Cases reported "Leukocytosis"

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1/9. Monocytic leukemia cutis diagnosed simultaneously with refractory anemia with monocytosis: a case report.

    A case of leukemia cutis (LC) of monocytic lineage in a patient with myelodysplastic syndrome (MDS) is presented. Cutaneous infiltrates were recognized concurrent with diagnosis of refractory anemia (RA) with monocytosis. skin infiltrates subsequently spontaneously regressed although MDS progressed with increasing monocytosis, anemia, and thrombocytopenia. death occurred 6 months after diagnosis with evolution of acute monoblastic leukemia complicated by sepsis. This case supports previous observations of poor prognosis associated with leukemia cutis. LC associated with MDS is reviewed including the role of monocytes.
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ranking = 1
keywords = myelodysplastic syndrome, myelodysplastic
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2/9. Cytogenetic and molecular studies of the philadelphia translocation in myelodysplastic syndromes. Report of two cases and review of the literature.

    We report two patients with a myelodysplastic syndrome and the philadelphia (Ph) chromosome. The first patient was a 73-year-old man who was diagnosed as having a chronic myelomonocytic leukemia in combination with features suggestive of a myeloproliferative syndrome. Chromosomal analysis showed a normal karyotype in the majority of cells, mixed with metaphases containing a standard Ph translocation, t(9;22)(q34;q11), as well as a translocation between chromosome 4 and 6: t(4;6)(p15;p12). Southern blot analysis showed breakpoint cluster region rearrangement as observed in classic chronic myeloid leukemia. The second patient was a 63-year-old man with a myelodysplastic syndrome, type refractory anemia. Cytogenetic study of bone marrow cells at the time of diagnosis revealed a normal karyotype: 46,XY. The initial myelodysplastic syndrome evolved to a myeloproliferative phase with progressive leukocytosis and thrombocytosis. During the terminal phase the Ph chromosome was discovered in 100% of the examined cells. We discuss the correlation between MDS and myeloproliferative diseases, the de novo acquisition of the Ph chromosome during the course of a myelodysplastic syndrome, and review the literature.
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ranking = 8
keywords = myelodysplastic syndrome, myelodysplastic
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3/9. Development of acute megakaryoblastic leukemia from a minor clone in a down syndrome patient with clinically overt transient myeloproliferative disorder.

    A down syndrome male showed leukocytosis from birth and was diagnosed as transient myeloproliferative disorder (TMD). Eight months later, his condition had progressed to myelodysplastic syndrome after spontaneous resolution, and it then evolved to acute megakaryoblastic leukemia (AMKL) at the age of 20 months. Sequencing analysis showed that the predominant TMD and AMKL clones had different GATA1 mutations, although a minor TMD clone identical to the AMKL clone was present at birth. These observations suggest that a minor clone rather than the predominant clone at the time of TMD may give rise to AMKL later on.
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ranking = 1
keywords = myelodysplastic syndrome, myelodysplastic
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4/9. Sweet's syndrome associated with sideroblastic anaemia.

    Previous reports have noted an association between Sweet's syndrome (acute febrile neutrophilic dermatosis) and leukaemia, and less commonly other haematological abnormalities. We report a previously unrecognised association between Sweet's syndrome (SS) and sideroblastic anaemia (myelodysplastic syndrome--refractory anaemia with ring sideroblasts). Both patients were males and one had prominent extracutaneous features of SS. Bone marrow cytogenetic studies were normal in this latter patient and neither patient showed progression to leukaemia.
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ranking = 1
keywords = myelodysplastic syndrome, myelodysplastic
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5/9. Phase II trial of fenretinide [N-(4-hydroxyphenyl) retinamide] in myelodysplasia: possible retinoid-induced disease acceleration.

    To determine the activity of fenretinide in patients with myelodysplastic syndromes, 15 patients were treated (300 mg/d starting dose, escalated to 400 mg/d) for a 12-week course. No responses were observed in 14 evaluable patients. Exacerbation of thrombocytopenia occurred in one patient with chronic myelomonocytic leukemia, who succumbed to an intracerebral hemorrhage after 3 weeks of treatment. Two patients with long-standing stable sideroblastic anemia experienced interval leukemic progression. In one patient, clinical features of chronic myelomonocytic leukemia appeared, characterized by a striking rise in peripheral monocyte count (0.49 x 10(9)/l to 10.8 x 10(9)/l) and hepatosplenomegaly, which resolved promptly after cessation of treatment. The second patient experienced evolution into acute myelomonocytic leukemia with cytogenetic progression. The drug was well tolerated with no patient having to discontinue treatment because of toxicity. We conclude that fenretinide lacks clinical efficacy in the treatment of myelodysplasia and in some patients may enhance leukemic progression.
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ranking = 1
keywords = myelodysplastic syndrome, myelodysplastic
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6/9. Red cell hypoplasia, thrombocytosis, and leucocytosis: myelodysplastic and proliferative syndrome.

    Three patients with chronic red cell aplasia also showed thrombocytosis or granulocytosis, or both. All had morphological evidence of myelodysplasia on examination of bone marrow aspirate but none had a detectable chromosomal abnormality. These patients seem to provide evidence of a separate entity within the spectrum of myelodysplastic and myeloproliferative disease.
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ranking = 1.7647322448984
keywords = myelodysplastic
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7/9. Neutrophilic dermatosis and myelodysplastic syndrome.

    Acute febrile neutrophilic dermatosis (AFND) is sometimes associated with leukemia. We present a patient with myelodysplastic syndrome who subsequently developed AFND, and we briefly review the association of AFND and myeloproliferative disorders.
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ranking = 5
keywords = myelodysplastic syndrome, myelodysplastic
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8/9. Refractory anemia with an excess of blasts developed into overt leukemia with leukothrombocytosis.

    A 72-year-old man with refractory anemia with an excess of blasts developed overt leukemia with leukothrombocytosis. Hematological and physical findings closely resembled those of an accelerated or blastic phase of chronic myelocytic leukemia. The cytogenetic anomaly of i(17q) was observed during the course. The present case is suggestive of the diversities of myelodysplastic syndromes (MDS), including relationships between MDS and myeloproliferative disorders (MPD) and acute leukemia.
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ranking = 1
keywords = myelodysplastic syndrome, myelodysplastic
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9/9. Hematological abnormalities and cholestatic liver disease in two patients with mevalonate kinase deficiency.

    We describe two patients with mevalonate kinase deficiency and prominent hematologic abnormalities and cholestatic liver disease. Patient R.B. was not anemic at birth, but developed petechiae and cutaneous extramedullary hematopoiesis, hepatosplenomegaly, leukocytosis, and recurrent febrile events without positive bacterial or viral cultures. Patient N.M. manifested minor anomalies, hepatosplenomegaly, anemia, thrombocytopenia, recurrent febrile crises, and facial rashes. Mevalonic aciduria was found by urinary organic acid analysis, and mevalonate kinase deficiency was documented in both. The clinical spectrum of normocytic hypoplastic anemia, leukocytosis, thrombocytopenia, and abnormal blood cell forms led to diagnoses of congenital infection, myelodysplastic syndromes, or chronic leukemia in these patients before recognition of mevalonate kinase deficiency. mevalonate kinase deficiency represents a single-gene abnormality that may be associated with significant hematologic findings. Recognition of the variability of this disorder with some patients manifesting only mild neurologic findings, yet significant hepatosplenomegaly, normocytic anemia, thrombocytopenia, and leukocytosis is important for all specialists who need to be aware of this organic aciduria.
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ranking = 1
keywords = myelodysplastic syndrome, myelodysplastic
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