No abstract available.
Anemia, Refractory

No abstract available.
Anemia, Refractory* ; Thrombocytosis*

No abstract available.
Anemia, Refractory* ; Cyclosporine*

No abstract available.
Anemia, Refractory* ; Azacitidine* ; Erythropoietin* ; Leukemia, Myelomonocytic, Chronic*

Anemia, Refractory ; Humans ; Male ; Middle Aged ; Thrombocytosis

OBJECTIVE: To evaluate the clinical efficacy of low dose combined chemotherapy（LDCC） for patients with relapsed and refractory aplastic anemia-paroxysmal nocturnal hemoglobinuria（AA-PNH） syndrome, and to analyze the advantages of LDCC in the treatment of AA-PNH syndrome. METHODS: The clinical characteristics and the curative effect of LDCC in 9 patients with relapsed and refractory AA-PNH syndrome were retrospectively analyzed. Five patients were treated with MP therapy［melphalan 2 mg/(m·d); prednisone 0.5 mg/(kg·d)］, and the other 4 patients were treated with HA therapy(HHT 2 mg/d iv drip, for 5 days; Ara-C 100 mg/d iv drip, for 5 days). The changes of PNH clone, dosage of corticosteroid, hemolysis and the relapse of disease, hematological parameters and adverse reactions were compared before and after therapy. All patients were treated for 1-2 courses. RESULTS: Seven out of 9 patients responded well, the dosage of corticosteroid and the bilirubin concentration decreased significantly and anemia was relieved in 7 patients (P<0.05). One patient relapsed in one year. PNH clone of 3 patients turned negative. Five patients did not rely on blood transfusion in 1 year. There was no bone marrow failure to be found in all patients. CONCLUSION The LDCC has better efficacy and safety in the treatment of patients with AA-PNH syndrome, moreover, the patients is more tolerant to LDCC, thus the LDCC may be a selection for treatment of patients with relapsed and refractory AA-PNH syndrome.
Anemia, Aplastic ; Anemia, Refractory ; Hemoglobinuria, Paroxysmal ; Hemolysis ; Humans ; Retrospective Studies

OBJECTIVE: To investigate the efficacy and safely of DAC and CAG/HAG preexcitation chemotherapy regimens for the treatment of patients with MDS-RAEB (refractory anemia with excess blasts, RAEB). METHODS: The clinical data of 86 MDS-RAEB patients were analyzed retrospectively from February 2014 to February 2018. According to therapeutic regimem, the 86 patients were divided into 2 groups: group A (41 patients) with DAC preexcitation chemotherapy regimen, and group B (45 patients) with CAG/HAG preexcitation chemotherapy regimen; and the disease control effect, effective treatment course, median survival time and incidence of adverse reactions were compared between these 2 groups. RESULTS: The CR rate and ORR rate were not significantly different between these 2 groups (P＞0.05). The mCR rate in group A was significantly higher than that in group B (P＜0.05). The numbers of cases obtained therapeutic efficacy at 2 rd and 3 rd conrse in group A significantly more than those in group B (P＜0.05), but the number of cases obtained efficacy at 1 st course in group B was significantly higher than that in group A (P＜0.05). The median OS time was not significanly different between 2 groups (P＞0.05). The duration of neutrophils deficiency in group A was significantly shorter than that in group B (P＜0.05). The transfusion volume of red blood cells and platelets in group A was significantly less than that of group B (P＜0.05). The incidence of neutropenia, anemia and thrombocytopenia of III-IV grade at different treatment courses of group A were significantly lower than that in group B (P＜0.05). The incidence of infection of III-IV grade in group A at 3rd treatment course was significantly lower than that in group B (P＜0.05). CONCLUSION Preexcitation chemotherapy regimens of DAC and CAG/HAG for the treatment of MDS-RAEB possess the same effects for disease control; application of DAC regimen can efficiently reduce the risk of adverse reaction, but CAG/HAG regimen can be helpful to accelerate the effective process of treatment.
Anemia, Refractory ; Anemia, Refractory, with Excess of Blasts ; drug therapy ; Humans ; Myelodysplastic Syndromes ; drug therapy ; Retrospective Studies ; Treatment Outcome

Refractory anemia with ringed sideroblasts (RARS) is an extremely rare type of myelodysplastic syndrome in children. We describe a 10-year-old boy with RARS presented with pancytopenia. He remained relatively stable with only a few transfusions until age of 20 years, when he underwent an allogeneic bone marrow transplantation (BMT) because of increased transfusion requirements. He remains in complete chimeric state at 20 months posttransplant with normal hematologic parameters. To our knowledge, this is the first description of successful BMT in a patient with childhood-onset RARS. The indication of BMT for this rare disorder in children is discussed.
Anemia, Refractory/therapy* ; Anemia, Sideroblastic/therapy* ; Bone Marrow Transplantation* ; Case Report ; Child ; Human ; Male ; Transplantation, Homologous

Translocation between chromosomes 1 and 19 is well documented in ALL. Here, we report a case of refractory anemia with ring sideroblasts associated with marked thrombocytosis with der(19)t(1;19). A 67-yr-old man was admitted to our hospital with anemia and thrombocytosis. The aspirated bone marrow showed erythroid and megakaryocytic hyperplasia and dyspoiesis. Iron staining showed that the ring sideroblasts increased in number. Bone-marrow cell karyotyping showed 46,XY,der(19)t(1;19)(q23;p13)[9]/46,XY,del(5)(q21)[2]/46,XY[9]. PCR analysis showed the absence of the TCF3-PBX1 rearrangement. The patient was treated with hydroxyurea and intermittent blood transfusion. It is known that t(1;19)(q23;p13) leads to a TCF3-PBX1 fusion gene, whose product is a powerful transcriptional activator that plays a key role in the development of ALL. However, t(1;19) has rarely been reported in myeloid neoplasms and the TCF3-PBX1 fusion gene has not been detected. This implies that other genes might be involved in the TCF3-PBX1 rearrangement, or an alternative TCF3-PBX1 fusion transcript with a different breakpoint has not been detected to date. Further research and case studies, including the use of molecular analysis techniques, are required to evaluate the clinical and prognostic significance of t(1;19) in the development of myeloid neoplasms.
Anemia ; Anemia, Refractory ; Blood Transfusion ; Bone Marrow ; Humans ; Hydroxyurea ; Hyperplasia ; Iron ; Karyotyping ; Polymerase Chain Reaction ; Thrombocytosis

BACKGROUND: Myelodysplastic syndrome (MDS) is characterized by signs of maturation disturbances in the both of peripheral blood (PB) and bone marrow (BM). Every hematologist who cares for patients with MDS will occasionally encounter difficulties in establishing the diagnosis, particularly in refractory anemia. Dysplastic features of cells in peripheral blood and bone marrow were studied to evaluate the significance of the degree of neutrophil hypogranulation (G-score) and the percentage of pseudo-pelgeroid polymorphs (PPP) in PB smear. METHODS: Cytoplasmic hypogranulation and nuclear abnormalites of the pelgeroid type of neutrophils were studied retrospectively on air dried peripheral blood and bone marrow smears stained with Wright reagent, and assayed according to Hast's method. In every cases, 100 neutrophils were analyzed as follows: the degree of hypogranulation was graded in arbiturary unit with the range 0 to 3. Neutrophils containing a single or bilobated nucleus with dense compact chromatin were counted as the pelgeroid type. Common morphological features of MDS were observed on bone marrow smears. A numetric level between 0 and 2 was assyed for two dysplastic features of the three hemoatopoiesis lineages. The score for total degree of bone marrow dysplasia (DysT) ranged between 0 and 12. RESULTS: There was a good correlation between PB and BM findings, of both C-score (r=0.5503, p<0.01) and PPP (r=0.8701, p<0.001). Patients with a high degree of bone marrow dysplasia had significantly higher PPP (r=0.5853, p<0.005) than those with less pronounced myelodysplasia. Patients with refractory anemia with excess blast (RAEB)/refractory anemia with excess blast in transformation (RAEB-T)/chronic myelomonocytic leukemia (CMML) had higher PPP, but lower G-score than those with refractory anemia (RA)/refractory anemia with ringed sideroblasts (RARS). CONCLUSIONS: The above results show that quantitative estimation of peripheral blood polymorph dysplasia by G-score and PPP seems to reflect the total degree of bone marrow dysplasia in MDS and may serve as a helpful method to bone marrow evaluation when the diagnosis is difficult.
Anemia ; Anemia, Refractory ; Bone Marrow ; Chromatin ; Cytoplasm ; Diagnosis ; Humans ; Leukemia ; Myelodysplastic Syndromes* ; Neutrophils ; Retrospective Studies