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The manifestations, diagnosis and management of the rarer chronic myeloid leukemias are reviewed, with special attention to problems that affect elderly patients. The spectrum of disorders includes atypical myeloproliferative syndrome, so-called Ph-negative CGL, chronic myelomonocytic leukemia, and leukemias characterized by chronic proliferation of neutrophil, eosinophil, or basophil leukocytes. These latter are sometimes difficult to differentiate from chronic nonleukemic proliferations of the index cells. Termination in an acute myeloid leukaemia that is usually refractory to treatment may occur in any of the above disorders but is not a constant event.

(2001). Management of the Chronic Leukemias: Special Considerations in the Elderly Patient. Part I. Chronic Lymphocytic Leukemias. Hematology: Vol. 6, No. 5, pp. 291-314.

Cutaneous cryptococcosis usually is associated with concurrent systemic infection and actually may develop before clinical manifestations of cryptococcal meningitis become apparent. It is rare for a cryptococcal infection to be localized only to the skin. A case of cutaneous cryptococcosis is described in an immunocompromised patient who initially had a rash and a positive serum cryptococcal antigen titer, but no central nervous system involvement. The papular pustular skin lesions disappeared after 8 weeks of therapy with amphotericin B, which was stopped secondary to progressive azotemia. Less than 2 months after therapy, the skin lesions recurred, again without evidence of systemic disease. Treatment with oral fluconazole resulted in a gradual resolution of the cutaneous lesions. The pathogenesis of cryptococcosis is discussed, with emphasis on the management of cutaneous cryptococcosis.

Background Adenoid cystic carcinoma is an uncommon tumor of the salivary gland. Little has been published on the chemotherapy of this neoplasm. Methods The literature on this disease is reviewed, and data from seven unpublished cases are presented. Results Four patients received cyclophosphamide. One responded, and another had pain relief. The literature review did not identify any single drug or combination that might be regarded as the treatment of choice. Conclusions Systematic, multi-institutional studies are required to determine appropriate systemic treatment for metastatic adenoid cystic carcinoma of salivary gland origin.

The Eastern Cooperative Oncology Group conducted a study of pentostatin (2'-deoxycoformycin) in 37 patients with hairy-cell leukemia. Among the 27 patients who met all the study's entry criteria, the response rate was 96 percent, with 16 patients (59 percent) entering complete remission and 10 patients (37 percent) partial remission. In one patient no response was observed. These results were not significantly changed by the inclusion of nine additional patients who were found retrospectively not to have fulfilled the entry criteria. When complete remission was attained, maintenance therapy was not given. Despite this, no patient has had a relapse, and the duration of complete remission ranges from 1 to 375 days. Pentostatin appears to be equally effective in untreated patients and in those who have progressive disease after splenectomy or after both splenectomy and treatment with interferon. Whether pentostatin is superior to splenectomy or interferon as therapy for hairy-cell leukemia will have to be assessed by direct comparison in randomized studies. Lengthy follow-up will be required to determine a median duration for the responses of hairy-cell leukemia to pentostatin.

Nine patients with hormone-resistant prostate carcinoma were treated with subcutaneous injection of recombinant human interferon-alpha 2 (rHuIFN-alpha 2), 5-10 X 10(6) U/m2, three times a week. One patient had a mixed clinical response with reduction of bone pain. The study was closed due to intolerable grade III and IV toxicities including weight loss (8/9), fatigue/malaise (7/9), central nervous system toxicity (4/9), leukopenia (3/9), and uncontrollable nausea and vomiting (2/9). These toxicities resulted in deterioration of performance status. It is concluded that rHuIFN-alpha 2, at least at the dose and schedule studied, should not be used for the treatment of patients with hormone-resistant prostate carcinoma.

The case is presented of a 2-year-old girl with mental retardation, talipes equinovarus, and patent ductus arteriosus, with metaphases of cultured peripheral blood lymphocytes possessing 49 chromosomes. The karyotype has 3 extra chromosomes in the group 6–12+X, and buccal smears show a percentage of cells with 4 chromatin bodies. The patient is therefore regarded as possessing five X chromosomes. Factors affecting the incidence of nuclear chromatin bodies in buccal cells and of drumsticks in polymorphonuclear leukocytes in individuals with multiple X chromosomes are discussed. The findings in this case are compared with those in XXXXY males and in the one XXXXX female previously described.