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1/107. Congenital erythroleukemia in a neonate with severe hypoxic ischemic encephalopathy.

    We report a case of a neonate who presented with hypoxic ischemic encephalopathy, persistent hypoglycemia and hypotension, intractable metabolic acidosis, renal failure and a coagulopathy but who, at autopsy, was found to have massive infiltration of nonhematopoietic tissues with blasts. The diagnosis of congenital erythroleukemia was confirmed by the detection of glycophorin A, a major erythrocyte membrane protein, on the surface of the blasts. The clinical presentation and course of the case described here have not previously been reported for this extremely rare condition.
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ranking = 1
keywords = leukemia
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2/107. Derivative (1;7)(q10;p10) in a patient with de novo acute erythroblastic leukemia (AML-M6).

    A rare association of der(1;7)(q10;p10) with de novo acute erythroblastic leukemia (AML-M6) in a 63-year-old male is reported. While this unbalanced 1;7 translocation, der(1;7), has been reported often in therapy-related myelodysplastic syndrome (t-MDS) or therapy-related acute myeloid leukemia (t-AML), its associations with de novo AML-FAB-M6 have rarely been reported. Although der(1;7) has been reported as a cytogenetic factor for poor prognosis in t-MDS/AML, our patient showed a good response to chemotherapy and obtained complete remission, although longer observation is required to evaluate the prognosis.
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ranking = 1.2
keywords = leukemia
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3/107. Acute erythroid leukemia after cyclophosphamide therapy for multiple myeloma: report of two cases.

    The diagnosis of multiple myeloma was made in two white men, aged 55 and 59 years. They were treated with cyclophosphamide for 98 and 44 months respectively. Patient 1 also received a nine-month course of combined therapy with melphalan, procarbazine, and prednisone. Both developed acute erythroid leukemia, 98 and 71 months after the original diagnosis of myeloma, and died of subarachnoid hemorrhage and cardiac arrest. Patient 1 developed squamous cell carcinoma of the skin with recurrence, and Patient 2 developed anaplastic carcinoma of the urinary bladder. Palliative radiation therapy was given. The development of erythroid leukemia plus carcinoma in these two men suggests mutagenic change secondary to cyclophosphamide therapy.
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ranking = 1.2
keywords = leukemia
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4/107. Erythroleukemia-like syndrome due to busulfan toxicity in polycythemia vera.

    Over a 19-year period, a patient with polycythemia vera who had undergone a splenectomy received six courses of busulfan for recurrent thrombocytosis. The total dose of busulfan given for the sixth course was greater than that used for the previous ones. Severe pancytopenia followed, which persisted for 4 months. During this period there was marked erythroid hyperplasia in the bone marrow with striking dyserythropoiesis; PAS-positive red cell precursors, as well as moderate numbers of circulating normoblasts and evidence of chronic and acute hemolysis, were present. All of these findings reverted to normal without therapy, and the polycythemic state eventually recurred. These events are interpreted as an unusual marrow reaction following busulfan overdosage rather than a transient erythroleukemia.
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ranking = 1.0020101775685
keywords = leukemia, precursor
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5/107. 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.
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ranking = 0.8
keywords = leukemia
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6/107. Sweet's syndrome and pneumocystis carinii pneumonia: two sequelae of low-dose cytosine arabinoside therapy in a patient with acute myeloid leukemia.

    cytosine arabinoside in low dose is sometimes employed for treating acute myeloid leukaemia. We report here a case of acute myeloid leukemia, treated with low-dose cytosine arabinoside, who developed acute febrile neutrophilic dermatosis and pneumocystis carinii pneumonia after attainment of remission. A direct effect of cytosine arabinoside on neutrophil function and an immunosuppressive potential in lower doses could be speculated.
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ranking = 1
keywords = leukemia
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7/107. Sialyl-Tn- and neuron-specific enolase-positive minimally differentiated erythroleukemia.

    Flow cytometric, immunochemical and molecular studies were performed on leukemic blasts from a patient with minimally differentiated erythroleukemia (AML-M6v). The blasts expressed CD36 and CD71 but not lymphoid antigens, myeloid antigens, CD41 or glycophorin A. Analysis of carbohydrate antigens showed that the blasts expressed the sialyl-Tn antigen. immunochemistry revealed that the blasts had neuron-specific enolase (NSE). serum sialyl-Tn and NSE levels were markedly increased. Finally, an erythroid lineage was confirmed in the presence of alpha-globin messages in the blasts. Sialyl-Tn and NSE expression in leukemic blasts may be useful to identify AML-M6v.
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keywords = leukemia
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8/107. Myelodysplastic syndrome progresses rapidly into erythroleukemia associated with synchronous double cancers of the stomach and the papilla of Vater.

    patients with myelodysplastic syndrome (MDS) show a relatively high incidence of developing cancers. However, it is extremely rare that synchronous double cancers develop in an MDS patient. We report a case of MDS that progressed rapidly into erythroleukemia (M6 by French-American-British classification) complicated by gastric cancer and carcinoma of the papilla of Vater. A 66-year-old man was admitted because of pancytopenia with peripheral blasts. A diagnosis of MDS (with refractory anemia with excess of blasts in transformation [RAEB-T]) was made by bone marrow examination. Chromosome analysis revealed 46,XY. An early gastric cancer was also diagnosed by endoscopic examination. The peripheral blasts gradually proliferated and the disease progressed to M6. A chromosome abnormality 46,XY,del(1)(q42) was detected at the leukemic transformation. A CAG (low-dose cytarabine and aclarubicin in combination with granulocyte colony-stimulating factor) regimen was started as a remission-induction therapy. However, obstructive jaundice developed and a marked dilatation of bile ducts was observed by abdominal computed tomography (CT). A carcinoma of the papilla of Vater was detected by endoscopy. As remission was achieved and the pancytopenia improved, the patient subsequently underwent a surgical jejuno-choledochostomy to manage the jaundice. However, the leukemia relapsed thereafter and additional chromosome abnormalities including der(5)t(5;10)(p15:q11) were observed.
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ranking = 1.2
keywords = leukemia
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9/107. Constitutive activation of Stat1 and Stat3 in primary erythroleukemia cells.

    Signal transducers and activators of transcription (Stat) proteins play important roles in the regulation of hematopoiesis as downstream molecules of cytokine signal transduction. Previously, we demonstrated that Stat1 and Stat3 are activated by erythropoietin (EPO) in a human EPO-dependent erythroleukemia cell line UT-7/EPO. We report here that Stat1 and Stat3 are constitutively activated in freshly isolated erythroleukemia cells. In addition, EPO promoted cell growth of these cells, accompanied by enhanced activities of Stat1 and Stat3. Furthermore, mutation in the Statl/Stat3-binding sites of the c-myc gene promoter clearly blocked its promoter activity in EPO-stimulated primary erythroleukemia cells. Thus, Stat1 and Stat3 may support cell growth in part via c-myc gene activation in primary erythroleukemia cells.
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ranking = 1.6
keywords = leukemia
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10/107. Isolated pentasomy of chromosome 8 in erythroleukemia.

    Pentasomy 8 as a sole anomaly in hematological disorders is rare. Only 2 such cases, one in acute monocytic leukemia and one in chronic myelomonocytic leukemia have been described in the literature to date. Here, we report the first case of a 42 year old man with erythroleukemia displaying a pentasomy 8 clone. Conventional cytogenetics of bone marrow cells showed 16 metaphases with pentasomy 8 and 9 with normal diploidy. fluorescence in situ hybridization (FISH) analysis using a whole chromosome painting probe and a centromeric probe specific for chromosome 8 confirmed the presence of pentasomy 8 and also revealed a low percentage of a trisomic and a tetrasomic clone. The patient died three days after diagnosis without chemotherapy. The findings suggest that pentasomy 8 is associated with a heterogeneous group of myeloid disorders and probably plays a specific role in the progression of myeloid neoplasia.
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ranking = 1.4
keywords = leukemia
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