Background
Thrombotic thrombocytopenic purpura (TTP) is a hematologic disease that can be fatal if not treated early. We aimed to describe the clinical characteristics of Mexican patients with idiopathic TTP.
Study design and methods
We conducted a retrospective study, including all adult patients diagnosed with idiopathic TTP from 2011 to 2017 in two Mexican centers. We further compared our results with the published literature.
Results
Twenty patients were included; 70% were female, with a median age of 38.5 years at diagnosis (range 16‐63). The median time from onset of symptoms to hospital admission was 1.5 days (range 0‐16). Most patients (85%) presented with at least one systemic manifestation at admission (including fever) and 90% had neurological symptoms, most of them major (70%) including loss of consciousness, transient focal abnormalities, headache, and confusion. Only one patient (5%) had the classical pentad at the time of admission. Kidney failure was present in 25% of patients and hemorrhagic symptoms in 60%. Digestive and cardiorespiratory symptoms were less common (45% and 15%, respectively). Median platelet count and lactate dehydrogenase were 10 500/μL and 1319 IU/L, respectively. Eighty percent of patients achieved remission following treatment. Patients admitted within the first 48 hours (after the onset of symptoms) tended to have better overall survival.
Conclusion
Clinical presentation in Mexican TTP patients is similar to that in other countries. Early admission and a high suspicion for the disease will avoid delays in the initial work‐up and initiation of therapy, further improving prognosis.
Angiosarcomas are neoplasms of blood or lymphatic vessels with aggressive behavior. We report the coexistence of this malignancy within soft tissue of the breast in a 49-year-old woman who was diagnosed with Klippel-Trenaunay-Weber syndrome (KTW-S) during childhood. The patient has no previous history of radiation therapy on the chest and does not have any known risk factor for developing angiosarcoma, except for her congenital disease. To the best of our knowledge, the association between soft tissue angiosarcoma of the breast and KTW-S has never been previously reported.
Introduction: The use of filgrastim biosimilars for healthy adult and pediatric donor mobilization in hematopoietic stem cell transplantation has been met with increased safety and efficacy concerns in contrast to generic small molecule drugs. In Mexico, several filgrastim-intended copies (FIC) have been available and marketed since 2001, while no clinical comparability studies to evaluate their use in this setting have been published and thus are not considered to be true biosimilars. In this study, we report our experience using three different FIC products currently available (Filatil, Dextrifyl, and Biofilgran). Methods: We retrospectively evaluated 118 related donors of all ages who received any brand 5 μg/kg subcutaneously twice daily for 4 days and were harvested in a single apheresis system on day 5. Results: Donors had a median age of 38 years (range, 1-69). A successful harvest defined as ≥2 × 10 6 CD34+ cells/kg of recipient weight was achieved in 95.8% of cases, with a median CD34+ cell dose of 9.4 × 10 6 /kg (range 1-42.8). A single apheresis session was performed in 89.8% of cases. No significant difference in cell yield between each brand was observed. All pediatric donors had a successful harvest with similar results to adult donors. No immediate severe adverse effects were documented in any case. Conclusions: In conclusion, three FICs available in Mexico were efficacious and without immediate severe adverse effects, resulting in significant cost savings.Evaluation of immunogenicity and establishment of a pharmacovigilance program with the use of FICs is warranted. K E Y W O R D S apheresis, biosimilar, filgrastim, g-CSF, healthy donor, hematopoietic stem cell transplantation, peripheral blood Presented in an abstract form in ASBMT/CIBMTR Tandem meetings, Orlando FL 2017.
patients with unidentified relapse at TPO start, TPO preceded diagnosis of relapse by median 62 days (range 22-281). Of the 9 patients who started TPO as inpatient, 7 subsequently died with median time from TPO to death 195 days (range 18-398). No significant toxicities to TPO were identified. The median time from TPO outpatient prescription to start was 22 days due to insurance delays (range 8-40). Conclusion: While causality is difficult to establish, TPO was associated with improvement of platelet counts in a subset of patients, with responses most likely to occur in outpatients with viral reactivation, GvHD, or primary poor engraftment. In 5 patients with unidentified relapse, start of TPO preceded a diagnosis of relapse by median 62 days. This should alert clinicians to assess for relapse when considering TPO. The start of TPO in inpatients generally reflects grave clinical outlook given low response rates and high number of subsequent deaths.
homing to the GI tract results in decreased GVHD severity and improved outcome in an established humanized mouse model of allo HCT. Methods: Sublethally irradiated RAG2-/-gc-/mice were transplanted with 30 million human PBMCs (huPBMCs). Vedolizumab or control IgG was given intraperitoneally at a dose of 4mg/kg body weight on day-1 and day +15 (preventive approach) or day +8 and day +22 (therapeutic approach). Time points were chosen in order to start a4b7 blockade prior to clinical onset of GVHD (preventive approach) and when animals started to get significantly suffer from GVHD (therapeutic approach). As non-GVHD controls, animals received sublethal irradiation without huPBMCs administration and received VDZ or control IgG to assess for direct conditioning and VDZ toxicity. Results: HuPBMC CD45 splenocyte analysis on day +28 after infusion demonstrated strong human leukocyte engraftment with more than 80% of spleen cells being huCD45 positive. Both preventive and therapeutic administration of Vedolizumab resulted in significantly improved survival in HuPBMC treated recipients when compared to IgG treated GVHD controls at week 8 (62.6% Vs 12.5% and 50% Vs 12.5% respectively). All recipients, which did not receive huPBMCs survived, indicating VDZ safety in this model. Clinical GVHD scores after day 21 after transplant were significantly lower in VDZ huPBMCs treated animals until end of analysis compared to control-treated recipients using both the preventive and therapeutic approaches, along with significant decreases in GI tract pathology scores on day +28. No differences in pathology were seen in liver or lung at this time point. Serum cytokine analysis revealed decreases in TNF (p<0.05) and IFNg (p<0.05) levels in Vedolizumab therapeutically treated recipients on day +28 and in TNF (p<0.05) in preventively treated recipients compared to control treated animals. Histopathologic analysis of T cell infiltration of small intestine and colon by immunohistochemistry for CD3 antibody revealed significantly lower infiltration of human T cells in VDZ huPBMCs treated group (p<0.05). Conclusion: Vedolizumab reduces the severity of intestinal GVHD by targeting donor T cell migration into the intestinal tract both when given early prior to onset of clinical disease and given at later time point, and improves overall survival both in the preventive and therapeutic setting. Given these promising results, targeting T cell homing with VDZ potentially presents a novel option for the management of intestinal GVHD.
Angiosarcomas are neoplasms of blood or lymphatic vessels with aggressive behavior. We report the coexistence of this malignancy within soft tissue of the breast in a 49-year-old woman who was diagnosed with Klippel-Trenaunay-Weber syndrome (KTW-S) during childhood. The patient has no previous history of radiation therapy on the chest and does not have any known risk factor for developing angiosarcoma, except for her congenital disease. To the best of our knowledge, the association between soft tissue angiosarcoma of the breast and KTW-S has never been previously reported.
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