Myelodysplastic syndrome at a large tertiary care community hospital: analysis according to the international prognostic scoring system.

The outcome of patients diagnosed myelodysplastic syndromes (MDS) between 1990 and 1997 from William Beaumont Hospital (WBH) was analyzed according to the International Prognostic Scoring System (IPSS) risk categorization. A retrospective study of 195 MDS patients wa s performed. Seventy-nine patients with MDS, in whom a karyotype was obtained and with an adequate follow-up were included in the final analysis. Cases of proliferative CMML (WBC > 12x10(9)/l) were excluded from the study. The overall median survival was 3.1 years, and median survival stratified by IPSS was 3.4, 4.1 and 0.5 years for the INT-1, INT-2 and high risk group and not yet reached for the low risk group. The overall survival by IPSS subcategorization were 6.88, 5.29, 5.30 and 2.12 years for the low, INT-1, INT-2, and high risk groups respectively. Cytogenetics were significant in predicting the overall survival. The IPSS score stratified patients into risk categories for development of AML. The risk of development into AML was 8, 8, 33 and 54% for the low, INT-1, INT-2 and high risk groups, respectively. We conclude that IPSS score can be useful in predicting survival and AML evolution in some MDS patients.

Letterer-Siwe disease in adults.

Histiocytosis X (HX) is a rare disorder of histiocytic proliferation characterized by a broad spectrum of clinicopathologic disease. An unusual case of Letterer-Siwe disease (LSD) or subacute disseminated HX in a 71-year-old woman is presented. The patient had a 3-year history of splenomegaly before skin lesions developed. She presented to our clinic at 1.5 years later and the diagnosis of HX was made by skin biopsy. Topical nitrogen mustard (NM) therapy resulted in complete clearing of cutaneous lesions. Her condition was stable over the next 10 months. However, she subsequently suffered a rapid and fatal dissemination of her disease. Systemic treatment with prednisone, vinblastine sulphate, and suppressin A (SA) (a calf thymus derived hormone preparation that specifically induces suppressor T-cells) was ineffective. Characteristic histopathologic, immunohistochemical, and electron microscopic findings of HX are illustrated. A review of the adult cases of LSD and treatment options for HX are presented and discussed.

Comparison of three treatment strategies for esophageal cancer within a single institution.

Fifty-seven patients with esophageal cancer were treated with curative intent between January 1979 and June 1985. Seventeen were treated with radical radiation therapy alone (TD 4000-6500 cGy in 200-250 cGy fractions). Twenty-five were treated using radiation therapy (3000 cGy in 200 cGy fractions, day 1-19, and 2600-3000 cGy in 200 cGy fractions, day 50-68) and concomitant chemotherapy (5-FU and Cis-platinum). Fifteen were treated preoperatively by radiation therapy (3000 cGy at 200 cGy fractions) and concomitant chemotherapy (5-FU and Cis-platinum) followed by esophagectomy in 2-3 weeks. Chi square tests showed no significant baseline differences between the patients in the three different treatment groups with respect to A.J.C. stage, T status, location of tumor or histology. Median survival and 2-year survival for the three treatment groups were RT alone: 5 months and 0%, RT and chemotherapy: 12 months and 37%, RT, chemotherapy and surgery 13 months and 38%. A Cox multivariate analysis revealed significant predictor variables for increased survival were treatment strategy, RT dose delivered and T status. Increased local control was seen with either multimodality approach compared to radiation therapy alone. Our data suggests that a multimodality approach is superior as a curative treatment strategy, compared to RT alone, in esophageal cancer. In our series no significant differences were seen with respect to treatment outcome between the two multimodality approaches used.