1/27. A case of relapsing acute disseminated encephalomyelitis with high dose corticosteroid treatment.We report a 16-month-old boy with acute disseminated encephalomyelitis (ADEM) who had an early relapse despite prompt treatment with high dose methylprednisolone. The second episode responded to intravenous immunoglobulin (IVIg). This case illustrates the probability of relapses or treatment failures in ADEM after steroid treatment, and the use of alternative drugs.- - - - - - - - - - ranking = 1keywords = treatment failure (Clic here for more details about this article) |
2/27. Allergic fungal sinusitis: learning from our failures.For more than five years, patients referred to the Department of otolaryngology-head and neck Surgery at the University of texas Southwestern Medical Center at Dallas with allergic fungal sinusitis (AFS) have been managed using a regimen combining surgery, perioperative corticosteroids, and immunotherapy for relevant antigens (fungal and non-fungal). The initial success of this program has been previously reported. Continued experience with this treatment plan, however, has yielded some cases of recurrence of AFS. Careful review of these cases implicate two major factors associated with treatment failure: (1) lack of compliance with immunotherapy, and (2) inadequate initial surgical extirpation of all allergic mucin. These cases and associated factors will be discussed.- - - - - - - - - - ranking = 1keywords = treatment failure (Clic here for more details about this article) |
3/27. syringomyelia--as a late complication of tuberculous meningitis.The aim of this paper is to demonstrate the unusual MR features of thoracic syringomyelia following TB meningitis and to discuss the neurosurgical aspect of the treatment of this rare entity. Four years after a TB meningitis episode, a 30 year-old female patient developed a progressive spastic paraparesis. MR studies revealed multiloculated syrinxes throughout the thoracic cord. She had a syringo-subarachnoid shunt with a silastic "T" tube inserted. On the first postoperative day, she showed a dramatic neurological improvement, but unfortunately her paraparesis progressed to the preoperative level within a month despite diminished size of the syrinxes on the control MRI examination. Two and a half years after the operation the patient complained of having a burning type of central pain, and further deterioration in neurological function. Thoracic spinal MRI examination demonstrated enlarged syringomyelic cavities. At the second operation syringo-peritoneal shunt insertion was performed via right T10-11 hemilaminectomy using a "T" tube. At present, 4 months after the second operation, the patient's neurological examination demonstrated decreased spasticity, and improved strength in the legs compared to the preoperative level. MRI is the first choice of investigation in detecting TB related myelopathy as it provides a greater detail of pathological changes within and around the spinal cord such as syrinx formation and arachnoiditis. The MR findings are also helpful in deciding the management and predicting the outcome. Presence of multifocal loculations and arachnoid adhesions is the likely cause of treatment failures and poor prognosis.- - - - - - - - - - ranking = 1keywords = treatment failure (Clic here for more details about this article) |
4/27. High-dose chlorambucil for the treatment of chronic lymphocytic leukaemia and low-grade non-Hodgkin's lymphoma.chlorambucil has been used for many years for the treatment of low-grade B-cell lymphoproliferative disorders, including chronic lymphocytic leukaemia and low-grade non-Hodgkin's lymphoma. There is evidence in the literature that increasing the dose of chlorambucil produces better results than 'standard' doses in terms of response rates and overall survival. There is also evidence that this approach may be at least as effective as the use of fludarabine, as well as being very much less expensive. We describe a high-dose chlorambucil (HDC) regimen, which involves a sustained but intermittent dose of chlorambucil, i.e. 30 mg/d for 4 d per week for 4 weeks, followed by a further four courses at fortnightly intervals for 8 weeks (a total of eight 4-d courses) given as a single drug over an initial 12-week period. The outcome of treatment in previously treated and untreated patients was excellent, with a median time to treatment failure of 33 months for the patient cohort overall and for previously treated and chemotherapy-naive patients of 13 and 104 months respectively. In patients previously treated with fludarabine, 78% had a response. Autoimmune haemolytic anaemia was reversed in one patient. Toxicity, both haematological and other, was minimal. We propose that escalated-dose chlorambucil regimens should be compared with fludarabine in randomized controlled trials, rather than 'standard' lower dose protocols.- - - - - - - - - - ranking = 1keywords = treatment failure (Clic here for more details about this article) |
5/27. Surgical management of acute complications and critical restenosis following carotid artery stenting.Carotid artery angioplasty with stenting (CAS) is being increasingly used in the treatment of extracranial carotid artery stenosis. As in other catheter-based approaches to the treatment of arterial disease, surgical intervention may be required because of either acute complications or correct critical restenosis. We have reviewed our experience managing early complications and critical in-stent restenoses after CAS in a tertiary care university hospital and a veterans Affairs Medical Center. During the last 5 years, 22 carotid arteries (21 patients) underwent CAS. One patient developed thrombosis and rupture of the carotid artery during stenting. Two other patients (3 arteries) developed critical restenosis within 12 months. Subsequent surgical reconstructions included an internal carotid artery (ICA)-to-external carotid artery (ECA) transposition and a common carotid artery (CCA)-to-ICA bypass with reversed saphenous vein (RSV). The patient who underwent CCA-to-ICA bypass later required subclavian-to-ICA bypass because of rapidly progressive intimal hyperplasia and subsequent occlusion of the CCA. The other patient has not had surgical repair because of his deteriorating condition and significant co-morbidities. During the same time period, two additional patients were referred from outside institutions specifically for surgical intervention after carotid stenting. One had delayed rupture of the carotid artery 1 day after stenting and underwent urgent surgical repair. Another patient had early, critical restenosis within the stent and underwent placement of a CCA-to-ICA interposition graft using RSV. Acute treatment failures after CAS can be successfully managed using standard surgical techniques. patients who develop critical in-stent restenosis requiring surgical repair may need more challenging surgical reconstructions to maintain cerebral perfusion.- - - - - - - - - - ranking = 1keywords = treatment failure (Clic here for more details about this article) |
6/27. Unusual presentation of central nervous system relapse with oculomotor nerve palsy in a case of CD56-positive acute myeloid leukemia following allogeneic stem cell transplantation.Allogeneic stem cell transplantation (allo-SCT) plays an important role in the treatment of infants and children with acute myelogenous leukemia (AML). Leukemic relapse after allo-SCT is responsible for a high rate of treatment failure. Extra-medullary relapse (EMR), without involvement of bone marrow, is rare compared to medullary relapse. CD56, the neural cell adhesion molecule, may contribute to the higher frequency of CNS relapse in CD56-positive AML. We observed an isolated EMR on the oculomotor nerve of a 17-month-old girl 12 weeks after cord blood transplantation (CBT), who was transplanted because of CD56-positive AML. diagnosis of relapse was suspected clinically and confirmed by magnetic resonance imaging (MRI), and fluorescence-activated cell sorter (FACS) and chimerism analysis of cerebrospinal fluid (CSF). Therapy consisted of intra-thecal chemotherapy, CNS irradiation, and systemic immunomodulation by cyclosporin A (CsA) and basiliximab withdrawal. Twenty-one months after relapse, the patient shows full remission of symptoms and previously described oculomotor nerve infiltration.- - - - - - - - - - ranking = 1keywords = treatment failure (Clic here for more details about this article) |
7/27. Successful treatment of juvenile myelomonocytic leukemia relapsing after stem cell transplantation using donor lymphocyte infusion.Juvenile myelomonocytic leukemia (JMML) is a rare pediatric malignancy. hematopoietic stem cell transplantation (SCT) is the only curative approach. However, relapse after SCT remains the major cause of treatment failure. Unlike most other pediatric malignancies, JMML may be susceptible to a graft-versus-leukemia (GVL) effect, although, unlike chronic myeloid leukemia, reports of response to donor lymphocyte infusions (DLIs) remain scanty. This is the first report that describes the successful treatment of relapsed JMML with DLI in the absence of further chemotherapy and provides definite proof of a GVL effect in JMML.- - - - - - - - - - ranking = 1keywords = treatment failure (Clic here for more details about this article) |
8/27. Recurrent, isolated factor x deficiency in myeloma: repeated normalization of factor X levels after cytostatic chemotherapy followed by late treatment failure associated with the development of systemic amyloidosis.We describe the case of a 64-year-old woman with isolated severe factor x deficiency associated with kappa light chain myeloma. At the time of diagnosis there was no evidence for amyloidosis. Complete remission (CR) of myeloma as well as normalization of factor X levels were achieved after cytostatic chemotherapy. Subsequently, factor x deficiency recurred twice without any evidence for relapse of myeloma. The first time factor X normalized again following cytostatic treatment, the second time, however, factor x deficiency was refractory to chemotherapy. Finally, relapse of myeloma became evident associated with rapidly progressing, systemic amyloidosis, which was fatal within a few months. Initially, factor X infusion studies showed a normal recovery, but when amyloidosis became overt the recovery decreased to 0%. We assume that factor x deficiency was due to a binding of factor X to kappa light chains associated with the proliferation of the malignant myeloma cell clone.- - - - - - - - - - ranking = 4keywords = treatment failure (Clic here for more details about this article) |
9/27. Successful treatment of JMML relapsed after unrelated allogeneic transplant with cytoreduction followed by DLI and interferon-alpha: evidence for a graft-versus-leukemia effect in non-monosomy-7 JMML.Relapse is the major cause of treatment failure after allogeneic transplantation of children with juvenile myelomonocytic leukemia (JMML), and the role of post-transplant immunomodulation is poorly understood. We report a 12-month-old child with JMML relapsed after unrelated marrow transplantation who received cytoreduction followed by donor lymphocyte infusion (DLI) with improvement, and after addition of interferon-alpha (IFN) achieved complete donor chimerism. He was weaned from IFN and has maintained complete remission for 19 months. This is the first published report of a patient with non-monosomy-7 JMML responding to post-transplant immunomodulation and suggests a role for DLI plus IFN in these patients.- - - - - - - - - - ranking = 1keywords = treatment failure (Clic here for more details about this article) |
10/27. Second allogeneic hematopoietic stem cell transplantation for juvenile myelomonocytic leukemia: case report and literature review.Juvenile myelomonocytic leukemia (JMML) is a rare clonal myeloproliferative disease in young childhood. hematopoietic stem cell transplantation (HSCT) is the only way to cure the disease, but relapse after HSCT remains a major cause of treatment failure. A 5-year-old girl with JMML, who had experienced a relapse after the first transplant, did not respond to donor lymphocyte infusion and withdrawal of immune-suppressing agents. She was successfully treated using a second transplant. Detailed reports from the English literature since 1988 relating to a total of 13 JMML patients undergoing a second transplant were reviewed. Seven of the 13 JMML patients (54%) were alive and disease-free, with a median follow-up of 53 months after the second transplant. Within the first 6 months following the initial transplant, 10 JMML patients suffered either autologous recovery (n = 6) or early relapse (n = 4). Seven of the 10 (70%) were alive, with a median survival period of 53 months after the second transplant. Six JMML patients underwent retransplantation within 6 months of the first transplant, with three of these (50%) alive at follow-ups of 24, 57, and 90 months after the second procedure. The authors conclude that a second transplant within 6 months may be worth considering for JMML patients who experience autologous recovery or earlier relapse after the first transplant.- - - - - - - - - - ranking = 1keywords = treatment failure (Clic here for more details about this article) |
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