Retroperitoneal and lower extremities muscle bleeding in acquired haemophilia A (AHA): Risk factors and implications in disability and survival.

Aim: To assess risk factors of retroperitoneal and lower extremity musculoskeletal bleed in acquired haemophilia (AHA) and perform an objective assessment of disability and influence on survival.

Methods: We included 49 patients with AHA from November 2017 to May 2023. The occurrence of any retroperitoneal or/and lower extremities bleeding manifestation was investigated.

Key Genes of the Immune System and Predisposition to Acquired Hemophilia A: Evidence from a Spanish Cohort of 49 Patients Using Next-Generation Sequencing.

Acquired hemophilia A (AHA) is a rare bleeding disorder caused by the presence of autoantibodies against factor VIII (FVIII). As with other autoimmune diseases, its etiology is complex and its genetic basis is unknown. The aim of this study was to identify the immunogenetic background that predisposes individuals to AHA.

Severe bleeding diathesis as onset of light-chain amyloidosis: combined excessive fibrinolysis and acquired von Willebrand disease in a young patient.

Our report shows a case of primary light-chain amyloidosis in a young patient that reflects the potential severity of bleeding diathesis associated with this plasma cell dyscrasia and the difficulty of diagnosis when only hemorrhagic manifestations are present at the onset of disease. The patient presented with recurrent and severe muscular bleeding secondary to associated acquired von Willebrand disease and fibrinolysis dysfunction. Treatment with bortezomib-cyclophosphamide and sequential hematopoietic stem cell transplantation solved coagulation alterations.

Acquired von Willebrand syndrome in a patient with small lymphocytic lymphoma and Sjögren's syndrome: which associated condition should be prioritized?

: Acquired von Willebrand syndrome (AVWS) is a rare hemorrhagic condition that poses both a diagnostic and a therapeutic challenge. Here we report a singular case of AVWS with two associated conditions, small lymphocytic lymphoma (SLL) and Sjögren's syndrome. The patient presented with recurrent and severe digestive bleeding that forced us to raise a curative attempt of AVWS.

Autoantibodies to types I and IV collagen and heart valve disease in systemic lupus erythematosus/antiphospholipid syndrome.

Introduction/objectives autoantibodies to types I and IV collagen have been described in rheumatic fever and infective endocarditis. We tried to elucidate if an autoimmune response against collagens I and IV exists, associated with heart valve disease in systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS). A cohort of 172 patients with SLE (n = 79), primary APS (PAPS, n = 83), and secondary APS (n = 10) were assessed for valvulopathy by transthoracic echocardiograms.

IgG4-Related Disease: Results From a Multicenter Spanish Registry.

IgG4-related disease (IgG4-RD) is a rare entity consisting of inflammation and fibrosis that has been described in multiple organs. Concrete diagnostic criteria have been established recently and there is a lack of large series of patients.To describe the clinical presentation, histopathological characteristics, treatment and evolution of a series of IgG4-RD Spanish patients.

Cardiac manifestations other than valvulopathy in antiphospholipid syndrome: long-time echocardiography follow-up study.

Aim: Non-valvular cardiac disease in the antiphospholipid syndrome (APS) has been scanty studied. We wanted to assess the prevalence and evolution of left myocardial disease, pulmonary hypertension and intracardiac thrombi in a cohort of APS patients.

Method: A total of 53 patients with APS, either primary (n = 34, 64%) or associated to lupus (n = 19, 36%) and 20 controls were included.

[Home intravenous antimicrobial therapy in multi-drug resistant microorganism infections].

Background: Although home intravenous antimicrobial infusion therapy (HIVAIT) has proved its safety and efficacy in a great number of common infections, there are few published studies about its role in the treatment of infections caused by multi-drug resistant microorganisms. Our objectives are to study clinical and epidemiological characteristics of patients with multi-drug resistant microorganism infections treated with HIVAIT, and its usefulness in this type of infections.

Methods: We analyzed all patients diagnosed of infections requiring HIVAIT and admitted to our Hospital at Home Unit (HHU) from March 2007 to February 2010.

[Home intravenous antibiotic therapy of hepatic abscess: Safety, efficacy and predictive factor of hospital readmission].

Background And Objective: Few data exists in the literature about intravenous antibiotic treatment of hepatic abscess in hospital based care units. Our objectives were to analyze the efficacy and safety of intravenous antibiotic therapy and the predictors of hospital readmission.

Patients And Methods: The study included all the patients admitted in our home care unit for intravenous antibiotic treatment of hepatic abscess from March 2006 to June 2009.

[Intravenous antibiotic treatment in a hospital based home care unit. Predictors of hospital readmission].

Background And Objective: Intravenous antibiotic therapy at home has showed its efficacy as an alternative to hospitalization care in many infectious pathologies. The objectives of this study are: a) to expose our experience, as hospital at home unit (HHU) integrated within a service of internal medicine, in the antibiotic treatment, and b) to define those parameters that can predict hospital readmissions.

Patients And Method: This study included all patients with infectious pathology and intravenous antibiotic therapy who were admitted in our HHU from March 2006 to March 2007.

Protein Z levels and anti-protein Z antibodies in patients with arterial and venous thrombosis.

Introduction: The thrombotic risk associated with protein Z (PZ) deficiency is unclear. Anti-protein Z (anti-PZ) has been described as a risk factor in unexplained embryo demise. The aim of our study was to evaluate a possible PZ deficiency and presence of anti-PZ antibodies on thrombotic diseases.