Abstract Introduction Hypereosinophilic syndrome (HES) is a very heterogeneous group of disorders with varied etiologies characterized by peripheral eosinophilia and eosinophilic tissue/end-organ damage. In the present study, the ability of a novel non-invasive clinical tool, three-dimensional speckle-tracking echocardiography (3DSTE) was investigated to reveal any change in left ventricular (LV) rotational mechanics in clinically asymptomatic HES patients without manifest organ damage as determined by conventional diagnostic methods. Methods The present study comprised 13 patients established diagnosis of HES. However, one patient with idiopathic HES has been excluded due to insufficient image quality. The remaining patient population contained 11 cases with idiopathic HES and one patient with acute T-lymphoma associated HES (mean age: 59.7 ± 13.7 years, 8 males). The control group consisted of 36 healthy volunteers (mean age: 52.9 ± 8.3 years, 23 males). All HES patients and controls underwent complete two-dimensional Doppler echocardiography and 3DSTE. Results Both LV apical rotation (4.86 ± 1.92 degree vs. 10.07 ± 3.92 degree, p < 0.0001) and LV twist (8.52 ± 2.79 degree vs. 14.41 ± 4.26 degree, p < 0.0001) showed significant deteriotations in most of HES patients. In 2 subjects absence of LV twist called as LV „rigid body rotation’ (RBR) was detected. One patient had 1.77 degree counterclockwise (abnormally directed) LV basal rotation and 14.29 degree counterclockwise (normally directed) LV apical rotation resulting in 12.59 degree LV apico-basal gradient. The other patient had normally directed -2.09 degree LV basal rotation and almost zero (-0.27 degree) LV apical rotation resulting in 1.82 degree LV apico-basal gradient. Conclusions Reduced LV apical rotation and twist could be demonstrated in HES. LV-RBR could be detected in some HES patients.
Idiopathic hypereosinophilic syndrome is characterized by a persistent eosinophil blood count of >1.5 × 109 cells/l and organ damage, independent of the primary and secondary causes of eosinophilia. The purpose of the present study was to assess the three-dimensional speckle tracking echocardiography-derived right atrial volumetric and functional properties between hypereosinophilic syndrome patients and matched controls.
Background:There is a well‐known connection between Hepatitis C virus (HCV) infection and B‐cell lymphomas (BCL). HCV infected patients have an increased risk of developing BCL. Hypothetically, lymphomagenesis can be induced by chronic antigen stimulation (analogous to Helicobacter pylori associated gastric marginal zone lymphoma) and/or a direct pro‐oncogenic effect of intracellular HCV proteins. Several studies have demonstrated that antiviral therapy may induce remission and improve prognosis of HCV‐associated lymphomas. On the other hand, HCV infection can cause sever complication during the immunochemotherapy and stem cell transplantation. These patients are at risk for HCV reactivation, accelerated liver disease progression, drug‐induced liver toxicity. Thus, HCV‐infected patients often suffer treatment delays, reduced dose intensity, and many times are excluded from bone marrow transplantation. Accordingly, there is some evidence of negative prognostic impact of HCV infection on survival.Aims:To draw attention to the HCV caused complications during lymphoma treatment.Methods:Here we present the case of a HCV‐infected patient with primary central nervous system (CNS) lymphoma complicated with HCV infection and review the relevant literature.Results:A 56‐year old woman with CNS lymphoma was treated with MATRIX (methotrexate, cytarabine, thiotepa, and rituximab). Initial serum serology showed reactivity to HBcAg and HCV. Polymerase chain reaction (PCR) detected no hepatits B virus DNA, however the copy number for hepatitis C virus (HCV) RNA was 104 850 000 IU/ml. She received HBV prophylaxis with lamivudine and subsequently entecavir against reactivation of HBV. After the 2nd cycle of chemotherapy her liver function tests became severely abnormal (AST: 514 U/L ALT: 264 U/L, ALP: 145 U/L, GGT: 1328 U/L, total Bi: 74.6 umol/L, conjugated Bi: 63.5 umol/L). The patient was asymptomatic regarding the lymphoma and in complete remission according to MRI scans. Further systemic chemotherapy had to be halted. The HBV DNA was still undetectable by PCR. Drug induced or mechanical cholestasis and the role of ongoing HCV infection was contemplated. The biliary tracts were not dilated on ultrasound scans. She had an ERCP with sphincterotomy and sofosbuvir plus ledipasvir plus ribavirin treatment for HCV was started. Meanwhile, intrathecal methotrexate therapy was administered 3‐weekly to bridge till next chemotherapy. The antiviral therapy was completed without any side effects and at 3 months, her HCV RNA PCR was negative with entirely normal liver function. The patient achieved sustained virological response. Two more cycles of MATRIX was delivered and an autologous stem cell transplantation with BCNU and thiothepa conditioning. Liver function remained normal ever since.Summary/Conclusion:This is a rare case where the role of HCV infection in liver damage during systemic chemotherapy is highly possible. We would like to draw attention to the liver vulnerability caused by chronic HCV infection. Nowadays HCV eradication is feasible with novel direct acting antiviral therapies, HCV‐infection should not contraindicate the adequate chemotherapy.
Background Idiopathic hypereosinophilic syndrome is characterized by a persistent eosinophil blood count of >1.5 × 10 9 cells/l and organ damage, independent of the primary and secondary causes of eosinophilia. The purpose of the present study was to assess the three-dimensional speckle tracking echocardiography-derived right atrial volumetric and functional properties between hypereosinophilic syndrome patients and matched controls. Methods A total of 11 patients with idiopathic hypereosinophilic syndrome and 22 age- and gender-matched healthy controls were enrolled in the study. Three-dimensional speckle tracking echocardiography was used for calculation of right atrial volumes, volume-based functional properties, and strain parameters. Results Significantly increased right atrial maximum (68.7 ± 33.1 ml vs. 40.3 ± 12.1 ml, respectively; p = 0.001) and minimum volumes (48.3 ± 31.0 ml vs. 28.3 ± 9.4 ml, respectively; p = 0.009), as well as right atrial volume before atrial contraction (58.6 ± 27.3 ml vs. 34.5 ± 11.8 ml, respectively; p = 0.001), were found in hypereosinophilic syndrome patients compared with controls. Total and passive right atrial stroke volumes proved to be significantly increased in hypereosinophilic syndrome patients. However, global and mean segmental strain parameters did not differ significantly between the groups. Conclusion Increased cyclic right atrial volumes and mild alterations in right atrial functional properties could be demonstrated in idiopathic hypereosinophilic syndrome patients.
Background: Chronic hepatitis C virus (HCV) infection is well known as a cause of hepatocellular carcinoma, while B-NHL as the second-most-common malignancy related to this infection is less appreciated. B-NHL in chronic hepatitis C frequently, but not exclusively, develops in pts with cryoglobulinaemia, another haematological extrahepatic manifestation of HCV infection. We report here on 5 cases of B-NHL found in pts with chronic hepatitis C. Patients. Since 1993, B-NHL has been diagnosed in 5 HCV-infected pts (1 man and 4 women; age: 55 to 63 ys) treated and followed up in the Regional Hepatology Centre in Szeged. In 4 cases, the recognition of chronic HCV infection preceded the diagnosis B-NHL; 2 of them had unsuccessful previous antiviral treatment. Cryoglobulinaemia was present in 3 pts, one of them with membranoproliferative glomerulonephritis and chronic renal failure. The type of B-NHL was aggressive diffuse large B-cell lymphoma (DLBCL) in 2 cases, nodal marginal zone lymphoma in 1, and indolent B-cell lymphoma diagnosed from a bone-marrow biopsy in 2 cases. Outcomes. Because of the progression of their haematologic disease, all 5 pts required chemotherapeutic treatment; 3 of them are currently in haematologic remission, while 2 courses of chemotherapy are ongoing. In one woman with marginal zone lymphoma, unsuccessful antiviral treatment was followed by chemotherapy because of the lymphoma progression. After successful completion of the chemotherapy, a second course of anti-HCV treatment with peginterferon plus ribavirin resulted in a sustained virological response; she is now free from HCV and in complete haematological remission. Conclusions: Besides other, better recognized HCV-related pathologies, B-NHL should also be borne in mind in the work-up and follow-up of HCV-infected patients. Chemotherapy and antiviral treatment can induce durable remission; thus, collaboration between hepatologists and haematologists is essential to optimize outcome in HCV-associated lymphomas.
Introduction: An acquired factor (F) VIII inhibitor in a pt without haemophilia A is a very rare disorder. Chronic HCV infection can have extrahepatic manifestations, especially autoimmune disorders, antibody formation and haematologic complications. Two case reports have described the occurrence of an acquired F VIII inhibitor in pts with CHC without exposure to IFN-α. Another case report described a pt with haemophilia A and HCV infection who developed an acquired F VIII inhibitor after treatment with IFN-α. The literature makes no mention of the production of F IX inhibitor in relation to CHC or treatment with PEG-IFN-α plus RBV.
Newborn A mice were injected either with a single i.v. dose (Group A) or with repeated doses of (B10 x A)F1 semiallogeneic spleen cells (SSC) (Group B). A similar degree of partial transplantation tolerance (TT) to B10 skin allografts was revealed in both groups. No signs of acute, rapidly fatal host-versus-graft disease (HVGD) (anemia, leukocytosis, severe thrombocytopenia, hepatic infarcts, gastrointestinal bleedings) were found, rather a chronic HVGD developed [moderate thrombocytopenia, autoimmune antithymocyte antibodies (ATA)] in both groups. The mortality due to lymphoproliferative disorders (LPD) was significantly higher in Group A. Thus, repeated perinatal injections of (B10 x A)F1 SSC into A mice did not increase the tolerogenic and the LPD-inducing effect either, and they did not elicit acute HVGD in contrast to observations in other F1 donor-recipient combinations [1]. Consequently, the development of acute HVGD depends on immunogenetic factors and not on the repeated administration of SSC.