The 16 patients with a quantitative and phenotypic abnormality encompassed nearly all patients with other unfavorable features. They included of 16 patients with latestage disease, 12 of 17 patients with a peripheral blood tcell clone, all patients with a cd4cd8 ratio of 10 or more, 4 of 5 patients with a chromosomal abnormality, and all patients with a sezary cell count of more than. Cd8 ratio in patchstage mf versus inflammatory mimics. The ratio of cd4 cd8 can be assessed and if there is restriction of one type this would indicate evidence of clonality. Mycosis fungoidessezary syndrome mfss flow cytometry. The disease, however, is not a fungal infection but rather a type of nonhodgkins lymphoma.
What are the histologic findings of mycosis fungoides in. Simplified flow cytometric assessment in mycosis fungoides. Mycosis fungoides mf is the most common primary cutaneous t cell lymphoma, which is characterised in its early stages by epidermotropism of small to mediumsized t lymphocytes with cerebriform nuclei. Cd8 ratio in patch stage mf versus inflammatory mimics. The aim of this study was to determine the contribution of the cd8cd3 ratio to the diagnosis of mycosis. Poikilodermatous mycosis fungoides rachel farleyloftus md, rajni mandal md, joann latkowski md dermatology online journal 16 11. Wang, md key facts terminology primary cutaneous tcell lymphoma characterized by epidermotropism clinical course showing stepwise evolution of patches, plaques, and tumors clinical issues overall indolent clinical course clinical stage is most important predictor of prognosis microscopic pathology skin biopsy findings may be. Mycosis fungoides american journal of clinical pathology. Alibert first described mycosis fungoides mf in 1806, and bazin later defined the natural evolution into the stages known today as patch, plaque, and tumor. Large cell transformation of mycosis fungoides mf is an uncommon phenomenon.
Mycosis fungoides mf arises from cells of lymphoid lineage 2, a key. Cd4 can also be useful to highlight the degree of t cell infiltration into. Neuere studien zeigen, dass cd4cd8negative lymphozyten. Nonetheless, there are unusual variants of mf with a decreased cd4. Mycosis fungoides, also known as alibertbazin syndrome or granuloma fungoides, is the most common form of cutaneous tcell lymphoma. Earlystage mfctcl stages ia and ib is most often managed in both dermatology and multidisciplinary settings. To analyze exclusively the lymphocytic infiltrates, cd4, which is also expressed by. Mycosis fungoides clinical images these clinical images show the plaques and patches invloving over half of the patients body, specifically, the chest, back, and palm. A case of mycosis fungoides university of california. Tox as a diagnostic and prognostic marker for mycosis fungoides. Mycosis fungoides is a malignant tcell lymphoma characterized by. Mycosis fungoides mf is the most common type of cutaneous tcell lymphoma ctcl and represents nearly 50 % of all primary cutaneous lymphomas. Tnmb staging of mycosis fungoides 25 skin t 1 patches, papules or plaques covering patches, papules or plaques covering 10% of the skin surface t 3 tumours t 4 generalized erythroderma lymph nodes n 0 no clinically abnormal lymph nodes. Cd4 is expressed in a tcell subset helperinducer and is found in approximately 80% of thymocytes and in 45% of peripheral blood lymphocytes.
Mycosis fungoides presenting as symmetric concentric patches. Whereas rapid progression has been attributed to cd8. Cd4positive tcell large granular lymphocytosis mimicking. However, there is no ratio number that proves the presence or absence of lymphoma. In the early stages of mycosis fungoides, the histopathology is nonspecific,ref81ref82ref83ref84 and the condition is often misdiagnosed as an inflammatory disorder. Mf progresses from patch to plaque stages, the relative pro portion of. Earlystage mf iaiia has an indolent course, although 25% of patients. Cd8 has been shown to be an important marker in the analysis of tcell mediated inflammatory dermatoses and is also useful. Figure 3 large, atypical lymphocytes within the papillary dermis. Mycosis fungoides is the most common form of cutaneous tcell lymphoma mfctcl. Mycosis fungoides mf is the most common primary cutaneous t cell lymphoma ctcl, which comprise a heterogeneous group of nonhodgkins lymphoma. The aim of this study was to determine the contribution of the cd8 cd3 ratio to the diagnosis of mycosis fungoides. These lymphocytes were of tcell origin as highlighted by a cd3 stain.
Symptoms include rash, tumors, skin lesions, and itchy skin. Mycosis fungoides including sezary syndrome treatment pdq. Furthermore, although rare, mf with cd81 phenotype could also be considered, given the near equal ratio of cd4. Cd8 ratio, a seemingly more attractive and simpler proposition, has yielded conflicting results, as most inflammatory dermatoses are also cd4 predominant. Mycosis fungoides mf is the most common type of cutaneous tcell lymphoma ctcl and represents nearly 50% of all primary cutaneous lymphomas. Originally described by alibert in 1806, mf is classically a disease of adults, although children and adolescents can be affected, and it typically has a. Immunohistochemical study combined with confocal microscopy could clarify this issue.
There is usually pigmentary incontinence and telangectatic vessels are common. Cd4 cd8 doublenegative epidermotropic cutaneous tcell lymphoma. Tumorspecific immune responses play an important role in the control of malignant disease, 1 and immunotherapy is now an effective therapy for many tumor subtypes. Treatment selection is generally guided by institutional experience, patient preference, and toxicity profile, as data from phase iii clinical trials are limited. Dense dermal infiltrates of atypical t cells with cerebriform nuclei. In about 10% of cases, mf can progress to lymph nodes and internal organs.
Epidemiology, etiology, genetics, and cytogenetic abnormalities. Cd4 positive large granular lymphocytosis occurring in a patient with stage ia mycosis fungoides mf that posed diagnostic and treatment dilemmas. We present a case of cd30positive large cell transformation and discuss its possible pathophysiology. The influence of the coexpression of cd4 and cd8 in cutaneous. Mycosis fungoides mycosis fungoides mf is the most common type of ctcl, accounting for 50% of all primary ctcl cases. Mycosis fungoides ubersicht altmeyers enzyklopadie. Mycosis fungoides variantsclinicopathologic features. Value of the cd8cd3 ratio for the diagnosis of mycosis. Dermal infiltrate of atypical t cells with cerebriform nuclei alone or clustered in epidermis and in small sheets in dermis. Although it is uncommon, it is the most common of the. As progression ensues, extracutaneous invasion is not uncommon. A pilot study patch stageearly mycosis fungoides mf is difficult.
A periodic acid schiff pas stain was negative for fungal organisms. The ihc profile of dermal lymphoid infiltrate in our study matched the reported pattern, i. Mycosis fungoides mf is the most common type of cutaneous tcell lymphoma. It was so named because alibert described the skin tumors of a severe case as having a mushroomlike appearance. It generally affects the skin, but may progress internally over time. The original staging system for ctcl was based on the tumour, lymph node, metastasis tnm system devised by bunn and lamberg in 1979. We retrospectively compared the immunophenotypic characteristics of 30 mycosis. Mycosis fungoides follows a slow, chronic indolent course and very often does not spread beyond the skin. Ten patients showed the coexpression of cd4 and cd8 and had a slightly lower rate of progressive. This is a case of a patient with hypopigmented patch of mycosis fungoides. Hodak e, david m, maron l, aviram a, kaganovsky e, feinmesser m. Ctcl diagnosis and staging what is cutaneous lymphoma. Based on these findings, the patient was advised to expose his face, which had previously been shielded, during his puva sessions. Symptoms of mf can include flat, red, scaly patches, thicker raised lesions calls plaques, and sometimes large nodules called tumors.
Immunophenotypic shift in a case of mycosis fungoides with. Mycosis fungoides mf represents the prototype of cutaneous t cell. Cd8 ratios are more likely to demonstrate poor clinical. Cd8 has been shown to be an important marker in the analysis of tcell mediated inflammatory.
After clinicopathologic correlation, the patient was diagnosed with stage ia mycosis fungoides. In contrast, abnor mal cd3tcrb antigen expression was seen in 3 of 6 cases 50% of tumor stage mycosis fungoides. The blood of 3 stage t2 patients was examined for evidence of sezary cells by 3 techniques. Fatima s, siddiqui s, tariq mu, ishtiaque h, idrees r, ahmed z, ahmed a. The influence of the coexpression of cd4 and cd8 in. Cd30positive large cell transformation of mycosis fungoides. The immunohistochemical profile for this case shows positivity in t cell markers, including cd3, cd4, cds, and cd8. Erythematous patches and plaques with fine scale and tumors that anatomically favor the buttocks and sunprotected areas of the trunk and limbs characterize this subtype.
The immunophenotype of neoplastic cells is similar to that observed in classic mycosis fungoides. Cd8 ratio may be similar to reactive infiltrates, in which the ratio tends to range from 1. Mycosis fungoides mf is characterized by epidermotropic skin infiltration by atypical t lymphocytes. A clinicopathological study of 60 cases from a tertiary care center. A high cd4 cd8 ratio in the lymphocytic infiltrates of clinically suspicious lesions is, however, considered to be highly suggestive for the histopathological diagnosis of mycosis fungoides. Patch stageearly mycosis fungoides mf is difficult to differentiate from benign dermatoses, despite several robust histologic criteria. Progression of mycosis fungoides occurs through divergence. It exhibits a protracted clinical course with slow progression from slightly scaly skin lesions patches. Histologic features, immunohistochemical profile, mycosis fungoides how to cite this article. Poikilodermatous mycosis fungoides is a rare form of cutaneous tcell lymphoma that is characterized clinically by localized or diffuse patches, which consist of telangiectases, mottled hyper and hypopigmentation, and atrophy.
Treatment selection is generally guided by institutional experience, patient preference, and toxicity profile, as data from phase iii clinical trials are. Patchstageearly mycosis fungoides mf is difficult to differentiate from benign. We undertook this blinded study of comparing the cd4. Value of the cd8cd3 ratio for the diagnosis of mycosis fungoides. If sezary cells cannot be used to determine tumor burden for b2, then one of the following modified iscl criteria, along with a positive clonal rearrangement of the tcell receptor tcr, may be used instead. Poikilodermatous mycosis fungoides is a rare form of cutaneous tcell lymphoma that is characterized clinically by localized or diffuse patches. The estimated annual incidence rate in the united states is only approximately 0. The disease begins with lightly erythematous patches that subsequently evolve. The ratio of cd4cd8 can be assessed and if there is restriction of one type this would indicate evidence of clonality. Quality nursing care is necessary for effective diagnosis and treatment of patients with mfctcl. Loss of cd7 andor cd26 in the cd4 tcells are typical aberrancies. Associated diseases mycosis fungoides has been described in patients with other haematological disorders. In three biopsies 2 patients, 1 with 2 sequential biopsies.
Skin, mycosis fungoides, cd8 stain lmp765 lmp digital. Taken together, these findings are consistent with mycosis fungoides. The presence of predominant cd4 lymphocytes in conjunction with epidermotropism, dermal fibrosis, and cytological atypia favor mf over an inflammatory dermatosis. Request pdf origin use of cd4, cd8, and cd1a immunostains in distinguishing mycosis fungoides from its inflammatory mimics. Intraocular involvement of mycosis fungoides associated with. Prototypical lesions of mf show an increase in the cd4. Wang, md key facts terminology primary cutaneous tcell lymphoma characterized by epidermotropism clinical course showing stepwise evolution of patches, plaques, and tumors clinical issues overall indolent clinical course clinical stage is most important predictor of prognosis microscopic pathology skin biopsy findings may be nondiagnostic in premycotic. Cd4 cd8 doublenegative phenotype appears to be associated with an unusual clinical presentation, it does not appear to confer prognostic significance. Inflammatory dermatoses demonstrate a mixture of cd4 and cd8 positive cells in a proportion of approximately 34. Cd8 ratio of 10, an absolute count of sezary cells per l, and demonstration of tcell clonality in the peripheral blood are diagnostic criteria. Cd8 ratio that has been reported in many patients with mf. Most studies include advanced lesions and data about early disease is limited. 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. Intraocular involvement of mycosis fungoides associated.
Cd4 can also be useful to highlight the degree of t cell infiltration into the epidermis. The name mycosis fungoides is very misleadingit loosely means mushroomlike fungal disease. Case report a 65yearold white woman initially noted a macular, nonscaly, nonpruritic, erythematous lesion on her right breast. Figure 4 folliculotropic variant of mycosis fungoides. Mycosis fungoides presenting as symmetric concentric. Further immunohistochemical stains performed include cd20, cd56, cd10, cd30 and ki67. Cd4 is expressed in the majority of tcell lymphomas including mycosis fungoides, a common form of cutaneous tcell lymphoma 1.
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