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Bilateral orbital granulocytic sarcoma (chloroma) preceding the blast phase of acute myelogenous leukemia: CT findings.

Granulocytic sarcoma (chloroma) is an uncommon malignant neoplasm associated with myelogenous leukemias. Its appearance may precede the clinical manifestations of the acute leukemic phase by months to years. We report the CT findings of a case of bilateral intraorbital granulocytic sarcoma which preceded the blast phase of acute myelogenous leukemia (AML) and led to its diagnosis. An awareness of granulocytic sarcoma and its CT appearance may expedite the diagnosis of AML or prompt close monitoring of those cases of granulocytic sarcoma antedating the appearance of AML.

Blast Crisis↗

Oral idarubicin in the treatment of acute myelogenous leukaemia and the blast phase of chronic myeloid leukaemia.

Fourteen patients with poor-risk acute myelogenous leukaemia (AML) and five patients with accelerated phase/blast crisis chronic myeloid leukaemia (CML) were treated with 3 days of oral idarubicin (25 mg/m2/day). No complete remissions or return to chronic phase CML were observed. A fall in the peripheral blast count was seen in all patients with the first cycle of treatment, and with subsequent cycles in CML patients, but all responses were transient, with eventual reemergence of peripheral blasts. In some patients, there was a clear cut improvement in symptoms such as bone and splenic pain. Five of the AML patients and all of the CML patients were treated as out-patients. In this group of patients oral idarubicin was found to be a useful drug for palliative treatment.

Administration, Oral↗

Cytogenetically distinct leukemic cell lines displaying in vitro specific proliferative and differentiation capacities may account for early disease relapse in the blast phase of CML.

The cytogenetic features and the proliferative and differentiation capabilities of blast cell fractions purified on a density gradient were studied in one patient with chronic myeloid leukemia (CML) in blast crisis, both at the emergence and at relapse of the disease. The results show that relapse was due to the appearance of a new leukemic cell line that was characterized by peculiar chromosomal, growth, and differentiation features, which seemingly accounted for early refractoriness to therapy and disease progression.

Adult↗

Interaction of cyclosporin A and etoposide. Clinical and in vitro assessment in blast phase of chronic myeloid leukaemia.

Combination chemotherapy has had a low impact on survival of blast crises in chronic myelogeneous leukaemia (CML) which may be due to drug resistance. This work attempted to correlate the clinical response and some experimental evidence for the MDR phenotype. Blast cells were positive for P-glycoprotein using APAAP assay. In vitro tests showed that etoposide was partially toxic to blast cells when used alone but had its toxicity increased by nearly sixfold when combined with cyclosporin A (CSA). The patient responded poorly to treatment with etoposide combined with mitoxantrone and high-dose ara-c. However, when etoposide was associated with CSA, this patient returned to the chronic phase reinforcing our in vitro studies. Because no serious toxicity was seen clinically, we are inclined to consider the circumvention protocol an useful strategy to treat blast crises of CML.

Adult↗

Intensive remission induction therapy for chronic myeloid leukemia in blast phase with a goal of post-remission bone marrow transplant--a pilot study.

An intensive protocol utilizing mitoxantrone, high-dose cytarabine, vincristine, etoposide and methylprednisolone as induction therapy for chronic myeloid leukemia in blast transformation is described. Fourteen patients were treated, with a remission/second chronic phase achieved in 64%. None of the 3 patients older than 50 yr responded. Complete hematological responses were seen in 9 of the 11 younger patients, 4 of whom also became BCR-ABL negative by Southern Blot analysis. Four patients went on to allogeneic bone marrow transplant. Median remission durations were 4.5 (1-5) and 8.5 (5-16) months in the non-transplanted and transplanted cohorts, respectively. Median survival is 1.5 (0.5-3), 9.5 (7-14) and 17 (14-61+) months in the non-responding, responding non-transplanted and transplanted cohorts, respectively. Toxicity, particularly gastrointestinal, was significant. This represents an aggressive protocol that should be reserved for patients who are potential transplant candidates.

Adolescent↗

In vivo differentiation of blast-phase chronic granulocytic leukemia. Expression of c-myc and c-abl protooncogenes.

A patient with chronic granulocytic leukemia in acute blastic transformation was treated with mithramycin, an RNA synthesis inhibitor, after in vitro exposure of her leukemic cells to mithramycin showed differentiation to normal appearing granulocytes. Mithramycin therapy in vivo resulted in a prompt and dramatic hematologic response. Before therapy, the patient's leukemic cells had high levels of transcription of the cellular myc and abl protooncogenes. After initiation of therapy, protooncogene mRNA decreased rapidly. These observations indicate that mithramycin can induce differentiation both in vitro and in vivo and suggest that such changes may be associated with altered oncogene expression.

Adolescent↗