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Related and unrelated nonmyeloablative hematopoietic stem cell transplantation for malignant diseases
Patients with advanced hematological malignancies ineligible for conventional myeloablative allogeneic hematopoietic stem cell transplantation (HSCT) due to advanced age or medical contraindications were enrol...
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Three German fibrinogen Aα-chain amyloidosis patients with the p.Glu526Val mutation
Plasma protein fibrinogen variants cause fibrinogen Aα-chain (AFib) amyloidosis, which presents with hypertension, proteinuria, and azotemia. Six AFib mutations have been reported thus far. We identified three...
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Effective prophylaxis of thromboembolic complications with low molecular weight heparin in relapsed multiple myeloma patients treated with lenalidomide and dexamethasone
The immunomodulatory drugs thalidomide and lenalidomide have enhanced activity in patients with multiple myeloma (MM). Their efficacy is increased with the addition of dexamethasone, but significant rates of v...
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Article
Amyloid in endomyocardial biopsies
The prognosis of cardiac amyloidosis depends on the nature and origin of the amyloid protein deposited. However, little is known about the prevalence and origin of amyloid in heart muscle biopsies. We therefor...
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Rapid assessment of longitudinal systolic left ventricular function using speckle tracking of the mitral annulus
Evaluation of left ventricular function (LV) is one of the most important tasks of echocardiography. Left ventricular longitudinal function has been recognized to differentiate myocardial disorders better than...
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Hereditary thrombocythemia caused by a thrombopoietin (THPO) gain-of-function mutation associated with multiple myeloma and congenital limb defects
Hereditary thrombocythemia (HT) has been described as a rare benign disorder caused by mutations in the thrombopoietin (THPO) or the c-Mpl receptor genes. Here we report two families with HT resulting from a T...
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Open AccessCardiac magnetic resonance strain imaging in systemic light chain amyloidosis
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Improvement of risk assessment in systemic light-chain amyloidosis using human placental growth factor
Vascular amyloid deposition is common in light-chain amyloidosis resulting in endothelial dysfunction. Human placental growth factor (PlGF), a member of the vascular endothelial growth factor family was found ...
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Open AccessQuantitative MR-neurographic parameters can determine and specify nerve injury in amyloid related polyneuropathy
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Open AccessMR-Neurography of the sural nerve in patients with hereditary amyloidosis
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Open AccessAmyloid in biopsies of the gastrointestinal tract—a retrospective observational study on 542 patients
In this retrospective observational study, we investigated the histopathological and demographic characteristics of amyloid in gastrointestinal biopsies. From the Amyloid Registry Kiel, we retrieved all cases ...
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Open AccessRegional differences in prognostic value of cardiac valve plane displacement in systemic light-chain amyloidosis
To compare the prognostic value of cardiac valve plane displacement (CVPD) on various locations in cardiac light chain (AL) amyloidosis.
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The impact of stem cell transplantation on the natural course of peripheral T-cell lymphoma: a real-world experience
The role of autologous stem cell transplantation (autoSCT) as consolidating treatment for peripheral T-cell lymphoma (PTCL) is unsettled. The aim of this analysis was to investigate the impact of autoSCT in th...
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European myeloma network recommendations on diagnosis and management of patients with rare plasma cell dyscrasias
The introduction of novel agents in the management of multiple myeloma and related plasma cell dyscrasias has changed our treatment approaches and subsequently the outcome of patients. Due to current advances,...
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Open AccessLocal vs. systemic pulmonary amyloidosis—impact on diagnostics and clinical management
Immunoglobulin-derived light-chain (AL) amyloidosis of lungs and bronchi can appear as a systemic and a local form. While systemic AL amyloidosis may need haemato-oncological care, the localised form can be tr...
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Open AccessDeferred autologous stem cell transplantation in systemic AL amyloidosis
High-dose melphalan with autologous stem cell transplantation (ASCT) can induce durable haematological and organ responses in systemic AL amyloidosis (AL). Stringent selection criteria have improved safety of ...
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Open AccessAL amyloidosis with a localized B cell neoplasia
Immunoglobulin light chain-derived (AL) amyloidosis may occur as a systemic disease usually with dismal prognosis and a localized variant with favorable outcome. We report 29 patients with AL amyloidosis and a...
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Open AccessCryo-EM structure of a light chain-derived amyloid fibril from a patient with systemic AL amyloidosis
Amyloid fibrils derived from antibody light chains are key pathogenic agents in systemic AL amyloidosis. They can be deposited in multiple organs but cardiac amyloid is the major risk factor of mortality. Here...
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Open AccessPrognostic value of novel imaging parameters derived from standard cardiovascular magnetic resonance in high risk patients with systemic light chain amyloidosis
The differentiated assessment of functional parameters besides morphological changes is essential for the evaluation of prognosis in systemic immunoglobulin light chain (AL) amyloidosis.
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Carpal tunnel syndrome and spinal canal stenosis: harbingers of transthyretin amyloid cardiomyopathy?
Carpal tunnel syndrome (CTS) and spinal canal stenosis can be frequently observed in the medical history of patients with transthyretin amyloidosis (ATTR), both in the hereditary (mt-ATTR) and wild-type (wt-AT...