RESUMEN
Systemic immunoglobulin light-chain (AL) amyloidosis is characterized by deposition of amyloid fibrils of light chains produced by clonal CD38+plasma cells, resulting in organ dysfunction. Cardiac involvement has a major prognostic value. Antiplasma cell chemotherapy reduces the synthesis of immunoglobulin light chains (precursors of amyloid deposits). We describe a case of AL amyloidosis in a 95-year-old patient. Our patient responded poorly to treatment with rituximab, cyclophosphamide-bortezomib-dexamethasone, and rituximab-bendamustine. Finally, the anti-CD38 antibody daratumumab was associated with the best hematologic responsiveness without significant adverse effects. In conclusion, our case suggests that daratumumab is an effective and well-tolerated alternative to chemotherapy in the treatment af AL amyloidosis in very elderly patients.
Asunto(s)
Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas , Humanos , Anciano , Anciano de 80 o más Años , Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas/complicaciones , Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas/diagnóstico , Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas/tratamiento farmacológico , Rituximab/uso terapéutico , Anticuerpos Monoclonales/uso terapéutico , Anticuerpos Monoclonales/efectos adversos , Cadenas Ligeras de Inmunoglobulina , Ciclofosfamida/uso terapéutico , Dexametasona/uso terapéuticoAsunto(s)
Anticuerpos Neutralizantes/inmunología , Vacuna BNT162/uso terapéutico , COVID-19/complicaciones , Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas/complicaciones , SARS-CoV-2/inmunología , Adulto , Anciano , Anciano de 80 o más Años , Anticuerpos Monoclonales/uso terapéutico , Anticuerpos Antivirales/inmunología , Antineoplásicos/uso terapéutico , COVID-19/inmunología , COVID-19/prevención & control , Femenino , Humanos , Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas/tratamiento farmacológico , Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas/inmunología , Cinética , Masculino , Persona de Mediana Edad , Estudios ProspectivosAsunto(s)
Vacuna BNT162/uso terapéutico , COVID-19/prevención & control , Inmunidad Humoral , Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas/complicaciones , Mieloma Múltiple/complicaciones , Anticuerpos Antivirales/inmunología , COVID-19/complicaciones , COVID-19/inmunología , Femenino , Humanos , Inmunoglobulina G/inmunología , Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas/inmunología , Masculino , Mieloma Múltiple/inmunología , Estudios ProspectivosRESUMEN
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.