RESUMEN
Cryoglobulins are abnormal serum immunoglobulins that tend to precipitate in intravascular compartments at temperatures lower than 37°C causing blood flow restriction to vital organs. They are divided into type I, II and III based on the immunoglobulin subtypes of the cryoprecipitates. Type II cryoglobulinemia is most commonly associated with viral infections, autoimmune diseases and lymphoproliferative disorders. Here, we reported an 80-year-old man who presented with fatigue, acute kidney injury, palpable purpura, anaemia and altered mental status. He was diagnosed with type II cryoglobulinemia with concomitant positive autoimmune markers, varicella IgM antibody and IgM hepatitis B core antibody. The patient responded well to intravenous and oral steroid treatment.
Asunto(s)
Enfermedades Autoinmunes/complicaciones , Varicela/complicaciones , Crioglobulinemia/complicaciones , Hepatitis B/complicaciones , Anciano de 80 o más Años , Enfermedades Autoinmunes/sangre , Biomarcadores/sangre , Varicela/sangre , Crioglobulinemia/sangre , Crioglobulinemia/clasificación , Hepatitis B/sangre , Humanos , MasculinoAsunto(s)
Antineoplásicos Inmunológicos/efectos adversos , Dermatomiositis/inducido químicamente , Trastuzumab/efectos adversos , Biopsia , Neoplasias de la Mama/tratamiento farmacológico , Dermatomiositis/diagnóstico , Dermatomiositis/patología , Femenino , Humanos , Persona de Mediana Edad , Piel/efectos de los fármacos , Piel/patologíaAsunto(s)
Anticuerpos Monoclonales Humanizados/uso terapéutico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Dexametasona/uso terapéutico , Amiloidosis de Cadenas Ligeras de las Inmunoglobulinas/tratamiento farmacológico , Lenalidomida/uso terapéutico , Mieloma Múltiple/tratamiento farmacológico , Anticuerpos Monoclonales Humanizados/farmacología , Protocolos de Quimioterapia Combinada Antineoplásica/farmacología , Dexametasona/farmacología , Femenino , Humanos , Lenalidomida/farmacología , Persona de Mediana EdadRESUMEN
Haploidentical stem cell transplantation provides a plausible alternative for the patients when a fully matched donor is unavailable. Historically, the decision of considering haploidentical transplant has remained elusive; however, with the recent advances, the consideration of haploidentical grafts as a treatment option has become more apparent for both allografting for diseases and engraftment failure. We are reporting here an anecdotal case of a successful haploidentical engraftment in a patient with the prior graft failure of an HLA-matched related donor. Since the patient was severely alloimmunized, desensitization protocol was utilized before the haploidentical transplant, and the patient after 8 months of her second allogeneic transplantation is doing great with successful engraftment, no relapse, and no graft-versus-host disease (GVHD). Numerous reports pertinent to haploidentical graft have shown favorable outcomes in the graft placement, a decline in the rate of GVHD, and an improvement in the morbidity and mortality in affected individuals. Based on the current reports, haploidentical transplantation might be more feasible and has meaningful implications in the situations where matched donors are infrequent.
RESUMEN
Current projections estimate that the number of newborns with sickle cell disease (SCD) globally will exceed 400,000 by 2050. Over the last three decades, increased newborn screening, supportive care, and use of hydroxyurea therapy, have decreased early childhood mortality among individuals affected with SCD. Despite hematopoietic cell transplantation (HCT) being curative in SCD, its impact on disease free survival remains unknown, especially in adults, partly due to previous limitations in donor options and perceived mortality in adults using myeloablative conditioning. Novel non-myeloablative or reduced intensity conditioning regimens have made HCT safer and applicable to adults with SCD who were previously excluded. Other reasons for limited use of HCT in SCD includes referral bias, provider preference, poor education of patient/families, perceived procedure risks, costs and limited logistic resources in both low income, middle income, and high income countries. Current data from landmark studies indicate that both the long-term disease-free survival and overall survival exceed 90% after matched sibling donor HCT in children. Improving transplant outcomes and expanding donor pool will increase the use of transplant for SCD, especially in affected adults. On-going investigations using matched umbilical cord units, unrelated matched donor, and related haploidentical donors, promises to make HCT a viable option for nearly all eligible patients with SCD. This systematic review focuses on the recent data for HCT for patients with adult SCD with particular emphasis on donor sources and availability as illustrated by a case presentation exhibiting real world issues.