1/11. Refractory anemia with ringed sideroblasts with a low IPSS score progressed rapidly with de novo appearance of multiple karyotypic abnormalities and into acute erythroleukemia (AML-M6A).We report here a case of refractory anemia with ringed sideroblasts (RARS) with a low risk group by the International Prognostic Scoring System (IPSS) at the time of diagnosis but had a rapid disease progression. Although the patient showed a normal male karyotype at the time of RARS diagnosis, his marrow cells had del(5)(q14) and add(17)(p12) abnormalities 2 months after the diagnosis, and later the marrow cells had multiple abnormalities and the patient expired 6 months after the initial diagnosis of RARS. The patient was diagnosed as having RARS with a low risk group by the IPSS classification, however, one should keep in mind that some patients with myelodysplastic syndromes with low risks by either the French-American-British (FAB) classification or the IPSS classification may have progressive disease and subsequential cytogenetic analysis could predict the disease progression.- - - - - - - - - - ranking = 1keywords = disease progression, progression (Clic here for more details about this article) |
2/11. Immunosuppressive therapy for patients with refractory anemia.Trials of immunosuppressive therapy have been reported in some case reports of hypoplastic myelodysplastic syndrome (MDS). In this study, we gave immunosuppressive therapies to eight patients with normo- or hyperplastic MDS of refractory anemia subtype without karyotypic abnormalities and analyzed the HLA-DRB1 type or the presence of paroxysmal nocturnal hemoglobinuria (PNH) neutrophils in these patients. Cyclosporin A (CyA) therapy was effective for improving cytopenia in four of the eight MDS patients. While the side effects of CyA were mostly mild and transient, one patient demonstrated karyotypic abnormality following CyA therapy and accelerated to refractory anemia with an excess of blasts. Additional antithymocyte globulin (ATG) therapy was effective in one of three nonresponders to CyA therapy. One patient died due to leukemic transformation after ATG therapy. When we analyzed the correlation between the response to CyA therapy and the HLA-DRB1 type, there were more responders with DRB1*1501 (three of four patients) than without (one of four patients), but a statistically significant difference was not evident between the two groups. In addition, the presence of PNH neutrophils was not correlated with the response to CyA and/or ATG therapy. These results indicate the usefulness of immunosuppressive therapies even for normo- or hyperplastic MDS patients. Further trials using more patients with a long follow-up period would be worthwhile in order to clarify the possibility of disease progression and in order to predict the response of patients.- - - - - - - - - - ranking = 0.5keywords = disease progression, progression (Clic here for more details about this article) |
3/11. 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.- - - - - - - - - - ranking = 0.0025449864464391keywords = progression (Clic here for more details about this article) |
4/11. Essential thrombocythemia developing into refractory anemia and complicated by acute myeloid leukemia.We report a case of essential thrombocythemia (ET) that climaxed in acute myeloid leukemia after developing into refractory anemia. The male patient had ET that was stable for 8 years on carboquone therapy. However, at the age of 72 years he developed an acute terminal illness that was characterized by severe pancytopenia, circulating myeloblasts, extensive bone marrow infiltration by myeloblasts, and an abnormal karyotype [46, XY, t(8q-; 20q )]. He subsequently died of severe bilateral pneumonia and heart failure. This case suggests that ET may be similar to polycythemia vera; progression to leukemia is unusual except after chemotherapy. Therefore, treatment of patients with asymptomatic ET may not be advisable.- - - - - - - - - - ranking = 0.0025449864464391keywords = progression (Clic here for more details about this article) |
5/11. multiple myeloma in pregnancy.This is a report on pregnancy complicated by multiple myeloma. Severe refractory anemia was present throughout the pregnancy and multiple myeloma was diagnosed in the second trimester. The anemia ceased after delivery but recurred one year later along with other signs of disease progression. The infant remained healthy after a 2-year follow-up.- - - - - - - - - - ranking = 0.5keywords = disease progression, progression (Clic here for more details about this article) |
6/11. Myelodysplastic syndrome transformed into Acute Lymphoblastic Leukaemia (FAB:L3).Myelodysplastic syndrome (MDS) is recognized as a preleukaemic disorder with a variable risk of transformation to acute myeloid leukaemia. Usually the blast cells in leukaemia are transformed after MDS displays a myeloid phenotype. Even though lymphoid progression had been reported previously, most displayed myeloid-lymphoid hybrid or early B phenotype. We report a case of an elderly man who had MDS transformed into Acute Lymphoblastic Leukaemia (ALL:L3) which is a rare lymphoid transformation.- - - - - - - - - - ranking = 0.0025449864464391keywords = progression (Clic here for more details about this article) |
7/11. Refractory anaemia with preleukaemic polyclonal haemopoiesis and the emergence of monoclonal erythropoiesis on disease progression.We describe a young woman with a myelodysplastic syndrome (MDS) of the type refractory anaemia (RA) which remained stable for 11 years and then underwent rapid progression manifested by bone marrow failure with the emergence of a complex clonal cytogenetic abnormality. Peripheral blood granulocytes, mononuclear cells and bone marrow erythroblasts were all polyclonal by X-inactivation analysis detected by the probe M27B during the preleukaemic phase. On disease progression, bone marrow erythroblasts developed an extremely skewed monoclonal pattern of X-inactivation. In some cases of MDS, therefore, polyclonal haemopoiesis can be detected for a considerable time during the preleukaemic phase and we report the demonstration of bone marrow erythroblasts changing from a polyclonal to a monoclonal pattern on disease progression.- - - - - - - - - - ranking = 3.0025449864464keywords = disease progression, progression (Clic here for more details about this article) |
8/11. Progression of refractory anaemia with ringed sideroblasts (RARS) to B-acute lymphoblastic leukaemia.The study describes progression of refractory anaemia with ring sideroblasts (RARS) of 3 years' duration to B-acute lymphoblastic leukaemia (ALL). Development of ALL in this patient was preceded by the deterioration of dyserythropoiesis, leukopenia and an increase of blasts in the bone marrow. Our case may represent an additional evidence to the hypothesis that a primary pathogenetic lesion in myelodysplastic syndrome occurs at the level of the pluripotent stem cell.- - - - - - - - - - ranking = 0.0025449864464391keywords = progression (Clic here for more details about this article) |
9/11. trisomy 13 in a patient with leukemic progression of myelodysplasia.Four months after the diagnosis of refractory anemia, a 60-year-old patient developed acute leukemia with blast cells that were poorly differentiated by morphology and clearly myeloid by immunophenotyping. cytogenetic analysis performed at leukemization showed trisomy 13. An extra copy of chromosome 13 has already been reported in a few cases of acute leukemia and myelodysplastic syndrome.- - - - - - - - - - ranking = 0.010179945785756keywords = progression (Clic here for more details about this article) |
10/11. Sustained improvement in anemia with low-dose recombinant human erythropoietin therapy in a patient with hypoplastic myelodysplastic syndrome and chromosomal abnormalities.We present a case report of a 55-year-old male patient with hypoplastic myelodysplastic syndrome (MDS, refractory anemia) in which a good response to recombinant human erythropoietin (rhEPO) has been maintained for more than 60 months. There is with no evidence of progression to high risk MDS or acute leukemia, although he was predicted to be a low-responder to rhEPO therapy because of very high serum EPO levels (5,260 mU/ml), a history of multiple transfusions, chromosomal abnormalities (47,XY, 8) and severe thrombocytopenia. Since he received rhEPO with no adverse effects, it may be valuable to try rhEPO treatment at least one time for low-risk MDS patients, depending on red cell transfusion requirements.- - - - - - - - - - ranking = 0.0025449864464391keywords = progression (Clic here for more details about this article) |
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