Cardiac amyloidosis (CA) still represents a frequently missed cause of heart failure with preserved ejection fraction (HFpEF). In the light of many new and effective therapies for immunoglobulin ...light chain amyloidosis (AL) and for transthyretin amyloidosis (ATTR), screening for amyloidosis as an important and potentially treatable diagnosis under the HFpEF becomes mandatory. A step-by-step algorithm for CA in HF patients was already provided by the guidelines. This review summarizes the role of all imaging modalities and biomarkers in the diagnosis and prognosis of both subtypes, the algorithm for diagnosis of CA, and new therapeutic options. It is the first Romanian publication which intends to bring altogether the current recommendations in the diagnosis and management of CA.
Waldenström macroglobulinemia (WM) is a low-grade B-cell clonal disorder with lymphoplasmacytic bone marrow involvement and monoclonal immunoglobulin M (IgM) with poor prognosis. Clinical features ...include anemia, thrombocytopenia, hepatosplenomegaly, lymphadenopathy and hyperviscosity. We report the case of a 42-year-old male who presented with unexplained weight loss. The diagnosis of WM was established after morphological and immunohistochemical examination of the patient's bone marrow along with an elevated serum IgM level. He started chemotherapy with cyclophosphamide, bortezomib, dexamethasone and, four months later, rituximab was added. Five months later the patient presented with relapse of the hematological disease, hyperviscosity syndrome and acute right heart failure. Echocardiography showed a dilated right ventricle with decreased global systolic function and severe systolic pulmonary artery pressure, with normal left cardiac function and volumes. Despite maximal medical therapy, the patient died in day six of hospitalization. This case is distinguished by the rapidly evolving rare haematological cancer despite optimal chemotherapy and a multidisciplinary approach associated with chemotherapy-related late right heart dysfunction in a young man, without any cardiovascular risk factors or previous myocardial disease.
Although acute myocarditis and coronary vasospasm are common differential diagnoses in the case of young patients with persistent ST elevation, the association of coronary vasospasm and acute ...fulminant myocarditis is a rare situation
. We present the case of a 21 year-old male who presented with chest pain, ECG changes and biomarker levels initially interpreted as ST elevation myocardial infarction (STEMI), in which severe coronary vasospasm was identified. Shortly after, he developed cardiogenic shock and fulminant acute myocarditis was suspected.
The Coronavirus disease 2019 (COVID-19) pandemic, caused by the novel severe acute respiratory syndrome coronavirus 2 (SARS-CoV2), exhibits a wide spectrum of manifestations, from asymptomatic ...presentations to acute respiratory failure, myocardial injury, arterial or venous thrombosis, multiorgan failure, and death. Although COVID-19 mainly disrupts the respiratory syndrome, it has been shown to have detrimental impact on cardiac morphology and function, resulting in a broad range of cardiovascular complications and poor outcome, increasing morbidity and mortality of these patients. This review will summarize the knowledge on characterization of myocardial injury in COVID 19, by using cardiac biomarkers, electrocardiographic, and cardiac multi-modality imaging findings for an early and accurate diagnosis, proper management, correct treatment, and follow-up of COVID-19 patients.
Transthyretin amyloid cardiomyopathy (ATTR-CM) is an infiltrative disorder characterized by extracellular myocardial deposits of amyloid fibrils, with poor outcome, leading to heart failure and ...death, with significant treatment expenditure. In the era of a novel therapeutic arsenal of disease-modifying agents that target a myriad of pathophysiological mechanisms, timely and accurate diagnosis of ATTR-CM is crucial. Recent advances in therapeutic strategies shown to be most beneficial in the early stages of the disease have determined a paradigm shift in the screening, diagnostic algorithm, and risk classification of patients with ATTR-CM. The aim of this review is to explore the utility of novel specific non-invasive imaging parameters and biomarkers from screening to diagnosis, prognosis, risk stratification, and monitoring of the response to therapy. We will summarize the knowledge of the most recent advances in diagnostic, prognostic, and treatment tailoring parameters for early recognition, prediction of outcome, and better selection of therapeutic candidates in ATTR-CM. Moreover, we will provide input from different potential pathways involved in the pathophysiology of ATTR-CM, on top of the amyloid deposition, such as inflammation, endothelial dysfunction, reduced nitric oxide bioavailability, oxidative stress, and myocardial fibrosis, and their diagnostic, prognostic, and therapeutic implications.
Background
Rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone (RCHOP) chemotherapy in non‐Hodgkin's lymphoma (NHL) has risk of cardiotoxicity.
Purpose
To determine the role of ...myocardial work and biomarkers in subclinical diagnosis and prediction of cardiotoxicity.
Methods
The 130 NHL patients (52 ± 9 years, 62% men) scheduled for RCHOP, with LVEF>50%, were evaluated at baseline, after third cycle and chemotherapy completion for 3D LVEF, 2D myocardial deformation (longitudinal, radial, circumferential strain ‐ LS, RS, CS) and myocardial work (global constructive work, waste work, work index and work efficiency ‐ GCW, GWW, GWI, GWE). NT‐pro‐BNP and troponin I were determined.
Results
After chemotherapy ended, 37 patients (28%) (group I) developed asymptomatic cardiotoxicity (8 mild form, 25 moderate form, 4 severe form); 93 patients (group II) did not. After third cycle, all patients had decreased LS, CS, RS, GCW, GWI, GWE and increased GWW, persistent after chemotherapy completion, with significant changes in group I. After third cycle, GWE and GCW were the best independent predictors for LVEF reduction; GWE decrease with>5% after third cycle predicted cardiotoxicity after chemotherapy completed (91% sensitivity, 94% specificity).
Conclusions
In NHL, myocardial work can diagnose subclinical cardiotoxicity and predict LVEF decline. These parameters should be used for sensitive evaluation of myocardial function during chemotherapy.
In non‐Hodgkin lymphoma patients, assessment of myocardial work and cardiac biomarkers is able to detect early chemotherapy‐induced cardiotoxicity and to predict further 3DLVEF decline. Therefore, these parameters can be incorporated in clinical protocols, for better and accurate monitoring of myocardial function during chemotherapy and for early intervention in oncological patients.
The following case report and literature review will emphasize the individualized therapeutic management of a complex prothrombotic pathology. The onset of acute portal vein thrombosis in a patient ...with atrial fibrillation and good compliance to anticoagulation with a direct oral anticoagulant, who associates significant thrombocytosis, after excluding predisposing inflammations, infections or solid neoplasia, raises the diagnostic suspicion of myeloproliferative disorder, and imposes a complex interdisciplinary approach.
Pituitary adenomas are intracranial tumors with controversial histopathology and heterogeneous clinical behaviour. Angiogenesis and tumor blood vessels' role in pathogenesis, remain one of the great ...pituitary tumor mysteries. No connection between tumor vessel heterogeneity, hormonal profile and biological behaviour has been reported. We aimed to study pituitary adenomas blood vessels concerning their immature, intermediate or mature phenotype and microvessel density, correlated with immunohistochemical hormonal profile and hormone values in serum and cerebrospinal fluid.
We classified pituitary adenomas according to hormone profile and we applied a double immunostaining highlighting both endothelial and perivascular cells for a more accurate assessment of blood vessel types.
Overall microvessel density was found to be highest in growth hormone-secreting adenomas (48.51 ± 12.15) and lowest in prolactinomas (29.15 ± 18.78). When we differentially counted tumor blood vessels we observed a predominance of immature and intermediate blood vessels compared to mature ones. A significant correlation was found between immature tumor blood vessels and tissue prolactin expression, as assessed by immunhistochemistry (p=0.044). A partial correlation was found between serum (p=0.036) and cerebrospinal prolactin values (p=0.006) with immature and intermediate blood vessels. Also, a partial correlation has been reported only between mature blood vessels and cerebrospinal fluid prolactin values (p=0.008). No correlation was obtained for other types of pituitary adenomas.
Our results suggest a strong involvement of prolactin with a dual role in pituitary adenomas vasculature remodelling by acting both on endothelial and perivascular cells, a finding that could partially explain discrepancies between clinical diagnosis and hormonal profile.
Pituitary adenomas represent the third most common primary intracranial tumor in neurosurgical practice. To understand the biological behaviour of the pituitary adenomas previous studies have ...determined the tumor proliferation rate using monoclonal antibodies targeted against the Ki-67 antigen. The aim of this study was to correlate the Ki-67 index with hormonal profiles of pituitary adenomas. The study included 50 pituitary adenomas. For histopathologic evaluation, the sections were stained with routine hematoxylin and eosin method. Additional paraffin sections from each tumor were immunostained using primary antibodies against the following pituitary hormones: somatotropin (STH), prolactin (PRL), adrenocorticotrophic hormone (ACTH), thyroid-stimulating hormone (TSH), follicle-stimulating hormone (FSH), and luteinizing hormone (LH). To detect the expression of Ki-67 we used a mouse anti-human monoclonal antibody (clone K2). The percentage of Ki-67 positive nuclei (Ki-67 labeling index) was assessed by counting approximately 1000 nuclei of the tumor cells at ×400 magnification. Out of the 50 tumor samples, 31 (62%) pituitary adenomas showed proliferative activity, and the proliferation rate was variable in this group. The overall mean Ki-67 labeling index was 1.59 ± 1.47, ranging from 0.3% to 6.6%. In 5 cases, the Ki-67 index was >3%, all of them being prolactinomas. The Ki-67 index was higher in PRL-secreting adenomas (mean ± SD was 3.37 ± 1.80, range 0.9 - 6.6%). Our study provides the evidence that a higher Ki-67 value is associated with pituitary adenomas that secrete PRL (prolactinomas and mixed STH/PRL-secreting adenomas).
We compared the immunoprofile of pituitary adenomas from Romania and Moldova. One hundred and eighty cases coming from Romania (94 cases, group 1) and Moldova (86 cases, group 2) were assessed by ...immunohistochemistry regarding all six basic hormones expressed in pituitary adenomas. Specific differences and similarities were found and stated for both groups. In group 1, 70% of cases were pituitary adenomas positive for one hormone, 13% were plurihormonal, while 17% were negative. In group 2, 50,3% of the cases expressed only one hormone and 12,5% were negative for all hormones. The highest difference was observed for plurihormonal adenomas, found in about 37,2% of cases for group 2 (2.86 times higher for group 2 compared with group 1). A higher incidence of GH-secreting adenomas characterized group “1,” while group “2” had the highest percent of LH-secreting adenomas, 55% of cases being positive. Triple association was noticed in 4.25% of cases of group 1 and in 8,13% out of total cases, from group 2. Four-hormone association was found only in group 2, noticed in 15,56% of the cases. The present paper highlights strong evidences of a particular and different immunoprofile of pituitary adenomas coming from Romania and Moldova.
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