Sara Velázquez-Sánchez-de-Cima

Nonalcoholic Fatty Liver Disease May Cause Thrombocytopenia

/l) were prospectively studied. Pa-tients with immune thrombocytopenia [7] , overt viral hepatitis or liver cirrhosis were excluded. The study was approved by the Ethics Committee of the Clinica Ruiz and informed consent was obtained from the patients in-cluded in both groups. Alpha 2 macroglobulin, haptoglo-bin, apolipoprotein A, bilirubin, gamma-glutamyl trans-peptidase, alanine aminotransferase, aspartate amino-transferase, glucose, cholesterol and triglycerides were measured in all patients. These biochemical markers were then analyzed. FibroTest was used for the quantitative as-sessment of fibrosis, SteatoTest for the quantitative as-sessment of steatosis, ActiTest for the quantitative assess-ment of necroinflammatory activity in chronic viral hep-atitis and NashTest for the categorical diagnosis of nonalcoholic steatohepatitis [5] . Patients with a score of >50% in either the SteatoTest or the NashTest coupled with a score of <50% in the FibroTest were defined as hav-ing NAFLD. Within the group of 93 consecutive patients studied due to their platelet count <100 × 10

Simultaneous romiplostin, eltrombopag, and prednisone were successful in severe thrombocytopenia of Evans syndrome refractory to hydrocortisone, splenectomy, intravenous IgG, and rituximab.

Abstract A 58-year-old woman presented with rheumatoid arthritis-associated Evans syndrome (simultaneous autoimmune hemolytic anemia and autoimmune thrombocytopenic purpura); she was treated unsuccesfully with steroids, romiplostin, rituximab, immunoglobulin G, and splenectomy. The platelet count responded to the combined use of prednisone, eltrombopag, and romiplostin. It may be more reasonable to use combined treatments than sequential monotherapies.

Primary Thrombophilia in México X A Prospective Study of the Treatment of the Sticky Platelet Syndrome

Introduction: The sticky platelet syndrome (SPS) is a common cause of thrombosis. There are no prospective studies concerning treatment. Objective: To analyze changes in platelet hyperaggregability of patients with SPS who were given antiplatelet drugs and to assess its association with rethrombosis. Methods: A total of 55 patients with a history of thrombosis and SPS phenotype were prospectively studied before and after treatment with aspirin and/or clopidogrel. Results: Patients were followed for 1 to 129 months, median 13. Of 55 patients, 40 received aspirin, 13 received aspirin + clopidogrel, and 2 received only clopidogrel. The platelet aggregation response to adenosine diphosphate and epinephrine significantly diminished after treatment, and only 2 patients developed rethrombosis 52 and 129 months after starting therapy, with the freedom from rethrombosis rate of the patients being 96.4% at 129 months. Conclusion: Using antiplatelet drugs, the platelet hyperreactivity of patients with the SPS phenotype was reverted; and this translated into a substantial decrease in the rethrombosis rate.

Oral versus Intravenous Fludarabine as Part of a Reduced-Intensity Conditioning for Allogeneic Stem Cell Transplantation

Background: In 2003, oral fludarabine was introduced in the USA for the treatment of patients with hematologic malignancies as an alternative to its intravenous (i.v.) formulation; in 2008, it was introduced in México while the i.v. formulation was withdrawn. Accordingly, i.v. fludarabine had to be replaced by oral fludarabine as part of the conditioning regimen employed to conduct allogeneic stem cell transplantation in México. Methods: Nonrandomized retrospective analysis of 55 patients conditioned with oral fludarabine compared with 113 patients conditioned with the i.v. formulation. In addition to fludarabine, the conditioning regimen included oral busulfan and i.v. cyclophosphamide. Donors were HLA-matched siblings. Results: The clinical features of the two groups were comparable. There were no statistical differences in time to neutrophil engraftment, time to platelet engraftment, acute graft versus host disease rate and nonrelapse mortality at day 100. The overall survival of patients allografted with oral fludarabine was better than those allografted with i.v. fludarabine: 62 and 33% at 67 months, respectively (p = 0.0006). Discussion: Oral fludarabine can replace its i.v. formulation as part of reduced-intensity conditioning regimens with no deleterious effect on any of the early transplantation outcomes.

Secondary malignancies after allogeneic hematopoietic stem cell transplantation using reduced-intensity conditioning and outpatient conduction

Abstract Background Patients given allogeneic hematopoietic stem cell transplants (HSCT) may develop secondary malignant neoplasms (SMN). Several variables have been identified but there are no data about the incidence of this complication in individuals given HSCT using reduced-intensity conditioning (RIC) methods. Objective Define the incidence of SMN in patients given HSCT using a RIC preparative regimen conducted on an outpatient basis. Materials and methods Patients given HSCT in two institutions between October 1998 and 2012 were analyzed. To appraise the SMN appearance, those patients dead were also regarded as censored at that moment, as well as those lost to follow up and those alive at the closing of the study. 95% Confidence intervals (CI) for the survival or failure estimate were calculated with the Greenwoods method. Results A total of 416 allografted patients with a Karnofsky performance index of 100% were included in the study. All patients received peripheral blood stem cells allografts. The conditioning regimen was delivered as an outpatient procedure in all individuals. No patient was given radiotherapy nor antithymocyte globulin during the conditioning. Three hundred and sixty five patients (88%) were never admitted to the hospital, whereas 12% were admitted because of grade III–IV acute graft versus host disease (aGVHD), fever, or mucositis. Median survival time was 15.7 months. Survival at 6 months (95% CI): 66.4% (61.5–70.8%), at 12 months: 53.3% (48.1–58.1%), at 60 months: 30.6% (30.5–41.5%). Eight patients with a SMN were identified in the group of 416 allografted patients, SMN rates (95% CI) were: one year post graft: 1.9% (0.7–4.9%), 5 years: 3.8% (1.6–9.2%), 10 years: 6.8% (2.6–17.7%) and 13 years post-graft: 20.2% (5.5–59.2%), the cumulative probability of SMN being 6.8 at 10 years. Since the number of expected cases in the general population is 0.62, the ratio of observed to expected cases is 12.9 (P < 0.001). This figure means that the risk of developing a malignant neoplasm in allografted individuals using our method is 12.9 times higher than that in the general population. There were three non-Hodgkins lymphomas, two M2 acute myelogenous leukemias, one hairy cell leukemia, one tongue epidermoid carcinoma, and one breast carcinoma. Conclusions We have found a low incidence of SMN in this group of Mexican patients allografted with the Mexican RIC method. Possible explanations for this difference are discussed, focusing on the RIC preparative regimen.

In pursuit of the graft-versus-myeloma effect: A single institution experience

Abstract Partly because of a potential graft-versus-myeloma effect, allogeneic stem cell transplantation is a potentially curative treatment modality in patients with multiple myeloma (MM). Initial attempts have been hampered by the high transplant-related mortality in this setting. With a reduction of toxicity, allogeneic transplant approaches with reduced-intensity conditioning (RIC) have been utilized, although they are subjected to continued disease progression and relapse following transplantation. We analyze here the experience of allografting four patients with MM in a single institution, along a 16-year period in which a total of 152 individuals were allografted, using an RIC regimen; three of the patients have had previous autografts. All patients engrafted successfully and a graft-versus-myeloma effect was shown in all of them. One patient relapsed in the face of graft-versus-host disease (GVHD). Three patients have died (two as a result of GVHD) and one is alive with a limited form of chronic GVHD. The graft-versus-myeloma effect can be induced by means of allogeneic transplantation but the morbidity and mortality associated with the procedure leads into a relatively small proportion of MM patients being cured.

Is Friday leukemia a non-entity?

Most hematologists are familiar with a subgroup of acute leukemias (AL) that first present on a Friday. Diagnostic and therapeutic efforts in caring for patients with AL are usually work-intensive procedures, and there are AL patients felt to present on a Friday afternoon, prompting an intense activity at the onset of the upcoming weekend. It is clear that all efforts at optimal processing of fresh material from AL blood and bone marrow in hospitals and study groups organizing multicenter clinical trials are mandatory regardless of the weekday. In a group of 197 patients with newly diagnosed AL, Wilop et al., in Germany, reported that Friday was significantly overrepresented compared with admissions on other working days, thus supporting the gut feeling of hematologists around the world. Wilop et al. speculate that this observation stems mainly from the health care system in which primary care physicians and health institutions not specializing in hematology elect to refer the AL patient with a weekend approaching. It has also been speculated that it reflects the state of mind of the patients who struggle through work all week and as the weekend approaches relax and realize that they do not feel well and subsequently present themselves for care. It has also been seen that many of these patients do have a history of presenting to primary care clinicians on a number of occasions before finally being taken seriously. On the other hand, the Friday afternoon admission of a patient with a potential diagnosis of leukemia creates perceived delays in treatment initiation. Although generally not felt to affect prognosis, the effect of a few days delay in chemotherapy for patients with AL has not been fully investigated. Wahl et al. were unable to demonstrate a significant effect of delay in chemotherapy initiation for patients with newly diagnosed ALL on the examined outcome variables. In a group of 675 consecutive patients with newly diagnosed AL (both myeloblastic and lymphoblastic) presenting in the Centro de Hematología y Medicina Interna of the Clinica RUIZ, in Puebla, México in a 30-year period, we have found that Friday leukemia represents 16% of the cases of AL and that theweekday distribution of theALcases occurred randomly (see Fig. 1). It is clear that the presentation of AL on Saturday or Sunday was significantly lower than on Monday through Friday (P< 0.001, χ test) because on these days only emergency patients are received; however, the differences in Monday to Friday presentation of AL patients are not significant (P= 0.06, χ test). Accordingly, our data do not support the observation of Wilop et al. in a smaller group of myelogenous AL patients, in Germany. Several differences that could account for this discrepancy can be mentioned: they included only myeloblastic leukemias and we included both myeloblastic and lymphoblastic, and they recruited patients from a university hospital whereas we recruited them from the private practice. It is interesting to mention that in our analysis, there is no difference in Monday to Friday presentation of either lymphoblastic (n= 432) or myeloblastic (n= 243) leukemias.