Spinal cord compression due to extramedullary hematopoiesis (EMH) is a rare complication of thalassemia and generally presents as paraparesis with sensory impairment. Complete paraplegia is extremely rare in EMH due to thalassemia although it is known to occur in polycythemia vera and sickle cell anemia. Treatment options mostly include surgery and/or radiotherapy. Whereas cases presenting with paraparesis have been treated with either surgery or radiotherapy with equal frequency and efficacy, almost all reported cases with paraplegia have been treated with surgery with or without radiation therapy. We hereby report a case of thalassemia intermedia with paraplegia treated successfully with radiotherapy.
Multiply transfused patients of severe aplastic anemia are at increased risk of graft rejection. Five such patients underwent peripheral blood stem cell transplantation from HLA-identical siblings with a fludarabine-based protocol. The conditioning consisted of fludarabine 30 mg/m 2 / day  6 days, cyclophosphamide 60 mg/kg/day  2 days and horse antithymocyte globulin (ATG)  4 days. Two different ATG preparations were used: ATGAM (dose 30 mg/kg/day  4 days) or Thymogam (dose 40 mg/kg/ day  4 days). Engraftment: median time to absolute neutrophil count (ANC) 40.5  10 9 /l was 11 days (range: 8-17) and median time to platelet count 420  10 9 /l was 11 days (range: 9-17). At a median follow-up of 171 days (range: 47-389), there has been no graft rejection and all patients are in complete remission. Acute GVHD (grade 1) occurred in one patient only. Chronic GVHD developed in two patients (extensive in one and limited in another). The transplants were performed in non-HEPA filter rooms. In only one patient, systemic antifungal therapy (voriconazole) was used. The use of Thymogam brand of ATG for conditioning is being reported for the first time. Our experience suggests that this fludarabine-based protocol allows rapid sustained engraftment in high-risk patients without significant immediate toxicity.
Imatinib mesylate therapy in CML patients is a generally well tolerated without any significant hematological adverse drug effects. However, complications like anemia and cytopenias have been described in literature. A very few case reports of bone marrow aplasia following imatinib therapy have been reported so far. We here report five patients of CML who developed bone marrow aplasia following imatinib therapy. Am. J. Hematol. 82:314-316, 2007. V V C 2006 Wiley-Liss, Inc.
Despite recent advances in the treatment of acute promyelocytic leukemia (APL), early mortality and relapses still occur. With the view to evaluate the role of FLT3 mutation in APL, 54 patients (median age 28 years, range11-57 years, male to female ratio 1.2:1, median TLC 8.4 x 10(9)/l, range 1-170 x 10(9)/l) were studied by reverse transcriptase-PCR. Forty-two patients (77%) achieved first remission (CR1). Ten (18.5%) of the 54 patients had internal tandem duplication of exons 11 and 12 of the FLT3 gene. The median TLC count was significantly higher in FLT3 positive cases (Median TLC 55.0 x 10(9)/l) as compared to FLT3 negative cases (Median TLC 6.8 x 10(9)/l) (p = 0.001). Induction CR was much lower (40%) in FLT3/ITD positive cases as compared to 86% of FLT3/ITD negative cases (p = 0.005). Early deaths too were significantly associated with FLT3/ITD positive cases (50 vs. 16% p = 0.033). The difference in the occurrence of bcr1 and bcr3 isoforms was not statistically significant between the two groups. The data suggest that the presence of FLT3/ITD in APL patients confers a poor prognosis.
Aims: Previous comparisons between AMPLATZER septal occluders and other designs were retrospective, non-randomised, non-concurrent and involved fewer patients. A prospective concurrent head-to-head comparison of AMPLATZER (ASO), Cera (CSO) and Figulla (FSO) septal occluders was planned to study the patient outcomes.Methods and results: The three occluders were serially allocated in a cycle of three to consecutively included patients. Demographic, procedural details and complications were analysed. After calculating a sample size of 122 patients in each group, additional patients were recruited to ensure at least 80% follow-up. Four hundred and fifty (450) consecutive patients equally divided among the three designs were comparable in all parameters. There were no major complications and procedural success was 99.6%. The defects and device sizes were similar in all groups; the delivery system was significantly smaller with the ASO. The FSO needed special deployment techniques less often and formed a cobra deformity more often, though this was not statistically significant. Patient outcome was similar among the groups at a follow-up of 12-47 months. Conclusions:The new occluders are comparable to the ASO with good outcomes and low complication rates in the current era. The new modified structural designs do not show any advantages in terms of procedural complications on early and midterm follow-up, but long-term studies are warranted.
Aim: This prospective, single arm, institutional study was designed to evaluate the response to Cyclosporin A (CsA) in adult patients with primary MDS with cytopenias. Material and Methods Patients aged 18 years or more, with primary MDS, normal renal and hepatic functions were eligible for inclusion after informed consent. The diagnosis of MDS was based on demonstrating features of dysplasia in one or more cell lines on peripheral blood smear and bone marrow examination. The criteria for cytopenia were one or more of the following: hemoglobin <10 g/dl; platelets <100x109/L; absolute neutrophil count <1.5x 109/L. Secondary causes of MDS were excluded by clinical and laboratory assessment. Other exclusion criteria were: response to therapeutic trial of vitamin B12 and folic acid, HIV infection and diagnosis of PNH. MDS was considered hypocellular if bone marrow (BM) cellularity was <30%, and in such cases dysplastic features had to be present in more than one cell line, so as to exclude hypoplastic anemia. CsA was used in doses of 3–5 mg/kg/day in 2 divided doses. Response was assessed using the International Working Group criteria for MDS (Cheson et al in Blood 2000, 96:3671–74), after a minimum follow up of 3 months. Results A total of 31consecutive patients with MDS who fulfilled inclusion criteria were assessed. Patient characteristics were: median age 38 years (range 20–70); male18, female 13; MDS type: RA-24, RAEB-7; BM: hypocellular-8 (25.8%), normocellular-19 (61.3%), hypercellular-4 (12.9%); transfusion dependent - 26 (83.8%). Response There were no remissions. Hematological improvement (HI) was seen in different cell lines. Erythroid response (HI-E) was seen in 18 (58%): major response in 10 (32.2%); type- 8 RA, 2 RAEB; cellularity - hypocellular 4, normocellular 6 minor response in 8 (25.8%); type- 7 RA, 1 RAEB; hypercellular-1, hypocellular-1, normocellular-6 and no response in 13 (41.9%); type RA- 9, RAEB-4; hypocellular- 3, normocellular-7, hypercellular-3. In terms of type of MDS, response occurred in 3/7 (42.8%) of RAEB (major in 2, minor in 1) and 15/24 (62.5%) of RA (major 8, minor 7). Neutropenia at onset was present in 14/31 (45.1%) patients only. Of these, neutrophil response (HI-N) major was seen in 1 (RA, hypocellular) who also showed major erythroid response. Thrombocytopenia at onset was present in 21/31 (67.7%) patients only. Of these, platelet response (HI-P) major was seen in 1 (RA, normocellular) and minor response was seen in 1 (RA, normocellular) and both also showed erythroid response. Median interval between diagnosis and treatment was 1 month (range 1–6). Median length of treatment: was 11 months (range 4–46). Effect in responders was seen in median of 3 months (range 1–6). There were no delayed responses seen after 6 months therapy. Relapse occurred in 9 patients. Two relapsed within 1 and 2 months of stopping treatment. 7 patients relapsed while on treatment at a median time of 6 months. There was no statistically significant difference in responses according to the type of MDS or BM cellularity using the Chi square test (p > 0.05). Conclusion: Anemia improved in 58% patients of MDS (RA and RAEB) and was sustained in 29% cases, irrespective of bone marrow cellularity. Further experience is needed to determine the duration of treatment, as relapses can occur both during therapy and on cessation of cyclosporin.
Background: Hemodynamically significant left to right shunts, once closed, decreases the left ventricular (LV) preload and increases the after load by isolating the low resistance pulmonary circulation from LV outflow circulation. The simultaneous reduction in the LV preload and increase in after load
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