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1/58. 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.
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ranking = 1
keywords = myelodysplastic syndrome, myelodysplastic
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2/58. Renal and adrenal gland localization of chronic myelomonocytic leukemia presenting as a kidney tumor.

    Chronic myelomonocytic leukemia (CMML) is a myelodysplastic syndrome (MDS) characterized by prominent monocytosis and an increase in bone marrow monocyte precursors in addition to dyshaematopoietic features (1). Extrahaematological manifestations including cutaneous, neurologic, and rheumatic symptoms have been recorded in association with CMML. Here, we report the first observation of renal, adrenal and perirenal involvement in CMML which presented as a kidney tumor.
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ranking = 1
keywords = myelodysplastic syndrome, myelodysplastic
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3/58. A group of previously not recognized cytogenetic abnormalities in myeloid hematological malignancies.

    We have identified a group of previously not reported chromosome abnormalities related to myeloid hematological malignancies. Cases 1 and 2 were observed to have an additional i(4)(p10) as the sole anomaly with similar clinical features of myeloid disorders; that is, acute nonlymphocytic leukemia (ANLL-M2) and myelodysplastic syndrome (MDS)-refractory anemia with an excess of blasts in transformation, respectively. fluorescence in situ hybridization studies with the use of a 4p-specific microdissection probe further confirmed the presence of an i(4)(p10) in these patients. Case 3 was diagnosed with ANLL-M1 and had an additional i(8)(p10) as the only change, also confirmed by a whole-chromosome painting procedure. In cases 4-6, deletions of 18q at breakpoints q12, q23, and q21 were identified as the sole anomaly in a myeloproliferative disorder (MPD), MPD, and MDS, respectively. X-autosome translocations other than t(X;10)(p11;p11) and t(X;11)(q13;q23) have not been reported as recurrent or primary changes in hematological disorders. In the present study, a t(X;9)(q26;q22) and t(X;5)(q13;q33) as the sole anomaly were found in cases 7 and 8, respectively. Both cases had the same diagnosis of MDS. Considering that trisomies 4 ( 4) and 8 ( 8) are common anomalies in MDS and ANLL, our findings strongly indicate that amplification of genes on 4p and 8p, but not on 4q and 8q, may play a crucial role in the pathogenesis of MDS and ANLL. In addition, genes on 18q12-23 and on Xq13-26 may be involved in the pathogenesis of myeloid disorders.
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ranking = 1
keywords = myelodysplastic syndrome, myelodysplastic
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4/58. Chronic myelomonocytic leukemia with t(7;11)(p15;p15) and NUP98/HOXA9 fusion.

    Translocation (7;11)(p15;p15) is a recently characterized chromosomal abnormality that results in fusion of the NUP98 gene on 11p15 and the HOXA9 gene on 7p15. It shows a strong racial predisposition, being found predominantly in Oriental patients, and has been reported almost exclusively in acute myeloid leukemia, often with associated myelodysplastic changes. In this report, we describe the unique occurrence of t(7;11)(p15;p15) and NUP98/HOXA9 fusion in a patient with chronic myelomonocytic leukemia, and suggest that the genetic lesion may involve multipotential myeloid stem cells.
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ranking = 0.33628111597713
keywords = myelodysplastic
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5/58. Chronic myelomonocytic leukemia with t(1;3)(p36;q21) and a synchronous gastric cancer.

    The translocation t(1;3)(p36;q21) has been reported previously in patients with the myelodysplastic syndrome and with acute nonlymphocytic leukemia. It has been reported in only 5 cases of chronic myelomonocytic leukemia and t(1;3)(p36;q21). We observed a case of chronic myelomonocytic leukemia with t(1;3)(p36;q21) complicated by a gastric cancer at the time of diagnosis.
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ranking = 1
keywords = myelodysplastic syndrome, myelodysplastic
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6/58. systemic vasculitis with bilateral perirenal haemorrhage in chronic myelomonocytic leukaemia.

    The cases of two patients with chronic myelomonocytic leukaemia associated with periarteritis nodosa-like, antineutrophil cytoplasmic antibody negative, systemic vasculitis, are reported. A 61 year old man was admitted with fever, diffuse myalgia, and abdominal pain. blood and bone marrow examination showed chronic myelomonocytic leukaemia. Vasculitis of the gall bladder was responsible for acalculous cholecystitis. A massive spontaneous bilateral perirenal haemorrhage occurred. A 73 year old woman with chronic myelomonocytic leukaemia had been followed up for one year when unexplained fever occurred. Two months after the onset of fever, sudden abdominal pain was ascribed to spontaneous bilateral renal haematoma related to bilateral renal arterial aneurysms. Neuromuscular biopsy showed non-necrotising periarteriolar inflammation. To our knowledge, systemic vasculitis has never been reported in chronic myelomonocytic leukaemia. In our two cases a non-random association is suggested because (a) chronic myelomonocytic leukaemia is a rare myelodysplastic syndrome, (b) spontaneous bilateral perirenal haematoma is not a usual feature of periarteritis nodosa.
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ranking = 1
keywords = myelodysplastic syndrome, myelodysplastic
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7/58. Chronic myelomonocytic leukemia developed 2 years after the onset of immune thrombocytopenic purpura like syndrome.

    An 80-year old man was diagnosed as having immune thrombocytopenic purpura based on epistaxis, purpura and by the platelet count 8 x 10(9)/l. prednisolone and gamma globulin were administered and the platelet count had been kept around 50 x 10(9)/l during his follow up. Two years from the onset of immune thrombocytopenic purpura he was admitted because of leukocytosis (79 x 10(9)/l with 79% monocytes), anemia and thrombocytopenia. Hypercellular bone marrow with dysplasia of three lineages was observed. In the bone marrow cytogenic analysis, a -6, clonal cytogenic abnormality was observed. 45XY, der(6), t(6;6)(q16;q23). He was diagnosed as having chronic myelomonocytic leukemia. This is a difficult case in which it was diagnosed as refractory thrombocytopenia as a subgroup of myelodysplastic syndrome, rather than immune thrombocytopenic purpura. which might have preceded the development of chronic myelomonocytic leukemia.
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ranking = 1
keywords = myelodysplastic syndrome, myelodysplastic
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8/58. Concurrent pernicious anemia and myelodysplastic syndrome.

    Megaloblastic anemia (MA) due to vitamin B12 deficiency is a reversible form of ineffective hematopoiesis. Myelodysplastic syndrome (MDS) is an acquired, irreversible disorder of ineffective hematopoiesis, characterized by stem cell dysfunction as a consequence of dna damage manifested in part by karyotype anomalies. Importantly, MA and MDS are generally considered mutually exclusive diagnoses. We report the case of a 73-year-old woman with a profound macrocytic anemia, monocytosis and neurologic symptoms. Low cobalamin levels and the presence of anti-intrinsic-factor antibodies definitively established a diagnosis of pernicious anemia. Replacement therapy resulted in resolution of neurologic findings and macrocytosis; however, the anemia and monocytosis persisted. Bone marrow biopsy revealed trilineage myelodysplasia, which together with the peripheral monocytosis suggested a diagnosis of chronic myelomonocytic leukemia. karyotype analysis revealed a clone with 45, XX, der(1;7)(q10;p10)-7 [20]. Eighteen months after documented vitamin B12 replenishment her MDS transformed to terminal acute myeloid leukemia with the same clonal abnormality. Reversible cytogenetic abnormalities have been observed with MA, occasionally including karyotypes typically associated with MDS or myeloid leukemias. These abnormalities, like the anemia, resolve with vitamin replacement. This case suggests that MA and MDS can occur simultaneously; clinicians should be aware that this phenomenon occurs. Whether acquired karyotype abnormalities from the MA were related to the MDS and subsequent myeloid leukemia in this woman is a speculative but intriguing consideration that is discussed.
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ranking = 4
keywords = myelodysplastic syndrome, myelodysplastic
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9/58. skin involvement in chronic myelomonocytic leukaemia as a predictor of transformation into acute myeloid leukaemia.

    We report on the case of a patient with myelodysplastic syndrome (MDS) who presented with leukaemia cutis preceding development of acute myeloid leukaemia. Leukaemic infiltration of the skin should be considered an early manifestation of leukaemic transformation and an indicator of poor prognosis in MDS.
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ranking = 1
keywords = myelodysplastic syndrome, myelodysplastic
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10/58. monosomy 15 in chronic myelomonocytic leukemia. description of a case and review of the literature.

    We report a 89-year-old female diagnosed with chronic myelomonocytic leukemia (CMMoL) presenting with a monosomy 15. To our knowledge, this is the second reported case of CMMoL with monosomy 15. On the other hand, monosomy 15 in complex karyotypes is a frequent chromosome aberration in myelodysplastic syndromes, particularly in refractory anemia with excess of blasts.
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ranking = 1
keywords = myelodysplastic syndrome, myelodysplastic
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