1/11. Myelodysplastic syndrome and associated skin lesions: a review of the literature.The skin involvement of the myelodysplastic syndrome (MDS) can take the form of either a neoplastic infiltration or various non specific lesions. The occurrence of these lesions may be the presenting feature of the disease (MDS) or may herald its progression to acute leukemia. Recognition and early diagnosis have therapeutic and prognostic significance.- - - - - - - - - - ranking = 1keywords = progression (Clic here for more details about this article) |
2/11. Smoldering acute myelogenous leukemia in the elderly.Out of 75 consecutive elderly AML patients who did not receive anti-leukemic treatment (52 pts) or failed to respond to differentiating agent (23 pts), 6 patients had survivals of 13.2 to 98 months with treatment restricted to supportive care. This cut-point is far longer than the median survival of the 235 elderly patients (3.5 mo.), either untreated (med. survival: 1 mo.) or treated (with treatment ranging from conventional induction to palliative chemotherapy) (4 mo.), admitted to our department within the same period of time. These cases of smoldering AML (4 women, 2 men) were all of AML2 FAB subtype (4 de novo, 2 post MDS) and presented with a significantly better performance status, lower WBC and circulating blast counts, higher platelet counts and with lower bone marrow infiltration than AML cases with more rapid progression. Cytogenetical analysis when available (3 pts) showed normal karyotypes and clonogenic assay performed in 3 of these patients showed a lack of (2 pts) or reduced in vitro leukemic cell growth (1 pt). The identification of specific characteristics of smoldering leukemia in the elderly might be an important development in the understanding of the physiopathology of acute leukemia and a tool for helping decision-making when selecting the time and intensity of cytotoxic treatment in these older patients.- - - - - - - - - - ranking = 1keywords = progression (Clic here for more details about this article) |
3/11. De novo appearance of t(7;13)(q10;q33) in the leukemic phase of myelodysplastic syndrome: a case report.Clonal cytogenetic abnormalities are found in about 50% of all patients with myelodysplastic syndrome (MDS) and the clinical implication of these abnormalities is now well documented. However, the de novo appearance of balanced translocations in MDS patients during the progression of the disease is rarely reported and the significance of the balanced translocation remain to be elucidated. We report here the first case of refractory anemia with excess blasts in transformation (RAEBt), in which a new chromosomal translocation, t(7;13)(q10;q33) appeared de novo in the AML phase. It has been revealed that rearrangements and deletions of chromosome 7, i.e. der(1;7)(q10;p10), are very complex and that multiple regions may contribute to the disease phenotype and progression. Our case suggests that the chromosomal region at 7q10, rather than 1p10, might be one of the hot spots for myeloid proliferative disorders, including MDS.- - - - - - - - - - ranking = 2keywords = progression (Clic here for more details about this article) |
4/11. Reversible acceleration of disease progression following cyclosporin A treatment in a patient with myelodysplastic syndrome.A 38-year-old Japanese man with myelodysplastic syndrome (MDS), whose bone marrow smears demonstrated hypercellularity, was treated with oral cyclosporin A (CsA) therapy. During the course of this therapy, the numbers of peripheral blood and bone marrow blasts increased and the level of serum lactate dehydrogenase increased. After discontinuation of CsA treatment, all of these levels rapidly decreased. We consider that CsA might accelerate disease progression in certain MDS cases.- - - - - - - - - - ranking = 508.96592404466keywords = disease progression, progression (Clic here for more details about this article) |
5/11. Lymphnode localization of extramedullary myeloid cell tumor in myelodysplastic syndrome: report of one case diagnosed by fine-needle cytology.Trilineage extramedullary myeloid tumor (EMT) is an uncommon medical condition mostly diagnosed in patients affected by acute or chronic myeloid leukemia or, more rarely, by a myelodysplastic syndrome, among which the most frequent is refractory anemia with excess of blasts in transformation (RAEB-t). The prognostic significance of EMT is still unclear, although the appearance of trilineage EMT is often considered to affect the outcome adversely. A 70-year-old lady with previous history of intestinal resection for colonic adenocarcinoma in 1995 and subsequently treated with 5-fuorouracyl developed a refractory anemia with excess of blasts (RAEB) in 1998. During the follow-up, a progression to RAEB-t was recorded. During chemotherapy for this condition, slight enlargement of left supraclavicular and right submandibular nodes was noticed. Fine-needle biopsy was performed with ancillary studies. A diagnosis of trilineage extramedullary myeloid tumor was reached. The patient was treated with low doses of chemotherapy with a good response lasting 12 months. The peculiar cytologic picture of this condition when corroborated by ancillary studies (immunocytochemistry and flow cytometry) is diagnostic of this rare condition. Furthermore, the extramedullary myeloid tumor in this case did not significantly affect the response to the chemotherapy of RAEB-t.- - - - - - - - - - ranking = 1keywords = progression (Clic here for more details about this article) |
6/11. Clonal karyotype evolution involving ring chromosome 1 with myelodysplastic syndrome subtype RAEB-t progressing into acute leukemia.Karyotypic evolution is a well-known phenomenon in patients with malignant hematological disorders during disease progression. We describe a 50-year-old male patient who had originally presented with pancytopenia in October 1992. The diagnosis of a myelodysplastic syndrome (MDS) FAB subtype RAEB-t was established in April 1993 by histological bone marrow (BM) examination, and therapy with low-dose cytosine arabinoside was initiated. In a phase of partial hematological remission, cytogenetic assessment in August 1993 revealed a ring chromosome 1 in 13 of 21 metaphases beside BM cells with normal karyotypes [46,XY,r(1)(p35q31)/46,XY]. One month later, the patient progressed to an acute myeloid leukemia (AML), subtype M4 with 40% BM blasts and cytogenetic examination showed clonal evolution by the appearance of additional numerical aberrations in addition to the ring chromosome [46,XY,r(1), 8,-21/45,XY,r(1), 8,-21,-22/46, XY]. Intensive chemotherapy and radiotherapy was applied to induce remission in preparation for allogeneic bone marrow transplantation (BMT) from the patient's HLA-compatible son. After BMT, complete remission was clinically, hematologically and cytogenetically (normal male karyotype) confirmed. A complete hematopoietic chimerism was demonstrated. A relapse in January 1997 was successfully treated using donor lymphocyte infusion and donor peripheral blood stem cells (PB-SC) in combination with GM-CSF as immunostimulating agent in April 1997, and the patient's clinical condition remained stable as of January 2005. This is an interesting case of a patient with AML secondary to MDS. With the ring chromosome 1 we also describe a rare cytogenetic abnormality that predicted the poor prognosis of the patient, but the patient could be cured by adoptive immunotherapy and the application of donor's PB-SC. This case confirms the value of cytogenetic analysis in characterizing the malignant clone in hematological neoplasias, the importance of controlling the quality of an induced remission and of the detection of a progress of the disease.- - - - - - - - - - ranking = 101.79318480893keywords = disease progression, progression (Clic here for more details about this article) |
7/11. Deletion (8)(q22) as the only chromosomal abnormality in a patient with RAEB-t with progression to acute myelocytic leukemia.Cytogenetic investigation of the bone marrow cells of an 88-year-old woman with refractory anemia with an excess of blasts in transformation with progression to acute myelocytic leukemia (AML), FAB classification M4, revealed a deleted chromosome #8 with the breakpoint at band q22 as the sole abnormality. This breakpoint is the same as that in t(8;21)(q22;q22), mostly found in patients with AML. This finding is discussed in relation to the possible oncogenesis of AML, which in this case may mean that the deletion of chromosome #8 at band 8q22 and the resultant loss of genetic material with possible antioncogenic activity is the critical event leading to malignant transformation in AML and not the translocation of the end of 21q next to 8q.- - - - - - - - - - ranking = 5keywords = progression (Clic here for more details about this article) |
8/11. Lineage-unrestricted hematologic response to granulocyte colony-stimulating factor in a patient with refractory anemia with excess blasts.We report a patient with refractory anemia with excess blasts who showed a lineage-unrestricted hematologic response to granulocyte colony-stimulating factor (G-CSF). After 17 months of a stable disease state, the patient developed pneumonia, progression of cytopenia, and reduced cellularity and blast mass in the bone marrow. He was given G-CSF to overcome the pneumonia. Not only the neutrophil count, but also the platelet count increased soon after initiation of the G-CSF therapy; both counts became normal on the fifth day of the G-CSF therapy. Additionally, the anemia improved gradually. The neutrophil and platelet counts were maintained in the normal range for 3 months after cessation of the G-CSF. in vitro studies showed that G-CSF alone stimulated megakaryocyte colony formation from bone marrow mononuclear cells (BMMNC), and accessory cells in the BMMNC were necessary for expression of this G-CSF-induced in vitro megakaryocytopoiesis. These results suggest that, in coordination with accessory cells, G-CSF stimulated megakaryocytopoiesis in the patient. This case provides valuable information for understanding the mechanisms of a lineage-unrestricted hematologic response to G-CSF, which is very rarely observed in MDS.- - - - - - - - - - ranking = 1keywords = progression (Clic here for more details about this article) |
9/11. Childhood myelodysplastic syndrome with clonal evolution progressing to acute megakaryoblastic leukemia (ANLL-M7).We treated a 16-month-old girl with myelodysplastic syndrome (MDS; refractory anemia with excess of blasts subtype, RAEB by FAB classification) that developed into acute megakaryoblastic leukemia (ANLL-M7). The blast cells were positive for CD41 shown by flow cytometry and for platelet peroxidase by electron microscopy. Cytogenetically, five kinds of abnormal karyotypes were apparent at the initial visit and karyotypic progression (clonal evolution) was also evident. These karyotypes were considered to be derived from the putative original clone, 48,XX, 6, 21. The observed karyotypes were considered 50,XX, 4,add(4)(q31), 6,add(7)(p22),add(10)(q24),add(12)(q11), 20, 21, mar[karyotype A];48,XX,add(4)(q31), 6,add(10)(q24),add(12)(q11), 21 [karyotype B];48,XX, 6,t(6;13)(p23;q14), 21 [karyotype C];51,XX, X, t(6;13)(p23;q14), der(6)t(6;13)(p23;q14), 21, 21, mar [karyotype D]; and 49,XX, X, -3,t(6;13)(p23;q14), der(6)t(6;13)(p23;q14), -12, 21, 21, mar [karyotype E]. It seems karyotypes B and C were derived from the putative clone; karyotype B developed into karyotype A; and karyotype C developed into karyotype E through karyotype D. After development of ANLL-M7, the cytogenetic study showed a karyotype with further karyotypic progression. The patient was treated with high-dose cytosine arabinoside (HD AraC) followed by allogeneic bone marrow transplantation. Despite intensive care, she died 3 months after the transplantation.- - - - - - - - - - ranking = 2keywords = progression (Clic here for more details about this article) |
10/11. Acute leukemias expressing p210-and p 190-type bcr/abl mRNAs: report of two cases and review of the literature.We report two patients with acute leukemias who expressed two types of bcr/abl mRNA. The first case was an 8-year-old boy with acute mixed leukemia in whom the Ph1 chromosome and p210/p190 types of bcr/abl mRNAs were detected at diagnosis. The second case was a 39-year-old male with acute nonlymphocytic leukemia transformed from myelodysplastic syndrome (refractory anemia with excess of blasts). In the latter case, the p210-type mRNA appeared after leukemic transformation, and the p190-type transcript was detected only during the late stage when the Ph1 chromosome was first observed. The leukemias in these two patients were aggressive in their clinical courses. We conclude that the dual expression of p210 and p190 types of bcr/abl is a factor indicating a poor prognosis, and that, in some patients, p190-type bcr/abl may contribute to disease progression.- - - - - - - - - - ranking = 101.79318480893keywords = disease progression, progression (Clic here for more details about this article) |
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