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Rise in cutaneous lymphoma calls for registry: causes unknown.

SEATTLE -- Some pediatric dermatologists believe they are seeing greatly increased numbers of children with cutaneous T-cell lymphomas, but exactly why remains a mystery.

Prompted by those reports, physicians at the annual meeting of the Society for Pediatric Dermatology called for the creation of a new registry to track the potential trend and identify possible causes

There may be a lot to be learned from a registry: Those at the meeting who reported the cutaneous T-cell lymphom; (CTCL) cases are not entirely clear whether something is going on that is increasing incidence or if some physicians have simply become better at diagnosing it. It is not known how CTCL in children typically progresses, nor is it known if treatment alters the natural history of the disease.

What is known is that some of these physicians are seeing many more cases where they rarely saw CTCL before: in children.

"Everybody recognizes that there is a big increase, and we really have no idea what to do," said Dr. Bernice Krafchik, who is head of the section of dermatology at the Hospital for Sick Children, Toronto, and who proposed the registry.

Dr. Krafchik said that she has seen 15 cases in the past 2 years. In her entire career prior to that, she had only one case. She called the situation "scary."

"We really do not know how to treat" these children, she said.

Dr. Neil Prose said he has begun taking note of patients because this is something he rarely saw before. He has 10 patients with what he believes may be CTCL.

Specifically intending to open a Pandora's box, Dr. Prose presented one of his pediatric cases at the meeting to find out if others were also seeing more cases and to learn if anyone could tell him anything about the general progression of CTCL in children. He was unaware that the registry was going to be proposed, said Dr. Prose of Duke University Medical Center, Durham, N.C.

His patient was a 12-year-old African American male who for 2 years had had progressively enlarging areas of macular hypopigmentation. Prior to coming to Dr. Prose, the patient had been treated with topical triamcinolone cream 0.1% and then topical tacrolimus. Neither was effective.

When Dr. Prose first saw the patient, he had three discrete areas of hypopigmentation: a large one on the right leg, a smaller one on the same leg, and one on the fight arm. Histology of a biopsy from the arm lesion revealed a lymphoid cell infiltrate in the papillary dermis with linear areas of lymphocytes at the dermal-epidermal junction. No cytologic atypia of the lymphoid cells was noted. Flow cytometry and staining revealed that the cells were CD3 positive, 50% of the dermal lymphocytes were CD4 and CD8 positive, and all the epidermal lymphocytes were CD8 positive.

Based on those findings, Dr. Prose diagnosed mycosis fungoides.

When he asked if anyone had any information on what kind of a prognosis he could expect for his patient, one audience member rose up to mention that he had a patient who progressed to systemic involvement and eventual death. Another audience member said that she believed she was seeing a number of cases that were spontaneously resolving.

Also presented at the meeting was the case of a 5-year-old boy referred to Children's Memorial Hospital in Chicago. Two months earlier, he had developed a diffuse erythematous eruption covered with yellowish scale on his back, posterior legs, buttocks, and groin, with some areas of sparing. Although the palms were also involved, there was only minimal involvement of the soles of the feet.

The boy's initial physicians diagnosed pemphigus foliaceus, but topical and oral corticosteroid treatment was of no avail, said Dr. Jon Dyer of the hospital.

Dr. Dyer said the patient had some areas of deeper erythema and scale that eventually became nodular and finally developed eschars.

A biopsy showed that the patient had a diffuse, upper dermal and epidermal infiltration of moderate-sized lymphoid cells. Further testing revealed that 60% of the isolated T cells were of one immunotype: dim staining for CD3, strongly staining for CD8, and positive for CD 16/56. Genetically, the cells were found to be clonal.

The patient was begun on narrow-band UVB treatment, with the idea that if the response were less than optimal, the physicians would consider adding bexarotene or some other oral retinoid, Dr. Dyer said. So far, however. all of the vatient's lesions have cleared and there is no systemic involvement.

During the discussion of the patient's treatment and prognosis, Dr. Dyer said that he was told by the assisting hematologist/oncologist that the patient probably would need to continue periodic UVB therapy for the rest of his life. Others noted that, in adults, CDS-positive T-cell lymphomas tend to have a poor outcome and that in Europe some physicians have begun to use PUVA in CTCL on the rationale that it might have deeper activity than UVB.

Meeting attendees were told that if they were interested in the proposed registry, they should contact Dr. Elena Pope of the Hospital for Sick Children at elena.pope@sickkids.ca.
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Title Annotation:Children's Health
Author:Kirn, Timothy F.
Publication:Family Practice News
Geographic Code:1USA
Date:Aug 15, 2003
Words:854
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