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At least 127 records · Page 7Linked to original sources
PubMed2002

More cell death in refractory anemia with excess blasts in transformation than in acute myeloid leukemia.

Refractory anemia with excess blasts in transformation (RAEB-T) is a subgroup of myelodysplastic syndrome (MDS) in which the bone marrow blast count ranges from 20% to 30%. The recently proposed World Health Organization Classification of Hematologic Malignancies eliminated this category from MDS by lowering the blast count cutoff for acute myeloid leukemia (AML) from 30% to 20%. However, MDS is distinguished from AML by a significant increase in apoptosis. To investigate the difference in apoptosis between RAEB-T, AML, and other categories of MDS, we prospectively analyzed fresh bone marrow samples using the Annexin V and mitochondrial potential assays. There was a significantly higher level of apoptosis in RAEB-T than in AML according to both assays, while no significant differences between RAEB-T and other categories of MDS were noted. The data suggest that RAEB-T is more likely to be an advanced stage of MDS and biologically different from AML.

Acute Disease↗
PubMed2003

[Successful T-cell-replete peripheral blood stem cell transplantation from an HLA 2 loci mismatched mother to her son with refractory anemia in myelodysplastic syndrome].

A 15-year-old male with refractory anemia in myelodysplastic syndrome underwent peripheral blood stem cell transplantation from his two-loci-mismatched haploidentical mother. The conditioning regimen was comprised of cyclophosphamide, busulfan, and fludarabine. Non-T-cell-depleted peripheral blood stem cells were infused with graft-versus-host disease (GVHD) prophylaxis consisting of tacrolimus and short-course methotrexate. Engraftment was confirmed on day 18, and complete chimera on day 29. Although the patient developed grade II acute GVHD following the transplantation, it responded rapidly to steroid administration. The subsequent course was uneventful, and he remains well in complete remission in 26 months after transplant. This successful experience suggests that maternal hematopoietic stem cell transplant for children, in specific immune tolerance based on the presence of microchimerism and compatibility of minor histocompatibility antigens between mother and child, may have given rise to the favorable preventing effect against severe GVHD.

Adolescent↗
PubMed2001

Successful treatment of refractory anemia by high-dose methylprednisolone associated with an increment in CD68-positive cells in bone marrow.

Refractory anemia has a relatively low incidence of the subsequent development of acute leukemia and a relatively long survival among the myelodysplastic syndromes (MDS). We observed hematological improvement due to high-dose methylprednisolone in 9 of 18 patients with refractory anemia. The patients' age range was from 28 to 78 years old (mean age: 54), including 14 male and 4 females. A complete response was obtained in 5 patients, minimal response in 4 patients, and no response in 9 patients. Laboratory data of peripheral blood counts and differential counts of bone marrow aspirates were not different, except that fewer chromosomal abnormalities (P = 0.086) were observed in the responder group. Side effects were seen in two patients but were controllable. Overall survival was significantly longer in the responder group (Log-rank P = 0.040, Wilcoxon P = 0.045). The overall survival of responders did not reach the median and 85% of the patients were alive after 180 months, while the median overall survival of the nonresponders was 61.8 months. Disease progression was more frequently seen in the non-responder group (P = 0.045). Furthermore, we investigated retrospectively immunohistochemical bone marrow staining, and a significantly higher percentage of CD68-positive (22.6% +/- 7.1%) and CD45RA-positive cells was observed in the responder group compared to the non-responder group (6.5% +/- 1.3%). Our present results indicate that high-dose methylprednisolone is valuable as a primary treatment before other immunosuppressive treatments, because of its ease of use. High efficacy with high-dose methylprednisolone is expected, especially in patients in which increments in CD68-positive cells in bone marrow are observed.

Adult↗
PubMedDate not supplied

Decreased deformability of red cells in refractory anemia and the abnormality of the membrane skeleton.

Rheological characteristics of red cells in two patients with refractory anemia (with single chromosomal abnormality of 20q- or 13q-, respectively) were investigated with the hematological and biochemical properties. (1) Whole blood viscosity was remarkably increased, and the red cell deformability was greatly impaired (the impairments were prominent in patient with 20q-). (2) The hematocrit of both patients was about half of the normal value. Remarkable anisocytosis with elliptocytes and poikilocytes was observed in the patient with 20q-, but the anisocytosis was not so prominent in the patient with 13q-. (3) 2,3-diphosphoglycerate content in red cells was markedly increased in both patients, but adenylate content was not. (4) The red cells were slightly resistant to osmotic hemolysis, but they were not heat-labile. (5) Structural abnormality of spectrin was suggested from the impaired dimer-dimer association in red cell membrane and from the different susceptibility of spectrin to tryptic digestion. In conclusion, the rheological impairments and the abnormal shape of red cells in refractory anemia probably originated from the structural abnormality of cytoskeletal proteins in membrane, and the functional and structural abnormality may be different among patients.

2,3-Diphosphoglycerate↗
PubMed2002

Multifocal progressive leukoencephalopathy occurring after refractory anemia and multiple infectious disorders consecutive to severe lymphopenia.

Progressive multifocal leukoencephalopathy (PML) is related to central nervous system infection with JC virus (JCV). This leukoencephalopathy occurs in immunocompromised patients such as those with acquired immunodeficiency syndrome (AIDS) or lymphoid malignancies. We describe here a patient with myelodysplastic syndrome who developed several life-threatening infections including listeriosis, tuberculosis, and PML. Listeriosis and recurrence of tuberculosis preceded the occurrence of PML. Neurologic features associated with major ataxia, speech disorders, and PML were documented by cranial magnetic resonance imaging showing typical features in the cerebellum and proven by polymerase chain reaction (PCR) detection of JCV DNA in the cerebrospinal fluid. No specific treatment was decided because of progression toward acute myeloid leukemia. In this case, PML occurred with no susceptibility and without immunosuppressive treatment. Our case adds further support to the association between the impairment of T-cell immune responses and myelodysplastic disorders.

Anemia, Refractory↗
PubMed1986

Deletion of the long arm of chromosome 5 (5q-) as a secondary event in the course of refractory anemia.

A 54-year-old chemical plant worker developed mild pancytopenia, with normal bone marrow morphology. Normal bone marrow cytogenetics were documented. The patient developed worsening anemia 5 years into his course. The bone marrow morphology remained normal, but four of 30 bone marrow metaphases examined showed deletion of the long arm of chromosome #5. Eight years into his course, the patient developed severe thrombocytopenia, and his bone marrow became hypercellular, with dysplastic changes. Deletion of the long arm of chromosome #5 was seen in all of 21 bone marrow metaphases examined. There had been no new exposure to potential mutagens during the course of the patient's illness. The occasional documentation of the late appearance of cytogenetic abnormalities during the course of clonal hematopoietic disorders implies that, in some cases at least, chromosomal abnormalities may not be primary pathogenetic events. The full expression of clonal disorders may require several pathogenetic events, which may occur in variable order.

Anemia, Refractory↗
PubMed1987

Late appearing 5q--marker in refractory anemia.

A patient with myelodysplasia indistinguishable from the 5q--syndrome is described. No clonal chromosome abnormalities were detected in the total of 120 bone marrow cells initially examined. At the next sampling, 3 years later, del(5q) was present in all analyzed metaphases. These findings indicate that del(5q) may be only one of the pathogenetically important events in the 5q--syndrome, and that submicroscopic changes may precede the visible chromosomal rearrangement.

Anemia, Refractory↗