Multicentric reticulohistiocytosis: the Mayo Clinic experience (1980–2017)

Rheumatology ◽  
2019 ◽  
Vol 59 (8) ◽  
pp. 1898-1905 ◽  
Author(s):  
Catalina Sanchez-Alvarez ◽  
Avneek Singh Sandhu ◽  
Cynthia S Crowson ◽  
David A Wetter ◽  
Gavin A McKenzie ◽  
...  
Keyword(s):  
Mayo Clinic ◽  
Treatment Options ◽  
Unmet Need ◽  
Skin Lesions ◽  
Multicentric Reticulohistiocytosis ◽  
Need For Treatment ◽  
Clinic Experience ◽  
Joint Effusions ◽  
Rheumatic Conditions ◽  
Record Review

Abstract Objectives Multicentric reticulohistiocytosis (MRH), a rare histiocytic disease that can mimic other rheumatic conditions, may be associated with cancer and other autoimmune disorders. To better understand the disorder and its other associations, we aimed to evaluate clinical correlates and outcomes of all patients with MRH seen at Mayo Clinic, Rochester between 1980 and 2017. Methods A retrospective medical record review was conducted to identify all patients with MRH between 1 January 1980 and 30 April 2017. Results We identified 24 patients with biopsy-proven MRH (58% female, 75% Caucasian, median age at diagnosis 52 years, median follow-up of 2.3 years). All patients had cutaneous and articular involvement; 23 (96%) patients had papulonodular skin lesions (87% periungual and dorsal hand) and seven (30%) mucosal nodules; and 22 (92%) patients had arthralgias, 21 (88%) joint effusions and 13 (54%) synovitis. Most frequently used therapies included corticosteroids, cyclophosphamide, methotrexate and bisphosphonates. Biologics were used in four patients. Nine patients had symptomatic resolution at 1 year and 12 partial improvement. Radiological findings included erosive changes in three (60%) patients and arthritis mutilans in two patients (40%). Twenty-nine per cent of patients had a concomitant autoimmune disease and 25% malignancy including melanoma, endometrial, peritoneal and lung carcinoma. The 5-year survival rate was 85% (95% CI: 74, 100%). Conclusion To our knowledge, this is the largest single-centre series of patients with MRH highlighting the rarity of the condition and an unmet need for treatment options that can allow sustained disease remission. It also highlights the need for a high vigilance for malignancy and autoimmune diseases.

scholarly journals A combination of potently neutralizing monoclonal antibodies isolated from an Indian convalescent donor protects against the SARS-CoV-2 delta variant

2021 ◽  
Author(s):  
Nitin Hingankar ◽  
Suprit Deshpande ◽  
Payel Das ◽  
Zaigham Abbas Rizvi ◽  
Alison Burns ◽  
...  
Keyword(s):  
Monoclonal Antibodies ◽  
Local Level ◽  
Treatment Options ◽  
Unmet Need ◽  
Mice Model ◽  
Middle Income ◽  
Virus Attachment ◽  
Vaccine Responses ◽  
Need For Treatment ◽  
Global Pandemic

Although efficacious vaccines have significantly reduced the morbidity and mortality due to COVID-19, there remains an unmet medical need for treatment options, which monoclonal antibodies (mAbs) can potentially fill. This unmet need is exacerbated by the emergence and spread of SARS-CoV-2 variants of concern (VOCs) that have shown some resistance to vaccine responses. Here we report the isolation of two highly potently neutralizing mAbs (THSC20.HVTR04 and THSC20.HVTR26) from an Indian convalescent donor, that neutralize SARS-CoV-2 VOCs at picomolar concentrations including the delta variant (B.1.617.2). These two mAbs target non-overlapping epitopes on the receptor-binding domain (RBD) of the spike protein thereby preventing the virus attachment to its host receptor, human angiotensin converting enzyme-2 (hACE2). Furthermore, the mAb cocktail demonstrated protection against the Delta variant at low antibody doses when passively administered in the K18 hACE2 transgenic mice model, highlighting their potential as cocktail for prophylactic and therapeutic applications. Developing the capacity to rapidly discover and develop mAbs effective against highly transmissible pathogens like coronaviruses at a local level, especially in a low- and middle-income country (LMIC) such as India, will enable prompt responses to future pandemics as an important component of global pandemic preparedness.

Stargardt and Fundus Flavimaculatus; Current and Developing Treatments

2021 ◽  
pp. 190-200
Keyword(s):  
Treatment Options ◽  
Unmet Need ◽  
Macular Dystrophy ◽  
Stargardt Disease ◽  
Need For Treatment ◽  
Complement Inhibitors ◽  
Abca4 Gene ◽  
Hereditary Retinal Degeneration ◽  
Pigment Epithelial Cells ◽  
Fundus Flavimaculatus

Stargardt macular dystrophy is a hereditary retinal degeneration that lacks effective treatment options. The pathophysiology of the disease is still not fully understood. While there are currently no available treatments for Stargardt disease, there are many categories of therapeutics under investigation to fulfill this unmet need for treatment. These include investigational visual cycle modulators, complement inhibitors, ABCA4 gene therapy, and subretinal transplantation of stem cell-derived retina pigment epithelial cells. Further trials are warranted to assess efficacy and safety in humans. In this review, the treatments investigated for the Stargardt disease are explained.

scholarly journals Multiple Reticulohistiocytomas in an 88-year-old Man: a Case Report

2013 ◽  
Vol 5 (2) ◽  
pp. 87-93
Author(s):  
Branislava Gajić ◽  
Tatjana Roš ◽  
Andrijana Arapović ◽  
Nada Vučković ◽  
Aleksandra Fejsa Levakov
Keyword(s):  
Treatment Options ◽  
Skin Lesions ◽  
Systemic Involvement ◽  
Multicentric Reticulohistiocytosis ◽  
Mucosal Involvement ◽  
Multinuclear Cells ◽  
Systemic Symptoms ◽  
One Year ◽  
Foamy Cytoplasm

Abstract Solitary and multiple reticulohistiocytomas, often referred to as diffuse cutaneous reticulohistiocytosis, that are generally restricted to skin, must be differentiated from multicentric reticulohistiocytosis. Multicentric reticulohistiocytosis is a rare histiocytic proliferative disease affecting joints, skin and mucous membranes, while arthropathy usually precedes skin and mucosal involvement. Other organs may also be involved, and 20% of patients have an associated internal malignancy. Multicentric reticulohistiocytosis has been reported to be associated with autoimmune diseases and malignancies. We present an 88-year-old man, with a sudden eruption of asymptomatic, firm, skin to brown colored papules and nodules, 6 to 17 mm in diameter, localized on forehead, arms, legs, and buttocks. There were no symptoms of joint or other organ involvement. Clinical and dermoscopic characteristics pointed to reticulohystiocytomas. Multiple excision biopsies of different tumors were performed and histopathology reports confirmed the diagnosis. In the case presented, reticulohistiocytosis was limited to the skin, exhibiting multiple reticlohistiocytomas with typical clinical, dermoscopic and rather peculiar histopathology presentation. Apart from this, some histologic features were seen in early lesions of multicentric reticulohistiocytosis, such as multinuclear cells dissociating collagen fibers with pale eosinophilic and foamy cytoplasm. Besides surgical excisions, no other treatment options were done. Laboratory and other tests showed no presence of extracutaneous illness, and no autoimmune or paraneoplastic processes. At one year follow up, the remaining tumors were of the same size, but there were no recurrences at excision sites, no signs of disease progression or systemic involvement. Since diffuse cutaneous reticulohistiocytosis without arthropathy as well as isolated reticulohistiocytomas have been described, in some cases of multiple reticulohistiocytomas even without systemic symptoms and signs, multicentric reticulohistiocytosis should be considered with an appropriate follow up. In such cases, skin lesions have the same histological features as lesions in multicentric reticulohistiocytosis, but they are not associated with joint problems or neoplasms.

scholarly journals Immunotherapy Predictive Molecular Markers in Advanced Gastroesophageal Cancer: MSI and Beyond

Cancers ◽  
2021 ◽  
Vol 13 (7) ◽  
pp. 1715
Author(s):  
Robin Park ◽  
Laercio Lopes ◽  
Anwaar Saeed
Keyword(s):  
Treatment Options ◽  
Unmet Need ◽  
Predictive Biomarkers ◽  
Phase Iii ◽  
Gastroesophageal Cancer ◽  
Programmed Death ◽  
Phase Iii Trials ◽  
Tumor Mutational Burden ◽  
Large Phase ◽  
Programmed Death 1

Advanced gastroesophageal cancer (GEC) has a poor prognosis and limited treatment options. Immunotherapy including the anti-programmed death-1 (PD-1) antibodies pembrolizumab and nivolumab have been approved for use in various treatment settings in GEC. Additionally, frontline chemoimmunotherapy regimens have recently demonstrated promising efficacy in large phase III trials and have the potential to be added to the therapeutic armamentarium in the near future. There are currently several immunotherapy biomarkers that are validated for use in the clinical setting for GEC including programmed death ligand-1 (PD-L1) expression as well as the tumor agnostic biomarkers such as mismatch repair or microsatellite instability (MMR/MSI) and tumor mutational burden (TMB). However, apart from MMR/MSI, these biomarkers are imperfect because none are highly sensitive nor specific. Therefore, there is an unmet need for immunotherapy biomarker development. To this end, several biomarkers are currently being evaluated in ongoing trials with some showing promising predictive potential. Here, we summarize the landscape of immunotherapy predictive biomarkers that are currently being evaluated in GEC.

scholarly journals MP-AzeFlu in Moderate-to-Severe Allergic Rhinitis: A Literature Review

2021 ◽  
pp. 1-10
Author(s):  
Ludger Klimek ◽  
William E. Berger ◽  
Jean Bousquet ◽  
Paul K. Keith ◽  
Peter Smith ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Allergic Rhinitis ◽  
Fluticasone Propionate ◽  
Treatment Options ◽  
Symptomatic Disease ◽  
Intranasal Corticosteroids ◽  
Need For Treatment ◽  
Ocular Symptoms ◽  
Symptom Complex

Allergic rhinitis (AR) is prevalent, and many patients present with moderate-to-severe symptomatic disease. The majority of patients are not satisfied with their AR treatment, despite the use of concurrent medications. These gaps underscore the need for treatment with more effective options for moderate-to-severe AR. The authors’ objective was to review systematically the efficacy and safety of MP-AzeFlu for the treatment of AR. The primary outcomes studied were nasal, ocular, and total symptoms. Other outcomes included time to onset and of AR control, quality of life, and safety. Searches of PubMed and Cochrane databases were conducted on May 14, 2020, with no date restrictions, to identify publications reporting data on MP-AzeFlu. Clinical studies of any phase were included. Studies were excluded if they were not in English, were review articles, did not discuss the safety and efficacy of MP-AzeFlu for AR symptoms. Treatment of AR with MP-AzeFlu results in effective, sustained relief of nasal and ocular symptoms, and faster onset and time to control compared with intranasal azelastine or fluticasone propionate. Long-term use of MP-AzeFlu was safe, with benefits in children, adults, and adults aged ≥65 years. Other treatment options, including fluticasone propionate and azelastine alone or the combination of intranasal corticosteroids and oral antihistamine, do not provide the same level of efficacy as MP-AzeFlu in terms of rapid and sustained relief of the entire AR symptom complex. Furthermore, MP-AzeFlu significantly improves patient quality of life. MP-AzeFlu is a currently available combination that may satisfy all these patient needs and expectations.

Solitary plasmacytoma of bone: mayo clinic experience

1989 ◽  
Vol 16 (1) ◽  
pp. 43-48 ◽  
Author(s):  
Deborah A. Frassica ◽  
Frank J. Frassica ◽  
Mark F. Schray ◽  
Franklin H. Sim ◽  
Robert A. Kyle
Keyword(s):  
Mayo Clinic ◽  
Solitary Plasmacytoma ◽  
Clinic Experience
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