Treatment of Bone Marrow Failure Syndrome with Integrated Traditional and Western Medicine

Aplastic anemia （AA） and myelodysplastic syndrome （MDS） are both included in the bone marrow failure syndromes （BMFS）. AA is a group of diseases characterized by hematopoietic stem/progenitor cell damage, oerioheral blood cvtooenia, andclinical manifestations including anemia, bleeding and infection, which eventually lead to bone marrow failure. The incidence rate of AA in China is 7.4/10^6, higher than that in Western countries, among which the morbidity of acute AA and chronic AA （CAA） is 1.4/10^6 and 6.0/10^6, respectively.

Effects of Angelica Polysaccharide on telomere length in mice with benzene-induced aplastic anemia

Objective:To investigate the changes in telomere length and the level of burst-forming units-erythrocyte(BFU-Es)and colony-forming unit-erythrocytes(CFU-Es)in mice with benzene-induced aplastic anemia(AA),and follow-up the therapeutic effects of Angelica Polysaccharide(AP).Methods:Male BALB/c mice(n=120)were randomly divided into three groups(1,2,3):normal control(n=24),AA control(n=48),and treated AA(n=48),respectively.Mice in Group 2 received benzene inhalation for 2.5 months and 1 ml distilled water p.o.per day for 2 weeks after the establishment of AA models.Similar procedure was applied to the mice in Group 3 and AP was given for 2 weeks after the establishment of AA models.Real-time polymerase chain reaction was used to survey relative telomere length measurement of the bone marrow cells.A BFU-Es and CFU-Es survey was done to follow-up the therapeutic effects of AP.Results:Compared with normal control,significant reductions of RBC,WBC,and platelet counts were found in peripheral blood of AA mice.After treatment with AP,counts of BFU-Es and CFU-Es were restored up to 66.8%and 77.25%,respectively,and length of telomere was restored up to 76.34%,of the normal levels.The telomere length in treated AA group was higher than the control AA group.Conclusion:The AP can protect the telomere length and differentiation of hemopoietic stem/progenitor cells,accelerate the recovery of BFU-Es and CFU-Es of AA mice,and then improve the bone marrow failure.

Does DKC1 Mutation Suffice to Define the Phenotype Severity of Hoyeraal-Hreidarsson Syndrome?

Both dyskeratosis congenita (DC) and Hoyeraal-Hreidarsson Syndrome (HHS) are rare inherited bone marrow failure conditions. HHS is considered to be a variant of DC in which neurological deficits and immunodeficiencies are also present. We describe a very interesting familial cluster where an invariant point mutation of DKC1 located in the exon 11 is observed in the carrier mother and in two decedent males. The older child developed the classical phenotype of HHS at a very early age. The second affected child remains poorly symptomatic, with only mild haematological changes. Telomere shortening, with different severity, is also present in both cases. This paper discusses the clinical spectrum of inherited BM failure syndromes from the perspective different clinical presentation within a family with a DKC1 mutation.

Toll-like receptor signaling in hematopoietic homeostasis and the pathogenesis of hematologic diseases

Toll-like receptors （TLRs）, which are found in innate immune cells, are essential mediators of rapid inflammatory responses and appropriate T-cell activation in response to infection and tissue damage. Accumulating evidence suggests that TLR signaling is involved in normal hematopoiesis and specific hematologic pathologies. Particular TLRs and their downstream signaling mediators are expressed not only in terminally differentiated innate immune cells but also in early hematopoietic progenitors. Sterile activation of TLR signaling is required to generate early embryonic hematopoietic progenitor cells. In adult animals, TLR signaling directly or indirectly promotes differentiation of myeloid cells at the expense of that of lymphoid cells and the self renewal of hematopoietic stem cells during infection and tissue damage. Activating mutations of the MyD88 gene, which codes for a key adaptor involved in TLR signaling, are commonly detected in B-cell lymphomas and other B-cell hematopathologies. Dysregulated TLR signaling contributes to the pathogenesis of many hematopoietic disorders, including bone marrow failure, myelodysplastic syndrome, and acute myeloid leukemia. Complete elucidation of the molecular mechanisms by which TLR signaling mediates the regulation of both normal and pathogenic hematopoiesis will prove valuable to the development of targeted therapies and strategies for improved treatment of hematopoietic disorders.