March 10, 2009 (San Francisco, California) — A retrospective analysis of patients with mycosis fungoides (MF), the most common form of cutaneous T-cell lymphoma, has revealed patterns of disease onset and progression that upset the current way of looking at the disease; it is not just a disease of older white men.
Grace Sun, PhD, conducted the study at the University of Texas MD Anderson Cancer Center, in Houston, and presented the findings during an oral poster session here at the American Academy of Dermatology 67th Annual Meeting.
Cutaneous T-cell lymphoma is most commonly found in whites in their 60s, with men outnumbering women 2:1. About 1500 cases a year are diagnosed nationwide, although that is likely to be a significant underdiagnosis.
A spate of aggressive cases in younger African American women raised the question of whether this was a "fluke" or something more, Dr. Sun said.
The researchers identified 1074 patients with MF who were diagnosed by or referred to the center between 1989 and 2007. Data were stratified by disease onset before or after age 40, disease state, sex, and race/ethnicity.
Surprisingly, early-onset MF occurred more frequently in African Americans (30/92; 32.6%) and Hispanics (31/87; 35.6%) than in whites (103/809; 12.7%). The results were strongly significant in African Americans (P = .0008) and Hispanics (P = .0002).
Women drove the differences in early age of onset, with statistically significant differences between African American (21/60, 35.0) and Hispanic (19/40; 47.5) women, and white (50/350, 14.3%) women. The differences among men trended in the same direction but did not reach statistical significance.
Dr. Sun found that Hispanics and whites were similarly likely to initially present with MF at stage 1, but "African Americans seemed to present at stage 4."
"Rapid progression, from a stable mild disease to stage 4 within 1 year at any time during their disease course," occurred in 1 white and 8 African American women under the age of 40. Six of the African American women died of their disease.
"The only 2 who survived had allogeneic bone marrow transplantation," 1 of them 6 years ago, Dr. Sun said.
The presentation of disease in patients under the age of 40 is very different from what is seen in the "traditional" cutaneous T-cell lymphoma patient, Dr. Sun pointed out. The early-stage patient is more likely to be a person of color, and the disease is significantly more likely to take an aggressive course in younger African American women.
These patients "need more aggressive therapy, including going to allogeneic transplantation earlier than we would think in other patients. This [disease] is not indolent in African American women," she said.
During a discussion, Dr. Sun noted that the African American women in the study "were very deficient in vitamin D." Researchers at MD Anderson are exploring what might affect vulnerability to the disease, as well as possible modulation of disease progression.
Isaac Brownell, MD, told Medscape Dermatology that cutaneous T-cell lymphomas "are notoriously difficult to diagnose" because, in most instances, it appears as an eczematous eruption and they will be treated with topical steroids, which results in at least temporary improvement. Dr. Brownell is from the Department of Medicine, Dermatology Service, at Memorial Sloan-Kettering Cancer Center, in New York City. He was not involved in the study.
A patient will have this type of recurrent eruption for many years before a biopsy is taken. But the histopathology of this disease is also "very difficult to diagnose under the microscope." He said that, in early disease, "the pathologist may notice some atypical lymphocytes, but it is not to a level where I can definitely call this [cutaneous T-cell lymphoma]."
Dr. Brownell pointed to a study from the 1970s indicating that typically it takes more than 4 years from the first visit to the correct diagnosis of the disease. That has not changed significantly.
One clue for clinicians in making the diagnosis is to look for distribution of symptoms; "the classic presentation is a bathing-trunk distribution, eczema confined to the pelvic girdle area," he offered.
"A loss of hair within the patch of eczema is also suggestive. I'm more aggressive in biopsying patients who present that way," said Dr. Brownell.
Early-stage cutaneous T-cell lymphoma has a very good prognosis; the percentage of people who progress is very low. "So missing that diagnosis isn't that critical" with most patients. However, he acknowledged that this new information from Houston is likely to make him more aggressive in following younger African American women who present with possible symptoms.
The study was funded by the National Institutes of Health. Dr. Sun and Dr. Brownell have disclosed no relevant financial relationships.
American Academy of Dermatology (AAD) 67th Annual Meeting: Poster P607. Presented March 9, 2009.
Medscape Medical News © 2009 Medscape
Cite this: Bob Roehr. AAD 2009: Younger African American Women at Higher Risk for T-Cell Lymphoma Progression - Medscape - Mar 10, 2009.
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