(Hematology AND Aplastic anemia)
- Autoimmune Hepatitis and Seronegative Hepatitis Associated With Myelodysplastic Syndrome in Children. [Journal Article]
- JPJ Pediatr Hematol Oncol 2016 Jul 27
- An association between hepatitis and aplastic anemia (AA) is known as hepatitis-associated AA, and is characterized by an acute attack of hepatitis followed by the development of AA. We report 2 clin...
An association between hepatitis and aplastic anemia (AA) is known as hepatitis-associated AA, and is characterized by an acute attack of hepatitis followed by the development of AA. We report 2 clinical cases of acute seronegative hepatitis in which pancytopenia with mild dysplasia developed after 3 months; however, neither of our cases fulfilled the histological criteria of AA, but rather myelodysplastic syndrome. This novel association bears considerable resemblance to hepatitis-associated AA, and raises the question of whether hepatitis-associated dysmyelopoiesis should be included in the spectrum of hypocellular myelodysplastic syndrome.
- High-dose Cyclophosphamide is Effective Therapy for Pediatric Severe Aplastic Anemia. [Journal Article]
- JPJ Pediatr Hematol Oncol 2016; 38(8):627-635
- CONCLUSIONS: Response rates and survival following high-dose cyclophosphamide in pediatric patients with SAA exceed those seen in adults and compare favorably to antithymocyte globulin/cyclosporine A with manageable infectious toxicity.
- Very Severe Aplastic Anemia During Treatment With Doxycycline. [Journal Article]
- AJAm J Ther 2016 Oct 18
- Vitamin D in haematological disorders and malignancies. [Review]
- EJEur J Haematol 2016 Oct 15
- Commonly known for its critical role in calcium homeostasis and bone mineralization, more recently vitamin D has been implicated in haematological cancer pathogenesis and shows promise as an anti-can...
Commonly known for its critical role in calcium homeostasis and bone mineralization, more recently vitamin D has been implicated in haematological cancer pathogenesis and shows promise as an anti-cancer therapy. Serum levels of 25(OH)D3 , the precursor to the active form of vitamin D, calcitriol, are typically lower in patients with haematological disease compared to healthy individuals. This often correlates with worse disease outcome. Furthermore, diseased cells typically highly express the vitamin D receptor (VDR), which is required for many of the anti-cancer effects observed in multiple in vivo and in vitro cancer models. In abnormal haematological cells, vitamin D supplementation promotes apoptosis, induces differentiation, inhibits proliferation, sensitizes tumor cells to other anti-cancer therapies, and reduces the production of pro-inflammatory cytokines. Although the dosage of vitamin D required to achieve these effects may induce hypercalcemia in humans, analogs and combinatorial treatments have been developed to circumvent this side effect. Vitamin D and its analogs are well tolerated in clinical trials and thus further investigation into the use of these agents in the clinic is warranted. Here we review the current literature in this field. This article is protected by copyright. All rights reserved.
- Evaluation of efficacy of alemtuzumab in 5 patients with aplastic anemia and/or myelodysplastic neoplasm. [Journal Article]
- WKWien Klin Wochenschr 2016 Oct 14
- Patients with aplastic anemia or hypoplastic myelodysplastic syndrome (MDS) may respond to immunosuppressive therapy, including the anti-CD52 antibody alemtuzumab. We analyzed treatment responses to ...
Patients with aplastic anemia or hypoplastic myelodysplastic syndrome (MDS) may respond to immunosuppressive therapy, including the anti-CD52 antibody alemtuzumab. We analyzed treatment responses to alemtuzumab in 5 patients with MDS or aplastic anemia (AA) evolving to MDS. Two patients with hypoplastic MDS (hMDS) showed a partial response (PR) to alemtuzumab. In both responding patients, a concomitant paroxysmal nocturnal hemoglobinuria (PNH) clone was detected before therapy. One responder relapsed after 15 months and underwent successful allogeneic stem cell transplantation. Both patients are still alive and in remission after 40 and 20 months, respectively. The other patients showed no response to alemtuzumab. One patient died from pneumonia 4 months after treatment. In summary, our data confirm that alemtuzumab is an effective treatment option for a subset of patients with MDS, even in the presence of a PNH clone.
- Aplastic anemia. [Journal Article]
- RKRinsho Ketsueki 2016; 57(10):1890-1899
- Treatments of aplastic anemia are comprised of supportive therapy and aplastic anemia-specific therapy aimed at restoring hematopoiesis. Supportive therapies include transfusion, G-CSF, and the admin...
Treatments of aplastic anemia are comprised of supportive therapy and aplastic anemia-specific therapy aimed at restoring hematopoiesis. Supportive therapies include transfusion, G-CSF, and the administration of iron chelation agents, as well as dealing specifically with individual symptoms. Aplastic anemia-specific treatments given with the aim of achieving hematopoietic recovery include immunosuppressive therapy, allogeneic hematopoietic stem cell transplantation, and anabolic hormone therapy. Although transplantation provides complete recovery of hematopoiesis (cure), there is a risk of death due to transplant-related complications. The most effective immunosuppressive therapy is a combination of anti-thymocyte globulin and cyclosporine. This treatment is also effective against the secondary, drug-induced and hepatitis-associated forms of aplastic anemia. In the management of aplastic anemia, a treatment is selected from among these options depending on the disease severity and the age of the individual case. The thrombopoietin receptor agonist eltrombopag appears to be effective and to provide tri-lineage recovery of hematopoiesis in some cases. Indications for its use are expected to expand in Japan.
- Changes in serum uric acid levels after allogeneic hematologic stem cell transplantation: A retrospective cohort study. [Journal Article]
- BRBlood Res 2016; 51(3):200-203
- CONCLUSIONS: HSCT is associated with significant changes in uric acid levels in patients with hematologic disorders.
- [The clinical feature and outcomes of severe aplastic anemia patients suffered from bacteremia following antithymocyte globulin]. [Journal Article]
- ZXZhonghua Xue Ye Xue Za Zhi 2016 Sep 14; 37(9):807-812
- Objective: To assess the clinical feature and outcomes of severe aplastic anemia (SAA) patients suffered from bacteremia following antithymocyte globulin (ATG). Methods: A total of 264 cases hospital...
Objective: To assess the clinical feature and outcomes of severe aplastic anemia (SAA) patients suffered from bacteremia following antithymocyte globulin (ATG). Methods: A total of 264 cases hospitalized in our hospital between Jan 2000 and July 2011 were enrolled into this study. We evaluated the associated pathogens of bacteremia, analyzed the risk factors by Logistic regression and estimated the overall survival (OS) by Kaplan-Meier method for the cohort of patients. Results: Bloodstream infections occurred in 49 patients, with a median age of 20 (4-62) years, including 38 cases with very SAA (VSAA) and 11 SAA patients. The median time of bacteremia was 13 (2-233) days following ATG administration. The most common microbiologically were Enterobacteriaceae (28.4% ), Pseudomonas aeruginosa (20.9% ) and Klebsiella pneumonia (14.9% ). Almost half (46.9% ) of these bacteria were resistant to most or all available antibacterial classes. Univariate and multivariate analyses demonstrated that VSAA, infections during previous week before ATG treatment were risk factors for bacteremia. The 3 and 6 months response rates (10.6% and 17.0% ) were poor in the patients with bloodstream infections, which were significantly lower than those patients without infections (35.6% and 55.6%, respectively, both P<0.001). The estimated 5-year OS were 36.4% (95%CI 21.3% to 51.5%) and 74.5% (95%CI 68.4% to 80.7%) in the two groups, respectively (P<0.001). Conclusions: ①VSAA has higher risk of bacteremia than SAA; ②Infections during previous week before ATG administration was a risk factor for bacteremia; ③ The outcomes of SAA or VSAA patients suffered from bacteremia following ATG was poor.
- Allogeneic hematopoietic stem cell transplantation for nonmalignant hematologic disorders using chemotherapy-only cytoreductive regimens and T-cell-depleted grafts from human leukocyte antigen-matched or -mismatched donors. [Journal Article]
- PHPediatr Hematol Oncol 2016 Oct 7; :1-12
- Nonmalignant hematologic disorders (NMHD) of childhood comprise a variety of disorders, including acquired severe aplastic anemia and inherited marrow failure syndromes. Patients with high-risk NMHD ...
Nonmalignant hematologic disorders (NMHD) of childhood comprise a variety of disorders, including acquired severe aplastic anemia and inherited marrow failure syndromes. Patients with high-risk NMHD without matched related donors fare poorly with allogeneic hematopoietic alternative donor stem cell transplantation (allo-HSCT) and are at high risk for developing graft-versus-host disease following unmodified grafts. The authors retrospectively analyzed data on 18 patients affected by NMHD, lacking a human leukocyte antigen (HLA)-identical sibling donor, who underwent an alternative donor allo-HSCT at their institution between April 2005 and May 2013. Fifty percent of the patients had received prior immunosuppressive therapy, 72% had a history of infections, and 56% were transfusion dependent at the time of transplant. Cytoreduction included a combination of 3 of 5 agents: fludarabine, melphalan, thiotepa, busulfan, and cyclophosphamide. Grafts were T-cell depleted. All evaluable patients engrafted. Five died of transplant complications. The cumulative incidence of graft-versus-host disease was 6%. No patient had recurrence of disease. Five-year overall survival was 77%. Age at transplant <6 years was strongly associated with better survival. Based on these results, transplant with chemotherapy-only cytoreductive regimens and T-cell-depleted stem cell transplants could be recommended for patients with high-risk NMHD, especially at a younger age.
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- Short-term efficacy and safety of antithymocyte globulin treatment in elderly patients with acquired aplastic anaemia. [Letter]
- BJBr J Haematol 2016 Oct 7