scholarly journals Successful treatment of acquired haemophila a associated with psoriasis: Case report

2005 ◽  
Vol 133 (Suppl. 2) ◽  
pp. 134-136
Author(s):  
Goran Marjanovic ◽  
Vesna Marjanovic ◽  
Ljubinka Velickovic ◽  
Mladen Milenovic ◽  
Lana Macukanovic-Golubovic ◽  
...  

Acquired factor VIII (FVIII) inhibitor causes a rare but lifethreatening form of bleeding disorder, owing to the formation of autoantibodies against FVIII. Treatment modalities include the use of immunosuppressive drugs, such as cyclophosphamide and corticosteroids, plasmapheresis, and i.v. immunoglobulin. The case of a 67-year-old patient with acquired FVIII inhibitor and psoriasis presented us with a serious bleeding complication. Bleeding reacted poorly to therapy with corticosteroids and cyclophosphamide. Treatment with cyclosporin, however, resulted in a prompt and complete response. The lack of side effects and the relatively quick response suggest that cyclosporin A should be tried as a frontline treatment for patients with acquired FVIII inhibitor.

2018 ◽  
Vol 11 (2) ◽  
pp. 425-430 ◽  
Author(s):  
Maurizio Lucchesi ◽  
Enrico Chiappa ◽  
Flavio Giordano ◽  
Francesco Mari ◽  
Lorenzo Genitori ◽  
...  

Introduction: Cardiac rhabdomyomas represent a frequent manifestation of tuberous sclerosis. Tumor growth, mainly prenatally, can result in intrauterine fetal or neonatal deaths in almost 10% of patients. Case Report: We treated 3 consecutive infants aged less than 12 months with sirolimus, an oral mTOR inhibitor. All patients achieved significant reductions in cardiac rhabdomyomas. A complete response was documented in 2 patients, while a partial response with tumor debulking greater than 50% was seen in the other one. The median time to best cardiac response was 1.9 months in all patients, and 3.3 months in those with complete response. The side effects profile was acceptable. Conclusion: Sirolimus may have a significant role in promoting natural regression of cardiac rhabdomyomas. Prospective clinical trials are needed.


2019 ◽  
Author(s):  
BA Högerle ◽  
EL Bulut ◽  
L Klotz ◽  
F Eichhorn ◽  
M Eichhorn ◽  
...  

2019 ◽  
Vol 12 (2) ◽  
pp. 126-134 ◽  
Author(s):  
Shahad Alsadik ◽  
Siraj Yusuf ◽  
Adil AL-Nahhas

Background: The incidence of pancreatic Neuroendocrine Tumours (pNETs) has increased considerably in the last few decades. The characteristic features of this tumour and the development of new investigative and therapeutic methods had a great impact on its management. Objective: The aim of this review is to investigate the outcome of Peptide Receptor Radionuclide Therapy (PRRT) in the treatment of pancreatic neuroendocrine tumours. Methods: A comprehensive literature search strategy was used based on two databases (SCOPUS, and PubMed). We considered all studies published in English, evaluating the use of PRRT (177Luteciuim- DOTA-conjugated peptides and 90Yetrium- DOTA- conjugated peptides) in the treatment of pancreatic neuroendocrine tumours as a standalone entity or as a subgroup within the wider category of Gastroenteropancreatic Neuroendocrine Tumours (GEP NETs). Results: PRRT was found to be an effective treatment modality as a monotherapy or in combination with other therapies in the treatment of non-operable and metastatic pNETs where other options are limited. Complete response was reported to be between 2-6% while partial response was achieved in up to 60% of cases. Survival analysis was also impressive. Progression Free Survival (PFS) reached a mean of 34 months and Overall Survival (OS) of 53 months. PRRT also proved to improve patients’ Quality of Life (QoL). Acute and sub-acute side effects like nephrotoxicity and haematotoxicity are usually mild and reversible. Conclusion: PRRT is well tolerated and effective treatment option for non-operable and/or metastatic pNETs. Side effects are usually mild and reversible. Larger randomized controlled trails need to be done to compare PRRT with other treatment modalities and to provide more detailed guidelines regarding patient selections, the choice of PRRT, follow up and response assessment to maximum potential benefit.


1970 ◽  
Vol 5 (4) ◽  
pp. 61-66
Author(s):  
Patrícia Resende Penido ◽  
Rhanna Junqueira Westin de Carvalho ◽  
Roger Willian Moraes Mendes

RESUMOIntrodução: A Arterite de Takayasu (AT) consiste em uma vasculopatia de origem indefinida, sendo de caráter crônico, que afeta a aorta e seus ramos principais. Em gestantes é uma condição complexa, em que o tratamento clínico é realizado com restrições. A utilização de corticoides tem sido favorável no controle inflamatório, principalmente naqueles casos em que antes da gravidez se fazia uso de imunossupressores. Casuística: Foi relatado um caso de uma gestante portadora de AT, através da análise de prontuário e de exames complementares, sendo realizado o pré-natal pelas equipes de Obstetrícia e Reumatologia, onde foi realizado tratamento clínico com corticoides, mostrando uma evolução satisfatória, ocorrendo apenas uma hospitalização que foi seguida de uma cesárea na trigésima sexta semana de gravidez, com recém nato saudável de 3.810g. Discussão: A AT pode estar associada a várias etiologias, sendo a gênese pouco conhecida. O diagnóstico na maioria das vezes é demorado, pela dificuldade da suspeita clínica, além de demandar o uso de técnicas de imagem mais sofisticadas. A gestação associada é fenômeno raro, já que as portadoras são orientadas a evitarem a gravidez. O controle clínico permitiu uma gestação sem grandes complicações e serviu como meio para levar a gestação até praticamente o fim do terceiro trimestre. Conclusão: Deve-se ressaltar o papel do acompanhamento clínico, especialmente com esta pan-arterite, mostrando os medicamentos que podem ser utilizados nesse grupo, especialmente quando se usava imunossupressores antes da gravidez. Palavras chave: Arterite de Takayasu, Gravidez, Vasculite. ABSTRACT:Introduction: Takayasu's arteritis (TA) consists of a vascular disease of unknown origin and chronic nature, which affects the aorta and its main branches. In pregnant women it is a complex condition, in which the clinical treatment is performed with restrictions. The use of corticosteroids has been favorable to control inflammation, especially in those cases when immunosuppressant had been used before pregnancy. Case Report: A case of a pregnant patient with TA has been reported through the analysis of medical records and laboratory tests. The Obstetrics and Rheumatology staff performed the prenatal care, and clinical treatment with corticosteroids was done showing a satisfactory outcome. There was only one hospitalization of the patient, which was followed by a cesarean in the thirty sixth week of pregnancy.  The newborn was a healthy one, weighing 3.810g. Discussion: TA may be associated with several etiologies, and the genesis is little known. The diagnosis most often takes time due to the difficulty of clinical suspicion, and requires the use of more sophisticated imaging techniques. The pregnancy is rare phenomenon, since the carriers are advised to avoid pregnancy. The clinical management allowed a pregnancy without major complications and served as a means to carry the pregnancy to almost the end of the third quarter. Conclusion: The role of the clinical follow-up should be noticed especially with this pan-arteritis, showing the drugs that can be used in this group, especially when there is use of immunosuppressive drugs before pregnancy. Keywords: Takayasu Arteritis, Pregnancy, Vasculitis.


2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
Freja Lærke Sand ◽  
Simon Francis Thomsen

Patients with severe chronic urticaria may not respond to antihistamines, and other systemic treatment options may either be ineffective or associated with unacceptable side effects. We present data on efficacy and safety of adalimumab and etanercept in 20 adult patients with chronic urticaria. Twelve (60%) patients obtained complete or almost complete resolution of urticaria after onset of therapy with either adalimumab or etanercept. Further three patients (15%) experienced partial response. Duration of treatment ranged between 2 and 39 months. Those responding completely or almost completely had a durable response with a mean of 11 months. Six patients (30%) experienced side effects and five patients had mild recurrent upper respiratory infections, whereas one patient experienced severe CNS toxicity that could be related to treatment with TNF-alpha inhibitor. Adalimumab and etanercept may be effective and relatively safe treatment options in a significant proportion of patients with chronic urticaria who do not respond sufficiently to high-dose antihistamines or in whom standard immunosuppressive drugs are ineffective or associated with unacceptable side effects.


2021 ◽  
pp. 030089162110272
Author(s):  
Ginevra Lolli ◽  
Beatrice Casadei ◽  
Cinzia Pellegrini ◽  
Lisa Argnani ◽  
Federica Cocito ◽  
...  

Objective: Peripheral T-cell lymphomas (PTCLs) are a group of heterogeneous T-cell malignancies representing 5%–10% of aggressive lymphomas. The prognosis is poor for patients with relapsed/refractory (R/R) disease, with a median overall survival of less than 6 months and no standardized treatments. We discuss the role of the phosphatidylinositol 3-kinase (PI3K) γδ inhibitor duvelisib as bridge to allotransplantation in a patient with R/R PTCL. Methods: Case report. Results: A 55-year-old woman diagnosed with relapsed nodal PTCL with T-follicular helper phenotype received PI3K γδ inhibitor duvelisib in the context of the phase II PRIMO clinical trial. After two cycles at a dose of 75 mg twice daily, the patient achieved complete response (CR), which was subsequently consolidated with human leukocyte antigen fully matched unrelated donor allotransplantation. No major toxicities were recorded during the duvelisib treatment period or during hospitalization for allotransplantation. At the latest follow-up, the patient was alive and still in CR 10 months posttransplant. Conclusions: Duvelisib should be further explored as a bridge to allotransplantation in patients with R/R PTCL, given the success and low toxicity in our patient.


2021 ◽  
pp. 239719832110043
Author(s):  
Paulina Śmigielska ◽  
Justyna Czarny ◽  
Jacek Kowalski ◽  
Aleksandra Wilkowska ◽  
Roman J. Nowicki

Eosinophilic fasciitis is a rare connective tissue disease of unknown etiology. Therapeutic options include high-dose corticosteroids and other immunosuppressive drugs. We present a typical eosinophilic fasciitis case, which did not respond to first-line treatment, but improved remarkably after infliximab administration. This report demonstrates that in case of initial treatment failure, infliximab might be a relatively safe and effective way of eosinophilic fasciitis management.


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