATION NUMBER: INPLASY2020110121.


Assuntos
Protocolos Clínicos , Hipertensão Portal/tratamento farmacológico , Medicina Tradicional Chinesa/normas , Esplenomegalia/tratamento farmacológico , Fibrose/complicações , Humanos , Hipertensão Portal/etiologia , Medicina Tradicional Chinesa/métodos , Metanálise como Assunto , Esplenomegalia/etiologia , Revisões Sistemáticas como Assunto , Resultado do Tratamento
3.
BMJ Case Rep ; 14(1)2021 Jan 12.
Artigo em Inglês | MEDLINE | ID: mdl-33436357

RESUMO

The objectives of this article are to present a case of type II cryoglobulinemic vasculitis, explain why mucosa-associated lymphoid tissue (MALT) lymphoma is an unusual cause of type II cryoglobulins and to discuss the aetiology, epidemiology, pathophysiology and treatment of cryoglobulinemic vasculitis. A 67-year-old woman presented with 4 months of weight loss, intermittent epistaxis and a purpuric skin rash. Prior to presentation, she was found to have an elevated rheumatoid factor. Further investigation revealed an acute kidney injury and elevated type II cryoglobulins suspicious for cryoglobulinemic vasculitis, which was confirmed by kidney biopsy. Additional workup for the weight loss included biopsy of newly found splenomegaly. Pathology revealed MALT lymphoma, a rare cause of type II cryoglobulinemic vasculitis. Successful medical therapy required treating the underlying malignancy with rituximab and high-dose steroids. After initial resolution of symptoms with this regimen, the patient's vasculitis worsened, which was thought to be secondary to undertreatment of the lymphoma. Bendamustine was added to further treat the lymphoma, after which the patient recovered and was able to discharge without recurrence of symptoms at 6 months.


Assuntos
Crioglobulinemia/etiologia , Linfoma de Zona Marginal Tipo Células B/complicações , Vasculite/etiologia , Lesão Renal Aguda/etiologia , Corticosteroides/uso terapêutico , Idoso , Antineoplásicos Imunológicos/uso terapêutico , Cloridrato de Bendamustina/uso terapêutico , Feminino , Humanos , Linfoma de Zona Marginal Tipo Células B/tratamento farmacológico , Rituximab/uso terapêutico , Esplenomegalia/etiologia
4.
AJR Am J Roentgenol ; 216(3): 698-703, 2021 03.
Artigo em Inglês | MEDLINE | ID: mdl-33439047

RESUMO

OBJECTIVE. Splenomegaly and thrombocytopenia are common complications in patients with cirrhosis. The present study aimed to evaluate changes in splenic volumes and platelet counts after TIPS insertion. MATERIALS AND METHODS. A total of 104 patients who had a diagnosis of portal hypertension and had undergone TIPS placement between November 2015 and August 2019 were enrolled in this retrospective cohort study. We retrospectively calculated splenic volumes before TIPS placement and at 1-2 and 6-12 months after TIPS placement and monitored the platelet count at 1, 3, 6, and 12 months after TIPS placement. RESULTS. The mean (± SD) portal pressure gradient before TIPS placement was 28.3 ± 4.6 mm Hg; after TIPS placement, it was 11.3 ± 4.5 mm Hg (p < .001). The mean splenic volume of all 104 patients before TIPS placement was 868 ± 409 cm3, and at 1-2 months after TIPS placement, it was 710 ± 336 cm3 (p < .001). Among the 43 patients for whom splenic volume data were available at both 1-2 and 6-12 months after TIPS placement, the mean splenic volume decreased from 845 ± 342 cm3 to 691 ± 301 cm3 and then to 674 ± 333 cm3, respectively. Correspondingly, the number of patients with severe thrombocytopenia decreased from 25 patients (35.7%) before the TIPS procedure to 16 patients (22.9%) in the 1-2 months after TIPS placement and then to 11 patients (15.7%) in the 6-12 months after TIPS implantation. The increase in the platelet count was significantly correlated with decreasing splenic volume (r2 = 0.3735; p < .001). CONCLUSION. In most patients, TIPS placement resulted in a significant decrease in splenic volume and a significant increase in the platelet count during the same period.


Assuntos
Hipertensão Portal/cirurgia , Cirrose Hepática/complicações , Derivação Portossistêmica Transjugular Intra-Hepática , Baço/patologia , Esplenomegalia/terapia , Trombocitopenia/terapia , Adulto , Idoso , Pressão Sanguínea , Feminino , Veias Hepáticas , Hepatite B/complicações , Hepatite C/complicações , Humanos , Hipertensão Portal/etiologia , Cirrose Hepática/virologia , Masculino , Pessoa de Meia-Idade , Tamanho do Órgão , Contagem de Plaquetas , Estudos Retrospectivos , Esplenomegalia/etiologia , Esplenomegalia/patologia , Trombocitopenia/etiologia
5.
Clin Imaging ; 69: 369-373, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-33070084

RESUMO

Acute splenic sequestration crisis, the sudden pooling of red blood cells in the spleen, is an emergent process typically seen in children with homozygous sickle cell disease. Splenic sequestration has rarely been reported in adults with heterozygous sickle cell conditions, including sickle cell beta(+)-thalassemia disease (HbS/ß+-thalassemia). Here we present a case of a 32-year-old man with HbS/ß+-thalassemia who suffered an acute splenic sequestration crisis. We review the CT and ultrasound appearance of splenic sequestration, which include splenic enlargement and an irregular rim of hypoenhancing or hypoechoic tissue at the periphery of the spleen, and discuss imaging differential considerations. To our knowledge, this is only the nineteenth case of acute splenic sequestration to be reported in an adult with HbS/ß+-thalassemia in the English literature, and only the second case in which ultrasound findings are reported.


Assuntos
Anemia Falciforme , Hiperesplenismo , Esplenopatias , Talassemia beta , Adulto , Criança , Humanos , Masculino , Esplenopatias/diagnóstico por imagem , Esplenomegalia/diagnóstico por imagem , Esplenomegalia/etiologia , Talassemia beta/complicações , Talassemia beta/diagnóstico por imagem
6.
Expert Opin Drug Saf ; 20(2): 139-154, 2021 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-33327810

RESUMO

INTRODUCTION: During the last decade, the development of small molecule inhibitors of Janus kinases (JAKi) contributed to revolutionize the therapeutic landscape of myelofibrosis (MF). JAKi proved to be effective in controlling disease-related symptoms and splenomegaly with remarkable inter-drug variability. However, in some cases the border between clinical efficacy of JAKi and dose-dependent toxicities is narrow leading to sub-optimal dose modifications and/or treatment discontinuation. AREAS COVERED: In the current review, the authors aimed at providing a comprehensive review of the safety profile of JAKi that are currently approved or in advanced clinical development. Also, a short discussion of promising JAKi in early clinical evaluation and molecules 'lost' early in clinical development is provided. Finally, we discuss the possible strategies aimed at strengthening the safety of JAKi while improving the therapeutic efficacy. EXPERT OPINION: Overall, JAKi display a satisfactory risk-benefit ratio, with main toxicities being gastrointestinal or related to the myelo/immunosuppressive effects, generally mild and easily manageable. However, JAKi may be associated with potentially life-threatening toxicities, such as neurological and infectious events. Thus, many efforts are needed in order to optimize JAKi-based therapeutic strategies without burdening patient safety. This could be attempted through drug combinations or the development of more selective molecules.


Assuntos
Inibidores de Janus Quinases/administração & dosagem , Mielofibrose Primária/tratamento farmacológico , Relação Dose-Resposta a Droga , Desenvolvimento de Medicamentos , Humanos , Inibidores de Janus Quinases/efeitos adversos , Mielofibrose Primária/fisiopatologia , Esplenomegalia/tratamento farmacológico , Esplenomegalia/etiologia
7.
BMJ Case Rep ; 13(12)2020 Dec 29.
Artigo em Inglês | MEDLINE | ID: mdl-33376090

RESUMO

Autoimmune hepatitis (AIH) is an autoimmune liver disease characterised by the presence of autoantibodies including antinuclear antibodies, anti-smooth muscle antibody and hypergammaglobulinaemia. Systemic lupus erythematosus (SLE) is a systemic disease that can affect multiple organs. Coexistence of AIH and SLE as an overlap syndrome occurs in about 1%-2.6% of the AIH cases. Since both conditions share common autoimmune features, their coexistence can pose a diagnostic dilemma which can result in a delay in treatment. We present here a challenging case of a middle-aged woman with AIH in remission who later developed new-onset fatigue, pleural effusion and splenomegaly.


Assuntos
Autoanticorpos/sangue , Hepatite Autoimune , Imunossupressores/administração & dosagem , Lúpus Eritematoso Sistêmico , Derrame Pleural , Esplenomegalia , Biópsia/métodos , Comorbidade , Diagnóstico Diferencial , Progressão da Doença , Feminino , Hepatite Autoimune/diagnóstico , Hepatite Autoimune/epidemiologia , Hepatite Autoimune/imunologia , Hepatite Autoimune/terapia , Humanos , Fígado/patologia , Testes de Função Hepática/métodos , Lúpus Eritematoso Sistêmico/epidemiologia , Lúpus Eritematoso Sistêmico/imunologia , Lúpus Eritematoso Sistêmico/fisiopatologia , Lúpus Eritematoso Sistêmico/terapia , Linfadenopatia/diagnóstico , Linfadenopatia/etiologia , Pessoa de Meia-Idade , Derrame Pleural/diagnóstico , Derrame Pleural/etiologia , Esplenomegalia/diagnóstico por imagem , Esplenomegalia/etiologia , Tomografia Computadorizada por Raios X/métodos , Resultado do Tratamento
8.
Medicine (Baltimore) ; 99(39): e22299, 2020 Sep 25.
Artigo em Inglês | MEDLINE | ID: mdl-32991435

RESUMO

INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) is a condition characterized by a hyperinflammatory state and persistent macrophage activation, resulting in reactive phagocytosis of the hematopoietic elements. In children, it is usually a hereditary disorder, while in adults it is usually acquired secondary to viral infections, collagenoses, or tumors. Although accounting for 10% of hematologic malignancies, HLH is rarely associated with multiple myeloma (MM) and other plasmacytic dyscrasias. PATIENT CONCERNS: A 64-year-old Brazilian man seeked medical care with a 3-month history of intermittent fever, weight loss, night sweats, and progressive anemic symptoms. DIAGNOSIS: Total blood count showed severe bicytopenia (normocytic-normochromic anemia and thrombocytopenia), biochemical exams showed elevation of creatinine, as well as monoclonal peak in serum protein electrophoresis, high IgA dosage, and serum immunofixation with IgA kappa paraprotein. Bone marrow biopsy showed 30% of monoclonal and phenotypically anomalous plasmocytes, confirming the diagnosis of MM. Diagnosis of HLH was established by the presence of clinical and laboratory criteria: fever, splenomegaly, cytopenias, hypofibrinogenemia, hyperferritinemia, elevation of triglycerides, and several figures of erythrophagocytosis in bone marrow aspirate. INTERVENTIONS: The patient experienced pulse therapy with methylprednisolone for hemophagocytic lymphohistiocytosis, followed by initial therapy for multiple myeloma with cyclophosphamide and dexamethasone. OUTCOMES: Once the diagnosis of MM and secondary hemophagocytic syndrome was established, the patient had a rapid clinical deterioration despite the established therapeutic measures, evolving with cardiovascular failure, acute liver failure, acute disseminated intravascular coagulation, worsening renal dysfunction requiring dialysis support, respiratory dysfunction, and lowering of consciousness, characterizing rapid multiple organ dysfunction, ultimately leading to the death of the patient. INNOVATION: Here, we aimed to describe the sixth reported case of HLH associated with MM, according to cases cataloged in the PubMed database, and the first case evaluated by 18-fluordeoxyglucose positron emission tomography (18-FDG-PETCT). CONCLUSION: Our case report seeks to provide support for a better clinical and laboratory characterization of this rare paraneoplastic entity associated with MM, and aims to call the attention of hematologists and intensivists to this condition that falls within the scope of the differential diagnosis of rapid onset multiple organ failure in patients with plasmacytic neoplasms.


Assuntos
Linfo-Histiocitose Hemofagocítica/tratamento farmacológico , Linfo-Histiocitose Hemofagocítica/etiologia , Mieloma Múltiplo/complicações , Mieloma Múltiplo/tratamento farmacológico , Anemia/sangue , Anemia/etiologia , Antineoplásicos Alquilantes/administração & dosagem , Antineoplásicos Alquilantes/uso terapêutico , Medula Óssea/patologia , Brasil/epidemiologia , Ciclofosfamida/administração & dosagem , Ciclofosfamida/uso terapêutico , Dexametasona/administração & dosagem , Dexametasona/uso terapêutico , Quimioterapia Combinada , Evolução Fatal , Febre/diagnóstico , Febre/etiologia , Glucocorticoides/administração & dosagem , Glucocorticoides/uso terapêutico , Humanos , Linfo-Histiocitose Hemofagocítica/sangue , Linfo-Histiocitose Hemofagocítica/diagnóstico , Masculino , Metilprednisolona/administração & dosagem , Metilprednisolona/uso terapêutico , Pessoa de Meia-Idade , Mieloma Múltiplo/metabolismo , Insuficiência de Múltiplos Órgãos/complicações , Paraproteinemias/sangue , Plasmócitos/patologia , Esplenomegalia/diagnóstico , Esplenomegalia/etiologia , Trombocitopenia/sangue , Trombocitopenia/etiologia , Perda de Peso
9.
Adv Clin Exp Med ; 29(7): 879-886, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32745379

RESUMO

BACKGROUND: The prognostic value and non-invasive predictors of splenomegaly in cirrhotic patients with hepatocellular carcinoma (HCC) after curative resection remain unknown. OBJECTIVES: To investigate the prognostic value and non-invasive predictors of splenomegaly in cirrhotic patients with HCC after curative resection. MATERIAL AND METHODS: The medical records of 78 patients with HCC and liver cirrhosis who underwent curative resection were retrospectively reviewed. The influence of spleen size, measured with clinically routine ultrasonography (USG), on overall and disease-free survival was evaluated using univariate and multivariate analyses. The efficiency of some frequently used blood-derived liver function parameters and non-invasive fibrosis markers to predict splenomegaly was also assessed. RESULTS: It was shown that tumor size >5 cm, the presence of microvascular invasion, tumor-node metastasis (TNM) stage III-IVA of the tumor, spleen size >11.45 cm, and age ≤52 years were associated with poor overall survival and/or disease-free survival in univariate analyses (all p < 0.05). In multivariate analyses, spleen size was identified as an independent predictor for both overall and disease-free survival (p < 0.001 and p = 0.012, respectively). On the other hand, platelet count, aspartate aminotransferase (AST) to platelet ratio index (APRI) and Fibrosis-4 index (FIB-4) scores were significantly different between small and large spleen groups (p = 0.026, 0.003 and 0.003, respectively), while statistical differences for albumin, alanine aminotransferase (ALT), AST, total bilirubin, AST to ALT ratio (AAR), and age-platelet index (API) were not found. Using receiver operating characteristic (ROC) curves, high powers of platelet count, APRI and FIB-4 in splenomegaly prediction were confirmed. CONCLUSIONS: Splenomegaly, which can be predicted by some non-invasive variables, serves as a strong determinant for postresectional prognoses of cirrhotic patients with HCC.


Assuntos
Carcinoma Hepatocelular , Neoplasias Hepáticas , Aspartato Aminotransferases , Carcinoma Hepatocelular/etiologia , Carcinoma Hepatocelular/cirurgia , Humanos , Cirrose Hepática/complicações , Neoplasias Hepáticas/etiologia , Neoplasias Hepáticas/cirurgia , Prognóstico , Curva ROC , Estudos Retrospectivos , Esplenomegalia/diagnóstico por imagem , Esplenomegalia/etiologia
11.
Surgery ; 168(3): 434-439, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32600882

RESUMO

BACKGROUND: Pancreatoduodenectomy with synchronous resection of the portal vein/superior mesenteric vein confluence may result in the development of left-sided portal hypertension. Left-sided portal hypertension presents with splenomegaly and varices and may cause severe gastrointestinal bleeding. The aim of the study is to review the incidence, treatment, and preventive strategies of left-sided portal hypertension. METHODS: A systematic literature search was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement to identify all studies published up to September 30, 2019 reporting data on patients with left-sided portal hypertension after pancreatoduodenectomy with venous resection. RESULTS: Eight articles including 829 patients were retrieved. Left-sided portal hypertension occurred in 7.7% of patients who had splenic vein preservation and 29.4% of those having splenic vein ligation. Fourteen cases of gastrointestinal bleeding owing to left-sided portal hypertension were reported at a mean interval of 28 months from pancreatoduodenectomy. Related mortality at 1 month was 7.1%. Treatment of left-sided portal hypertension consisted of splenectomy in 3 cases (21%) and colectomy in 1 (7%) case, whereas radiologic, endoscopic procedures or conservative treatments were effective in the other cases (71%). CONCLUSION: Left-sided portal hypertension represents a potentially severe complication of pancreatoduodenectomy with venous resection occurring at greater incidence when the splenic vein is ligated and not reimplanted. Left-sided portal hypertension-related gastrointestinal bleeding although rare can be managed depending on the situation by endoscopic, radiologic procedures or operative intervention with low related mortality.


Assuntos
Hipertensão Portal/epidemiologia , Veias Mesentéricas/cirurgia , Pancreaticoduodenectomia/efeitos adversos , Veia Porta/cirurgia , Complicações Pós-Operatórias/epidemiologia , Carcinoma Ductal Pancreático/cirurgia , Colectomia/estatística & dados numéricos , Tratamento Conservador/estatística & dados numéricos , Hemorragia Gastrointestinal/epidemiologia , Hemorragia Gastrointestinal/etiologia , Hemorragia Gastrointestinal/terapia , Humanos , Hipertensão Portal/etiologia , Hipertensão Portal/terapia , Incidência , Ligadura/efeitos adversos , Neoplasias Pancreáticas/cirurgia , Pancreaticoduodenectomia/métodos , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/terapia , Esplenectomia/estatística & dados numéricos , Esplenomegalia/epidemiologia , Esplenomegalia/etiologia , Esplenomegalia/terapia , Resultado do Tratamento
12.
Pediatrics ; 146(1)2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32532792

RESUMO

An 8-year-old boy with no significant past medical history presented to his pediatrician with 5 days of fever, diffuse abdominal pain, and pallor. The pediatrician referred the patient to the emergency department (ED), out of concern for possible malignancy. Initial vital signs indicated fever, tachypnea, and tachycardia. Physical examination was significant for marked abdominal distension, hepatosplenomegaly, and abdominal tenderness in the right upper and lower quadrants. Initial laboratory studies were notable for pancytopenia as well as an elevated erythrocyte sedimentation rate and C-reactive protein. Computed tomography (CT) of the abdomen and pelvis showed massive splenomegaly. The only significant history of travel was immigration from Albania 10 months before admission. The patient was admitted to a tertiary care children's hospital and was evaluated by hematology-oncology, infectious disease, genetics, and rheumatology subspecialty teams. Our multidisciplinary panel of experts will discuss the evaluation of pancytopenia with apparent multiorgan involvement and the diagnosis and appropriate management of a rare disease.


Assuntos
Febre/diagnóstico , Leishmaniose Visceral/complicações , Pancitopenia/diagnóstico , Esplenomegalia/diagnóstico , Criança , Diagnóstico Diferencial , Febre/etiologia , Humanos , Leishmania/isolamento & purificação , Leishmaniose Visceral/diagnóstico , Masculino , Pancitopenia/etiologia , Esplenomegalia/etiologia , Tomografia Computadorizada por Raios X
13.
Surgery ; 168(2): 267-273, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32536489

RESUMO

BACKGROUND: The ligation of the splenic vein during pancreaticoduodenectomy with synchronous resection of the spleno-mesenteric-portal venous confluence has been associated with the development of left portal hypertension despite preservation of the natural confluence with the inferior mesenteric vein. This study aimed to assess whether a left splenorenal venous shunt might mitigate clinical signs of left portal hypertension associated with splenic vein ligation. METHODS: We retrospectively evaluated the presence of left portal hypertension based on biologic and radiologic parameters in patients undergoing pancreaticoduodenectomy with synchronous resection of the spleno-mesentericoportal confluence between January 1, 2012, and December 31, 2018. We compared several parameters between patients undergoing splenic vein ligation with preservation of the inferior mesenteric vein confluence and a splenorenal venous shunt: the early and late spleen volumes and spleen volume ratios, an early and late platelet count, the presence of thrombocytopenia, the presence of varices, and digestive bleeding in the long-term. RESULTS: There were 114 consecutive patients: 36 with splenic vein ligation and 78 with splenorenal venous shunt. All had a pancreaticogastrostomy. Patients with splenic vein ligation had a comparable baseline and early and late platelet counts. Although baseline splenic volumes were comparable between the 2 groups (242 ± 115 mL vs 261 ± 138 mL; P = .51), patients with splenic vein ligation showed a statistically significant greater splenic volume beyond the 6th postoperative months (334 ± 160 mL vs 241 ± 111 mL; P = .004), higher early and late spleen volume ratios (1.42 ± 0.67 vs 1.10 ± 0.3; P = .001 and 1.38 ± 0.38 vs 0.97 ± 0.4; P = .0001) than patients with splenorenal venous shunt. Splenic vein ligation was also associated with a higher rate of varices (81% vs 50%; P = .002) and more frequent varices with a caliber greater than 1 cm (57% vs 36%; P = .05) and more colonic varices (33% vs 12%; P = .01). Only 1 patient had long-term digestive bleeding (splenic vein ligation). CONCLUSION: The left splenorenal shunt decreases clinical signs of left portal hypertension associated with splenic vein ligation and inferior mesenteric vein confluence preservation.


Assuntos
Hipertensão Portal/etiologia , Ligadura/efeitos adversos , Pancreaticoduodenectomia/efeitos adversos , Veia Esplênica/cirurgia , Derivação Esplenorrenal Cirúrgica , Idoso , Estudos de Coortes , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias Pancreáticas/cirurgia , Contagem de Plaquetas , Estudos Retrospectivos , Esplenomegalia/etiologia , Varizes/etiologia
15.
Ann Hematol ; 99(7): 1441-1451, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32417942

RESUMO

Myelofibrosis (MF) is a chronic myeloproliferative neoplasm which can lead to massive splenomegaly secondary to extramedullary hematopoiesis. Patients frequently exhibit debilitating symptoms including pain and early satiety, in addition to cellular sequestration causing severe cytopenias. JAK 1/2 inhibitors, such as ruxolitinib and fedratinib, are the mainstay of therapy and produce significant and durable reductions in spleen volume. However, many patients are not eligible for JAK 2 inhibitor therapy or become refractory to treatment over time. Novel therapies are in development that can reduce the degree of splenomegaly for some of these patients. However, splenectomy, splenic irradiation, and partial splenic artery embolization remain valuable therapeutic options in select patients. In this review, we will discuss currently available pharmacologic therapies and describe promising drugs currently in development. We will also delve into the efficacy and safety concerns of splenectomy, splenic irradiation, and partial splenic artery embolization. Finally, we will propose a treatment algorithm to help guide clinicians in the management of symptomatic splenomegaly in patients with MF.


Assuntos
Mielofibrose Primária/complicações , Mielofibrose Primária/terapia , Esplenomegalia/etiologia , Esplenomegalia/terapia , Embolização Terapêutica/métodos , Humanos , Inibidores de Proteínas Quinases/uso terapêutico , Baço/irrigação sanguínea , Baço/patologia , Baço/cirurgia , Esplenectomia/métodos , Artéria Esplênica/patologia , Artéria Esplênica/cirurgia
16.
Am Surg ; 86(4): 334-340, 2020 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-32391757

RESUMO

Different kinds of complications after splenectomy in hepatolenticular degeneration patients with hypersplenism have been reported in the past decades, but studies on pancreatic fistula and the corresponding targeted prevention and treatment after splenectomy still remain much unexplored. The present work investigated the pathogenic factors of pancreatic fistula after splenectomy and the variation tendency of amylase in drainage fluid, aiming to verify the significance of monitoring amylase in the abdominal drainage fluid in the early diagnosis of pancreatic fistula after splenectomy. One hundred sixty-seven patients with hepatolenticular degeneration and hypersplenism who underwent splenectomy in the First Affiliated Hospital of Anhui University of Traditional Chinese Medicine from January 2016 to August 2018 were selected and analyzed. The amylase in the abdominal drainage fluid was monitored routinely after splenectomy. We also conducted the statistics on the incidence of different types of pancreatic fistula and analyzed the influence factors of pancreatic fistula formation. After splenectomy, biochemical fistula occurred in 11 patients (6.6%), grade B fistula in six patients (3.6%), grade C fistula in one patient (0.6%), and the incidence of pancreatic fistula was 4.2 per cent (biochemical fistula excluded). The amylase in the peritoneal drainage fluid was closely concerned with the incidence of pancreatic fistula according to our statistics. Furthermore, by analyzing the different influence factors of pancreatic fistula, Child-Pugh grading of liver function (P = 0.041), pancreatic texture (P = 0.029), degree of splenomegaly (P = 0.003), and operative method (P = 0.001) were supposed to be closely related to the formation of pancreatic fistula. Monitoring of amylase in peritoneal drainage fluid is regarded as an important physiological parameter in the early diagnosis of pancreatic fistula after splenectomy, which provides effective clinical reference and plays a significant role in preventing the occurrence and development of pancreatic fistula.


Assuntos
Amilases/análise , Líquido Ascítico/química , Degeneração Hepatolenticular/cirurgia , Fístula Pancreática/etiologia , Complicações Pós-Operatórias/epidemiologia , Esplenectomia/efeitos adversos , Esplenomegalia/cirurgia , Adolescente , Adulto , Idoso , Biomarcadores/análise , Criança , Drenagem , Feminino , Degeneração Hepatolenticular/complicações , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Monitorização Fisiológica , Fístula Pancreática/epidemiologia , Fístula Pancreática/prevenção & controle , Esplenomegalia/etiologia , Adulto Jovem
18.
Am J Trop Med Hyg ; 103(1): 485-493, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32372751

RESUMO

Tropical splenomegaly is often associated with malaria and schistosomiasis. In 2014 and 2015, 145 Congolese refugees in western Uganda diagnosed with splenomegaly during predeparture medical examinations underwent enhanced screening for various etiologies. After anecdotal reports of unresolved splenomegaly and complications after U.S. arrival, patients were reassessed to describe long-term clinical progression after arrival in the United States. Post-arrival medical information was obtained through medical chart abstraction in collaboration with state health partners in nine participating states. We evaluated observed splenomegaly duration and associated clinical sequelae between 130 case patients from eastern Congo and 102 controls through adjusted hierarchical Poisson models, accounting for familial clustering. Of the 130 case patients, 95 (73.1%) had detectable splenomegaly after arrival. Of the 85 patients with records beyond 6 months, 45 (52.9%) had persistent splenomegaly, with a median persistence of 14.7 months (range 6.0-27.9 months). Of the 112 patients with available results, 65 (58.0%) patients had evidence of malaria infection, and the mean splenomegaly duration did not differ by Plasmodium species. Refugees with splenomegaly on arrival were 43% more likely to have anemia (adjusted relative risk [aRR]: 1.43, 95% CI: 1.04-1.97). Those with persistent splenomegaly were 60% more likely (adjusted relative risk [aRR]: 1.60, 95% CI: 1.15-2.23) to have a hematologic abnormality, particularly thrombocytopenia (aRR: 5.53, 95% CI: 1.73-17.62), and elevated alkaline phosphatase (aRR: 1.57, 95% CI: 1.03-2.40). Many patients experienced persistent splenomegaly, contradicting literature describing resolution after treatment and removal from an endemic setting. Other possible etiologies should be investigated and effective treatment, beyond treatment for malaria and schistosomiasis, explored.


Assuntos
Anemia/epidemiologia , Eosinofilia/epidemiologia , Malária/epidemiologia , Refugiados , Esquistossomose/epidemiologia , Esplenomegalia/epidemiologia , Trombocitopenia/epidemiologia , Adolescente , Adulto , Fosfatase Alcalina/sangue , Anemia/sangue , Anti-Helmínticos/uso terapêutico , Antimaláricos/uso terapêutico , Combinação Arteméter e Lumefantrina/uso terapêutico , Estudos de Casos e Controles , Criança , Pré-Escolar , Estudos de Coortes , República Democrática do Congo/etnologia , Progressão da Doença , Eosinofilia/sangue , Feminino , Hepatite A/epidemiologia , Hepatite B/epidemiologia , Hepatite C/epidemiologia , Humanos , Imunoglobulina M , Lactente , Malária/complicações , Malária/diagnóstico , Malária/tratamento farmacológico , Masculino , Pessoa de Meia-Idade , Reação em Cadeia da Polimerase , Praziquantel/uso terapêutico , Esquistossomose/complicações , Esquistossomose/tratamento farmacológico , Esplenomegalia/sangue , Esplenomegalia/etiologia , Trombocitopenia/sangue , Estados Unidos/epidemiologia , Adulto Jovem
19.
Zhongguo Xue Xi Chong Bing Fang Zhi Za Zhi ; 32(3): 323-325, 2020 Jan 14.
Artigo em Chinês | MEDLINE | ID: mdl-32468802

RESUMO

The etiology, pathology, clinical features and prognosis of megalosplenic advanced schistosomiasis have their specific features, and therefore, the perioperative management of this disorder has special countermeasures. The review analyzes the difficult problems in the perioperative management of megalosplenic advanced schistosomiasis, including ultra - low platelet counts, extensive and severe adhesive splenomegaly, massive hemorrhage during surgery and portal vein thrombosis, and proposes countermeasures to tackle these problems, with aims to guide the clinical treatment and cure of schistosomiasis, thereby improving the prognosis, reducing complications and improving the quality of life.


Assuntos
Esquistossomose , Esplenectomia , Esplenomegalia , Humanos , Período Perioperatório , Qualidade de Vida , Esquistossomose/complicações , Esplenectomia/efeitos adversos , Esplenomegalia/etiologia , Esplenomegalia/cirurgia , Trombocitopenia/etiologia , Trombose Venosa/etiologia
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