The Experts below are selected from a list of 360 Experts worldwide ranked by ideXlab platform
Masafumi Iijima - One of the best experts on this subject based on the ideXlab platform.
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autopsy case of cd4 cd8 cutaneous t cell lymphoma presenting disseminated Pagetoid Reticulosis with aggressive granulomatous invasion to the lungs and pancreas
Pathology International, 2005Co-Authors: Eisuke Shiozawa, Toshiko Yamochionizuka, Bungo Saito, Emi Takaba, Masafumi Iijima, Mari Shibata, Tokio Nakada, Akira Shiokawa, Michiaki TakimotoAbstract:Pagetoid Reticulosis is a rare cutaneous T-cell lymphoma with striking epidermotropism similar to that present in Paget's disease. There are two forms of Pagetoid Reticulosis: localized and disseminated. Reported herein is an autopsy case of disseminated Pagetoid Reticulosis with CD4–/CD8– phenotype T cells and massive invasion of the lungs and pancreas. The abnormal cells in the epidermis expressed a protein derived from a rearranged T-cell receptor β gene, and this feature was used to confirm the monoclonality of these cells by polymerase chain reaction. At present, the World Health Organization (WHO) classification system considers Pagetoid Reticulosis to be an indolent form of primary cutaneous T-cell lymphoma and a variant of mycosis fungoides/Sezary syndrome with prominent epidermotropism. Some differences have been observed between Pagetoid Reticulosis and mycosis fungoides in terms of clinical course, tumor cell phenotype, and genetic findings; and these differences are highlighted in the present case. The relation between disseminated Pagetoid Reticulosis, CD4–/CD8– cutaneous T-cell lymphoma, and γδ T-cell lymphoma, including whether Pagetoid Reticulosis is a variant of mycosis fungoides, remains unclear.
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autopsy case of cd4 cd8 cutaneous t cell lymphoma presenting disseminated Pagetoid Reticulosis with aggressive granulomatous invasion to the lungs and pancreas
Pathology International, 2005Co-Authors: Eisuke Shiozawa, Toshiko Yamochionizuka, Bungo Saito, Emi Takaba, Masafumi Iijima, Mari Shibata, Tokio Nakada, Akira Shiokawa, Masafumi Takimoto, Hidekazu OtaAbstract:Pagetoid Reticulosis is a rare cutaneous T-cell lymphoma with striking epidermotropism similar to that present in Paget's disease. There are two forms of Pagetoid Reticulosis: localized and disseminated. Reported herein is an autopsy case of disseminated Pagetoid Reticulosis with CD4(-)/CD8(-) phenotype T cells and massive invasion of the lungs and pancreas. The abnormal cells in the epidermis expressed a protein derived from a rearranged T-cell receptor beta gene, and this feature was used to confirm the monoclonality of these cells by polymerase chain reaction. At present, the World Health Organization (WHO) classification system considers Pagetoid Reticulosis to be an indolent form of primary cutaneous T-cell lymphoma and a variant of mycosis fungoides/Sezary syndrome with prominent epidermotropism. Some differences have been observed between Pagetoid Reticulosis and mycosis fungoides in terms of clinical course, tumor cell phenotype, and genetic findings; and these differences are highlighted in the present case. The relation between disseminated Pagetoid Reticulosis, CD4(-)/CD8(-) cutaneous T-cell lymphoma, and gammadelta T-cell lymphoma, including whether Pagetoid Reticulosis is a variant of mycosis fungoides, remains unclear.
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disseminated Pagetoid Reticulosis ketron goodman disease six year follow up
Journal of The American Academy of Dermatology, 2002Co-Authors: Tokio Nakada, Hirohiko Sueki, Masafumi IijimaAbstract:Pagetoid Reticulosis consists of 2 types: Woringer-Kolopp and Ketron-Goodman disease (K-G). Compared with the former, K-G may have disseminated lesions and a guarded prognosis. We encountered a case of K-G in a 67-year-old man with disseminated plaques on the neck, the trunk, and both extremities. Histologic specimens demonstrated medium- to large-sized, atypical cells infiltrating in the lower epidermis. The phenotype (CD3(+), CD4(-), CD8(-), CD45RO(-), CD45RA(+)) and ultrastructural findings suggest that these cells were immature T cells. Although PUVA was initially effective, new plaques in which atypical cells are still present histologically, have appeared through a 6-year follow-up. Literature review revealed the high rate of recurrence. These findings suggest that K-G is an epidermotropic or immature T-cell variant of mycosis fungoides. In patients with K-G, therefore, long-term observation is necessary: Disappearance of cutaneous lesions may not mean cure.
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disseminated Pagetoid Reticulosis ketron goodman disease six year follow up
Journal of The American Academy of Dermatology, 2002Co-Authors: Tokio Nakada, Hirohiko Sueki, Masafumi IijimaAbstract:Abstract Pagetoid Reticulosis consists of 2 types: Woringer-Kolopp and Ketron-Goodman disease (K-G). Compared with the former, K-G may have disseminated lesions and a guarded prognosis. We encountered a case of K-G in a 67-year-old man with disseminated plaques on the neck, the trunk, and both extremities. Histologic specimens demonstrated medium- to large-sized, atypical cells infiltrating in the lower epidermis. The phenotype (CD3 + , CD4 − , CD8 − , CD45RO − , CD45RA + ) and ultrastructural findings suggest that these cells were immature T cells. Although PUVA was initially effective, new plaques in which atypical cells are still present histologically, have appeared through a 6-year follow-up. Literature review revealed the high rate of recurrence. These findings suggest that K-G is an epidermotropic or immature T-cell variant of mycosis fungoides. In patients with K-G, therefore, long-term observation is necessary: Disappearance of cutaneous lesions may not mean cure. (J Am Acad Dermatol 2002;47:S183-6.)
E Berti - One of the best experts on this subject based on the ideXlab platform.
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primary cutaneous cd8 positive epidermotropic cytotoxic t cell lymphomas a distinct clinicopathological entity with an aggressive clinical behavior
American Journal of Pathology, 1999Co-Authors: E Berti, Dario Tomasini, Maarten H Vermeer, Chris J L M Meijer, E Alessi, Rein WillemzeAbstract:Cutaneous T cell lymphomas (CTCL) generally have the phenotype of CD3+, CD4+, CD45RO+ memory T cells. CTCL expressing a CD8+ T cell phenotype are extremely rare and ill-defined. To elucidate whether these CD8+ CTCL represent a distinct disease entity, the clinical, histological, and immunophenotypical features of 17 CD8+ CTCL were reviewed. None of the 17 cases expressed markers characteristic of natural killer cells or γ/δ T cells. Nine of 17 cases showed the characteristic clinical and histological features as well as clinical behavior of well defined types of CTCL, such as mycosis fungoides (2 cases), Pagetoid Reticulosis (2 cases), lymphomatoid papulosis (2 cases), and CD30+ large T cell lymphoma (2 cases), all of which usually express a CD4+ T cell phenotype, and 1 case of subcutaneous panniculitis-like T cell lymphoma. The other 8 cases formed a homogeneous group showing a distinctive set of clinicopathological and immunophenotypical features, not consistent with that of other well defined types of CTCL. Clinical characteristics included presentation with generalized patches, plaques, papulonodules, and tumors mimicking disseminated Pagetoid Reticulosis; metastatic spread to unusual sites, such as the lung, testis, central nervous system, and oral cavity, but not to the lymph nodes; and an aggressive course (median survival, 32 months). Histologically, these lymphomas were characterized by band-like infiltrates consisting of pleomorphic T cells or immunoblasts, showing a diffuse infiltration of an acanthotic epidermis with variable degrees of spongiosis, intraepidermal blistering, and necrosis. The neoplastic cells showed a high Ki-67 proliferation index and expression of CD3, CD8, CD7, CD45RA, βF1, and TIA-1 markers, whereas CD2 and CD5 were frequently lost. Expression of TIA-1 pointed out that these lymphomas are derived from a cytotoxic T cell subset. The results of this and other studies reviewed herein suggest that these strongly epidermotropic primary cutaneous CD8+ cytotoxic T cell lymphomas represent a distinct type of CTCL with an aggressive clinical behavior.
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primary cutaneous cd8 positive epidermotropic cytotoxic t cell lymphomas a distinct clinicopathological entity with an aggressive clinical behavior
American Journal of Pathology, 1999Co-Authors: E Berti, Dario Tomasini, Maarten H Vermeer, Chris J L M Meijer, E Alessi, Rein WillemzeAbstract:Cutaneous T cell lymphomas (CTCL) generally have the phenotype of CD3+, CD4+, CD45RO+ memory T cells. CTCL expressing a CD8+ T cell phenotype are extremely rare and ill-defined. To elucidate whether these CD8+ CTCL represent a distinct disease entity, the clinical, histological, and immunophenotypical features of 17 CD8+ CTCL were reviewed. None of the 17 cases expressed markers characteristic of natural killer cells or γ/δ T cells. Nine of 17 cases showed the characteristic clinical and histological features as well as clinical behavior of well defined types of CTCL, such as mycosis fungoides (2 cases), Pagetoid Reticulosis (2 cases), lymphomatoid papulosis (2 cases), and CD30+ large T cell lymphoma (2 cases), all of which usually express a CD4+ T cell phenotype, and 1 case of subcutaneous panniculitis-like T cell lymphoma. The other 8 cases formed a homogeneous group showing a distinctive set of clinicopathological and immunophenotypical features, not consistent with that of other well defined types of CTCL. Clinical characteristics included presentation with generalized patches, plaques, papulonodules, and tumors mimicking disseminated Pagetoid Reticulosis; metastatic spread to unusual sites, such as the lung, testis, central nervous system, and oral cavity, but not to the lymph nodes; and an aggressive course (median survival, 32 months). Histologically, these lymphomas were characterized by band-like infiltrates consisting of pleomorphic T cells or immunoblasts, showing a diffuse infiltration of an acanthotic epidermis with variable degrees of spongiosis, intraepidermal blistering, and necrosis. The neoplastic cells showed a high Ki-67 proliferation index and expression of CD3, CD8, CD7, CD45RA, βF1, and TIA-1 markers, whereas CD2 and CD5 were frequently lost. Expression of TIA-1 pointed out that these lymphomas are derived from a cytotoxic T cell subset. The results of this and other studies reviewed herein suggest that these strongly epidermotropic primary cutaneous CD8+ cytotoxic T cell lymphomas represent a distinct type of CTCL with an aggressive clinical behavior.
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primary cutaneous γ δ t cell lymphoma presenting as disseminated Pagetoid Reticulosis
Journal of Investigative Dermatology, 1991Co-Authors: E Berti, E Alessi, A Cerri, S Cavicchini, Domenico Delia, D Soligo, R CaputoAbstract:Abstract The first case of primary γ/δ cutaneous T-cell lymphoma (CTCL) with a fatal outcome is reported. The patient had the clinical and histopathologic features of disseminated page told Reticulosis, a rare form of CTCL characterized by a strong epidermotropic lymphoid infiltrate. Extensive immuno-cytochemical studies showed that the neoplastic cells were almost exclusively localized in the epidermis, expressed the γ/δ variant of the T-cell receptor (CD3 + , TCR-δ-1 + ) and were CD5 + , CD7 + , CD27 + , CD29 + , CD43 + , CD44 + , CD45 + , CD45RA + , CD54 + , CD69 + , but βF1 − , Tiγa − , BB3 − , A13 − , CD2 − , CD4 − , CD8 − , CD11a − , CD49d − , CD25 − , CD30 − , and HLA-DR − . A comparison of our results with those of the literature, which have not included γ/δ T-cell receptor analysis, suggests that some reported cases of Pagetoid Reticulosis may have phenotypes similar to our case. Electron microscopy studies demonstrated that the γ/δ T lymphocytes were villous, containing dense and multivesicular bodies, and formed close contacts with the surrounding keratinocytes, suggesting that these cells should have a role in the skin-associated lymphoid tissue. The proliferating cells in our case might represent the neoplastic counterpart of the recently reported CD2 − subset of normal human peripheral blood γ/δ T lymphocytes.
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primary cutaneous γ δ t cell lymphoma presenting as disseminated Pagetoid Reticulosis
Journal of Investigative Dermatology, 1991Co-Authors: E Berti, E Alessi, A Cerri, S Cavicchini, Domenico Delia, D Soligo, R CaputoAbstract:The first case of primary gamma/delta cutaneous T-cell lymphoma (CTCL) with a fatal outcome is reported. The patient had the clinical and histopathologic features of disseminated Pagetoid Reticulosis, a rare form of CTCL characterized by a strong epidermotropic lymphoid infiltrate. Extensive immuno-cytochemical studies showed that the neoplastic cells were almost exclusively localized in the epidermis, expressed the gamma/delta variant of the T-cell receptor (CD3+, TCR-delta-1+) and were CD5+, CD7+, CD27+, CD29+, CD43+, CD44+, CD45+, CD45RA+, CD54+, CD69+, but beta F1-, Ti gamma a-, BB3-, A13-, CD2-, CD4-, CD8-, CD11a-, CD49d-, CD25-, CD30-, and HLA-DR-. A comparison of our results with those of the literature, which have not included gamma/delta T-cell receptor analysis, suggests that some reported cases of Pagetoid Reticulosis may have phenotypes similar to our case. Electron microscopy studies demonstrated that the gamma/delta T lymphocytes were villous, containing dense and multivesicular bodies, and formed close contacts with the surrounding keratinocytes, suggesting that these cells should have a role in the skin-associated lymphoid tissue. The proliferating cells in our case might represent the neoplastic counterpart of the recently reported CD2- subset of normal human peripheral blood gamma/delta T lymphocytes.
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Primary cutaneous gamma/delta T-cell lymphoma presenting as disseminated Pagetoid Reticulosis
'Wiley', 1991Co-Authors: E Berti, E Alessi, A Cerri, S Cavicchini, Domenico Delia, D Soligo, R CaputoAbstract:The first case of primary \u3b3/\u3b4 cutaneous T-cell lymphoma (CTCL) with a fatal outcome is reported. The patient had the clinical and histopathologic features of disseminated page told Reticulosis, a rare form of CTCL characterized by a strong epidermotropic lymphoid infiltrate. Extensive immuno-cytochemical studies showed that the neoplastic cells were almost exclusively localized in the epidermis, expressed the \u3b3/\u3b4 variant of the T-cell receptor (CD3+, TCR-\u3b4-1+) and were CD5+, CD7+, CD27+, CD29+, CD43+, CD44+, CD45+, CD45RA+, CD54+, CD69+, but \u3b2F1-, Ti\u3b3a-, BB3-, A13-, CD2-, CD4-, CD8-, CD11a-, CD49d-, CD25-, CD30-, and HLA-DR-. A comparison of our results with those of the literature, which have not included \u3b3/\u3b4 T-cell receptor analysis, suggests that some reported cases of Pagetoid Reticulosis may have phenotypes similar to our case. Electron microscopy studies demonstrated that the \u3b3/\u3b4 T lymphocytes were villous, containing dense and multivesicular bodies, and formed close contacts with the surrounding keratinocytes, suggesting that these cells should have a role in the skin-associated lymphoid tissue. The proliferating cells in our case might represent the neoplastic counterpart of the recently reported CD2-subset of normal human peripheral blood \u3b3/\u3b4 T lymphocytes
Tokio Nakada - One of the best experts on this subject based on the ideXlab platform.
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autopsy case of cd4 cd8 cutaneous t cell lymphoma presenting disseminated Pagetoid Reticulosis with aggressive granulomatous invasion to the lungs and pancreas
Pathology International, 2005Co-Authors: Eisuke Shiozawa, Toshiko Yamochionizuka, Bungo Saito, Emi Takaba, Masafumi Iijima, Mari Shibata, Tokio Nakada, Akira Shiokawa, Michiaki TakimotoAbstract:Pagetoid Reticulosis is a rare cutaneous T-cell lymphoma with striking epidermotropism similar to that present in Paget's disease. There are two forms of Pagetoid Reticulosis: localized and disseminated. Reported herein is an autopsy case of disseminated Pagetoid Reticulosis with CD4–/CD8– phenotype T cells and massive invasion of the lungs and pancreas. The abnormal cells in the epidermis expressed a protein derived from a rearranged T-cell receptor β gene, and this feature was used to confirm the monoclonality of these cells by polymerase chain reaction. At present, the World Health Organization (WHO) classification system considers Pagetoid Reticulosis to be an indolent form of primary cutaneous T-cell lymphoma and a variant of mycosis fungoides/Sezary syndrome with prominent epidermotropism. Some differences have been observed between Pagetoid Reticulosis and mycosis fungoides in terms of clinical course, tumor cell phenotype, and genetic findings; and these differences are highlighted in the present case. The relation between disseminated Pagetoid Reticulosis, CD4–/CD8– cutaneous T-cell lymphoma, and γδ T-cell lymphoma, including whether Pagetoid Reticulosis is a variant of mycosis fungoides, remains unclear.
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autopsy case of cd4 cd8 cutaneous t cell lymphoma presenting disseminated Pagetoid Reticulosis with aggressive granulomatous invasion to the lungs and pancreas
Pathology International, 2005Co-Authors: Eisuke Shiozawa, Toshiko Yamochionizuka, Bungo Saito, Emi Takaba, Masafumi Iijima, Mari Shibata, Tokio Nakada, Akira Shiokawa, Masafumi Takimoto, Hidekazu OtaAbstract:Pagetoid Reticulosis is a rare cutaneous T-cell lymphoma with striking epidermotropism similar to that present in Paget's disease. There are two forms of Pagetoid Reticulosis: localized and disseminated. Reported herein is an autopsy case of disseminated Pagetoid Reticulosis with CD4(-)/CD8(-) phenotype T cells and massive invasion of the lungs and pancreas. The abnormal cells in the epidermis expressed a protein derived from a rearranged T-cell receptor beta gene, and this feature was used to confirm the monoclonality of these cells by polymerase chain reaction. At present, the World Health Organization (WHO) classification system considers Pagetoid Reticulosis to be an indolent form of primary cutaneous T-cell lymphoma and a variant of mycosis fungoides/Sezary syndrome with prominent epidermotropism. Some differences have been observed between Pagetoid Reticulosis and mycosis fungoides in terms of clinical course, tumor cell phenotype, and genetic findings; and these differences are highlighted in the present case. The relation between disseminated Pagetoid Reticulosis, CD4(-)/CD8(-) cutaneous T-cell lymphoma, and gammadelta T-cell lymphoma, including whether Pagetoid Reticulosis is a variant of mycosis fungoides, remains unclear.
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disseminated Pagetoid Reticulosis ketron goodman disease six year follow up
Journal of The American Academy of Dermatology, 2002Co-Authors: Tokio Nakada, Hirohiko Sueki, Masafumi IijimaAbstract:Pagetoid Reticulosis consists of 2 types: Woringer-Kolopp and Ketron-Goodman disease (K-G). Compared with the former, K-G may have disseminated lesions and a guarded prognosis. We encountered a case of K-G in a 67-year-old man with disseminated plaques on the neck, the trunk, and both extremities. Histologic specimens demonstrated medium- to large-sized, atypical cells infiltrating in the lower epidermis. The phenotype (CD3(+), CD4(-), CD8(-), CD45RO(-), CD45RA(+)) and ultrastructural findings suggest that these cells were immature T cells. Although PUVA was initially effective, new plaques in which atypical cells are still present histologically, have appeared through a 6-year follow-up. Literature review revealed the high rate of recurrence. These findings suggest that K-G is an epidermotropic or immature T-cell variant of mycosis fungoides. In patients with K-G, therefore, long-term observation is necessary: Disappearance of cutaneous lesions may not mean cure.
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disseminated Pagetoid Reticulosis ketron goodman disease six year follow up
Journal of The American Academy of Dermatology, 2002Co-Authors: Tokio Nakada, Hirohiko Sueki, Masafumi IijimaAbstract:Abstract Pagetoid Reticulosis consists of 2 types: Woringer-Kolopp and Ketron-Goodman disease (K-G). Compared with the former, K-G may have disseminated lesions and a guarded prognosis. We encountered a case of K-G in a 67-year-old man with disseminated plaques on the neck, the trunk, and both extremities. Histologic specimens demonstrated medium- to large-sized, atypical cells infiltrating in the lower epidermis. The phenotype (CD3 + , CD4 − , CD8 − , CD45RO − , CD45RA + ) and ultrastructural findings suggest that these cells were immature T cells. Although PUVA was initially effective, new plaques in which atypical cells are still present histologically, have appeared through a 6-year follow-up. Literature review revealed the high rate of recurrence. These findings suggest that K-G is an epidermotropic or immature T-cell variant of mycosis fungoides. In patients with K-G, therefore, long-term observation is necessary: Disappearance of cutaneous lesions may not mean cure. (J Am Acad Dermatol 2002;47:S183-6.)
R Caputo - One of the best experts on this subject based on the ideXlab platform.
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primary cutaneous γ δ t cell lymphoma presenting as disseminated Pagetoid Reticulosis
Journal of Investigative Dermatology, 1991Co-Authors: E Berti, E Alessi, A Cerri, S Cavicchini, Domenico Delia, D Soligo, R CaputoAbstract:Abstract The first case of primary γ/δ cutaneous T-cell lymphoma (CTCL) with a fatal outcome is reported. The patient had the clinical and histopathologic features of disseminated page told Reticulosis, a rare form of CTCL characterized by a strong epidermotropic lymphoid infiltrate. Extensive immuno-cytochemical studies showed that the neoplastic cells were almost exclusively localized in the epidermis, expressed the γ/δ variant of the T-cell receptor (CD3 + , TCR-δ-1 + ) and were CD5 + , CD7 + , CD27 + , CD29 + , CD43 + , CD44 + , CD45 + , CD45RA + , CD54 + , CD69 + , but βF1 − , Tiγa − , BB3 − , A13 − , CD2 − , CD4 − , CD8 − , CD11a − , CD49d − , CD25 − , CD30 − , and HLA-DR − . A comparison of our results with those of the literature, which have not included γ/δ T-cell receptor analysis, suggests that some reported cases of Pagetoid Reticulosis may have phenotypes similar to our case. Electron microscopy studies demonstrated that the γ/δ T lymphocytes were villous, containing dense and multivesicular bodies, and formed close contacts with the surrounding keratinocytes, suggesting that these cells should have a role in the skin-associated lymphoid tissue. The proliferating cells in our case might represent the neoplastic counterpart of the recently reported CD2 − subset of normal human peripheral blood γ/δ T lymphocytes.
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primary cutaneous γ δ t cell lymphoma presenting as disseminated Pagetoid Reticulosis
Journal of Investigative Dermatology, 1991Co-Authors: E Berti, E Alessi, A Cerri, S Cavicchini, Domenico Delia, D Soligo, R CaputoAbstract:The first case of primary gamma/delta cutaneous T-cell lymphoma (CTCL) with a fatal outcome is reported. The patient had the clinical and histopathologic features of disseminated Pagetoid Reticulosis, a rare form of CTCL characterized by a strong epidermotropic lymphoid infiltrate. Extensive immuno-cytochemical studies showed that the neoplastic cells were almost exclusively localized in the epidermis, expressed the gamma/delta variant of the T-cell receptor (CD3+, TCR-delta-1+) and were CD5+, CD7+, CD27+, CD29+, CD43+, CD44+, CD45+, CD45RA+, CD54+, CD69+, but beta F1-, Ti gamma a-, BB3-, A13-, CD2-, CD4-, CD8-, CD11a-, CD49d-, CD25-, CD30-, and HLA-DR-. A comparison of our results with those of the literature, which have not included gamma/delta T-cell receptor analysis, suggests that some reported cases of Pagetoid Reticulosis may have phenotypes similar to our case. Electron microscopy studies demonstrated that the gamma/delta T lymphocytes were villous, containing dense and multivesicular bodies, and formed close contacts with the surrounding keratinocytes, suggesting that these cells should have a role in the skin-associated lymphoid tissue. The proliferating cells in our case might represent the neoplastic counterpart of the recently reported CD2- subset of normal human peripheral blood gamma/delta T lymphocytes.
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Primary cutaneous gamma/delta T-cell lymphoma presenting as disseminated Pagetoid Reticulosis
'Wiley', 1991Co-Authors: E Berti, E Alessi, A Cerri, S Cavicchini, Domenico Delia, D Soligo, R CaputoAbstract:The first case of primary \u3b3/\u3b4 cutaneous T-cell lymphoma (CTCL) with a fatal outcome is reported. The patient had the clinical and histopathologic features of disseminated page told Reticulosis, a rare form of CTCL characterized by a strong epidermotropic lymphoid infiltrate. Extensive immuno-cytochemical studies showed that the neoplastic cells were almost exclusively localized in the epidermis, expressed the \u3b3/\u3b4 variant of the T-cell receptor (CD3+, TCR-\u3b4-1+) and were CD5+, CD7+, CD27+, CD29+, CD43+, CD44+, CD45+, CD45RA+, CD54+, CD69+, but \u3b2F1-, Ti\u3b3a-, BB3-, A13-, CD2-, CD4-, CD8-, CD11a-, CD49d-, CD25-, CD30-, and HLA-DR-. A comparison of our results with those of the literature, which have not included \u3b3/\u3b4 T-cell receptor analysis, suggests that some reported cases of Pagetoid Reticulosis may have phenotypes similar to our case. Electron microscopy studies demonstrated that the \u3b3/\u3b4 T lymphocytes were villous, containing dense and multivesicular bodies, and formed close contacts with the surrounding keratinocytes, suggesting that these cells should have a role in the skin-associated lymphoid tissue. The proliferating cells in our case might represent the neoplastic counterpart of the recently reported CD2-subset of normal human peripheral blood \u3b3/\u3b4 T lymphocytes
Sabine Kohler - One of the best experts on this subject based on the ideXlab platform.
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clinicopathologic features and treatment outcomes in woringer kolopp disease
Journal of The American Academy of Dermatology, 2008Co-Authors: Natalie Viakhireva, Sabine Kohler, Christine Cesca, Richard T HoppeAbstract:Woringer-Kolopp disease, also known as Pagetoid Reticulosis, is an exceedingly rare variant of mycosis fungoides. Accurate diagnosis and effective treatment is essential to prevent progression to debilitating disease. We identified 7 patients with Woringer-Kolopp disease treated at our institution. We review the major clinical and pathologic characteristics of this disease, focusing on treatment strategies and patient outcomes. All of our patients were successfully treated with skin-directed therapies including topical steroids, topical nitrogen mustard, psoralen plus ultraviolet A, narrow-band ultraviolet B, and radiation therapy. Our observations confirm that Woringer-Kolopp disease carries an excellent prognosis, and support that the most effective and appropriate treatment for recalcitrant or severe Woringer-Kolopp disease is localized radiation therapy.
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Pagetoid Reticulosis woringer kolopp disease an immunophenotypic molecular and clinicopathologic study
Modern Pathology, 2000Co-Authors: Bijan Haghighi, Roger A. Warnke, Phillip E Leboit, Christian A Sander, Bruce R Smoller, Sabine KohlerAbstract:Pagetoid Reticulosis (Woringer-Kolopp Disease): An Immunophenotypic, Molecular, and Clinicopathologic Study
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Pagetoid Reticulosis woringer kolopp disease an immunophenotypic molecular and clinicopathologic study
Modern Pathology, 2000Co-Authors: Bijan Haghighi, Roger A. Warnke, Phillip E Leboit, Christian A Sander, Bruce R Smoller, Sabine KohlerAbstract:Pagetoid Reticulosis (PR), also known as Woringer-Kolopp disease, is a form of cutaneous T-cell lymphoma that demonstrates striking epidermotropism on histologic examination. We present the histologic, immunologic, and molecular findings for seven patients who had PR. The patients ranged in age from 33 to 67 years. All patients presented with one or several thick plaques involving the distal extremities except for one patient, who presented with a tongue lesion. Immunohistochemical staining of the atypical lymphoid cells demonstrated a T-cell phenotype in all cases. In one of four frozen cases, the neoplastic cells were of T-helper cell phenotype (CD4 positive). Four of seven cases demonstrated a T-cytotoxic/suppressor cell phenotype (CD8 positive). The T-cell subset for the remaining two cases could not be determined. CD30 positivity and a high growth fraction as indicated by staining with Ki-67 were seen in three of seven and three of four cases, respectively. Genotypic analysis performed on three of our cases revealed T-cell receptor (gamma and/or beta) rearrangement, indicating a clonal proliferation. The clinical follow-up ranged from 15 months to 13 years. Four of seven patients are alive and free of disease after treatment with excision or local irradiation. One patient relapsed twice after treatment with radiation and photochemotherapy with 8-methoxypsoralen and UVA and was then lost to follow-up. The lesions of another patient resolved spontaneously but recurred at the same and in an additional site 5 years later. One patient recurred after electron beam therapy. The recurrent lesion improved with radiation therapy and local wound care but never resolved completely. The patient died of unrelated causes. Our findings suggest that PR is a distinct clinicopathologic entity, separate from unilesional mycosis fungoides, demonstrating a slow disease course. The disease is a clonal cutaneous T-cell lymphoma with relatively consistent clinical and histopathologic findings but a heterogeneous immunophenotypic profile.
