Post-transplant cyclophosphamide has become a promising medical option after allogeneic HSCT. In this study we aimed to evaluate the efficacy of cyclophosphamide and cyclosporine combination in acute and chronic graft-versus-host disease (GvHD) prophylaxis in acute myeloid leukemia (AML) cases scheduled for allogeneic hematopoietic stem cell transplantation (allo-HSCT). Retrospective analysis of data from 40 cases who underwent allogeneic HSCT under GvHD prophylaxis with cyclophosphamide and cyclosporine combination between April 2016 and August 2017 was made. Cyclophosphamide was given at daily doses of 50 mg/kg on post-transplant 3rd and 4th days, and cyclosporine was applied at daily doses of 3 mg/kg/day starting from the 5th post-transplant day. Cyclosporine dose was tapered beginning from the 45th postoperative day and completely discontinued on the 90th post-transplant day. Mean age was 38.25 ± 15.25 years. Posttransplant median follow-up was six months (6–17 months). Post-transplant, the number of deaths and mortality rates related and unrelated to transplantation were 5 (12.5%), and 2 (5%), respectively. Acute GvHD was diagnosed in 7 cases (17.5%), and relapse was noted in 9 cases (22.5%). Myeloablative or reduced intensity conditioning was performed in 22 (55%) and 18 (45%) patients, respectively. The distribution of the donors was as follows: match-related (n = 26; 65%), match-unrelated (n = 9, 22.5%) and haploidentical donors (n = 5; 12.5%). There was no statistically significant correlation between the transplant-related and unrelated mortality and parameters under investigation.Cyclophosphamide use appears to be a highly effective and promising strategy for acute GvHD prophylaxis in non-haploidentical allogeneic HSCT cases. Identification of the impact of cyclophosphamide use on the development of chronic GvHD needs further investigation.
1 December 2021 in Transfusion and Apheresis Science
Essential thrombocythemia (ET) is a Philadelphia chromosome (Ph)-negative myeloproliferative neoplasm. It is characterized by thrombocytosis and megakaryocytic hyperplasia of the bone marrow with JAK2V617F mutation. Iron-refractory iron deficiency anemia (IRIDA) is an autosomal recessive disorder, which is mainly characterized by iron deficiency anemia not responding to oral iron intake, but partially responding to parenteral iron therapy. Recently, it has been shown that IRIDA has stemmed from mutations in the gene TMPRSS6, which encodes a transmembrane serine protease (matriptase- 2) expressed by the liver. Renal cell carcinoma (RCC) accounts for 2–3% of all cancers. As the most common solid lesion in the kidneys, it represents approximately 90% of all renal malignancies. Approximately 30% of patients with symptomatic RCCs seem to display paraneoplastic syndromes. The symptom that may result from erythrocytosis is the most wellknown paraneoplastic hematological event. Here, we report a patient who presents with coexistence of RCC and thrombocytosis, which hasn’t been caused by hormonal factors that are produced in tumor cells. This patient has been therefore diagnosed with ET. The patient who was expected to display RCC with polycythemia, conversely present with IRIDA.
Primary immune thrombocytopenia (ITP) is an acquired autoimmune disease characterized by isolated thrombocytopenia. While first-line treatments focus on inhibiting autoantibodies and platelet destruction, second- and third-line treatments include splenectomy and Thrombopoietin receptor agonists (TPO-RAs). In this study, we aimed to compare the efficiency and toxicities of splenectomy and Eltrombopag as second-line treatments in ITP. We retrospectively analyzed patients who were diagnosed with ITP and followed between 2015 and 2020. Patients who underwent splenectomy or received Eltrombopag treatment as 2nd or further line therapy were included. For subgroup analyses, patients were further stratified according to whether they received Eltrombopag in the second or third line of treatment. There were 38 patients in the splenectomy group and 47 patients in the Eltrombopag group. Mean age of patients in splenectomy and Eltrombopag groups 43.2 and 50.5, respectively. Time to response is significantly shorter in the splenectomy arm (p= 0.001). However, response rates at the 3rd, 6th, 12th, and 24th months, total duration of response and adverse events did not exhibit a statistically significant difference between groups. Response rates at the 1st, 3rd, 6th, 12th, and 24th months and the total duration of response did not exhibit a statistically significant difference between the Eltrombopag subgroups. Eltrombopag treatment was ceased in 20 patients after a median of 54.1 months (1-151). Among them, 12 patients (60%) did not experience a loss of response. When we compare the splenectomy and eltrombopag arms, even though time to achieving response is in favor of splenectomy, this advantage disappears when overall response rates and response rate at the 2nd year are considered. Using eltrombopag in the 2nd or 3rd line of therapy does not yield any difference in terms of time to achieving response.