ABSTRACT
Introducción. El síndrome hemofagocítico (SH) es una proliferación benigna de histiocitos, asociado con fagocitosis de elementos hemopoyéticos. El cuadro clínico se caracteriza por: fiebre, linfadenopatía, hepatoesplenomegalia, citopenias profundas y coagulopatías. Se ha asociado a: virus, bacterias, hongos, parásitos y neoplasias. Caso clínico. Se informa el caso de un SH después de trasplante de médula ósea autólogo y purga con mafosfamida en un paciente con leucemia aguda mieloblástica M-4 sin donador HLA compatible, en primera remisión completa con serología y reacción en cadena de la polimerasa (PCR) para citomegalovirus negativos (CMV) antes del trasplante, todas sus transfusiones se aplicaron con filtros leuco-reductores. La toma de injerto fue en el día +17. En el día +28 presentó neutropenia de 200 /mL, el aspirado de médula ósea mostró hemofagocitosis y PCR para CMV fue positiva, en leucocitos, plasma y orina; después de tratamiento con ganciclovir e inmunoglobulina intravenosa la PCR se negativizó y la médula ósea se observó sin evidencia de hemofagocitosis; no se documentaron otras infecciones o neoplasias.Conclusión. De acuerdo a las características clínicas y estudios de laboratorio, se consideró que la hemofagocitosis intramedular estuvo asociada a la infección por CMV.
Subject(s)
Humans , Male , Child , Histiocytosis, Non-Langerhans-Cell/diagnosis , Bone Marrow Purging/methods , Bone Marrow Transplantation/adverse effects , Leukemia, Myelomonocytic, AcuteABSTRACT
During the last 30 years in vivo blood cell separation, generally referred to apheresis, has established a central role in both blood donor programmes and therapeutics. The technological advances in apheresis equipment has made procedures safer, faster and more effective. This article will review the use of apheresis in clinical medicine with emphasis on plasma exchange and peripheral blood stem cell collection. Plasma exchange now has a pivotal role in the management of a range of disorders, specially those with autoimmune pathogenesis. However, Plasma exchange should be practised as one component of an integrated and frequently multidisciplinary approach to management. The harvesting of allogeneic or autologous of peripheral blood haemopoietic stem cells is increased and it has become the principle indication for apheresis in many haematology units. A well coordinated protocol approach to this procedure is important if adequate haemopoietic stems cells are to be collected and safely cyropreserved. This requires successful cooperation between medical, nursing and scientific personnel.
Subject(s)
Antigens, CD34 , Bone Marrow Purging/methods , Cytapheresis/methods , Hematopoietic Stem Cell Mobilization , Humans , Immune System Diseases/therapy , Plasma Exchange/adverse effects , Plasmapheresis/methodsABSTRACT
OBJECTIVE: To study the outcome of oral busulfan and intravenous cyclophosphamide (BuCY 2 regimen) followed by allogeneic bone marrow transplant (BMT) in a cohort of patients with Philadelphia chromosome (Ph+) chronic myeloid leukaemia (CML) in a single centre. METHODS: From 1991 to March 1998, a total of 27 consecutive Ph+ CML patients received busulfan 4 mg/kg/day over 4 days and cyclophosphamide 60 mg/kg/day over 2 days followed by infusion of HLA-identical sibling haematopoietic stem cells. All except one (who received peripheral blood stem cells) were given donor bone marrow cells. Post-transplant graft versus host disease (GVHD) prophylaxis included a short course of methotrexate (on days +1, +3, +6 and +11) and cyclosporine till day +180. RESULTS: With a median follow-up of 30.5 months (1-55+ months), 14 patients (52%) are alive free from relapse. Early mortality was relatively high with 10 patients (37%) dying within first 100 days post-transplant. Acute GVHD developed in 14 patients (52%) inspite of GVHD prophylaxis with methotrexate and cyclosporine; six had grade I/II and eight grade III/IV. Chronic GVHD developed in five of 15 patients who lived beyond 70 days. CONCLUSION: Allogeneic BMT appears to result in eradication of CML and ensure disease free survival in about half of the young patients. However, efforts should be on to minimise early mortality.