Primary non-Hodgkin lymphoma of liver is a very rare malignancy. The disease is poorly understood and few clinical studies have been conducted to help elucidate the natural course of disease, pathogenesis, optimal the...Primary non-Hodgkin lymphoma of liver is a very rare malignancy. The disease is poorly understood and few clinical studies have been conducted to help elucidate the natural course of disease, pathogenesis, optimal therapy, response to therapy, and survival. Here, we present an interesting case of primary non-Hodgkin lymphoma (NHL) originating in liver. A literature review of clinical features, diagnosis, and management is also provided.展开更多
BACKGROUND： A preoperative diagnosis of primary hepatic lymphoma（PHL） can have profound therapeutic and prognostic implications. Because of the rarity of PHL, however, there are few reports on diagnostic imaging. W...BACKGROUND： A preoperative diagnosis of primary hepatic lymphoma（PHL） can have profound therapeutic and prognostic implications. Because of the rarity of PHL, however, there are few reports on diagnostic imaging. We reviewed the clinical and radiologic findings of 29 patients with PHL, the largest series to date, to evaluate the diagnostic features of this disease.METHODS： Clinical data and radiologic findings at presentation were retrospectively reviewed for 29 patients with pathologically confirmed PHL from January 2005 to June 2013. Imaging studies, including ultrasound（US）（n=29） and contrast-enhanced computed tomography（CECT）（n=24）, were performed within 2 weeks before biopsy or surgery.RESULTS： Among the 29 patients, 23（79%） were positive for hepatitis B virus（HBV） and 26（90%） had a significantly elevated level of serum lactate dehydrogenase（LDH）. There were two distinct types of PHL on imaging： diffuse（n=5） and nodular（n=24）. Homogeneous or heterogeneous hepatomegaly was the only sign for diffuse PHL on both US and CECT, without any definite hepatic mass. For the nodular type, 63%（15/24） of patients had solitary lesions and 38%（9/24） had multiple lesions. On US, seven patients displayed patchy distribution with an indistinct tumor margin and a rich color flow signal. CECT showed rim-like enhancement（n=3） and slightly homogeneous or heterogeneous enhancement（n=14） in the arterial phase and isoenhancement（n=5） and hypoenhancement（n=12） in the portal venous and late phases. Furthermore, in five patients, CT revealed that hepatic vessels passed through the lesions and were not displaced from the abnormal area or appreciably compressed.CONCLUSIONS： The infiltration type of PHL was associated with the histologic subtype. Considered together with HBV positivity and elevated LDH, homogeneous or heterogeneous hepatomegaly may indicate diffuse PHL, whereas patchy distribution with a rich color flow signal on US or normal vessels extending through the lesion on CECT may be the diagnostic indicators of nodular PHL.展开更多
Primary mediastinal neuroendocrine tumors are a rare malignancy that accounts for < 10% of all mediastinal tumors. The case presented here involves a 52-yearold man who had been suffering for 3 mo from chronic coug...Primary mediastinal neuroendocrine tumors are a rare malignancy that accounts for < 10% of all mediastinal tumors. The case presented here involves a 52-yearold man who had been suffering for 3 mo from chronic cough, anorexia and substantial weight loss, as well as 2 wk of jaundice prior to his admission. A computed tomography scan showed a 4.3 cm × 6.6 cm mediastinal mass with multiple liver nodules scattered along both hepatic lobes. Endoscopic ultrasound showed a large heterogeneous hypoechoic mass at the mediastinum with multiple target-like nodules in the liver. Fine-needle aspiration specimens revealed numerous, small, round cells with hyperchromatic nuclei, scarce cytoplasm, and frequent mitotic features. Immunohistochemical study revealed positive results for AE1/AE3, CD56 and chromogranin A, with negative findings for synaptophysin, CK20, vimentin, CK8/18 and CD45. The patient was subsequently diagnosed with a poorly differentiated neuroendocrine carcinoma, small cell type. A bone marrow biopsy also revealed extensive involvement by the carcinoma.展开更多
A 59-year-old woman presented with the clinical symptoms and radiologic investigations of a liver lesion suspect of metastasis. However, postoperative histopathology revealed a primary hepatic lymphoma (PHL). The case...A 59-year-old woman presented with the clinical symptoms and radiologic investigations of a liver lesion suspect of metastasis. However, postoperative histopathology revealed a primary hepatic lymphoma (PHL). The case of a patient with a solitary PHL, which was treated by resection and subsequent chemotherapy, will be discussed with a short overview of the literature.展开更多
Acute on chronic liver failure(ACLF) is rarely the initial manifestation of a malignant process or precipitated by the initiation of anti-viral treatment with a nucleoside or nucleotide agent. We report an unusual cas...Acute on chronic liver failure(ACLF) is rarely the initial manifestation of a malignant process or precipitated by the initiation of anti-viral treatment with a nucleoside or nucleotide agent. We report an unusual case of ACLF temporally associated with initiation of Entecavir for treatment of chronic hepatitis B. Early Hodgkin's lymphoma(HL) was unmasked with initiation of the antiviral treatment which may have exacerbated ACLF. To the best of our knowledge, this has not been described in the literature. In reviewing our patients clinical course and liver autopsy, he developed a severe acute exacerbation of his chronic hepatitis B virus coinciding with the institution of antiviral therapy and the underlying HL perhaps modulating the overall degree of hepatic injury.展开更多
Acute liver failure(ALF) is an uncommon disease in the United States,affecting more than 2 000 people each year.Of all the various causes,malignant infiltration is one of the least well known and carries with it a hig...Acute liver failure(ALF) is an uncommon disease in the United States,affecting more than 2 000 people each year.Of all the various causes,malignant infiltration is one of the least well known and carries with it a high mortality.We describe a case of ALF as the presenting manifestation of peripheral T-cell lymphoma in an elderly woman.By reporting this case,we hope to increase early recognition of this disease process in order to potentially improve treatment outcomes.展开更多
Acute liver failure is a rare presentation of hematologic malignancy. Acute on chronic liver failure(ACLF) is a newly recognized clinical entity that describes acute hepatic decompensation in persons with preexisting ...Acute liver failure is a rare presentation of hematologic malignancy. Acute on chronic liver failure(ACLF) is a newly recognized clinical entity that describes acute hepatic decompensation in persons with preexisting liver disease. Diffuse large B-cell lymphoma(DLBCL) is an aggressive non-Hodgkin's lymphoma(NHL) with increasing incidence in older males, females and blacks. However, it has not yet been reported, to present with acute liver failure in patients with preexisting chronic liver disease due to human immunodeficiency virus(HIV)/hepatitis C virus(HCV) co-infection. We describe a case of ACLF as the presenting manifestation of DLBCL in an elderly black man with HIV/HCV coinfection and prior Hodgkin's disease in remission for three years. The rapidly fatal outcome of this disease is highlighted as is the distinction of ACLF from decompensated cirrhosis. Due to the increased prevalence of HIV/HCV co-infection in the African American 1945 to 1965 birth cohort and the fact that both are risk factors for chronic liver disease and NHL we postulate that the incidence of NHL presenting as ACLF may increase.展开更多
Plasmablastic lymphoma(PBL)is a very rare B-cell lymphoproliferative disorder was with an aggressive clinical behavior that recently characterized by the World Health Organization.Although PBL is most commonly observe...Plasmablastic lymphoma(PBL)is a very rare B-cell lymphoproliferative disorder was with an aggressive clinical behavior that recently characterized by the World Health Organization.Although PBL is most commonly observed in the oral cavity of human immunodeficiency virus(HIV)-positive patients,it can also be observed at extra-oral sites in HIV-negative patients.Epstein-Barr virus(EBV)may be closely related the pathogenesis of PBL.PBL shows different clinicopathological characteristics between HIV-positive and-negative patients.Here,we report a case of PBL of the liver in a 79-yearold HIV-negative male.The patient died approximately1.5 mo after examination and autopsy showed that the main lesion was a very large liver mass.Histopathological examination of the excised lesion showed large-cell lymphoma with plasmacytic differentiation diffusely infiltrating the liver and involving the surrounding organs.The neoplastic cells were diffusely positive for CD30,EBV,Bob-1,and CD38.The autopsy findings suggested a diagnosis of PBL.To our knowledge,the present case appears to be the first report of PBL with initial presentation of the liver in a patient without HIV infection.展开更多
Post transplantation lymphoproliferative disorder is a lymphoid/plasmacytic proliferation that develops as a consequence of immunosuppression in a recipient of a solid organ/bone marrow/stem cell allograft. The incide...Post transplantation lymphoproliferative disorder is a lymphoid/plasmacytic proliferation that develops as a consequence of immunosuppression in a recipient of a solid organ/bone marrow/stem cell allograft. The incidence reported in literature in post liver transplant patients is 1% - 2%. This case was of a 16-year-old boy who underwent liver transplantation for fulminant hepatic failure and subsequently developed PTLD. Morphologically and immunohistochemically, it was Hodgkin’s lymphoma like PTLD, which was the least common type reported in literature.展开更多
A significant association between hepatitis C virus(HCV) infection and B-cell lymphoma has been reported by epidemiological studies, most of them describing a strong relationship between indolent lymphomas and HCV. Fu...A significant association between hepatitis C virus(HCV) infection and B-cell lymphoma has been reported by epidemiological studies, most of them describing a strong relationship between indolent lymphomas and HCV. Furthermore, the curative potential of antiviral therapy on HCV related indolent lymphomas supports a specific role for the virus in lymphomagenesis. These observations are reinforced by numerous laboratory experiments that led to several hypothetical models of B-cell transformation by HCV. Diffuse large B-cell lymphoma(DLBCL), the most common lymphoma subtype in the western countries, has been associated to HCV infection despite its aggressive nature. This association seems particularly prominent in some geographical areas. Clinical presentation of HCV-associated DLBCL has consistently been reported to differ from the HCVnegative counterpart. Nevertheless, histopathology, tolerance to standard-of-care chemo-immunotherapy(R-CHOP or CHOP-like regimens) and final outcome of HCV-positive DLBCL patients is still matter of debate.Addition of rituximab has been described to enhance viral replication but the probability of severe hepatic complications remains low, with some exceptions(i.e., hepatitis B virus or immune immunodeficiency virus coinfected patients, presence of grade > 2 transaminases elevation, cirrhosis or hepatocarcinoma). HCV viral load in this setting is not necessarily directly associated with liver damage. Overall, treatment of HCV associated DLBCL should be performed in an interdisciplinary approach with hepatologists and hematologists with close monitoring of liver function. Available reports reveal that the final outcome of HCV-positive DLBCL that receive standard immunochemotherapy is not inferior to their HCV-negative counterpart. This review summarizes data on epidemiology, pathogenesis and therapeutic approach on HCV-associated DLBCL. Several issues that are matter of debate like clinical management of patients with transaminase elevation, criteria for discontinuing or starting immuno-chemotherapy, as well as the exact role of monoclonal antibodies will be analyzed.展开更多
We reported a case of non-Hodgkin's lymphoma where liver involvement was the predominant clinical manifestation. A 27-year old man presented wiht markedly elevated serum aspartate aminotrasferase, alanine aminotra...We reported a case of non-Hodgkin's lymphoma where liver involvement was the predominant clinical manifestation. A 27-year old man presented wiht markedly elevated serum aspartate aminotrasferase, alanine aminotransferase and lactate dehydrogenase, reduced prothrombin activity, thrombocytopenic purpura and hepato-splenomegaly without adenopathy. Viral, toxic,autoimmune and metabolic liver diseases were excluded.Bone marrow biopsy showed an intracapillary infiltration of T-lymphocytes with no evidence of lipid storage disease.Because of a progressive spleen enlargement, splenectomy was performed. Histological examination showed lymphomatous intrasinuses invasion of the spleen.Immunohistochemical investigation revealed the T phenotype of the neoplastic cells: CD45+, CD45RO+,CD3+, CD4-, CD8-, TIA1-. About 50 % of the lymphoid cells expressed CD56 antigen. The diagnosis of hepatosplenic T cell lymphoma was done. The patient was treated with chemotherapy, which induced a complete remission. Eighteen months later, he had a first relapse with increased aspartate aminotransferase, alanine aminotransferase, lactate dehydrogenase,thrombocytopenic purpura and blast in the peripheral blood.In spite of autologous bone marrow transplantation, he died twenty months after the diagnosis. Even in the absence of a mass lesion or lymphoadenopathy, hepatosplenic Tcell lymphoma should be considered in the differential diagnosis of a patient whose clinical course is atypical for acute hepatic dysfunction.展开更多
Vanishing bile duct syndrome(VBDS) has been described in different pathologic conditions including infection, ischemia, adverse drug reactions, autoimmune diseases, allograft rejection, and humoral factors associated ...Vanishing bile duct syndrome(VBDS) has been described in different pathologic conditions including infection, ischemia, adverse drug reactions, autoimmune diseases, allograft rejection, and humoral factors associated with malignancy. It is an acquired condition characterized by progressive destruction and loss of the intra-hepatic bile ducts leading to cholestasis. Prognosis is variable and partially dependent upon the etiology of bile duct injury. Irreversible bile duct loss leads to significant ductopenia, biliary cirrhosis, liver failure, and death. If biliary epithelial regeneration occurs, clinical recovery may occur over a period of months to years. VBDS has been described in a number of cases of patients with Hodgkin's lymphoma(HL) where it is thought to be a paraneoplastic phenomenon. This case describes a 25-year-old man found on liver biopsy to have VBDS. Given poor response to medical treatment, the patient underwent transplant evaluation at that time and was found to have classical stage ⅡB HL. Early recognition of this underlying cause or association of VBDS, including laboratory screening, and physical exam for lymphadenopathy are paramount to identifying potential underlying VBDS-associated malignancy. Here we review the literature of HL-associated VBDS and report a case of diagnosed HL with biopsy proven VBDS.展开更多
AIM:To estimate the characteristics of Color Doppler findings and the results of hepatic radionuclide angiography (HRA) in secondary Hodgkin's hepatic lymphoma.METHODS:The research included patients with a diagnos...AIM:To estimate the characteristics of Color Doppler findings and the results of hepatic radionuclide angiography (HRA) in secondary Hodgkin's hepatic lymphoma.METHODS:The research included patients with a diagnosis of Hodgkin's lymphoma with metastatic focal lesions in the liver and controls.Morphologic characteristics of focal liver lesions and hemodynamic parameters were examined by pulsed and Color Doppler in the portal,hepatic and splenic veins were examined.Hepatic perfusion index (HPI) estimated by HRA was calculated.RESULTS:In the majority of patients,hepatomegaly was observed.Lesions were mostly hypoechoic and mixed,solitary or multiple.Some of the patients presented with dilated splenic veins and hepatofugal blood flow.A pulse wave was registered in the centre and at the margins of lymphoma.The average velocity of the pulse wave was higher at the margins (P>0.05).A continuous venous wave was found only at the margins of lymphoma.There was no linear correlation between lymphoma size and velocity of pulse and continuous wave (r=390,P<0.01).HPI was significantly lower in patients with lymphomas than in controls (P<0.05),pointing out increased arterial perfusion in comparison to portal perfusion.CONCLUSION:Color Doppler ultrasonography is a sensitive method for the detection of neovascularization in Hodgkin's hepatic lymphoma and estimation of its intensity.Hepatic radionuclide angiography can additionally help in the assesment of vascularisation of liver lesions.展开更多
AIM: To investigate the short-term and long-term ef-ficacy and safety of laparoscopic splenectomy (LS) for treatment of splenic marginal zone lymphoma (SMZL). METHODS: A total of 18 continuous patients who were diagno...AIM: To investigate the short-term and long-term ef-ficacy and safety of laparoscopic splenectomy (LS) for treatment of splenic marginal zone lymphoma (SMZL). METHODS: A total of 18 continuous patients who were diagnosed with SMZL and underwent LS in our department from 2008 to 2012 were reviewed. The perioperative variables and long-term follow-up were evaluated. To evaluate the efficacy and safety of this procedure better, we also included 34 patients with liver cirrhosis who underwent LS, 49 patients with immune thrombocytopenia (ITP) who underwent LS, and 20 patients with SMZL who underwent open splenectomy (OS). The results observed in the different groups were compared.RESULTS: No differences were found in the sex and Child-Pugh class of the patients in SMZL-LS, SMZL-OS, ITP, and liver cirrhosis groups. The splenic length of the patients in the SMZL-LS group was similar to that in the SMZL-OS and liver cirrhosis groups but significantly longer than in the ITP group. The SMZL-LS group had a significantly longer operating time compared with the SMZL-OS, ITP, and liver cirrhosis groups, and the SMZL-LS group exhibited significantly less blood loss compared with the SMZL-OS group. No difference was found in the length of the postoperative hospital stay between the SMZL-LS, SMZL-OS, ITP, and liver cirrhosis-LS groups. After surgery, 6 (33.3%) SMZL-LS patients suffered slight complications. During mean fol-low-up periods of 13.6 and 12.8 mo, one patient from the SMZL-LS group and two from the SMZL-OS group died as a result of metastasis after surgery. None of the ITP and liver cirrhosis patients died. CONCLUSION: LS should be considered a feasible and safe procedure for treatment of SMZL in an effort to improve the treatment options and survival of patients.展开更多
Primary hepatic peripheral T-cell lymphoma(H-PTCL) is one of the rarest forms of non-Hodgkin lymphoma. We report a patient who presented with worsening jaundice, abdominal pain, and vomiting. Laboratory values were si...Primary hepatic peripheral T-cell lymphoma(H-PTCL) is one of the rarest forms of non-Hodgkin lymphoma. We report a patient who presented with worsening jaundice, abdominal pain, and vomiting. Laboratory values were significant for elevated total bilirubin, alkaline phosphatase, and liver aminotransferases. Following a liver biopsy, histopathology revealed several large dense clusters of atypical T-lymphocytes which were CD2+, CD3+, CD5+, CD7-, CD4+, CD8-, CD56-, CD57-, CD30+ by immunohistochemistry. The proliferation index was approximately 70% by labeling for ki67/mib1. The above histological profile was consistent with peripheral T-cell lymphoma of the liver. Epstein-Barr viral serology indicated a remote infection, a likely risk factor for PTCL. Bone marrow biopsy was negative for malignancy, further supporting hepatic origin.展开更多
AIM To explore the ability of superb microvascular imaging(SMI) in differential diagnosis of focal liver lesions(FLLs) and to compare SMI morphology findings to those of color Doppler ultrasound and enhanced imaging.M...AIM To explore the ability of superb microvascular imaging(SMI) in differential diagnosis of focal liver lesions(FLLs) and to compare SMI morphology findings to those of color Doppler ultrasound and enhanced imaging.METHODS Twenty-four patients with 31 FLLs were included in our study,with diagnoses of hemangioma(HE)(n = 17),hepatocellular carcinoma(HCC)(n = 5),metastatic lesions(n = 5),primary hepatic lymphoma(n = 1),focal nodular hyperplasia(FNH)(n = 2),and adenoma(n = 1). Nine lesions were pathologically diagnosed,and 22 lesions were radiologically confirmed,all of which were evaluated by at least two types of enhanced imaging techniques. All patients had undergone SMI. Patients were divided into subgroups based on pathological and radiological diagnoses to analyze SMI manifestations. We also compared the SMI manifestations of the most common malignant FLLs(HCCs and metastatic lesions) with those of the most common benign FLLs(HEs).RESULTS HEs were classified into three SMI subgroups: diffuse dot-like type(n = 6),strip rim type(n = 8),and nodular rim type(n = 3). The sizes of the three types of HEs were significantly different(P = 0.00,< 0.05). HCCs were classified into two subgroups: diffuse honeycomb type(n = 2) and non-specific type(n = 3). Four of the metastatic lesions were the strip rim type,and the other metastatic lesion was the thick rim type,which is the same as that of lymphoma. FNH was described as a spoke-wheel type,and adenoma as a diffuse honeycomb type. The SMI types of HCCs and metastatic lesions were significantly different from those of HEs(P = 0.048,< 0.05).CONCLUSION SMI technology enables microvascular evaluation of FLLs without using any contrast agent. For HEs,lesion size may affect SMI performance. SMI is able to provide useful information for differential diagnosis of HCCs and metastatic lesions from HEs.展开更多
The hepatitis C virus(HCV) infected patients are prone to develop bone marrow or various tissue infiltrates with monoclonal B cells, monoclonal B lymphocytosis or different types of B cell non-Hodgkin's lymphoma(B...The hepatitis C virus(HCV) infected patients are prone to develop bone marrow or various tissue infiltrates with monoclonal B cells, monoclonal B lymphocytosis or different types of B cell non-Hodgkin's lymphoma(BCNHL), of which the most common are splenic marginal zone BCNHL, diffuse large BCNHL and follicular lymphoma. The association between chronic HCV infection and non Hodgkin's lymphoma has been observed especially in areas with high prevalence of this viral infection. Outside the limitations of some studies that have been conducted, there are also geographic, environmental, and genetic factors that contribute to the epidemiological differences. Various microenvironmental signals, such as cytokines, viral antigenic external stimulation of lymphocyte receptors by HCV antigens, and intercellular interactions contribute to B cell proliferation. HCV lymphotropism and chronic antigenic stimulation are involved in B-lymphocyte expansion, as mixted cryoglobulinemia or monoclonal gammopathy of undetermined significance, which can progress to BCNHL. HCV replication in B lymphocytes has oncogenic effect mediated by intracellular HCV proteins. It is also involved in an important induction of reactive oxygen species that can lead to permanent B lymphocyte damage, as DNA mutations, after binding to surface B-cell receptors. Posttransplant lymphoproliferative disorder could appear and it has a multiclonal potentiality that may develop into different types of lymphomas. The hematopoietic stem cell transplant made for lymphoma in HCV-infected patients can increase the risk of earlier progression to liver fibrosis and cirrhosis. HCV infected patients with indolent BCNHL who receive antiviral therapy can be potentially cured. Viral clearance was related to lymphoma response, fact that highlights the probable involvement of HCV in lymphomagenesis. Direct acting antiviral drugs could be a solution for the patients who did not tolerate or respond to interferon, as they seem to be safe and highly effective. The use of chemotherapy in combination with rituximab for the treatment of BCNHL in patients infected with HCV can produce liver dysfunction. The addition of immunotherapy with rituximab can increase the viral replication, and severe complications can occure especially in patients co-infected with hepatitis B virus or immune immunodeficiency virus, in those with hepatocarcinoma,cirrhosis, or liver cytolysis. But the final result of standard immunochemotherapy applied to diffuse large BCNHL patients with HCV infection is not notably worse than in those without this viral infection. The treatment of patients chronically infected with HCV and having BCNHL is complex and requires a multidisciplinary approach and the risk / benefit ratio of rituximab treatment must be evaluated especially in those with liver cytolysis.展开更多
文摘Primary non-Hodgkin lymphoma of liver is a very rare malignancy. The disease is poorly understood and few clinical studies have been conducted to help elucidate the natural course of disease, pathogenesis, optimal therapy, response to therapy, and survival. Here, we present an interesting case of primary non-Hodgkin lymphoma (NHL) originating in liver. A literature review of clinical features, diagnosis, and management is also provided.
文摘BACKGROUND： A preoperative diagnosis of primary hepatic lymphoma（PHL） can have profound therapeutic and prognostic implications. Because of the rarity of PHL, however, there are few reports on diagnostic imaging. We reviewed the clinical and radiologic findings of 29 patients with PHL, the largest series to date, to evaluate the diagnostic features of this disease.METHODS： Clinical data and radiologic findings at presentation were retrospectively reviewed for 29 patients with pathologically confirmed PHL from January 2005 to June 2013. Imaging studies, including ultrasound（US）（n=29） and contrast-enhanced computed tomography（CECT）（n=24）, were performed within 2 weeks before biopsy or surgery.RESULTS： Among the 29 patients, 23（79%） were positive for hepatitis B virus（HBV） and 26（90%） had a significantly elevated level of serum lactate dehydrogenase（LDH）. There were two distinct types of PHL on imaging： diffuse（n=5） and nodular（n=24）. Homogeneous or heterogeneous hepatomegaly was the only sign for diffuse PHL on both US and CECT, without any definite hepatic mass. For the nodular type, 63%（15/24） of patients had solitary lesions and 38%（9/24） had multiple lesions. On US, seven patients displayed patchy distribution with an indistinct tumor margin and a rich color flow signal. CECT showed rim-like enhancement（n=3） and slightly homogeneous or heterogeneous enhancement（n=14） in the arterial phase and isoenhancement（n=5） and hypoenhancement（n=12） in the portal venous and late phases. Furthermore, in five patients, CT revealed that hepatic vessels passed through the lesions and were not displaced from the abnormal area or appreciably compressed.CONCLUSIONS： The infiltration type of PHL was associated with the histologic subtype. Considered together with HBV positivity and elevated LDH, homogeneous or heterogeneous hepatomegaly may indicate diffuse PHL, whereas patchy distribution with a rich color flow signal on US or normal vessels extending through the lesion on CECT may be the diagnostic indicators of nodular PHL.
文摘Primary mediastinal neuroendocrine tumors are a rare malignancy that accounts for < 10% of all mediastinal tumors. The case presented here involves a 52-yearold man who had been suffering for 3 mo from chronic cough, anorexia and substantial weight loss, as well as 2 wk of jaundice prior to his admission. A computed tomography scan showed a 4.3 cm × 6.6 cm mediastinal mass with multiple liver nodules scattered along both hepatic lobes. Endoscopic ultrasound showed a large heterogeneous hypoechoic mass at the mediastinum with multiple target-like nodules in the liver. Fine-needle aspiration specimens revealed numerous, small, round cells with hyperchromatic nuclei, scarce cytoplasm, and frequent mitotic features. Immunohistochemical study revealed positive results for AE1/AE3, CD56 and chromogranin A, with negative findings for synaptophysin, CK20, vimentin, CK8/18 and CD45. The patient was subsequently diagnosed with a poorly differentiated neuroendocrine carcinoma, small cell type. A bone marrow biopsy also revealed extensive involvement by the carcinoma.
文摘A 59-year-old woman presented with the clinical symptoms and radiologic investigations of a liver lesion suspect of metastasis. However, postoperative histopathology revealed a primary hepatic lymphoma (PHL). The case of a patient with a solitary PHL, which was treated by resection and subsequent chemotherapy, will be discussed with a short overview of the literature.
文摘Acute on chronic liver failure(ACLF) is rarely the initial manifestation of a malignant process or precipitated by the initiation of anti-viral treatment with a nucleoside or nucleotide agent. We report an unusual case of ACLF temporally associated with initiation of Entecavir for treatment of chronic hepatitis B. Early Hodgkin's lymphoma(HL) was unmasked with initiation of the antiviral treatment which may have exacerbated ACLF. To the best of our knowledge, this has not been described in the literature. In reviewing our patients clinical course and liver autopsy, he developed a severe acute exacerbation of his chronic hepatitis B virus coinciding with the institution of antiviral therapy and the underlying HL perhaps modulating the overall degree of hepatic injury.
文摘Acute liver failure(ALF) is an uncommon disease in the United States,affecting more than 2 000 people each year.Of all the various causes,malignant infiltration is one of the least well known and carries with it a high mortality.We describe a case of ALF as the presenting manifestation of peripheral T-cell lymphoma in an elderly woman.By reporting this case,we hope to increase early recognition of this disease process in order to potentially improve treatment outcomes.
文摘Acute liver failure is a rare presentation of hematologic malignancy. Acute on chronic liver failure(ACLF) is a newly recognized clinical entity that describes acute hepatic decompensation in persons with preexisting liver disease. Diffuse large B-cell lymphoma(DLBCL) is an aggressive non-Hodgkin's lymphoma(NHL) with increasing incidence in older males, females and blacks. However, it has not yet been reported, to present with acute liver failure in patients with preexisting chronic liver disease due to human immunodeficiency virus(HIV)/hepatitis C virus(HCV) co-infection. We describe a case of ACLF as the presenting manifestation of DLBCL in an elderly black man with HIV/HCV coinfection and prior Hodgkin's disease in remission for three years. The rapidly fatal outcome of this disease is highlighted as is the distinction of ACLF from decompensated cirrhosis. Due to the increased prevalence of HIV/HCV co-infection in the African American 1945 to 1965 birth cohort and the fact that both are risk factors for chronic liver disease and NHL we postulate that the incidence of NHL presenting as ACLF may increase.
文摘Plasmablastic lymphoma(PBL)is a very rare B-cell lymphoproliferative disorder was with an aggressive clinical behavior that recently characterized by the World Health Organization.Although PBL is most commonly observed in the oral cavity of human immunodeficiency virus(HIV)-positive patients,it can also be observed at extra-oral sites in HIV-negative patients.Epstein-Barr virus(EBV)may be closely related the pathogenesis of PBL.PBL shows different clinicopathological characteristics between HIV-positive and-negative patients.Here,we report a case of PBL of the liver in a 79-yearold HIV-negative male.The patient died approximately1.5 mo after examination and autopsy showed that the main lesion was a very large liver mass.Histopathological examination of the excised lesion showed large-cell lymphoma with plasmacytic differentiation diffusely infiltrating the liver and involving the surrounding organs.The neoplastic cells were diffusely positive for CD30,EBV,Bob-1,and CD38.The autopsy findings suggested a diagnosis of PBL.To our knowledge,the present case appears to be the first report of PBL with initial presentation of the liver in a patient without HIV infection.
文摘Post transplantation lymphoproliferative disorder is a lymphoid/plasmacytic proliferation that develops as a consequence of immunosuppression in a recipient of a solid organ/bone marrow/stem cell allograft. The incidence reported in literature in post liver transplant patients is 1% - 2%. This case was of a 16-year-old boy who underwent liver transplantation for fulminant hepatic failure and subsequently developed PTLD. Morphologically and immunohistochemically, it was Hodgkin’s lymphoma like PTLD, which was the least common type reported in literature.
基金Supported by In part by grants of AViLL/AIL(Associazione Vicentina per le Leucemie,i Linfomi e il Mieloma/Associazione Italiana Leucemie)(Vicenza,Italy)the Hematology Project Foundation(HPF,Fondazione Progetto Ematologia,Vicenza,Italy)
文摘A significant association between hepatitis C virus(HCV) infection and B-cell lymphoma has been reported by epidemiological studies, most of them describing a strong relationship between indolent lymphomas and HCV. Furthermore, the curative potential of antiviral therapy on HCV related indolent lymphomas supports a specific role for the virus in lymphomagenesis. These observations are reinforced by numerous laboratory experiments that led to several hypothetical models of B-cell transformation by HCV. Diffuse large B-cell lymphoma(DLBCL), the most common lymphoma subtype in the western countries, has been associated to HCV infection despite its aggressive nature. This association seems particularly prominent in some geographical areas. Clinical presentation of HCV-associated DLBCL has consistently been reported to differ from the HCVnegative counterpart. Nevertheless, histopathology, tolerance to standard-of-care chemo-immunotherapy(R-CHOP or CHOP-like regimens) and final outcome of HCV-positive DLBCL patients is still matter of debate.Addition of rituximab has been described to enhance viral replication but the probability of severe hepatic complications remains low, with some exceptions(i.e., hepatitis B virus or immune immunodeficiency virus coinfected patients, presence of grade > 2 transaminases elevation, cirrhosis or hepatocarcinoma). HCV viral load in this setting is not necessarily directly associated with liver damage. Overall, treatment of HCV associated DLBCL should be performed in an interdisciplinary approach with hepatologists and hematologists with close monitoring of liver function. Available reports reveal that the final outcome of HCV-positive DLBCL that receive standard immunochemotherapy is not inferior to their HCV-negative counterpart. This review summarizes data on epidemiology, pathogenesis and therapeutic approach on HCV-associated DLBCL. Several issues that are matter of debate like clinical management of patients with transaminase elevation, criteria for discontinuing or starting immuno-chemotherapy, as well as the exact role of monoclonal antibodies will be analyzed.
文摘We reported a case of non-Hodgkin's lymphoma where liver involvement was the predominant clinical manifestation. A 27-year old man presented wiht markedly elevated serum aspartate aminotrasferase, alanine aminotransferase and lactate dehydrogenase, reduced prothrombin activity, thrombocytopenic purpura and hepato-splenomegaly without adenopathy. Viral, toxic,autoimmune and metabolic liver diseases were excluded.Bone marrow biopsy showed an intracapillary infiltration of T-lymphocytes with no evidence of lipid storage disease.Because of a progressive spleen enlargement, splenectomy was performed. Histological examination showed lymphomatous intrasinuses invasion of the spleen.Immunohistochemical investigation revealed the T phenotype of the neoplastic cells: CD45+, CD45RO+,CD3+, CD4-, CD8-, TIA1-. About 50 % of the lymphoid cells expressed CD56 antigen. The diagnosis of hepatosplenic T cell lymphoma was done. The patient was treated with chemotherapy, which induced a complete remission. Eighteen months later, he had a first relapse with increased aspartate aminotransferase, alanine aminotransferase, lactate dehydrogenase,thrombocytopenic purpura and blast in the peripheral blood.In spite of autologous bone marrow transplantation, he died twenty months after the diagnosis. Even in the absence of a mass lesion or lymphoadenopathy, hepatosplenic Tcell lymphoma should be considered in the differential diagnosis of a patient whose clinical course is atypical for acute hepatic dysfunction.
基金Supported by National Institute of Health,NIH 5 T32 DK 7356-37(BN)
文摘Vanishing bile duct syndrome(VBDS) has been described in different pathologic conditions including infection, ischemia, adverse drug reactions, autoimmune diseases, allograft rejection, and humoral factors associated with malignancy. It is an acquired condition characterized by progressive destruction and loss of the intra-hepatic bile ducts leading to cholestasis. Prognosis is variable and partially dependent upon the etiology of bile duct injury. Irreversible bile duct loss leads to significant ductopenia, biliary cirrhosis, liver failure, and death. If biliary epithelial regeneration occurs, clinical recovery may occur over a period of months to years. VBDS has been described in a number of cases of patients with Hodgkin's lymphoma(HL) where it is thought to be a paraneoplastic phenomenon. This case describes a 25-year-old man found on liver biopsy to have VBDS. Given poor response to medical treatment, the patient underwent transplant evaluation at that time and was found to have classical stage ⅡB HL. Early recognition of this underlying cause or association of VBDS, including laboratory screening, and physical exam for lymphadenopathy are paramount to identifying potential underlying VBDS-associated malignancy. Here we review the literature of HL-associated VBDS and report a case of diagnosed HL with biopsy proven VBDS.
基金Supported by The grant of the Republic of Science of Serbia (M145033,Chief investigatior:Professor Vladimir Obradovic)
文摘AIM:To estimate the characteristics of Color Doppler findings and the results of hepatic radionuclide angiography (HRA) in secondary Hodgkin's hepatic lymphoma.METHODS:The research included patients with a diagnosis of Hodgkin's lymphoma with metastatic focal lesions in the liver and controls.Morphologic characteristics of focal liver lesions and hemodynamic parameters were examined by pulsed and Color Doppler in the portal,hepatic and splenic veins were examined.Hepatic perfusion index (HPI) estimated by HRA was calculated.RESULTS:In the majority of patients,hepatomegaly was observed.Lesions were mostly hypoechoic and mixed,solitary or multiple.Some of the patients presented with dilated splenic veins and hepatofugal blood flow.A pulse wave was registered in the centre and at the margins of lymphoma.The average velocity of the pulse wave was higher at the margins (P>0.05).A continuous venous wave was found only at the margins of lymphoma.There was no linear correlation between lymphoma size and velocity of pulse and continuous wave (r=390,P<0.01).HPI was significantly lower in patients with lymphomas than in controls (P<0.05),pointing out increased arterial perfusion in comparison to portal perfusion.CONCLUSION:Color Doppler ultrasonography is a sensitive method for the detection of neovascularization in Hodgkin's hepatic lymphoma and estimation of its intensity.Hepatic radionuclide angiography can additionally help in the assesment of vascularisation of liver lesions.
文摘AIM: To investigate the short-term and long-term ef-ficacy and safety of laparoscopic splenectomy (LS) for treatment of splenic marginal zone lymphoma (SMZL). METHODS: A total of 18 continuous patients who were diagnosed with SMZL and underwent LS in our department from 2008 to 2012 were reviewed. The perioperative variables and long-term follow-up were evaluated. To evaluate the efficacy and safety of this procedure better, we also included 34 patients with liver cirrhosis who underwent LS, 49 patients with immune thrombocytopenia (ITP) who underwent LS, and 20 patients with SMZL who underwent open splenectomy (OS). The results observed in the different groups were compared.RESULTS: No differences were found in the sex and Child-Pugh class of the patients in SMZL-LS, SMZL-OS, ITP, and liver cirrhosis groups. The splenic length of the patients in the SMZL-LS group was similar to that in the SMZL-OS and liver cirrhosis groups but significantly longer than in the ITP group. The SMZL-LS group had a significantly longer operating time compared with the SMZL-OS, ITP, and liver cirrhosis groups, and the SMZL-LS group exhibited significantly less blood loss compared with the SMZL-OS group. No difference was found in the length of the postoperative hospital stay between the SMZL-LS, SMZL-OS, ITP, and liver cirrhosis-LS groups. After surgery, 6 (33.3%) SMZL-LS patients suffered slight complications. During mean fol-low-up periods of 13.6 and 12.8 mo, one patient from the SMZL-LS group and two from the SMZL-OS group died as a result of metastasis after surgery. None of the ITP and liver cirrhosis patients died. CONCLUSION: LS should be considered a feasible and safe procedure for treatment of SMZL in an effort to improve the treatment options and survival of patients.
文摘Primary hepatic peripheral T-cell lymphoma(H-PTCL) is one of the rarest forms of non-Hodgkin lymphoma. We report a patient who presented with worsening jaundice, abdominal pain, and vomiting. Laboratory values were significant for elevated total bilirubin, alkaline phosphatase, and liver aminotransferases. Following a liver biopsy, histopathology revealed several large dense clusters of atypical T-lymphocytes which were CD2+, CD3+, CD5+, CD7-, CD4+, CD8-, CD56-, CD57-, CD30+ by immunohistochemistry. The proliferation index was approximately 70% by labeling for ki67/mib1. The above histological profile was consistent with peripheral T-cell lymphoma of the liver. Epstein-Barr viral serology indicated a remote infection, a likely risk factor for PTCL. Bone marrow biopsy was negative for malignancy, further supporting hepatic origin.
文摘AIM To explore the ability of superb microvascular imaging(SMI) in differential diagnosis of focal liver lesions(FLLs) and to compare SMI morphology findings to those of color Doppler ultrasound and enhanced imaging.METHODS Twenty-four patients with 31 FLLs were included in our study,with diagnoses of hemangioma(HE)(n = 17),hepatocellular carcinoma(HCC)(n = 5),metastatic lesions(n = 5),primary hepatic lymphoma(n = 1),focal nodular hyperplasia(FNH)(n = 2),and adenoma(n = 1). Nine lesions were pathologically diagnosed,and 22 lesions were radiologically confirmed,all of which were evaluated by at least two types of enhanced imaging techniques. All patients had undergone SMI. Patients were divided into subgroups based on pathological and radiological diagnoses to analyze SMI manifestations. We also compared the SMI manifestations of the most common malignant FLLs(HCCs and metastatic lesions) with those of the most common benign FLLs(HEs).RESULTS HEs were classified into three SMI subgroups: diffuse dot-like type(n = 6),strip rim type(n = 8),and nodular rim type(n = 3). The sizes of the three types of HEs were significantly different(P = 0.00,< 0.05). HCCs were classified into two subgroups: diffuse honeycomb type(n = 2) and non-specific type(n = 3). Four of the metastatic lesions were the strip rim type,and the other metastatic lesion was the thick rim type,which is the same as that of lymphoma. FNH was described as a spoke-wheel type,and adenoma as a diffuse honeycomb type. The SMI types of HCCs and metastatic lesions were significantly different from those of HEs(P = 0.048,< 0.05).CONCLUSION SMI technology enables microvascular evaluation of FLLs without using any contrast agent. For HEs,lesion size may affect SMI performance. SMI is able to provide useful information for differential diagnosis of HCCs and metastatic lesions from HEs.
文摘The hepatitis C virus(HCV) infected patients are prone to develop bone marrow or various tissue infiltrates with monoclonal B cells, monoclonal B lymphocytosis or different types of B cell non-Hodgkin's lymphoma(BCNHL), of which the most common are splenic marginal zone BCNHL, diffuse large BCNHL and follicular lymphoma. The association between chronic HCV infection and non Hodgkin's lymphoma has been observed especially in areas with high prevalence of this viral infection. Outside the limitations of some studies that have been conducted, there are also geographic, environmental, and genetic factors that contribute to the epidemiological differences. Various microenvironmental signals, such as cytokines, viral antigenic external stimulation of lymphocyte receptors by HCV antigens, and intercellular interactions contribute to B cell proliferation. HCV lymphotropism and chronic antigenic stimulation are involved in B-lymphocyte expansion, as mixted cryoglobulinemia or monoclonal gammopathy of undetermined significance, which can progress to BCNHL. HCV replication in B lymphocytes has oncogenic effect mediated by intracellular HCV proteins. It is also involved in an important induction of reactive oxygen species that can lead to permanent B lymphocyte damage, as DNA mutations, after binding to surface B-cell receptors. Posttransplant lymphoproliferative disorder could appear and it has a multiclonal potentiality that may develop into different types of lymphomas. The hematopoietic stem cell transplant made for lymphoma in HCV-infected patients can increase the risk of earlier progression to liver fibrosis and cirrhosis. HCV infected patients with indolent BCNHL who receive antiviral therapy can be potentially cured. Viral clearance was related to lymphoma response, fact that highlights the probable involvement of HCV in lymphomagenesis. Direct acting antiviral drugs could be a solution for the patients who did not tolerate or respond to interferon, as they seem to be safe and highly effective. The use of chemotherapy in combination with rituximab for the treatment of BCNHL in patients infected with HCV can produce liver dysfunction. The addition of immunotherapy with rituximab can increase the viral replication, and severe complications can occure especially in patients co-infected with hepatitis B virus or immune immunodeficiency virus, in those with hepatocarcinoma,cirrhosis, or liver cytolysis. But the final result of standard immunochemotherapy applied to diffuse large BCNHL patients with HCV infection is not notably worse than in those without this viral infection. The treatment of patients chronically infected with HCV and having BCNHL is complex and requires a multidisciplinary approach and the risk / benefit ratio of rituximab treatment must be evaluated especially in those with liver cytolysis.
