We hope to increase awareness of this rare CD30-positive lymphoproliferative disorder subtype by better defining the clinical spectrum in which this entity can present. [ncbi.nlm.nih.gov]

• Anorexia

  The most common adverse effects (AE) include gastrointestinal disturbance such as nausea and anorexia, hepatotoxicity and hematological toxicity. [ispub.com]

• Loss of Appetite

  General symptoms, usually present in cases with extracutaneous involvement, include loss of appetite, weight loss, fatigue, and night sweats (B symptoms). Etiology Etiology is unknown. [orpha.net]

  You may also have symptoms like: Fatigue Fever Loss of appetite Night sweats Weight loss With cutaneous ALCL, you may first notice one or more raised, red bumps on the skin that don't go away. These are tumors. [webmd.com]

• Ulcer

  We present a case of primary cutaneous anaplastic large cell lymphoma manifesting with widespread erythema around an ulcer. [ncbi.nlm.nih.gov]

• Skin Lesion

  Distinction was made between patients first presenting with solitary or localized lesions (n=63), with multifocal skin lesions (n=6), and patients with a skin relapse (n=22). Radiation doses, treatment response, and follow-up were evaluated. [ncbi.nlm.nih.gov]

  The staging of PCALCL is based on a TNM system : T: T1 Only a single spot or area of the skin is involved. T2 Multiple skin lesions in one area or adjacent areas of the body. [clfoundation.org]

• Eruptions

  To our knowledge, this is the first case of a generalized CD8+, CD30+ eruption with features of both mycosis fungoides and primary cutaneous ALCL arising following an episode of solitary primary cutaneous CD8-positive ALCL. [ncbi.nlm.nih.gov]

  Case 113 of the workshop is a characteristic example of such a lymphomatoid drug eruption resulting from phenytoin. In most cases, however, the cause is unknown. [academic.oup.com]

  FIGURE 1 Widespread scaly eruption with ulcerated nodules and scarring hypochromic spots FIGURE 2 Lesions on scalp She did outpatient treatment with a specialist since the onset of disease, but she only received a definitive diagnosis in 2007 (after six [scielo.br]

• Erythema

  A 63-year-old Japanese woman was referred to our hospital with a red nodule and indurated erythema in 1997. [ncbi.nlm.nih.gov]

  Case 213 had a 10-year history of recurrent self-remitting subcutaneous nodules clinically diagnosed as erythema nodosum before a diagnosis of PCGD-TCL with predominantly panniculitis-like involvement of subcutis and deep dermis was made. [academic.oup.com]

• Skin Disease

  Patients with widespread systemic and cutaneous disease at first presentation should be considered to have the systemic form with skin involvement. [emedicine.medscape.com]

  The disease recurs in the skin in 39% of the cases. [academic.oup.com]

• Withdrawn

  Skin and tissue over the bone is first numbed with local anesthetic, and a needle is inserted into the bone through which a bone marrow sample is withdrawn. The sample is then examined under a microscope by a pathologist. [rarediseases.org]

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]

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]

Differential diagnosis and treatment of primary, cutaneous, anaplastic large cell lymphoma: not always an easy task. Int J Hematol. 2009;90:226-9. [scielo.br]

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]

Usually favorable prognosis (10 year disease related survival of 90%) May show partial or complete spontaneous regression of skin lesions (10% - 40%) Frequent skin relapses Multifocality / nodal involvement have a similar prognosis as skin only lesions [pathologyoutlines.com]

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 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]

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]

[…] 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]

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]

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 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]

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]

Any significant medical condition, laboratory abnormality, or psychiatric illness that would prevent the patient from participating in the study. 2. Any condition that confounds the ability to interpret data from the study. 3. [clinicaltrialsregister.eu]

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]