Cutaneous cd30positive lymphoproliferative disorders. Cutaneous pathology clusterin expression in cutaneous cd30 positive lymphoproliferative disorders and their histologic simulants background. The spectrum of primary cutaneous cd30positive lymphoproliferative disorders includes primary cutaneous anaplastic large cell lymphoma calcl. Treatment of primary cutaneous anaplastic large cell lymphoma. Lymphoproliferative disorders lpds refer to several conditions in which lymphocytes are produced in excessive quantities. Primary cutaneous cd30 positive tcell lymphoproliferative disorders. An update on lymphoproliferative disorders disclosures uscap. Lpd are a heterogeneous group comprising lymphomatoid papulosis lyp, primary cutaneous anaplastic large cell lymphoma pcalcl, transformed mycosis. Despite expression of cd30 by the neoplastic cells as the hallmark of these. Extracutaneous dissemination may occur, especially to regional lymph nodes. Who classification of tumours of haematopoietic and lymphoid tissues, 4th edition, 2008. Localized radiotherapy at doses of 34 to 44 gy is firstline treatment of pcalcl, but the use of lowdose radiotherapy for pcalcl has not been reported. Cutaneous cd30positive tcell lymphoproliferative disorders.
Primary cutaneous cd30 positive lymphoproliferative disorder the term cd30 positive lymphoproliferative disorders. Clinicopathologic and microenvironmental analysis of primary. Cutaneous cd30 positive lymphoproliferative disorders are a spectrum of disease from waxing and waning lymphomatoid papulosis to persistent alk negative primary cutaneous anaplastic large cell lymphoma. Primary cutaneous cd30positive tcell lymphoproliferative disorders are the second most common subgroup of cutaneous tcell lymphomas. Some lesions have overlapping features of type a and b with a spectrum of epidermotropic lymphocytes and large atypical cells and have been classified by us as type ab 9. Pcalcl is considered a skin lymphoma, but is sometimes considered a variant of systemic alcl. Observation is preferred for asymptomatic patients with limited lesions.
Lowdose radiotherapy for primary cutaneous anaplastic. The treatment for patients with lyp depends on the presence or absence of symptoms. Calcl is comprised of large cells with anaplastic, pleomorphic, or immunoblastic cytomorphology, and indeed, more than 75% of the tumor cells express the cd30 antigen. However, strong and homogeneous cd30 expression in most neoplastic cells is restricted to fewer entities, mainly three groups of lymphoid neoplasms. Swerdlow, md 1 department of pathology, division of hematopathology, university of. Eortc, iscl, and usclc consensus recommendations for the. Primary cutaneous alcl, which affects the skin primarily. Pdf molecular alterations in primary cutaneous cd30. Cd30positive primary cutaneous lymphoproliferative disorders. Mum1 multiple myeloma oncogene 1 is expressed in systemic alcl and classical hodgkin lymphoma.
A form of primary tcell lymphoma of the skin which is made up of cd30 positive anaplastic lymphoid cells. Eortc, iscl, and usclc consensus recommendations for the treatment of primary cutaneous cd30 positive lymphoproliferative disorders. Primary lymphomas that present in the skin with no evidence of extracutaneous disease at the time of diagnosis secondary lymphomas systemic lymphomas that. Current classification systems recognize lymphomatoid papulosis lyp, primary cutaneous anaplastic large cell lymphoma, and borderline cases. Tlpd is a provisional entity with uncertain malignant potential according to the latest revision of the who classification for lymphoid neoplasms. Clinically, lyp is characterized by erythematous to brown papules and small nodules measuring less than 2 cm. We sought to highlight the importance of correlation between pathological findings and clinical presentation for correct classification of the disease. They typically occur in people who have a compromised immune system. Mycosis fungoides is not discussed in this subsection but is addressed further on. Primary cutaneous cd30positive tcell lymphoproliferative. Cutaneous bcell lymphoproliferative disorders american. The stanford experience in lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma. Primary cutaneous anaplastic largecell lymphoma case. The condition generally has a very good prognosis with skin lesions usually regressing on.
Primary cutaneous cd30 lymphoproliferative disorders. This is a pdf file of an unedited manuscript that has been. Pdf primary cutaneous cd30positive tcell lymphoproliferative disorders are the second most common subgroup of cutaneous tcell. They include two clinically different entities with some overlapping features and borderline cases. Nccn clinical practice guidelines in oncology nccn. One of the most frequently asked questions on cutaneous lymphomas is how to differentiate lymphomatoid papulosis lyp from cutaneous anaplastic cd30. They are sometimes equated with immunoproliferative disorders, but technically lymphoproliferative disorders are a subset of immunoproliferative disorders, along with hypergammaglobulinemia and. They represent a welldefined spectrum encompassing lymphomatoid papulosis lyp, primary cutaneous anaplastic largecell lymphoma pcalcl, and borderline lesions. The 2018 esmo clinical practice guidelines on primary cutaneous lymphomas are based on results from recent studies and analyses. Nos and b of a tumour on the left leg at the time of relapse primary cutaneous cd30. Cutaneous lymphoproliferative disorders slideshare. Cd30positive primary cutaneous lymphoproliferative. Report of the 2011 society for hematopathologyeuropean association for haematopathology workshop steven h. Outcomes of rare patients with a primary cutaneous cd30.
Primary cutaneous cd30positive tcell lymphoproliferative disorders are the second most. Because of overlapping histological features, it is impossible to distinguish alcl from lyp on histological grounds. Challenges in the diagnosis of primary cutaneous cd30. How i treat primary cutaneous cd30 lymphoproliferative disorders. Clusterin is a ubiquitous 80 kda heterodimeric glycoprotein previously shown to be expressed on tumor cells of systemic and, to a lesser extent, primary cutaneous anaplastic large cell lymphoma pcalcl.
642 758 573 24 1232 620 205 398 371 1282 953 929 388 725 1077 776 52 885 58 778 1142 1401 954 413 952 1207 977 756 773 603 299 473 556 1296 932 903