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Blood ; 136(Supplement 1):11-11, 2020.
Article in English | PMC | ID: covidwho-1339039


Introduction: The COVID-19 pandemic, caused by the SARS-CoV-2 virus, has become a global health crisis since it was first reported in December 2019. In a subset of infected subjects, pneumonia, multi-organ failure, and eventually death can occur. Frail patients and those with comorbidities are believed to be at increased risk of severe manifestations of SARS-CoV-2 infection. Patients with light chain (AL) amyloidosis have a hematologic malignancy that causes multi-organ dysfunction and can be at higher risk of complications and death. The International Society of Amyloidosis (ISA) has issued a guidance (Kastritis et al. BJH 2020, for patients with amyloidosis during the pandemic and called for an international data collection in April 2020. Aim of this study is to report the preliminary data of the ongoing international survey regarding systemic AL amyloidosis and COVID-19.Methods: The survey was proposed by the ISA Board and approved by the coordinating institution's Ethics Committee. All members of the ISA were invited to participate by email and a link for participation is online on ISA website. RedCap software was used for the data collection.Results: Twelve Institutions requested the access to the data collection system from 7 countries. At the data lock of July 26, 2020, 29 patients with systemic amyloidoses were collected from 7 different Institutions. Systemic AL amyloidosis patients reported so far were 19: 12 from the Pavia Amyloidosis Research and Treatment Center (Italy), 3 from the Boston Medical Center (USA), and 1 patient each from the Columbia University Hospital (New York, USA), Hospital Clinic (Barcelona, Spain), Clinica Universitaria de Navarra (Navarra, Spain) and Amyloidosis Centrum (Heidelberg, Germany). Eleven (58%) had heart involvement, 8 (42%) had kidney and two or more organs were involved in 9 patients (47%). The most frequent comorbidities reported were history of hypertension in 7 (37%) and cardiovascular diseases in 3 (16%). Four (21%) patients were newly diagnosed and treatment-naïve at the time SARS-CoV-2 infection was documented. The remaining 15 patients had received a median number of 2 previous lines of therapy (range 1-3). Nine (47%) patients were on active chemotherapy at the time of COVID-19 infection. Five were receiving daratumumab combinations, and the 4 remaining patients were on cyclophosphamide, bortezomib and dexamethasone, oral melphalan and dexamethasone, lenalidomide and ixazomib. Relevant concomitant medications were anti-hypertensive drugs in 26% of cases and diuretics in 21%. One patient was on dialysis. COVID-19-related symptoms were fever 11 (58%), cough 8 (42%), anosmia and ageusia. Pneumonia was documented in 10 (53%) patients, 5 of whom had acute respiratory distress syndrome (ARDS) (26%). Four of them were treated with non-mechanical ventilation and one accessed intensive care support. Three of the 5 patients with severe COVID-19 had heart involvement, 2/5 had concomitant heart and kidney involved and 3 was infected while on active chemotherapy. Azytromicin was used in 6 (26%) cases, which was in combination with hydroxycloroquine in 4 of them. Three patients received steroids as treatment for SARS-CoV-2 infection, while anticoagulant therapy was used only in two cases. Lopinavir, tocilizumab and sarilumab were used in one patient each. Four patients (21%) died in the whole cohort. Three had ARDS and one patient died few weeks after the recovery of COVID-19 infection. All deceased patients had heart involvement, 2 were on active therapy (daratumumab plus bortezomib and ixazomib plus dexamethasone). Two patients with kidney involvement at diagnosis, one with ARDS and one with a radiological documented pneumonia treated with non-mechanical ventilation recovered from COVID-19 but developed subsequent worsening of renal function, requiring dialysis in one case.Conclusions: The fatality rate and the proportion of patients with severe COVID-19 in this series is n the higher range of reports from the general population. Severe SARS-CoV-2 infection can result in renal failure in patients with renal AL amyloidosis.

Amyloid ; 27(4): 217-222, 2020 Dec.
Article in English | MEDLINE | ID: covidwho-889371


The ISA Nomenclature Committee met electronically before and directly after the XVII ISA International Symposium on Amyloidosis, which, unfortunately, had to be virtual in September 2020 due to the ongoing COVID-19 pandemic instead of a planned meeting in Tarragona in March. In addition to confirmation of basic nomenclature, several additional concepts were discussed, which are used in scientific amyloid literature. Among such concepts are cytotoxic oligomers, protofibrils, primary and secondary nucleation, seeding and cross-seeding, amyloid signature proteins, and amyloid plaques. Recommendations for their use are given. Definitions of amyloid and amyloidosis are confirmed. Possible novel human amyloid fibril proteins, appearing as 'classical' in vivo amyloid, were discussed. It was decided to include fibulin-like extracellular matrix protein 1 (amyloid protein: AEFEMP1), which appears as localised amyloid in portal veins. There are several possible amyloid proteins under investigation, and these are included in a new Table.

Amyloid/classification , Amyloidogenic Proteins/classification , Amyloidosis/classification , Terminology as Topic , Amyloid/genetics , Amyloid/metabolism , Amyloidogenic Proteins/genetics , Amyloidogenic Proteins/metabolism , Amyloidosis/diagnosis , Amyloidosis/genetics , Amyloidosis/pathology , COVID-19 , Congresses as Topic , Coronavirus Infections , Education, Distance/organization & administration , Gene Expression , Humans , Pandemics , Pneumonia, Viral
Br J Haematol ; 190(3): 346-357, 2020 08.
Article in English | MEDLINE | ID: covidwho-457351


The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)-associated coronavirus disease 2019 (COVID-19) is primarily manifested as a respiratory tract infection, but may affect and cause complications in multiple organ systems (cardiovascular, gastrointestinal, kidneys, haematopoietic and immune systems), while no proven specific therapy exists. The challenges associated with COVID-19 are even greater for patients with light chain (AL) amyloidosis, a rare multisystemic disease affecting the heart, kidneys, liver, gastrointestinal and nervous system. Patients with AL amyloidosis may need to receive chemotherapy, which probably increases infection risk. Management of COVID-19 may be particularly challenging in patients with AL amyloidosis, who often present with cardiac dysfunction, nephrotic syndrome, neuropathy, low blood pressure and gastrointestinal symptoms. In addition, patients with AL amyloidosis may be more susceptible to toxicities of drugs used to manage COVID-19. Access to health care may be difficult or limited, diagnosis of AL amyloidosis may be delayed with detrimental consequences and treatment administration may need modification. Both patients and treating physicians need to adapt in a new reality.

Coronavirus Infections/complications , Immunoglobulin Light-chain Amyloidosis/complications , Pneumonia, Viral/complications , Antiviral Agents/adverse effects , Antiviral Agents/therapeutic use , Betacoronavirus , COVID-19 , Coronavirus Infections/drug therapy , Coronavirus Infections/epidemiology , Health Services Accessibility , Humans , Immunoglobulin Light-chain Amyloidosis/drug therapy , Immunologic Factors/adverse effects , Immunologic Factors/therapeutic use , Pandemics , Pneumonia, Viral/drug therapy , Pneumonia, Viral/epidemiology , SARS-CoV-2