Comparison of various criteria in predicting treatment response and prognosis of patients with myelodysplastic syndrome treated with azacitidine.

This study was performed to identify whether cytogenetics, International Prognostic Scoring System (IPSS), or World Health Organization Classification-Based Prognostic Scoring System are predictive of the efficacy of azacitidine in patients with myelodysplastic syndrome (MDS). We retrospectively reviewed the clinical records of 113 patients with MDS treated with azacitidine. The "response alternating disease natural history," "cytogenetic response," and "hematologic improvement" were assessed by serial bone marrow biopsy, cytogenetic study, and hemogram analyses. The complete and partial remission rates were 17.6% and 3.9% in 51 evaluable patients. There were no significant differences in response rate in the different cytogenetic/IPSS/WPSS groups. The overall hematologic response (HR) rate was 49.6%, and the HR rate was significantly greater in patients classed as "very high" risk according to the WPSS compared with other patient groups. The 1-year overall survival (OS) rate was higher among patients with HR compared with those without HR (80.9% vs 63.3%, p = 0.046), and the 1-year OS rate among patients classed as being at high risk by each criteria was similar to that of patients classed as being at low risk. The hazard ratio of death among patients with HR compared with those without HR was 0.17 (95% CI 0.04-0.69) for high + very high risk group based on WPSS. Patients in the WPSS high-risk group had an increased HR rate compared with other patient groups, and the achievement of HR was associated with a significant increase in OS. Azacitidine showed similar efficacy in all patient groups, even in patients with poor cytogenetics and in high-risk groups.

Multiple intracranial tuberculomas mimicking granulocytic sarcomas in acute myeloid leukemia.

The diagnosis of incracranial tuberculoma in immune-compromised hosts is often difficult because conventional magnetic resonance (MR) imaging of tuberculoma reveals various findings and neurologic symptoms are not typical. Here, we report a case of a 54-yr old man with multiple intracranial tuberculoma who was treated for acute myeloid leukemia. He complained of right-side paresthesia after the third consolidation chemotherapy without leukemic relapse and fever. MR imaging of the brain showed multiple ring-enhanced lesions in the cerebrum, cerebellar hemisphere, and pons. The lesions appeared to mimic a metastatic tumor or abscess. Cerebrospinal fluid analysis showed no abnormal cells, but the level of adenosine deaminase was elevated (28.8 IU/L, normal 0-8). Stereotactic brain biopsy was performed, but only reactive gliosis was observed. To confirm diagnosis, an open brain biopsy was performed. The histopathology demonstrated chronic granulomatous inflammation with caseous necrosis. Tuberculous-polymerase chain reaction of the biopsy showed a positive result. He was treated with anti-tuberculosis medication and a high dose of steroid. Paresthesia improved, and follow-up brain MR imaging showed the decreased size and numbers of ring-enhanced lesions and improvement of perilesional edema 1 month after treatment. Here, we report on an interesting case of intracranial tuberculoma in acute myeloid leukemia.

Dermatomyositis associated with cancer of unknown primary site.

Dermatomyositis (DM) is an uncommon inflammatory myopathy with characteristic rash accompanying, or more often preceding, muscle weakness. There is a well-recognized association between DM and several cancers, such as ovarian cancer, breast cancer, melanoma, colon cancer, and non-Hodgkin lymphoma. We report the first case of cancer of unknown primary site associated with DM. A 62-yr-old woman presented to us with both shoulder painful swelling and facial edema. She was diagnosed previously as cancer of unknown primary site, histologically confirmed with squamous cell carcinoma in a pelvic mass. For the following days, she complained of erythematous face followed by progressive weakness of the proximal muscles of upper and lower limbs. The laboratory tests showed an increased muscle enzyme and acute phase reactants. The electromyogram showed the typical findings of DM. After the treatment with high dose steroid and methotrexate, the proximal motor weakness improved, and she received palliative radiation therapy.

Pilot remission induction therapy with idarubicin, plus an intensified dose of ara-C and priming with granulocyte colony-stimulating factor for acute myeloid leukemia.

BACKGROUND: The sensitization of leukemic cells with hematopoietic growth factors can enhance the cytotoxicity of chemotherapy in acute myeloid leukemia (AML). Intensified remission induction (RI) therapy can also improve the treatment results for AML. Therefore, the current trial attempted to evaluate the efficacy and toxicity of granulocyte colony-stimulating factor (G-CSF) priming and a dose intensification of Ara-C in RI chemotherapy for AML. METHODS: A total of 29 patients with newly diagnosed AML received G-CSF-priming RI chemotherapy consisting of idarubicin (12 mg/m2, days 1-3), G-CSF (150 microg/m2, days 3-8) and Ara-C (500 mg/m2, b.i.d., days 4-8), and the outcomes were compared with those of a historical group treated with a standard regimen consisting of idarubicin (12 mg/m2, days 1-3) and Ara-C (100 mg/m2, days 1-7). RESULTS: There was no difference in sex, age, subtype and cytogenetic risk between the two groups. The complete remission rate and treatment-related mortality were 72 and 17% for the G-CSF-primed group (p = 0.89) and 71 and 10% for the historical group (p = 0.32), respectively. The time to neutrophil recovery (25 vs. 24 days, p = 0.17) and platelet recovery (24 vs. 23 days, p = 0.23) did not differ significantly between the two groups. Similarly, the duration of fever was not significantly different (5 vs. 7 days, p = 0.58). Thirteen patients (45%) experienced fever and 5 patients (17%) manifested skin rashes during the G-CSF priming. After a median follow-up of 336 days, the 1-year overall survival, disease-free survival and event-free survival rates were 72 vs. 63% (p = 0.83), 74 vs. 56% (p = 0.059) and 53 vs. 38% (p = 0.32), respectively. CONCLUSION: The sensitization of leukemic cells with growth factors and dose intensification seem to be clinically applicable means to enhance the efficacy of RI chemotherapy only in selected patients with AML, thereby warranting further studies focusing on specific subgroups of AML patients.