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]

• Constitutional Symptom

  There were no constitutional symptoms. He was initially treated with oral antibiotics and topical steroids by a local physician as the condition was presumed to be of infectious origin, but there was no improvement. [jdrntruhs.org]

• Splenomegaly

  Hepatomegaly was present in three cases, one ALK+ ALCL and two ALK− ALCL cases, while none had splenomegaly. Bone marrow (BM) involvement was present in one case of ALK+ ALCL. [journal.sajc.org]

  Abdominal examination did not reveal any hepato-splenomegaly or abnormal mass. The scrotal examination showed both normal testes, and a small 3X2 cms ulcerative nodular lesion of right testicular skin. [jpma.org.pk]

  Close attention should be paid to lymphadenopathy (the systemic form of anaplastic large cell lymphoma has systemic lymphadenopathy other than regional lymphadenopathy associated with skin lesions), pleural effusions, pulmonary parenchymal lesions, splenomegaly [emedicine.medscape.com]

• Hepatomegaly

  Hepatomegaly was present in three cases, one ALK+ ALCL and two ALK− ALCL cases, while none had splenomegaly. Bone marrow (BM) involvement was present in one case of ALK+ ALCL. [journal.sajc.org]

• 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]

• Papule

  […] cutaneous T cell lymphoma Median age 60 years, M:F ratio 2 - 3:1, occasionally children Common form of cutaneous T cell lymphoma in HIV+ individuals Sites Trunk, face, extremities, buttocks Clinical features Solitary or localized nodules / tumors, sometimes papules [pathologyoutlines.com]

  Help me code for diagnosis year : Abstractor Notes Most patients present with solitary or localized nodule s or tumors, and sometimes papule s, and often show ulceration. Extracutaneous dissemination occurs in lymph node s. [seer.cancer.gov]

  It reaches an overall peak in the sixth decade of life and an average of 50% of cases are diagnosed in patients aged 61. 2 Most patients present with solitary or localized nodules, papules or plaques. [scielo.br]

  […] of skin-homing chemokine receptors may play a role in confining C-ALCL to skin CLINICAL ISSUES Epidemiology Age Median: 60 years Gender M:F = 2-3:1 Site Common sites: Face, trunk, and extremities Presentation Solitary nodule or localized nodules or papules [basicmedicalkey.com]

  A subset of patients with transformed mycosis fungoides (T-MF) also has either LyP papules or tumors expressing CD30 ( 6, 7 ). [clincancerres.aacrjournals.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]

• Alopecia

  General symptoms associated with Sezary syndrome include fevers, weight loss, bald patches on the scalp (alopecia), and a general feeling of ill health (malaise). [rarediseases.org]

  Vomiting, neutropenia, and alopecia were not seen, and no evidence of liver damage was observed. Infusion reactions and cytokine release syndrome, reported with other antibodies, were not observed with SGN-30. [clincancerres.aacrjournals.org]

• Skin Rash

  Elsewhere, the skin showed a widespread poikilodermatous red rash with several interspersed scaly patches that was considered clinically to be poikilodermatous MF. [academic.oup.com]

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]

• Enlargement of the Liver

  […] of the liver and/or spleen (hepatosplenomegaly). [rarediseases.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]