Apoptosis in cd30positive lymphoproliferative disorders of the skin. Mycosis fungoides mf is generally considered an indolent cd30 negative. D, pigmented purpuriclike dermatitis on the left inner thigh. Mf patients at risk of disease progression can now be identified and an international consortium has been established to address the prognostic relevance of specific biologic factors and.
The clinical behavior of this lymphoma differs from that of other extranodal lymphomas and thus requires a particular pretreatment evaluation and treatment strategy. Most often there are oval or ringshaped annular pink dry patches on covered skin. Mycosis fungoides is not discussed in this subsection but is addressed further on. The major differential diagnosis for a primary cutaneous tcell lymphoproliferative disorder with cd30 ki1 positivity includes primary cutaneous anaplastic large cell lymphoma, lymphomatoid papulosis, pagetoid reticulosis and transformed mycosis fungoides mf. Mycosis fungoides nord national organization for rare. Mf presents itself initially as patches or plaques that can develop into a tumor over. Microabstracttransformed mycosis fungoides mf can occur in early and late stages of mf and in skin andor lymph nodes but risk factors associated with transformation of mf are not well known. Sgn35 in cd30positive lymphoproliferative disorders. Mycosis fungoides in any stage may suddenly become much more aggressive, progressing rapidly to more advanced stages see the images below. Significance of cd30 expression by epidermotropic t cells. Lymphomatoid papulosis associated with mycosis fungoides. C, dyschromic patches with fine scale on the right upper arm. They may spontaneously disappear, remain the same size, or slowly enlarge. Lymphocytes in mycosis fungoides have a characteristic clear space halo around them and are seen lining up along the dermalepidermal junction.
Mycosis fungoides variantsclinicopathologic features. Most tcell skin lymphomas begin as patches of dry, discoloured skin usually. Of 202 patchplaque phase mf cases studied by immunohistochemistry, we encountered 4. Mycosis fungoides mf is the most common type of cutaneous tcell lymphoma ctcl and represents nearly 50% of all primary cutaneous lymphomas.
Cd30positive primary cutaneous lymphoproliferative disorders. Cd20 antigen in mycosis fungoides nejm journal watch. Conventional mf begins as eczematoid or psoriasiform patches and. Sometimes a red rash is more than an annoying skin problem. Early stage mycosis fungoides with focal cd30positive large cell. A, clinical photograph shows patches and plaques on the trunk. Cd30positive large cell transformation of mycosis fungoides.
A diagnosis of mycosis fungoides may be made by a thorough clinical evaluation and a variety of specialized techniques and tests including dna cytophotometry, nuclear contour analysis, and analysis of tcell receptor gene rearrangement. It has been classified according to the type of skin lesions patches, plaques and tumours, the presence or absence of large. Herein, we present a 45yearold woman with patch stage mf stage ib who developed a papule within one patch, which revealed large cell transformation with. The prolonged clinical course may be undulating and. It may be evident as a new, solitary nodule within a classic mycosis fungoides patch or. This condition is one of the most common types of tcell lymphoma. Patients with mycosis fungoides mf are at increased risk of a second malignant neoplasm, especially lymphoma. Local radiation for cutaneous tcell lymphoma other than. Of 202 patchplaque phase mf cases studied by immunohistochemistry, we encountered 4 patients with patchphase mf at stage ia or ib, whose lesions contained a high proportion greater than. These kinds of neoplasms initially present as skin involvement and, as such, have been classified as cutaneous tcell lymphomas.
Ctcls correspond to 75%80% of pcls, and mycosis fungoides mf and its. At the same time, malignant t cells in the lymph node biopsy stained positive for cd30, and the diagnosis of transformed mf was established. Mycosis fungoides mf comprises about 65% of cutaneous tcell lymphomas. Mycosis fungoides mf is a clinical diagnosis that requires strong correlation with histopathologic and sometimes molecular findings to exclude benign inflammatory diseases, more aggressive primary cutaneous lymphomas, and extracutaneous lymphomas that can involve the skin. Mycosis fungoides and ctcl are often used interchangeably, which is imprecise, as mycosis fungoides is just one type of ctcl. Mycosis fungoides mf, the most common cutaneous tcell lymphoma, is usually indolent, but transformation to aggressive behavior occurs in about 10% of cases. Tcell lymphomas after mycosis fungoides mf, accounting for approximately 30%. A case of exuberant cutaneous lymphomagenesis in the setting of chronic patch mycosis fungoides jason c. Sgn35 in cd30positive lymphoproliferative disorders alcl, mycosis fungoides mf, and extensive lymphomatoid papulosis lyp the safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Mycosis fungoides is characterized by a scaly, red rash that develops on the skin, particularly on areas that are not usually exposed to the sun.
N2 mycosis fungoides mf, the most common type of primary cutaneous tcell lymphoma, undergoes large cell transformation in only a minority of cases that present in patch stage. Large cell transformation of mycosis fungoides on fine. Early stage mycosis fungoides with focal cd30positive. Early stage mycosis fungoides with focal cd30positive large cell transformation author links open overlay panel poju lai 1 2 yuping hsiao 1 3 jengdong hsu 4 shiowjiuan wey 1 show more. Epidermotropic infiltrate of atypical small to medium. Large cell transformation lct in mf has been defined by the presence of large cells at least four times larger than a small lymphocyte exceeding 25% of. Case report early stage mycosis fungoides with focal cd30positive large cell transformation poju lai1,2, yuping hsiao1,3, jengdong hsu4, shiowjiuan wey1, 1 department of dermatology, chung shan medical university hospital, taichung, taiwan, roc 2 institute of biochemistry and biotechnology, chung shan medical university, taichung, taiwan, roc 3 institute of medicine, school of medicine. The image shows positive staining with cd3 depicting epidermotrophic string of pearls infiltrate along the basal layer. A case of exuberant cutaneous lymphomagenesis in the. Mycosis fungoides mf represents the most common type of cutaneous tcell lymphomas. Learn more about how it is diagnosed, treated and its prognosis. Among the cutaneous cd30positive lymphomas, we highlight the systemic.
Cd30 expression and proliferative fraction in nontransformed. Little is known, however, about cd30 expression in nontransformed mf, whether it simply reflects the proliferative fraction and if. A sign of mycosis fungoides is a red rash on the skin. Mycosis fungoides american journal of clinical pathology. The name mycosis fungoides is historical and confusing. In patch stage mycosis fungoides, the skin lesions are flat. Large numbers of cd30positive tumor cells are not typically observed in mycosis fungoides mf, but cd30 expression may occur on large cells of mf that have transformed into highgrade large cell lymphoma.
Request pdf mycosis fungoides with cd30positive cells in the epidermis. Mycosis fungoides, also known as alibertbazin syndrome or granuloma fungoides, is the most common form of cutaneous tcell lymphoma. Mycosis fungoides genetic and rare diseases information. Retrospective analysis of prognostic factors in 187 cases. Large cell transformation of mycosis fungoides mf is an uncommon phenomenon. Mycosis fungoides variantsclinicopathologic features, differential diagnosis, and treatment rein willemze, md, phd m ycosis fungoides mf is the most common type of cutaneous tcell lymphoma ctcl and accounts for approximately 50% of all primary cutaneous lymphomas. Mycosis fungoides mycosis fungoides is the most common type of cutaneous lymphoma, representing almost 50% of all lymphomas arising primarily in the skin. Patch, plaque, tumour mycosis fungoides abstract mycosis fungoides is a frequent cutaneous lymphoma contributing to an estimated half 50% of the emerging dermal lymphomas.
Positive large cell transformation of mycosis fungoides. It is defined as a tumor composed of smallmediumsized, epidermotropic thelper lymphocytes but tcytotoxic variants are not uncommon and tumor cells may be mediumlarge in advanced stages. Mycosis fungoides with cd30positive cells in the epidermis. Mycosis fungoides is the most common form of cutaneous tcell lymphoma ctcl. In univariate analysis, risk factors associated with disease.
We present a case of cd30positive large cell transformation and discuss its possible pathophysiology. Mycosis fungoides mf usually has an indolent course, 1 with a diseasespecific 5year survival rate of 88%. Primary cutaneous cd30positive tcell lymphoproliferative disorders are the. Primary cutaneous cd30positive lymphoproliferative disorder the. Sluzevich, md,a han tun, md,b demetriastrauchjacks,md,b and liuyan jiang, mdc. Mycosis fungoides is a disease in which tcell lymphocytes a type of white blood cell become malignant cancerous and affect the skin.
We describe a 52yearold patient with a history of unilesional. A biopsy of a nodule showed atypical cd30 cells, which were positive for cd3 and cd4, but p53negative. It exhibits a protracted clinical course with slow progression from slightly scaly skin lesions patches to infiltrated plaques. Symptoms include rash, tumors, skin lesions, and itchy skin. In patch and plaque stage mf, a high number greater than 50% of cd30. Patients presenting with involvement of regional lymph nodes have a good.
Screening for large cell transformation lct and cd30 expression might be helpful for prognosis and more aggressive or targeted therapies. Large numbers of cd30positive tumor cells are not typically observed in mycosis fungoides mf, but cd30 expression may occur on large cells of mf that have transformed into highgrade large cell. Mycosis fungoides rarely occurs before age 40 years. It can be a sign of illness, including a blood cancer with a big name. T cell lymphomas after mycosis fungoides mf, accounting for approximately 30%. Types of tcell skin lymphoma including mycosis fungoides and sezary syndrome.
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