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. Recurrence of primary cutaneous anaplastic large cell. Primary cd30 cutaneous lymphoproliferative disease pclpd is a spectrum of indolent cutaneous tcell lymphomas. They represent a welldefined spectrum encompassing lymphomatoid papulosis lyp, primary cutaneous anaplastic largecell lymphoma pcalcl, and. Primary cutaneous cd30positive tcell lymphoproliferative disorders are the second most common subgroup of cutaneous tcell lymphomas.
However, strong and homogeneous cd30 expression in most neoplastic cells is restricted to fewer entities, mainly three groups of lymphoid neoplasms. The spectrum of primary cutaneous cd30 ki1positive lymphoproliferative disorders. Jun 15, 2000 primary cutaneous tcell lymphomas ctcl represent a heterogeneous group of neoplasms derived from skinhoming t cells. Cutaneous pathology clusterin expression in cutaneous cd30 positive lymphoproliferative disorders and their histologic simulants background. Cutaneous cd30positive tcell lymphoproliferative disorders. Both disease entities share overlapping clinical, histopathological. These disorders primarily present in patients who have a compromised immune system. This is a spectrum of disorders that ranges from the more benign lymphomatoid papulosis which almost always regresses spontaneously. Current classification systems recognize lymphomatoid papulosis lyp, primary cutaneous anaplastic large cell lymphoma, and borderline cases. Primary cutaneous anaplastic large cell lymphoma mdpi.
Bekkenk mw, geelen fa, van voorst vader pc, of ctcl with variable expression of cd30. Patients with calcl should not have the clinical evidence of mycosis fungoides with large cell transformation, which may be cd30 postive or cd30. Primary cutaneous anaplastic large cell lymphoma seer. Primary cutaneous cd30positive t cell lymphoproliferative disorders. Due to this factor, there are instances of these conditions being equated with immunoproliferative disorders. Final diagnosis cd30 positive lymphoproliferative disorder.
It often occurs in elderly patients, presenting at a median age of 60 years, although it may occur at any age. Cd30 ki1positive lymphoproliferative disorders of the skinclinicopathologic. Incorrect diagnosis can result in overly aggressive therapy. Immunohistochemistry revealed atypical cells to be positive for cd30, cd3, partially for cd15, and. Molecular studies of primary cutaneous anaplastic large cell lymphoma reveal an. Primary cutaneous anaplastic large cell lymphoma calcl is composed of large cells with an anaplastic, pleomorphic, or immunoblastic cytomorphology, the majority of which express the cd30 antigen. Detection of alk expression in cd30 h lymphoproliferative disorders with antip80 only cases of cutaneous cd30 f lymphoproliferative disorders reactive with autip80 were analyzed. Primary cutaneous cd30 positive lymphoproliferative disorders represent the second most common subgroup of cutaneous tcell lymphomas and include lymphomatoid papulosis, primary cutaneous anaplastic large cell lymphoma, and borderline lesions. Reports of borderline lesions of primary cutaneous cd30 positive tcell lymphoproliferative disorders are few the prognosis seems to be favorable, but long term followup is required borderline lesions. In addition to making the distinction between primary cutaneous anaplastic largecell lymphoma pcalcl and lymphomatoid papulosis lyp, the two entities within this. Primary cutaneous cd30positive large tcell lymphoma in an 80. Pdf eortc, iscl, and usclc consensus recommendations for.
Eortc, iscl, and usclc consensus recommendations for the. The annual incidence of these lymphomas is low, at 710 cases per 100 million population. Lowdose radiotherapy for primary cutaneous anaplastic. Comprising this group are lymphomatoid papulosis lyp, primary cutaneous anaplastic lymphoma calcl, and borderline cases.
Eortc, iscl, and usclc consensus recommendations for the treatment of primary cutaneous cd30 positive lymphoproliferative disorders. Observation is preferred for asymptomatic patients with limited lesions. Little is known about tumor microenvironment and there is almost no literature about pdl1 expression. Primary cutaneous anaplastic large cell lymphoma uptodate. Primary cutaneous anaplastic large cell lymphoma is characterized by the presence of solitary or localized nodules or tumors located on the extremities. The stanford experience in lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma howard l. Cd30positive primary cutaneous lymphoproliferative.
Clinicopathologic correlation is essential for proper diagnosis and management. Primary cutaneous cd30positive tcell lymphoproliferative. Lymphomatoid papulosis lesions appear as small selfhealing papules, with a necrotic center that often appears in clusters and recurs in the same region of the body. The primary intention of the analysis of the national cancer institute surveillance, epidemiology, and end results seer database was to report epidemiologic information and overall survival of patients with pclpd. In some patients, both diseases may coexist at initial diagnosis or develop over the course of the disease. Primary cutaneous t cell lymphoma pctl refers to cases of t cell lymphoma that. The term cd30positive lymphoproliferative disorders encompasses entities such as anaplastic large cell lymphoma primary cutaneous and. Lymphoproliferative disorders lpds refer to a specific class of diagnoses, comprising a group of several conditions, in which, lymphocytes are produced in excessive quantities.
Most primary cutaneous cd30 positive lymphoproliferative. Most primary cutaneous cd30positive lymphoproliferative. They include two clinically different entities with some overlapping features and borderline cases. What is primary cutaneous cd30positive lymphoproliferative. Clinicopathologic and microenvironmental analysis of. Cd30 primary cutaneous tcell lymphoproliferative disorders are the second most common subgroup of cutaneous tcell lymphomas after mycosis fungoides mf, accounting for approximately 30% of cases. A report from the dutch cutaneous lymphoma group on the longterm followup data of 219 patient and guidelines for diagnosis and treatment. Primary cutaneous anaplastic large cell lymphoma is characterized by the presence of solitary or localized nodules or tumors located on the. Primary cutaneous cd30 positive tcell lymphoproliferative disorders are the second most common subgroup of cutaneous tcell lymphomas. Despite expression of cd30 by the neoplastic cells as the hallmark of these. Primary cutaneous cd30 positive t cell lymphoproliferative disorders are the second most common category of cutaneous t cell lymphoma in the world health organization who classification of lymphoid neoplasms. Pdf cutaneous cd30positive lymphoproliferative disorders. New insights, future directions in primary cutaneous. Primary cutaneous anaplastic largecell lymphoma fulltext.
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. Mycosis fungoides associated with lesions in the spectrum. Cd30positive primary cutaneous lymphoproliferative disorders. Lyp is associated with development of lymphoma in nearly 20% of. Most primary cutaneous cd30 positive lymphoproliferative disorders have a cd4positive cytotoxic tcell phenotype jean alain kummer, maarten h. Lyp may be distinguished from calcl by clinicopathological correlation. Who classification of tumours of haematopoietic and lymphoid tissues, 4th edition, 2008. Meij er, and rein willemze departments of pathology and dermatology, free university hospital, amsterdam, the netherlands. Primary and secondary cutaneous cd30 1 interestingly, response to the drug does not corre lymphoproliferative disorders.
Primary cutaneous cd30 lymphoproliferative disorders cd30 lpds are the second most common form of cutaneous tcell lymphomas ctcls and represent a spectrum of diseases, includinglymphomatoidpapulosislypandprimarycutaneousanaplasticlargecelllymphomapcalcl. This case illustrates the diagnostic challenges posed by a group of disorders known as cd30 positive lymphoproliferative disorders of the skin. Primary cutaneous cd30 positive tcell lymphoproliferative disorders include pcalcl, lyp, and other miscellaneous entities. Lowdose radiotherapy for primary cutaneous anaplastic large. Cutaneous cd30positive lymphoproliferative disorders. Polymorphism of the cd30 promoter microsatellite repressive. Cutaneous bcell lymphoproliferative disorders american. Clinicopathologic and microenvironmental analysis of primary. The workshop included an example of a posttransplant lymphoproliferative disorder of bcell lymphoma, unclassifiable, with features intermediate between burkitt lymphoma and dlbcl type that was an myc with bcl6 double hit lymphoma which arose 17 years after transplantation. These findings were supported by a tcell receptor clonality assessment by the. In three of four cases w ith primary cutaneo us kil or lyp type a. A phase ii study of sgn30 in cutaneous anaplastic large. Th2 cytokine mrna expression in primary cutaneous cd30.
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