To examine the efficacy of intensified maintenance chemotherapy, we conducted a prospective multicenter trial in adult patients with newly diagnosed acute promyelocytic leukemia treated with alltrans retinoic acid and chemotherapy. Of the 302 registered, 283 patients were assessable and 267 (94%) achieved complete remission. Predicted 6-year overall survival in all assessable patients and disease-free survival in patients who achieved complete remission were 83.9% and 68.5%, respectively. A total of 175 patients negative for PML-RAR␣ at the end of consolidation were randomly assigned to receive either intensified maintenance chemotherapy (n ؍ 89) or observation (n ؍ 86). Predicted 6-year disease-free survival was 79.8% for the observation group and 63.1% for the chemotherapy group, showing no statistically significant difference between the 2 groups (P ؍ .20). Predicted 6-year survival of patients assigned to the observation was 98.8%, which was significantly higher than 86.2% in those allocated to the intensified maintenance (P ؍ .014). These results indicate that the intensified maintenance chemotherapy did not improve disease-free survival, but rather conferred a significantly poorer chance of survival in acute promyelocytic leukemia patients who have become negative for the PML-RAR␣ fusion transcript after 3 courses of intensive consolidation therapy. (Blood. 2007;110:59-66)
A new megakaryoblastic cell line (CMK), which also exhibits erythroid and myeloid markers, was established from a Down's syndrome patient suffering from acute megakaryoblastic leukaemia. The CMK cells were found to be positive in reactions with anti-platelet antibodies (anti-glycoproteins IIb/IIIa and Ib, and Plt-1). Platelet peroxidase (PPO) reactivity was found to be associated with the nuclear envelope and the endoplasmic reticulum but not with the Golgi apparatus. Some cells possessed cytoplasmic granules with the characteristics of alpha-granules and demarcation membranes. Karyotyping revealed near-tetraploidy (modal chromosome number of 95; ranging 87-98) and a translocation der(17)t(11;17), also found in the original leukaemic cells, confirming that the cells were derived from the patient's malignant blasts. The CMK cells were also found to be positive in reaction with anti-glycophorin A antibody, as well as with anti-myeloid antibodies (MY4, MY7 and MY9). Treatment of CMK cells with phorbol ester 12-O-tetradecanoylphorbol-13-acetate (TPA) greatly enhanced the reactivity with anti-platelet antibodies, increased the number of cells in which cytoplasm was dissociated into numerous segments and suppressed the reactivity with anti-glycophorin A. The proliferation of CMK cells was stimulated by interleukin-3 (IL-3) and granulocyte-macrophage colony stimulation factor (GM-CSF). This cell line should be a useful tool for analysing the basis of the afferent association between megakaryoblastic leukaemia and Down's syndrome, as well as for further study of megakaryocytic differentiation.
These results indicate that fatal hemorrhage represents a major obstacle in curing APL, and that patients with such high-risk features may benefit from more aggressive supportive care.
Summary:Case reportA 40-year-old man was diagnosed with AML in January We report an unusual case of a patient who was cured of one autoimmune disease (palmoplantar pustular pso-1992. Bone marrow cytology revealed M6 by the FAB classification with trilineage myelodysplasia. The patient riasis (PPP)) but developed another autoimmune disease (autoimmune thyroiditis) after allogeneic BMT. A 40-went into complete remission following induction chemotherapy with daunorubicin, 6-mercaptopurine, and behenoyl year-old man suffering from AML with PPP underwent allogeneic BMT from his HLA-identical sister for the cytosine arabinoside. After receiving three courses of consolidation chemotherapy, he developed skin lesions on the treatment of AML. The patient experienced complete clearance of the cutaneous PPP despite the cessation of palms and soles ( Figure 1). The skin lesions were diagnosed as PPP by histological examination in February 1993. immunosuppressive therapy for over 2 years. However, he developed hyperthyroidism with anti-thyroglobulin They were treated with combinations of topical corticosteroids and etretinate, but showed only partial response. antibodies 5 months after BMT, although he had showed normal thyroid functions without anti-thyrogloIn November 1993, after myeloablation with BU (16 mg/kg) and CY (120 mg/kg), the patient received an bulin antibodies before BMT. The donor had no history of thyroid diseases and showed normal thyroid funcallogeneic BMT from his HLA-identical younger sister, who did not suffer from PPP. The mixed lymphocyte reactions but was positive for anti-thyroglobulin antibodies. Thus, even when the donor is in a subclinical state, autotion was negative. Although the patient's elder sister had died of hyperthyroidism, his younger sister had no history immune thyroiditis may be transferred from donors to recipients by BMT.of thyroid diseases and showed normal thyroid functions, but was positive for anti-thyroglobulin antibodies. Keywords: autoimmune thyroiditis; palmoplantar pustular psoriasis; bone marrow transplantation; autoimmunity CYA and MTX were used for GVHD prophylaxis. The hematological follow-up showed stable engraftment with complete hematopoietic recovery and sustained complete chimerism. There were no signs of acute GVHD. After his It is well known that the hematopoietic and immune system of recipients is eventually replaced by donor-derived cells after allogeneic BMT. Experimental work in animal models has demonstrated that BMT can be used to treat systemic and organ-specific autoimmune diseases. 1 In humans, the resolution of immune-mediated diseases after BMT has recently been reported. [2][3][4] On the other hand, autoimmune diseases have also been transferred from donors to recipients in both mice 5 and humans 6-8 by BMT. We report the unusual case of a patient who was cured of palmoplantar pustular psoriasis (PPP) but developed autoimmune thyroiditis after allogeneic BMT. This is the first case report in which a pre-existing immune-mediated disease was cured while ...
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