Presentation
To our knowledge, the present case seems to be the first description of pagetoid reticulosis presenting at the site of a previously treated dermal anaplastic large cell lymphoma. [ncbi.nlm.nih.gov]
Entire Body System
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Rigor
As a scientist by training with an emphasis on targeted cancer therapies, Siegall built Seattle Genetics on a foundation of scientific innovation, rigorous research and drug development practices as well as a passion for helping patients. [adcreview.com]
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Soft Tissue Mass
Computed tomography (CT) scan showed the presence of soft tissue mass with irregular outlines arising from the skin. There was no nodal or any other organ involvement in the body. [jcytol.org]
Immune System
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Axillary Lymphadenopathy
There was no cervical and axillary lymphadenopathy and hepatosplenomegaly. The other systemic examinations were unremarkable. Routine investigations revealed a normal hemogram and urine examination. [e-ijd.org]
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Axillary Pain
In this report, we present an unusual case of an 8-year-old boy with a noduloulcerativelesion present on the upper back with axillary painful lymphadenopathy, initially mimicking as scrofuloderma which was later histopathologically and immunohistochemically [ijpd.in]
Skin
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Pruritic Rash
The lesions began as eczema located in upper and lower limbs that have evolved to a widespread scaly and quite pruritic rash with papules and nodules which ulcerated and spontaneously regressed, leaving permanent hypochromic stains ( Figures 1 and 2 ) [scielo.br]
Workup
A systemic workup of the patient excluded other potential causes. The neonate was treated with wide excision, but chemotherapy or radiation therapy was not administered, as the patient's parents did not consent to such treatment. [ncbi.nlm.nih.gov]
Distinguishing primary cutaneous anaplastic lymphoma (PC-ALCL) from its systemic counterpart requires a complete clinical and laboratory workup. [cancerjournal.net]
Histologic examination is the first diagnostic step in the diagnostic workup of clinically suspected CD30 LPDs. [bloodjournal.org]
Microbiology
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Human Papillomavirus
Detection of human herpesvirus 6 and human papillomavirus 16 in cervical carcinoma. Am J Pathol. 1994;145:1509–16. [infectagentscancer.biomedcentral.com]
Treatment
There are several perils and pitfalls that exist regarding the differential diagnosis, the possible progression, and the treatment of PC-ALCL. [ncbi.nlm.nih.gov]
Management and treatment If lesions are solitary or localized to a single area, excision and/or radiation therapy is performed. [orpha.net]
Prognosis
Nevertheless, PC-ALCL must be distinguished from secondary skin lesions in systemic ALCL, which confer a poor prognosis, and other CD30 PCLPDs, reactive conditions, or borderline cases. [ncbi.nlm.nih.gov]
Prognosis The prognosis is generally good, with a five-year survival rate of 90%. In 25% of cases, spontaneous regression is observed. Relapses, generally without any extracutaneous dissemination, may sometimes occur (approximately in 30% of cases). [orpha.net]
Etiology
Etiology Etiology is unknown. [orpha.net]
The systemic and primary cutaneous types of anaplastic large cell lymphomas have been differentiated on clinical and immunophenotypical findings, but further research is required to elucidate their exact etiologies and pathogeneses. [ncbi.nlm.nih.gov]
Background: The etiology of primary cutaneous anaplastic large-cell CD30 lymphoma is largely unknown, and although an infectious involvement has been suspected, the implication of infectious agents in its pathogenesis is still unclear. [adolfosebastiani.it]
[…] cutaneous anaplastic large cell lymphoma (C-ALCL) Synonyms Primary cutaneous CD30( ) T-cell lymphoproliferative disorder This term also includes lymphomatoid papulosis Definitions Cutaneous lymphoma composed of large T cells that express CD30 ( 75%) ETIOLOGY [basicmedicalkey.com]
Epidemiology
Summary Epidemiology The prevalence of primary C-ALCL is unknown but it accounts for approximately 9% of cutaneous lymphomas. The male/female ratio is of 3:2. [orpha.net]
Additional cases are needed to delineate the epidemiology of this rare entity: you are welcome to submit a paper to our new Case Report section. Genetic alterations in primary cutaneous CD30 anaplastic large cell lymphoma. [atlasgeneticsoncology.org]
pleomorphic or immunoblastic cytomorphology that express the CD30 antigen by the majority ( 75%) of the tumor cells ( WHO 2008 ) Terminology Also called regressing atypical histiocytosis (European Organisation for Research and Treatment of Cancer) Epidemiology [pathologyoutlines.com]
Epidemiology and Mortality Age: 60 years median age (can occur in children, although rare) Race: female predominance Sources Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds): WHO Classification of Tumours of Haematopoietic [seer.cancer.gov]
[…] immunosuppressive drugs Gene expression profiling has failed to show genes that clearly distinguish C-ALCL from ALK(-) systemic ALCL Increased expression of skin-homing chemokine receptors may play a role in confining C-ALCL to skin CLINICAL ISSUES Epidemiology [basicmedicalkey.com]
Pathophysiology
Pathophysiology ALK positive and ALK negative ALCL have different gene expression profiles and seem to be biologically distinct entities. The pathophysiology of both subtypes of ALCL is incompletely understood. [cancertherapyadvisor.com]
Prevention
As such histopathologic findings often represent close mimickers of neoplastic and infectious etiologies, knowledge of the potential for systemic manifestations of taxane therapy in patients treated topically may prevent potentially costly diagnostic [ncbi.nlm.nih.gov]
How can Primary Cutaneous Anaplastic Large Cell Lymphoma be Prevented? Presently, the cause of Primary Cutaneous Anaplastic Large Cell Lymphoma is unknown and there are no known methods to prevent its formation. [dovemed.com]
None of the available therapies for LYP appears unequivocally effective in preventing LYP-associated second lymphomas, although this point has not been systematically addressed in prospective studies. [bloodjournal.org]
Ruling out these conditions will prevent unnecessarily aggressive treatment for primary cutaneous CD30 LCL, an indolent disease with a very favourable prognosis. [jpma.org.pk]
A good skincare regime with regular use of moisturisers helps to prevent dryness and irritation. If you have stage 1 disease, this may be all you need. [lymphomas.org.uk]