Under Pressure: Post-Transplant Lymphoproliferative Disease: A Case of Pulmonary Artery External Compression.
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ABSTRACT:
SUBMITTER: Freitas D
PROVIDER: S-EPMC8387807 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:AimTo investigate the prognostic value of early post-transplant hemodynamic measurements on 5-year mortality in cardiac recipients (HTx).MethodsA right heart catheterization was performed in 290 heart transplantation (HTx) recipients at a one-year post-HTx evaluation. To study the effect of post-HTx hemodynamic variables on 5-year outcome, the cohort was stratified into several subgroups. For right atrial pressure (RAP), mean pulmonary artery pressure (MPAP), pulmonary artery wedge pressure (PAWP), and pulmonary vascular resistance (PVR), patients with values from the upper 10th percentile (high), were compared with those with values from the remaining lower 90th percentile (normal). For cardiac index (CI), patients with values from the lower 10th percentile (low) were compared with those with values from the remaining upper 90th percentile (normal).ResultsDeath or re-transplantation within 5 years after the one-year control occurred in 44 patients (13%). Of those, death or re-HTx was related to graft failure in 20 of cases (45%) and non-cardiac causes in 24 of cases (55%). The risk of death or re-HTx was higher in the subgroup with MPAP above 23 mmHg than those equal to or below this value [hazard ratio 3.22, 95% confidence interval (CI) 1.49-6.97; P = 0.003]. The association remained significant despite adjustment for several comorbidities. There were no differences in outcome between subgroups stratified with respect to high versus low RAP, PAWP, CI or PVR.ConclusionElevated pulmonary artery pressure at a first annual evaluation after HTx was the only hemodynamic variable that predicted impaired outcome in cardiac recipients.
| S-EPMC6872849 | biostudies-literature
Project description:Post-transplant lymphoproliferative disorder (PTLD) is a rare complication. It represents a spectrum of lymphoid proliferations which occur in the setting of immunosuppression and organ transplantation. There are no reported cases or recommendations for the treatment of residual masses post rituximab of PTLD.A patient with a long standing history of immunosuppression due to multiple kidney transplants starting in 1979, presented with a very large palpable hard abdominal mass (2004) after a fourth renal transplant. There was a past history of heavy immune suppression. CT scans revealed a conglomerate mass involving the right native kidney and two prior right sided renal allografts that crossed the midline. Biopsy of the large right retroperitoneal mass revealed large B cell lymphoma (CD 20 positive); consistent with post-transplant lymphoproliferative disorder (PTLD).Management of bulky PTLD, in a highly sensitized, heavily immune suppressed patient is not well described in the literature. The mainstay of therapy is IR and Ritixumab (R) monotherapy and combination R-CHOP. CHOP chemotherapy has an associated mortality rate of up to 38%. Radiotherapy is often considered over surgery and surgery has been most frequently used when associated with bowel complications. In this case report we describe upfront Ritiximab followed by consolidation resection and cytotoxic chemotherapy as a management strategy to reduce toxicity.The approach taken by our surgical team illustrates the benefits of disease debulking in certain cases of PTLD, by guiding further therapy and spacing and reducing chemotherapy in immune suppressed patients.
| S-EPMC5537377 | biostudies-other
Project description: Not available
| S-EPMC7141437 | biostudies-literature
Project description:Unravelling the heterogenous molecular landscape of pediatric post-transplant lymphoproliferative disorders
| PRJEB50295 | ENA
Project description:BACKGROUND:Although the transpulmonary gradient (TPG) and pulmonary vascular resistance (PVR) are commonly used to differentiate heart failure patients with pulmonary vascular disease from those with passive pulmonary hypertension (PH), elevations in TPG and PVR may not always reflect pre-capillary PH. Recently, it has been suggested an elevated diastolic pulmonary artery pressure-to-pulmonary capillary wedge pressure gradient (DPG) may be a better indicator of pulmonary vascular remodeling, and therefore, may be of added prognostic value in patients with PH being considered for cardiac transplantation. METHODS:Using the United Network for Organ Sharing (UNOS) database, we retrospectively reviewed all primary adult (age > 17 years) orthotropic heart transplant recipients between 1998 and 2011. All patients with available pre-transplant hemodynamic data and PH (mean pulmonary artery pressure ? 25 mm Hg) were included (n = 16,811). We assessed the prognostic value of DPG on post-transplant survival in patients with PH and an elevated TPG and PVR. RESULTS:In patients with PH and a TPG > 12 mm Hg (n = 5,827), there was no difference in survival at up to 5 years post-transplant between high DPG (defined as ?3, ?5, ?7, or ?10 mm Hg) and low DPG (<3, <5, <7, or <10 mm Hg) groups. Similarly, there was no difference in survival between high and low DPG groups in those with a PVR > 3 Wood units (n = 6,270). Defining an elevated TPG as > 15 mm Hg (n = 3,065) or an elevated PVR > 5 (n = 1,783) yielded similar results. CONCLUSIONS:This large analysis investigating the prognostic value of DPG found an elevated DPG had no effect on post-transplant survival in patients with PH and an elevated TPG and PVR.
| S-EPMC3955214 | biostudies-literature
Project description:Post-transplant lymphoproliferative disorders (PTLDs) are life-threatening complications arising after solid organ or hematopoietic stem cell transplantations. Although the majority of these lymphoproliferations are of B cell origin, and are frequently associated with primary Epstein-Barr virus (EBV) infection or reactivation in the post-transplant period, rare cases of T cell and natural killer (NK) cell-originated PTLDs have also been described. A general assumption is that PTLDs result from the impairment of anti-viral and anti-tumoral immunosurveillance due to the long-term use of immunosuppressants in transplant recipients. T cell impairment is known to play a critical role in the immune-pathogenesis of post-transplant EBV-linked complications, while the role of NK cells has been less investigated, and is probably different between EBV-positive and EBV-negative PTLDs. As a part of the innate immune response, NK cells are critical for protecting hosts during the early response to virus-induced tumors. The complexity of their function is modulated by a myriad of activating and inhibitory receptors expressed on cell surfaces. This review outlines our current understanding of NK cells in the pathogenesis of PTLD, and discusses their potential implications for current PTLD therapies and novel NK cell-based therapies for the containment of these disorders.
| S-EPMC8068932 | biostudies-literature
Project description:PTLD is a rare but severe complication of hematopoietic or solid organ transplant recipients, with variable incidence and timing of occurrence depending on different patient-, therapy-, and transplant-related factors. The pathogenesis of PTLD is complex, with most cases of early PLTD having a strong association with Epstein-Barr virus (EBV) infection and the iatrogenic, immunosuppression-related decrease in T-cell immune surveillance. Without appropriate T-cell response, EBV-infected B cells persist and proliferate, resulting in malignant transformation. Classification is based on the histologic subtype and ranges from nondestructive hyperplasias to monoclonal aggressive lymphomas, with the most common subtype being diffuse large B-cell lymphoma-like PTLD. Management focuses on prevention of PTLD development, as well as therapy for active disease. Treatment is largely based on the histologic subtype. However, given lack of clinical trials providing evidence-based data on PLTD therapy-related outcomes, there are no specific management guidelines. In this review, we discuss the pathogenesis, histologic classification, and risk factors of PTLD. We further focus on common preventive and frontline treatment modalities, as well as describe the application of novel therapies for PLTD and elaborate on potential challenges in therapy.
| S-EPMC9740763 | biostudies-literature
Project description:BackgroundAlthough rare, external compression of the left main coronary artery (LMCA) by a pulmonary arterial aneurysm (PAA) as a consequence of pulmonary arterial hypertension causing stable angina pectoris is well described. However, acute myocardial infarction is extremely rare, particularly with a full array of electrocardiographic, biochemical, and echocardiographic features, as in this scenario.CaseIn this case, a 62-year-old man with a past history of severe fibrotic lung disease was hospitalised with chest pain. The patient had dynamic anterolateral ischaemic changes on electrocardiography and serially elevated high-sensitivity troponin I. Transthoracic echocardiography revealed impaired left ventricular ejection fraction with anterolateral hypokinesis. Coronary angiography with intracoronary imaging revealed external compression of the LMCA. Computer tomography (CT) scans confirmed new PAA, compared to previous scans. The patient was successfully treated by percutaneous coronary stent implantation.ConclusionProgressive dilatation of the pulmonary artery due to pulmonary arterial hypertension can result in acute MI secondary to external compression of the LMCA. Clinicians should be mindful of acute coronary syndromes in patients with long-standing pulmonary hypertension presenting with chest pain.
| S-EPMC7925067 | biostudies-literature
Project description:BACKGROUND:Post-transplant lymphoproliferative disorder (PTLD) of T cell type has been rarely reported. Accurate diagnosis of this life-threatening rare form of PTLD is important for the treatment strategy. CASE PRESENTATION:A 7-year-old boy had severe diarrhea and weight loss progressively at 7?years post-living donor liver transplantation (LDLT) for biliary atresia. Endoscopy in the gastrointestinal (GI) tract revealed multiple erosions and ulcer lesions with prominent intraepithelial lymphocytosis in the duodenum and terminal ileum. Immunohistochemical examination demonstrated that these accumulated lymphocytes mainly comprised small- to medium-sized T cells expressing CD3, CD4, CD5, CD7, and CD103, but lacking CD8, CD56, and Epstein-Barr virus-encoded small RNAs. In addition, T cell receptor ? gene rearrangement was detected by polymerase chain reaction analysis. Comprehensively, the lesions were best interpreted as post-transplant indolent T cell lymphoproliferative disorder (LPD) of the intestine. Clinical remission was achieved by reducing the immunosuppressant. CONCLUSION:A rarely reported indolent type of T cell LPD in post-LDLT was diagnosed by direct inspection and histological investigation. Although the histological classification and therapeutic strategy for post-transplant indolent T cell LPD have not been established, reducing immunosuppression allowed complete remission in our case. To prevent the incidence of PTLD and de novo malignancy, developing a methodology to set a proper dose of immunosuppressant is required.
| S-EPMC7316934 | biostudies-literature
Project description:BackgroundPulmonary artery aneurysms (PAAs) are rare and they are infrequently diagnosed due to the non-specificity of their symptoms. However, their related complications, mainly described in patients with pulmonary hypertension (PH), are associated with significant morbidity and mortality.Case summaryWe report the case of a 64-year-old woman previously operated on for pulmonary valve stenosis, who presented with rapid worsening of dyspnoea and sudden onset of chest pain. Physical examination did not show heart failure symptoms, and an echocardiogram showed significant but not severe pulmonary regurgitation with preserved right and left ventricular function. Estimated pulmonary artery (PA) pressure was normal. As myocardial ischaemia was suspected the patient underwent a coronary computed tomography angiography that showed compression of the left main coronary artery by a large PAA. Early diagnosis led to surgery that solved her symptoms.DiscussionComprehensive medical evaluation of symptomatic patients with PA dilatation, even in the absence of PH, is key to rule out the possibility of serious complications as soon as possible.
| S-EPMC6426109 | biostudies-literature