scholarly journals How I treat primary cutaneous CD30+ lymphoproliferative disorders

Blood ◽  
2019 ◽  
Vol 134 (6) ◽  
pp. 515-524 ◽  
Author(s):  
Michi M. Shinohara ◽  
Andrei Shustov
Keyword(s):  
Cell Lymphoma ◽  
Large Cell ◽  
Lymphoproliferative Disorders ◽  
Classification Systems ◽  
Team Approach ◽  
Consensus Guidelines ◽  
Lymphomatoid Papulosis ◽  
Current Classification ◽  
Best Disease ◽  
Systemic Therapies

Abstract The primary cutaneous CD30+ lymphoproliferative disorders are a family of extranodal lymphoid neoplasms that arise from mature postthymic T cells and localize to the skin. Current classification systems recognize lymphomatoid papulosis (LyP), primary cutaneous anaplastic large cell lymphoma, and borderline cases. In the majority of patients, the prognosis of primary cutaneous CD30+ lymphoproliferative disorders is excellent; however, relapses are common, and complete cures are rare. Skin-directed and systemic therapies are used as monotherapy or in combination to achieve the best disease control and minimize overall toxicity. We discuss 3 distinct presentations of primary cutaneous CD30+ lymphoproliferative disorder and present recommendations for a multidisciplinary team approach to diagnosis, evaluation, and management of these conditions in keeping with existing consensus guidelines.

scholarly journals Lack of the t(2;5) or other mutations resulting in expression of anaplastic lymphoma kinase catalytic domain in CD30+ primary cutaneous lymphoproliferative disorders and Hodgkin's disease

Blood ◽  
1996 ◽  
Vol 88 (5) ◽  
pp. 1765-1770 ◽  
Author(s):  
GS Wood ◽  
DL Hardman ◽  
R Boni ◽  
R Dummer ◽  
YH Kim ◽  
...  
Keyword(s):  
Hodgkin's Disease ◽  
Catalytic Domain ◽  
Cell Lymphoma ◽  
Hodgkin’S Disease ◽  
Large Cell ◽  
Lymphoproliferative Disorders ◽  
Lymphomatoid Papulosis ◽  
Anaplastic Lymphoma ◽  
Large Cell Lymphoma ◽  
Inappropriate Expression

The t(2;5) (p23;q35) chromosomal translocation has been found in a high proportion of lymph node-based CD30+ large cell lymphomas of T-cell lineage. This translocation is believed to result in the expression of a fusion protein containing the catalytic domain of anaplastic lymphoma kinase (ALK) under the control of the promoter for nucleophosmin, a nucleolar phosphoprotein. Expression of ALK activity, which does not normally occur in lymphocytes, is postulated to be involved in the pathogenesis of lymphomas bearing the t(2;5) translocation. Several primary cutaneous lymphoproliferative disorders and Hodgkin's disease are also known to contain CD30+ large lymphoid cells. To determine the role of the t(2;5) translocation in these diseases, we developed a DNA- based polymerase chain reaction (PCR)/Southern blot assay to detect this translocation at the genomic level in lymphomatoid papulosis (14 cases), primary cutaneous CD30+ large cell lymphoma of T-lineage (10 cases) and Hodgkin's disease (13 cases). Two cases of pityriasis lichenoides were also studied. The t(2;5) translocation was not present in any of these specimens. To determine if some other somatic mutation might have resulted in inappropriate expression of ALK catalytic domain, we devised an RNA-based reverse transcriptase-PCR assay to detect transcripts encoded by this ALK region. None were found in the six additional cases of lymphomatoid papulosis that were studied. In aggregate, these results strongly suggest that inappropriate expression of ALK is not involved in the pathogenesis of these CD30+ lymphoproliferative disorders, and that lymph node-based CD30+ large cell lymphoma is a disease that is biologically distinct from skin- based CD30+ lymphoproliferative disorders and Hodgkin's disease. Using methods developed for this report, we also cloned and sequenced the t(2;5) genomic junctional sequences present in the SUP-M2 and SU-DHL-1 cell lines. These intron sequences will be useful for mapping t(2;5) breakpoint clusters.

scholarly journals EORTC, ISCL, and USCLC consensus recommendations for the treatment of primary cutaneous CD30-positive lymphoproliferative disorders: lymphomatoid papulosis and primary cutaneous anaplastic large-cell lymphoma*

Blood ◽  
2011 ◽  
Vol 118 (15) ◽  
pp. 4024-4035 ◽  
Author(s):  
Werner Kempf ◽  
Katrin Pfaltz ◽  
Maarten H. Vermeer ◽  
Antonio Cozzio ◽  
Pablo L. Ortiz-Romero ◽  
...  
Keyword(s):  
Anaplastic Large Cell Lymphoma ◽  
Cell Lymphoma ◽  
Large Cell ◽  
The United States ◽  
Lymphoproliferative Disorders ◽  
Cutaneous Lymphoma ◽  
European Organization ◽  
Lymphomatoid Papulosis ◽  
Cutaneous Lymphomas ◽  
Large Cell Lymphoma

AbstractPrimary cutaneous CD30+ lymphoproliferative disorders (CD30+ LPDs) are the second most common form of cutaneous T-cell lymphomas and include lymphomatoid papulosis and primary cutaneous anaplastic large-cell lymphoma. Despite the anaplastic cytomorphology of tumor cells that suggest an aggressive course, CD30+ LPDs are characterized by an excellent prognosis. Although a broad spectrum of therapeutic strategies has been reported, these have been limited mostly to small retrospective cohort series or case reports, and only very few prospective controlled or multicenter studies have been performed, which results in a low level of evidence for most therapies. The response rates to treatment, recurrence rates, and outcome have not been analyzed in a systematic review. Moreover, international guidelines for staging and treatment of CD30+ LPDs have not yet been presented. Based on a literature analysis and discussions, recommendations were elaborated by a multidisciplinary expert panel of the Cutaneous Lymphoma Task Force of the European Organization for Research and Treatment of Cancer, the International Society for Cutaneous Lymphomas, and the United States Cutaneous Lymphoma Consortium. The recommendations represent the state-of-the-art management of CD30+ LPDs and include definitions for clinical endpoints as well as response criteria for future clinical trials in CD30+ LPDs.

scholarly journals The t(2;5) chromosomal translocation is not a common feature of primary cutaneous CD30+ lymphoproliferative disorders: comparison with anaplastic large-cell lymphoma of nodal origin [see comments]

Blood ◽  
1996 ◽  
Vol 87 (8) ◽  
pp. 3437-3441 ◽  
Author(s):  
JF DeCoteau ◽  
JR Butmarc ◽  
MC Kinney ◽  
ME Kadin
Keyword(s):  
Age Of Onset ◽  
Cell Lymphoma ◽  
Chromosomal Translocation ◽  
Large Cell ◽  
Borderline Case ◽  
Lymphoproliferative Disorders ◽  
Response To Treatment ◽  
Lymphomatoid Papulosis ◽  
Polymerase Chain ◽  
Large Cell Lymphoma

Primary cutaneous CD30+ lymphoproliferative disorders (LPDs), including lymphomatoid papulosis (LyP), anaplastic and nonanaplastic CD30+ large- cell lymphoma, and borderline cases, comprise a clinical and histologic spectrum. Primary cutaneous and primary nodal CD30+ anaplastic large- cell lymphomas (ALCLs) are distinct clinical entities that have identical morphologic features but differ in age of onset, immunophenotype, and prognosis. It can be difficult to distinguish primary cutaneous from nodal ALCLs that secondarily involve the skin, which is important because these diseases differ significantly in response to treatment and clinical outcome. The t(2;5) chromosomal translocation is highly associated with primary CD30+ ALCL of nodal origin. The possible occurrence of t(2;5) in primary cutaneous CD30+ LPDs has not been studied extensively, and it remains to be determined if expression of this translocation can be used to distinguish primary cutaneous ALCL from nodal ALCL that secondarily involves the skin. To address these issues, we studied 43 cases of cutaneous and nodal CD30+ LPDs using reverse transcriptase-polymerase chain reaction (RT-PCR) and/or immunohistochemistry. We found no evidence for the t(2; 5) translocation in 14 cases of primary cutaneous CD30+ LPDs, which included 10 cases of LyP, three cases of primary cutaneous CD30+ ALCL, and one borderline case. These findings were in marked contrast to CD30+ ALCL of nodal origin, in which 19 of 29 (66%) cases were positive for t(2;5), including all five cases with secondary skin involvement. Our results support the hypothesis that (1) primary cutaneous CD30+ LPDs (including LyP) and primary nodal ALCL are distinct diseases that differ in clinical behavior and pathogenesis and (2) differential expression of t(2;5) can help to distinguish between primary cutaneous CD30+ LPDs and ALCL of nodal origin.

scholarly journals Lack of the t(2;5) or other mutations resulting in expression of anaplastic lymphoma kinase catalytic domain in CD30+ primary cutaneous lymphoproliferative disorders and Hodgkin's disease

Blood ◽  
1996 ◽  
Vol 88 (5) ◽  
pp. 1765-1770 ◽  
Author(s):  
GS Wood ◽  
DL Hardman ◽  
R Boni ◽  
R Dummer ◽  
YH Kim ◽  
...  
Keyword(s):  
Hodgkin's Disease ◽  
Catalytic Domain ◽  
Cell Lymphoma ◽  
Hodgkin’S Disease ◽  
Large Cell ◽  
Lymphoproliferative Disorders ◽  
Lymphomatoid Papulosis ◽  
Anaplastic Lymphoma ◽  
Large Cell Lymphoma ◽  
Inappropriate Expression

Abstract The t(2;5) (p23;q35) chromosomal translocation has been found in a high proportion of lymph node-based CD30+ large cell lymphomas of T-cell lineage. This translocation is believed to result in the expression of a fusion protein containing the catalytic domain of anaplastic lymphoma kinase (ALK) under the control of the promoter for nucleophosmin, a nucleolar phosphoprotein. Expression of ALK activity, which does not normally occur in lymphocytes, is postulated to be involved in the pathogenesis of lymphomas bearing the t(2;5) translocation. Several primary cutaneous lymphoproliferative disorders and Hodgkin's disease are also known to contain CD30+ large lymphoid cells. To determine the role of the t(2;5) translocation in these diseases, we developed a DNA- based polymerase chain reaction (PCR)/Southern blot assay to detect this translocation at the genomic level in lymphomatoid papulosis (14 cases), primary cutaneous CD30+ large cell lymphoma of T-lineage (10 cases) and Hodgkin's disease (13 cases). Two cases of pityriasis lichenoides were also studied. The t(2;5) translocation was not present in any of these specimens. To determine if some other somatic mutation might have resulted in inappropriate expression of ALK catalytic domain, we devised an RNA-based reverse transcriptase-PCR assay to detect transcripts encoded by this ALK region. None were found in the six additional cases of lymphomatoid papulosis that were studied. In aggregate, these results strongly suggest that inappropriate expression of ALK is not involved in the pathogenesis of these CD30+ lymphoproliferative disorders, and that lymph node-based CD30+ large cell lymphoma is a disease that is biologically distinct from skin- based CD30+ lymphoproliferative disorders and Hodgkin's disease. Using methods developed for this report, we also cloned and sequenced the t(2;5) genomic junctional sequences present in the SUP-M2 and SU-DHL-1 cell lines. These intron sequences will be useful for mapping t(2;5) breakpoint clusters.

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