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Biomedical subjects

H S Zackheim

Publications and source records attributed to H S Zackheim.

At least 55 records · Page 3Linked to original sources
PubMed1989

Combined total body X-ray irradiation and total skin electron beam radiotherapy with an improved technique for mycosis fungoides.

Twelve consecutive patients with advanced stage mycosis fungoides (MF) were treated with combined total body X ray irradiation (TBI) and total skin electron beam radiotherapy (EBRT). Six had generalized plaque disease and dermatopathic nodes, three had tumor stage disease and node biopsy positive for mycosis fungoides, and three had erythroderma/Sezary syndrome. The treatment regimen consisted of split course total body X ray irradiation, given in twice weekly 15 cGy fractions to 75 cGy, then total skin electron beam radiation therapy given in once weekly 400 cGy fractions to a total dose of 2400 cGy. Underdosed areas and areas of greatest initial involvement were boosted 400 cGy twice weekly for an additional 1200 cGy. This was followed by a second course of total body X ray irradiation, to a total dose of 150 cGy. The total skin electron beam radiotherapy technique is a modification of an established six position EBRT technique for mycosis fungoides. Measurements to characterize the beam with and without a lexan scattering plate, demonstrated that the combination of no-plate beams produced better dose uniformity with a much higher dose rate. This improved technique is particularly advantageous for elderly and/or frail patients. Nine (75%) of the 12 patients achieved complete response (CR). The other three had significant improvement with greater than 80% clearing of their disease and resolution of symptoms. All six patients with generalized plaque disease achieved complete response and remained free of disease from 2 to 16 months. Two of three node positive patients also achieved complete response; one, with massive biopsy-documented mycosis fungoides nodal disease and deep open tumors, remained relapse-free over 2 years. Only one of the three patients with erythroderma/Sezary syndrome achieved a complete response, which was short lived. Therapy was well tolerated. No significant hematological toxicity occurred. Although total body X ray irradiation and total skin electron beam radiotherapy produced excellent palliation of patients with advanced stage mycosis fungoides, new strategies to provide more effective systemic treatment are needed.

Adult↗
PubMed1989

Low-dose methotrexate for the Sézary syndrome.

Seventeen patients with Sézary syndrome were treated with low-dose methotrexate for periods of up to 5 years (median follow-up 22 months). Seven patients (41%) achieved a complete response, and six patients (35%) achieved a partial response (more than 50% improvement). As of their latest evaluation, 10 of 17 (59%) were in a state of either complete or partial response. The estimated 5-year survival rate was 71% (standard error 13%). Toxicity was minor and self-limiting in only one patient. Clinical improvement was accompanied by a decrease in the circulating Sézary cell count.

Adolescent↗
PubMed1988

Canine epidermotropic cutaneous lymphoma.

The histologic features of an epidermotropic cutaneous lymphoma in a dog are presented. The neoplasm, which was intensely epidermotropic, thoroughly invaded the follicular and apocrine gland epithelium. The intense epidermotropism was retained in the tumor stage. By comparison to human mycosis fungoides, the cell nucleus was less convoluted and the infiltrate was less polymorphic. The lymphoid cell appeared to undergo neoplastic transformation into a cell with a large pale nucleus, often with a prominent nucleolus and variable cytoplasm. In situ marker studies are needed to clarify the cell type in this neoplasm. These studies could have relevance for the pathogenesis of human epidermotropic lymphoma.

Animals↗
PubMed1988

Granulomatous variants of cutaneous T-cell lymphoma. The histopathology of granulomatous mycosis fungoides and granulomatous slack skin.

Granulomatous mycosis fungoides is an unusual histologic variant of mycosis fungoides, a condition that is ordinarily indolent. Granulomatous slack skin, like granulomatous mycosis fungoides, shows epidermotropism, granulomatous inflammation, a clonal T-helper cell population, and progression to systemic lymphoma in some cases. Unlike granulomatous mycosis fungoides, it is characterized clinically by bulky, pendulous skin folds. The similarities between the two conditions prompted us to compare the histologic features. We reviewed 24 biopsies from 10 patients with granulomatous mycosis fungoides. These showed several distinct histologic patterns, including three cases that mimicked granuloma annulare. We also reviewed biopsy specimens from four patients with granulomatous slack skin. These specimens had a more stereotypic appearance, with permeation of the entire dermis and subcutis by lymphocytes, marked epidermotropism, and a more even distribution of granulomas and giant cells within the infiltrate. Biopsies of fully developed lesions of granulomatous slack skin showed elastolysis involving the full thickness of the dermis--a feature not seen in any of our granulomatous mycosis fungoides cases. Biopsy specimens from granulomatous mycosis fungoides and granulomatous slack skin may be mistaken for nonneoplastic granulomatous dermatitides, but they can usually be distinguished from these by the presence of epidermotropism or atypical lymphocytes. Because several of our patients with granulomatous mycosis fungoides died after courses of unremarkable length, it seems unlikely that the presence of granulomas is invariably correlated with a more benign course than nongranulomatous mycosis fungoides.

Adolescent↗
PubMed1987

Mycosis fungoides beginning in childhood and adolescence.

Mycosis fungoides is a form of cutaneous T cell lymphoma usually occurring in mid to late adulthood. We report 12 patients with mycosis fungoides whose eruption began before age 20 years, including one patient with histologic documentation at 5 years of age. The onset of mycosis fungoides during the first two decades may be more common than is generally recognized and should be included in the differential diagnosis of chronic dermatoses in children and adolescents.

Adolescent↗
PubMed1985

Phaeohyphomycotic cyst of the skin caused by Exophiala jeanselmei.

A dermosubcutaneous nodule presenting with a superficial papular vascular component occurred in a man receiving long-term prednisone therapy. The histologic findings were characteristic of a phaeohyphomycotic cyst, and a black mold, Exophiala jeanselmei, was isolated. Despite treatment with ketoconazole and local heat, the lesion persisted and was excised. The fungus could be seen in the excised tissue, but cultures were sterile. Subcutaneous phaeohyphomycosis is a relatively uncommon, but recently more frequently recognized type of opportunistic fungus infection.

Cysts↗
PubMed1985

Lymphomatoid papulosis associated with plaque-stage and granulomatous mycosis fungoides.

A 47-year-old black man had typical papulonodular lesions of lymphomatoid papulosis (LyP) with concurrent plaque-stage mycosis fungoides (MF). Both diagnoses were confirmed histologically. This supports the concept that LyP is part of the spectrum of cutaneous T-cell lymphoproliferative disorders. The patient also had a large nodule and a deeply infiltrated plaque, each of which exhibited a deep granulomatous reaction. These were interpreted as representing granulomatous MF. Clinically, there was an evolution from a predominance of LyP lesions to a predominance of MF plaques. Topical carmustine therapy resulted in a substantial decrease in the number and size of both LyP and MF lesions. Both lesion types involuted with hypopigmentation.

Administration, Topical↗
PubMed1985

Topical carmustine therapy for lymphomatoid papulosis.

Seven patients with lymphomatoid papulosis were treated with solutions of topical carmustine, a nitrosourea compound. Recently used schedules have employed 10 mg of carmustine in dilute alcohol applied to the total skin surface daily for four to 17 weeks (total dosage, 280 to 1,180 mg). All patients experienced a rapid reduction in the number and size of lesions. Maintenance therapy consisted of local applications of carmustine (2 to 4 mg/mL of 95% ethanol) to individual new papules. This method was effective in suppressing disease activity and reduced by half the average life cycle of individual lesions. However, long-term lesion-free remissions were not seen. Bone marrow depression did not occur.

Administration, Topical↗
PubMed1984

Circulating Sézary cells. A new preparatory method for their identification and enumeration.

Although Sézary cells are not specific for the Sézary syndrome, the enumeration of these circulating abnormal lymphoid cells is important in the evaluation of patients with mycosis fungoides, Sézary syndrome, and benign dermatoses that might be confused with these entities. Various techniques have been developed for this purpose, but none of the techniques have been widely accepted as practical and accurate. We describe a new method of preparing blood specimens for the identification and enumeration of circulating Sézary cells. Peripheral blood was separated on a density gradient and the mononuclear cell fraction was embedded in plastic. Semithin sections were cut, stained for alpha-naphthyl acetate esterase, and examined by light microscopy. We found that the percentage of Sézary cells correlated well with the clinical diagnosis of Sézary syndrome in the subjects who were studied.

Adult↗
PubMed1983

Topical carmustine (BCNU) for mycosis fungoides and related disorders: a 10-year experience.

A 10-year experience in eighty-six patients confirms the effectiveness of topical carmustine (BCNU) in mycosis fungoides (MF). Complete remission (CR) was achieved in 84% of those with less than 10% involvement (stage IA), median CR, 12 months, and in 52% with greater than 10% involvement (stage IB), median CR, 23 months. The probability of freedom from relapse for 1 year was 72% in stage IA and 37% in stage IB. No deaths in stages IA or IB were attributable to MF. Including all causes of death, the probability of 5-year survival for stage IA was 93% and for stage IB, 48%. Good results were obtained with only local BCNU in fourteen patients with mostly less than 5% involvement. Five of seven with poikilodermatous MF, two with parapsoriasis en plaques (PEP), and three with lymphomatoid papulosis did well. Persistent local therapy cleared deeply infiltrated lesions in some patients. With present schedules, the hazard of bone marrow depression is slight. Erythematous reactions and telangiectasia are troublesome but have not been accompanied by premalignant changes.

Administration, Topical↗