- GPRASP proteins negatively regulate HSC engraftment and proliferation
- Outcome of relapsed/refractory Burkitt lymphoma/leukemia
- Clonal hematopoiesis in anemia in older individuals
- Rapid diagnosis of heparin-induced thrombocytopenia
- Preexistent TP53 mutations in SCD patients developing AML following transplant
In this week’s episode we’ll learn about novel insights into the link between innate immune responses and activation of coagulation during gram-negative sepsis, examine the impact of GPRASP proteins on hematopoietic stem cell transplantation, and explore the dynamics of clonal hematopoiesis in anemia of older individuals.
Scleromyxedema is a rare skin and systemic disorder associated with monoclonal gammopathy. In a retrospective study of 33 patients, the authors documented the efficacy of intravenous immunoglobulin in the treatment of mild and moderate cases of scleromyxedema and of antimyeloma therapy in severe cases.
Hematopoietic stem cell transplantation (HSCT) depends on engraftment and proliferation of donor HSCs. Morales-Hernández and colleagues report that GPRASP proteins are negative regulators of HSCs and that silencing of Gprasp1 or Gprasp2 increases HSC survival, engraftment, and proliferation through downregulation of CXCR4. These findings suggest that disruption of this pathway could enhance HSCT.
Progressive or relapsed Burkitt lymphoma or leukemia in children and adolescents after BFM-type first-line therapy
While survival for pediatric, adolescent, and young-adult Burkitt lymphoma/leukemia (BL) is over 90%, the prognosis for those with relapsed or primary refractory disease is extremely poor. Woessmann et al reviewed the 3-year survival of 157 out of 1979 children (8%) and adolescents with relapsed or refractory BL and identified risk factors for the failure of intensive salvage therapy and stem cell transplantation.
Anemia increases with age and one-third of cases are unexplained. The authors examined the relationship of clonal hematopoiesis (CH) to the anemia of aging. While common age-related mutations were not increased in the anemia population, deleterious mutations, including TP53 and SF3B1, were increased. However, the higher prevalence of CH was insufficient to account for unexplained anemia, the etiology of which remains elusive.