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《BAD原發(fā)性皮膚T細胞淋巴瘤的治療指南》內容預覽

This article is restricted to the management of primaryCTCL, and specifically of mycosis fungoides and Se′zarysyndrome. Two sections devoted to primary cutaneousCD30+ lymphoproliferative disorders and rare CTCLvariants are found towards the end of the article. It isrecommended that all patients, possibly with the excep-tion of those with early stages of mycosis fungoides (IA)or with lymphomatoid papulosis, should be reviewed bya multidisciplinary team (MDT) which should include adermatologist, a clinical or medical (haemato)oncolo-gist, and a dermatopathologist or pathologist withconsiderable experience of the diagnosis and manage-ment of primary CTCL. In addition, a central review of allpathology would be desirable, consistent with currentrecommendations from the Royal College of Pathologistsfor specialized pathology services. Subsequent manage-ment should ideally be shared between the cancer centreand the local referring physician in a cancer unit. TheMDT should ideally be supported by an accreditedlaboratory for immunophenotypic and molecular diag-nostic studies in lymphoma.

All patients should have adequate diagnostic biop-sies for histology, immunophenotypic and preferablymolecular studies. This is advised even for stage IAdisease as studies have shown that patients with adetectable T-cell clone have a shorter duration ofresponse and higher rate of failure to respond. Thesefindings should be interpreted with the clinicalfeatures in order to make a specific diagnosis basedon the WHO classification for primary CTCL. This iscritical because treatment and prognosis can varywidely depending on the diagnostic category. Occa-sionally, multiple skin biopsies are required to make adiagnosis and often the opinion of other dermatopa-thologists experienced in cutaneous lymphoma isrequired. The patient should be examined fully andany bulky palpable pe**heral nodes should be biop-sied, preferably by excision rather than by core or fineneedle biopsy. Staging computed tomographic (CT)scans of the chest, abdomen and pelvis are indicatedin all those patients with non-mycosis fungoides CTCLvariants or with stage IIA/B ? III ? IV mycosis fungoides,but not in those with stage IA ? IB disease or lymph-omatoid papulosis. Bone marrow aspirate or trephinebiopsies are indicated in all patients with CTCLvariants (except lymphomatoid papulosis), and shouldbe considered in stage IIB ? III ? IV mycosis fungoidesand also in patients with pe**heral blood involve-ment (as indicated by the presence of Se′zary cellcounts representing > 5% of the total lymphocytecount). Pe**heral blood samples should be taken forroutine haematology, biochemistry, serum lactatedehydrogenase (LDH), Se′zary cells, lymphocyte sub-sets, CD4 ? CD8 ratio, human T-cell lymphotropic virus(HTLV)-I serology and T-cell receptor (TCR) geneanalysis of pe**heral blood mononuclear cells. Thesetests are necessary to distinguish patients withHTLV-I-associated adult T-cell leukaemia ? lymphoma(ATLL) and other T-cell leukaemias such asT-prolymphocytic leukaemia (T-PLL), and also toidentify those with T-cell clones in pe**heral bloodas a marker of tumour burden and as a prognosticindicator. On the basis of these findings a specificclinicopathological diagnosis should be establishedand all patients should be accurately staged, providingprognostic data (Table 1)

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