RESUMO
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.
RESUMO
BACKGROUND: Visceral leishmaniasis (VL), or kala-azar, is a common comorbidity in patients with AIDS in endemic areas. Many patients continue to experiences relapses of VL despite virological control, but with immunological failure. These patients remain chronically symptomatic with hypersplenism, for example with anemia, leukopenia, and thrombocytopenia, and are at risk of severe co-infection due to low CD4+ count. Therefore, in this study, splenectomized patients with VL and HIV infection were investigated to understand why the CD4+ count fails to recover in these patients, evaluating the importance of spleen mass for hypersplenism and immunological failure. METHODS: From a retrospective open cohort of 13 patients who had previously undergone splenectomy as salvage therapy for relapsing VL, 11 patients with HIV infection were investigated. This study compared the patients' complete blood cell count (CBC) and CD4+ and CD8+ cell counts before and after splenectomy with respect to spleen weight. RESULTS: CBC was substantially improved after splenectomy, indicating hypersplenism. However, to the best of our knowledge, this is the first study to show that spleen mass is strongly and negatively correlated with CD4+ cell count (ρ = -0.71, P = 0.015). CONCLUSIONS: This finding was unexpected, as the spleen is the most extensive lymphoid tissue and T-lymphocyte source. After reviewing the literature and reasoning, we hypothesized that the immunological failure was secondary to CD4+ loss initially by apoptosis in the spleen induced by productive HIV infection and, subsequently, by pyroptosis sustained by parasitic infection in spleen macrophages.
Assuntos
Síndrome da Imunodeficiência Adquirida , Infecções por HIV , Hiperesplenismo , Leishmaniose Visceral , Humanos , Leishmaniose Visceral/epidemiologia , Infecções por HIV/complicações , Hiperesplenismo/complicações , Estudos Retrospectivos , Cemitérios , Síndrome da Imunodeficiência Adquirida/complicações , Recidiva Local de Neoplasia/complicações , Linfócitos T CD4-PositivosRESUMO
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.
RESUMO
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.
Assuntos
Humanos , Criança , Adolescente , Hipertensão Portal/diagnóstico , Hipertensão Portal/etiologia , Hipertensão Portal/tratamento farmacológico , Veia Porta , Vasodilatação , Seguimentos , Cirrose Hepática/complicaçõesRESUMO
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.
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.
Assuntos
Hipertensão Portal , Adolescente , Criança , Seguimentos , Humanos , Hipertensão Portal/diagnóstico , Hipertensão Portal/tratamento farmacológico , Hipertensão Portal/etiologia , Cirrose Hepática/complicações , Veia Porta , VasodilataçãoRESUMO
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.
Assuntos
Humanos , Esquistossomose , Varizes Esofágicas e Gástricas/cirurgia , Varizes Esofágicas e Gástricas/diagnóstico por imagem , Baço/cirurgia , Baço/diagnóstico por imagem , Estudos Retrospectivos , Seguimentos , Hemorragia Gastrointestinal/cirurgia , Hemorragia Gastrointestinal/etiologia , Hemorragia Gastrointestinal/diagnóstico por imagem , Recidiva Local de NeoplasiaRESUMO
We present a case of a patient with hypersplenism secondary to portal hypertension due to hepato-splenic schistosomiasis, which was accompanied by severe and refractory thrombocytopenia. We performed spleen ablation and measured the total spleen and ablated volumes with contrast-enhanced computed tomography and volumetry. No major complications occurred, thrombocytopenia was resolved, and platelet levels remained stable, which allowed for early treatment of the patient's underlying disease. Previous work has shown that splenic radiofrequency ablation is an attractive alternative treatment for hypersplenism induced by liver cirrhosis. We aimed to contribute to the currently sparse literature evaluating the role of radiofrequency ablation (RFA) in the management of hypersplenism. We conclude that splenic RFA appears to be a viable and promising option for the treatment of hypersplenism.
Assuntos
Ablação por Cateter , Hiperesplenismo/cirurgia , Hepatopatias Parasitárias/parasitologia , Esquistossomose mansoni/parasitologia , Trombocitopenia/cirurgia , Meios de Contraste , Humanos , Hiperesplenismo/diagnóstico , Hiperesplenismo/parasitologia , Hipertensão Portal/diagnóstico , Hipertensão Portal/parasitologia , Hepatopatias Parasitárias/complicações , Hepatopatias Parasitárias/diagnóstico , Masculino , Pessoa de Meia-Idade , Contagem de Plaquetas , Valor Preditivo dos Testes , Esquistossomose mansoni/complicações , Esquistossomose mansoni/diagnóstico , Trombocitopenia/diagnóstico , Trombocitopenia/parasitologia , Tomografia Computadorizada por Raios X , Resultado do TratamentoRESUMO
BACKGROUND: Schistosomiasis is a tropical disease. Patients who develop hepatosplenic schistosomiasis have clinical findings including periportal fibrosis, portal hypertension, cytopenia, splenomegaly and gastrointestinal hemorrhage. OBJECTIVE: The aim of this study was to analyze the hemostatic and hematologic findings of patients with schistosomiasis and correlate these to the size of the spleen. METHODS: Fifty-five adults with hepatosplenic schistosomiasis and 30 healthy subjects were selected through a history of contact with contaminated water, physical examination and ultrasound characteristics such as periportal fibrosis and splenomegaly in the Gastroenterology Service of the Universidade Federal de Pernambuco. Blood samples were collected to determine liver function, blood counts, prothrombin (international normalized ratio), partial thromboplastin time and fibrinogen and D-Dimer levels using the Pentra 120 hematological analyzer (HORIBA/ABX), Density Plus (test photo-optical Trinity Biotech, Ireland) and COBAS analyzer 6000 (Roche). Furthermore, the longitudinal size of the spleen was measured by ultrasound (Acuson X analyzer 150, Siemens). The Student t-test, the Fisher test and Pearson's correlation were used to analyze the results with statistical significance being set for a p-value < 0.05. RESULTS: The mean age was higher for the Study Group than for the Control Group (54 ± 13.9 vs. 38 ± 12.7 years). The average longitudinal diameter of the spleen was 16.9 cm (Range: 12.3-26.3 cm). Anemia is a common finding in patients with schistosomiasis (36.3%). The mean platelet and leukocyte counts of patients were lower than for the Control Group (p-value < 0.001). Moreover, the international normalized ratio (1.42 vs. 1.04), partial thromboplastin time (37.9 vs. 30.5 seconds) and D-Dimer concentration (393 vs. 86.5 ng/mL) were higher for the Study Group compared to the Control Group. CONCLUSION: This study suggests that hematological and hemostatic abnormalities are associated with splenomegaly, hypersplenism and portal hypertension.
RESUMO
BACKGROUND Schistosomiasis is a tropical disease. Patients who develop hepatosplenic schistosomiasis have clinical findings including periportal fibrosis, portal hypertension, cytopenia, splenomegaly and gastrointestinal hemorrhage. OBJECTIVE The aim of this study was to analyze the hemostatic and hematologic findings of patients with schistosomiasis and correlate these to the size of the spleen. METHODS Fifty-five adults with hepatosplenic schistosomiasis and 30 healthy subjects were selected through a history of contact with contaminated water, physical examination and ultrasound characteristics such as periportal fibrosis and splenomegaly in the Gastroenterology Service of the Universidade Federal de Pernambuco. Blood samples were collected to determine liver function, blood counts, prothrombin (international normalized ratio), partial thromboplastin time and fibrinogen and D-Dimer levels using the Pentra 120 hematological analyzer (HORIBA/ABX), Density Plus (test photo-optical Trinity Biotech, Ireland) and COBAS analyzer 6000 (Roche). Furthermore, the longitudinal size of the spleen was measured by ultrasound (Acuson X analyzer 150, Siemens). The Student t-test, the Fisher test and Pearson's correlation were used to analyze the results with statistical significance being set for a p-value < 0.05. RESULTS The mean age was higher for the Study Group than for the Control Group (54 ± 13.9 vs. 38 ± 12.7 years). The average longitudinal diameter of the spleen was 16.9 cm (Range: 12.3-26.3 cm). Anemia is a common finding in patients with schistosomiasis (36.3%). The mean platelet and leukocyte counts of patients were lower than for the Control Group (p-value < 0.001). Moreover, the international normalized ratio (1.42 vs. 1.04), partial thromboplastin time (37.9 vs. 30.5 seconds) and D-Dimer concentration (393 vs. 86.5 ng/mL) were higher for the Study Group compared to the Control Group...
Assuntos
Humanos , Adulto , Hiperesplenismo , Esquistossomose , Esquistossomose mansoni , Esplenomegalia , TrombocitopeniaRESUMO
Visceral leishmaniasis (VL), also known as Kala-azar, is a systemic infection caused by a protozoan (Leishmania) and, in its classic form, is a serious illness associated with malnutrition, anemia, hepatosplenomegaly, infectious processes and coagulopathies. The effect of splenectomy in patients with visceral leishmaniasis is not well defined; however, it is known that the spleen is the largest reservoir of infected cells belonging to the reticulo endothelial system. Therefore, the surgical procedure is an option for the debulking of parasites, providing a cure for refractory VL and minimizing the complications of hypersplenism.
A leishmaniose visceral (LV) ou calazar é uma infecção sistêmica causada por um protozoário (Leishmania) e na sua forma clássica é uma doença grave. Cursa com desnutrição, anemia, hepatoesplenomegalia, processos infecciosos e coagulopatias. O papel da esplenectomia em pacientes com leishmaniose visceral não é bem definido; entretanto, sabe-se que o baço é o maior reservatório de células parasitadas do sistema reticulo endotelial e o procedimento cirúrgico é, dessa forma, uma opção para debulking de parasitas, propiciando a cura da LV refratária e minimizando as complicações do hiperesplenismo.