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Primary Cutaneous Lymphoma


Presentation

  • The term "primary cutaneous lymphoma" refers to cutaneous lymphomas that present in the skin with no evidence of extracutaneous disease at the time of diagnosis.[ncbi.nlm.nih.gov]
  • Case Presentation A 78–year old woman presented to the dermatology department with a 5–week history of multiple erythematous nodules on left cheek (Figure 1). These nodules did not bleed and only caused mild itching occasionally.[austinpublishinggroup.com]
Nasal Ulcer
  • Figure 2 Clinical presentation of PCAECyTCL in two different patients. ( a ) Oral and nasal ulcerated macules (Case 25). ( b ) Extensive annular erosive plaques some with hemorrhagic features (Case 25). ( c ) Annular and arcuate ulcerated plaques and[nature.com]
Lax, Wrinkled Skin
  • Granulomatous Slack Skin This variant of MF is characterized by lax, wrinkled skin masses, especially in the body folds. The groin and axilla are common sites of involvement. Erythema and pruritus of the affected areas can occur.[rarediseases.org]

Workup

  • Thus, while this review provides an overview and guideline for the workup of lymphoid lesions of the skin, the practitioner should understand the importance of clinical correlation as well as appropriate utility of available resources (such as clonality[ncbi.nlm.nih.gov]
  • Distinguishing primary cutaneous anaplastic lymphoma (PC-ALCL) from its systemic counterpart requires a complete clinical and laboratory workup.[cancerjournal.net]
  • Follow-up Patients should be seen in an outpatient setting at least every 6 months for a clinical workup. They do not require any additional laboratory workup unless enlargement of the regional lymph nodes has occurred.[emedicine.medscape.com]

Treatment

  • Five of six patients relapsed following initial treatment. Four patients were treated with primary radiation therapy, three of which had relapses outside the treatment area.[ncbi.nlm.nih.gov]
  • Based on this classification we will provide a summary of the current medical literature in diagnosis, treatment, and prognosis for primary cutaneous lymphomas with emphasis on new treatment strategies.[scholars.northwestern.edu]
  • Treatments for cutaneous lymphoma Treatment for skin lymphomas depends on how widespread the disease is.[cityofhope.org]

Prognosis

  • (associated with a poor prognosis).[orpha.net]
  • Primary cutaneous T- and B-cell lymphomas are a heterogenous group of diseases with varied clinical presentations and prognosis.[scholars.northwestern.edu]
  • Primary cutaneous diffuse large B-cell lymphoma, other Large B-cell lymphoma has an excellent prognosis. Blastic plasmacytoid dendritic cell neoplasm and in travascular large B-cell lymphoma have a poor prognosis.[dermnetnz.org]
  • Survival curves were similar to those reported in the literature, except for Sézary syndrome, which had a better prognosis in this sample. 2015 The International Society of Dermatology.[ncbi.nlm.nih.gov]
  • The prognosis of patients with MF is above all dependent on the stage of the disease [ 3 ].[annonc.oxfordjournals.org]

Etiology

  • These two cases, in consideration of the rarity of nodular lymphoma in children, promote speculative considerations, especially in regard to etiology and therapy.[ncbi.nlm.nih.gov]
  • Cutaneous T-Cell Lymphoma: Epidemiology, Etiology, and Classification. Leukemia & Lymphoma , Nov2003 Supplement 3, Vol. 44 Issue s3, pS49-S54, Burg G y otros: WHO/EORTC classification of cutaneous lymphomas 2005. Histological and molecular aspects.[iqb.es]
  • (Etiology) Lymphocytes are a type of white blood cells that are responsible for providing immunity in the human body. B-cells and T-cells are the two different types of lymphocytes.[dovemed.com]
  • Most probably it is a multifactorial and multistep process, with various etiologic factors intervening over a long period. The disease likely begins as a hyperreactive inflammatory process.[emedicine.medscape.com]

Epidemiology

  • Epidemiological data on PCLs according to the World Health Organization/European Organization for Research and Treatment of Cancer classification (WHO-EORTC) has not been investigated in Korea to date.[ncbi.nlm.nih.gov]
  • 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]
  • Herein, we review primary CTCLs, including epidemiological data, a brief summary of clinical presentations, immunophenotype, molecular signatures and differential diagnoses.[jcp.bmj.com]
Sex distribution
Age distribution

Pathophysiology

  • Pathophysiology The development of cutaneous lymphoma is only partially understood. Most probably it is a multifactorial and multistep process, with various etiologic factors intervening over a long period.[emedicine.medscape.com]

Prevention

  • United States Preventive Services Task Force Overstates Cosmetic Harms of Skin Cancer Screening by Joseph F. Sobanko, MD; Kimberly Shao, BS; Rebecca L. Pearl, PhD; and Sancy Leachman, MD PhD Dr.[jcadonline.com]
  • How can Primary Cutaneous, Small-Medium, CD4-Positive, T-Cell Lymphoma be Prevented? Currently, it is not possible to prevent Primary Cutaneous, Small-Medium, CD4-Positive, T-Cell Lymphoma.[dovemed.com]
  • […] and early detection of cervical cancer: American Cancer Society, American Society for Colposcopy and Cervical Pathology, and American Society for Clinical Pathology screening guidelines for the prevention and early detection of cervical cancer.[nccn.org]
  • Residency University of Pa Medical Ctr - Dermatology Other Education University of Pa Medical Ctr - Dermatology Internship Sinai Hospital of Baltimore - Internal Medicine Medical School McGill University - Medical Doctor Member Board American Board of Preventive[uvmhealth.org]
  • Oral pyridoxine 300 mg was administered daily from the beginning of Peg-Doxo treatment until 1 month after its discontinuation to prevent the palmar-plantar erythrodysesthesia (PPE).[haematologica.org]

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