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Resumen La amiloidosis por depósito de cadenas livianas de inmunoglobulinas (AL) es una enfermedad poco frecuen te y subdiagnosticada. El mejor tratamiento disponible al momento es el trasplante autólogo de médula ósea (TMO). El compromiso cardíaco es el principal determi nante pronóstico en esta patología y en ocasiones un impedimento para recibir el TMO. Se presenta el caso de un varón de 44 años que consultó por signos y síntomas de insuficiencia cardiaca (IC) con biomarcadores cardia cos elevados. Se realizó un ecocardiograma transtorácico donde se objetivó aumento de espesores parietales con hipoquinesia global y fracción de eyección deteriorada en grado leve (50%). El paciente se internó en unidad coronaria para balance negativo y para estudio etiológico del cuadro. Ante la sospecha de enfermedad infiltrativa, se solicitaron un centellograma óseo con pirofosfato y cadenas livianas libres en suero. El centellograma óseo resultó no sugestivo para amiloidosis por transtiretina y las cadenas livianas libres mostraron una relación me nor a 0.26 con predominio lambda. Se realizó una biopsia de encía que confirmó el diagnóstico de amiloidosis AL. Posterior al diagnóstico comenzó tratamiento qui mioterápico específico con Ciclofosfamida, Bortezomib y Dexametasona (esquema CYBORD) y Daratumumab. Evolucionó con IC refractaria por lo que ingresó a lista de trasplante cardiaco, recibiendo el mismo al poco tiempo con buena evolución. Esto permitió reiniciar el esquema quimioterápico y en segundo término finalmente recibir el TMO, con buena evolución.
Abstract Light chain amyloidosis (AL) is a rare and underdi agnosed disease. The best treatment available is au tologous bone marrow transplantation (BMT). Cardiac involvement is the main prognostic determinant in this pathology and sometimes an impediment to re ceive BMT. We present a clinical case of a 44-year-old who consulted for signs and symptoms of heart failure (HF) with elevated cardiac biomarkers. A transthoracic echocardiogram showed increased wall thickness with global hypokinesia and mildly impaired ejection fraction (50%). The patient was admitted to the coronary unit for treatment with diuretics and for etiological study of the condition. In view of the suspicion of infiltrative disease, a bone scintigraphy with pyrophosphate and free light chains in serum were requested. The bone scintigraphy was not suggestive of transthyretin amyloidosis and the free light chains showed a ratio of less than 0.26 with lambda predominance. A gum biopsy was per formed and confirmed the diagnosis of AL amyloidosis. After diagnosis, specific chemotherapy treatment with Cyclophosphamide, Bortezomib and Dexamethasone (CYBORD scheme) and Daratumumab was started. He evolved with refractory HF so it was decided to admit him to the cardiac transplantation list, receiving the same soon after, with good evolution. This allowed the patient to restart the chemotherapy regimen and finally receive BMT, with good evolution.
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PURPOSE: This study evaluated mid-term clinical and radiological results of autologous bone marrow transplantation (BMT) for early stage osteonecrosis of the femoral head (ONFH) and analyzed prognostic factors. MATERIALS AND METHODS: From November 2003 to April 2008, 101 hips of 93 patients with early stage ONFH who underwent autologous BMT were followed for at least five years. For clinical results, preoperative and postoperative Harris hip scores (HHS) were evaluated and survival rate was obtained at the point of performing total hip arthroplasty or femoral head collapse progression. Radiologic results were assessed by changes in necrosis size on magnetic resonance imaging performed preoperative and postoperatively. For evaluation of prognostic factors, survival rate was analyzed according to age, gender, etiology, stage, necrosis size, and location. RESULTS: Averaged HHS at latest follow up showed no significant change in comparison with preoperative HHS. Of 101 hips, 35 hips required arthroplasty and six hips were running head collapse. Groups with use of steroid, lateral location of necrosis, large size of necrosis, or large necrotic angles showed lower survival rate. However, age, gender, and stage had no effect. CONCLUSION: In early days, autologous BMT for early ONFH can be considered as a treatment for improvement of clinical features and delay of radiologic progress. However, after some years, there was no effect compared with the natural course of ONFH.
Subject(s)
Humans , Arthroplasty , Arthroplasty, Replacement, Hip , Bone Marrow Transplantation , Follow-Up Studies , Head , Hip , Magnetic Resonance Imaging , Necrosis , Osteonecrosis , Risk Factors , Running , Survival RateABSTRACT
Objective To explore the functional expression and temporality of MDR1 gene in bone marrow of rabbits after autologous bone marrow transplantation with MDR1 transferred bone marrow mononuclear cells. Methods The supernatant of the amphotropic virus producer cell line PA317-HaMDR1/A was collected and concentrated to cocultivate with the bone marrow mononuclear cells of the rabbits. After large dose of chemotherapy with cyclophosphamide,the transferred cells were autotransplanted into the bone marrow. The integration,transfection rate and physiological function of MDR1 gene were tested by PCR,SP immunohistochemical method and daunorubicin (DNR) extrusion test respectively. Results After autologous bone marrow transplantation had been executed for 1-4 months,the integration of MDR1 gene in genome of bone marrow mononuclear cells was detected by PCR,and the expression rates of P-gp in cells tested by SP immunohistochemical method were 9.5%,8.5%,6.0% and 3.5% respectively. The physiological function of MDR1 gene in bone marrow cells was proved by DNR extrusion test. Conclusion After the autotransplantation with bone marrow mononuclear cells transferred by MDR1 gene,the MDR1 gene can implant into the bone marrow of rabbits and has expressed functionally for 4 months,which has provided a basis for further research on chemoprotection experiment of the MDR1 gene transferred into the bone marrow cells.
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Objective To evaluate the clinical effects of autologous bone marrow transplantation(ABMT) in the treatment of patients with acute leukemia.Methods A retrospective study was accomplished on the ABMT in the treatment of 35 patients with acute leukemia from Oct 1999 to Oct 2004.The median age of the patients was 32.5(9～55) years.Of the 35 patients,26 cases were acute non-lymphocytic leukemia(ANLL) and 9 cases were acute lymphoblastic leukemia(ALL).The patients were pretreated with melphalan(140～180mg/m~2),cyclophosphamide(120mg/kg) and arabinosylcytosin(3g/m~2).Results All patients engrafted successfully.The median follow-up duration was 756(186～1950) days.The 3-year probabilities of disease-free-survival(DFS) for ANLL and ALL were(65.4%?8.9)% and(33.3?13.6)% respectively,and the probabilities of relapse were(30.6?9.2)% and(60.7?25.5)%,respectively.Conclusion To decrease relapse and increase DFS,patients with acute leukemia who have no chance for allogene haemopoietic stem cell transplantation are recommended for ABMT.
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Objective This study aimed to investigate whether more intensive induction regimen plus autologous bone marrow transplantation and 13-cis-retinoic acid can alter the extremely poor prognosis of high-risk neuroblastoma patients.Methods Five children with stage Ⅳ neuroblastoma were studied. One was under one year old, which was founded by neuroblastoma screening. The oldest one was 5 year old. The duration of illness before admission was from 7 days to 4 months. Primary site of the disease was adrenal gland in four patients and retroperitoneal in one. All of the patients had multiple metastasis. Urine VMA/Cr was more than normal in two patients. NSE elevated in all 5 patients. The MYCN amplification more than 10 copies in three patients. All of the patients received high-dose chemotherapy, radical removal of the original tumor and metastatic retroperitoneal lymph nodes, local radiation, plus supralethal preconditioning regimen followed by autologous bone marrow transplantation as well as 13-cis-retinoic acid.Results All the five patients got a complete response after the induction regimen. Follow-up 4-33 months, five patients keep complete responses and three patients disease-free survival, two patients had mild renal failure.Conclusions High-dose induction chemotherapy plus autologous bone marrow transplantation as well as 13-cis-retinoic acid may can improve the prognosis of high-risk neuroblastoma patients.
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5.16 log of K562, U937, HIMeg and HL60 cells, respectively, but spare 23.37 ? 25.66% of normal CFU-GM (0.63 log killing). When HL60 cells mixed with normal mononucleated bone marrow cells in 1:9 ratio were studied, the killing of HL60 colonies was more than 4.29 log. The result suggested that photocarcinorin plus light could eliminate leukemic progenitors, and might be used in clinical ABMT. Several factors that might affect the photosensitization effect on cells were discussed.