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This neoplasm is not reportable.
Name
ICD-O-3 Morphology
Effective
2021 and later
Reportability
This neoplasm is not reportable
Primary Site(s)
No primary site specified
Cutaneous primary sites only (C440-C449, C510-C512, C518-C519, C600-C602, C608-C609, C632)
This hematopoietic disease is not reportable for the U.S.
Canada, refer to your specific province for reportability requirements.
This hematopoietic disease is not reportable for the U.S.
Canada, refer to your specific province for reportability requirements.
Abstractor Notes
Primary cutaneous CD4 positive small or medium T-cell lymphoproliferative disorder (PCSM-LPD) is part of the Mature T-cell and NK-cell neoplasms lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B20)
These lesions usually present as a solitary plaque or nodule, most commonly on the face, neck, or upper trunk. Patients are asymptomatic, a single slow growing skin lesion is the sole manifestation of this disease.
These lesions usually present as a solitary plaque or nodule, most commonly on the face, neck, or upper trunk. Patients are asymptomatic, a single slow growing skin lesion is the sole manifestation of this disease.
Alternate Names
Primary cutaneous CD4 positive small/medium T-cell lymphoma [OBS]
Definition
Primary cutaneous CD4-positive small or medium T-cell lymphoproliferative disorder (PCSM-LPD) is characterized by a predominance of small to medium-sized CD4-positive pleomorphic T cells within a solitary skin lesion, without evidence of the plaques typical of mycosis fungoides. (WHO 5th edition)
Genetics Data
This data item does not apply
Immunophenotyping
This data item does not apply
Corresponding ICD-10 Codes (Cause of Death codes only)
D47.7 Other specified neoplasms of uncertain or unknown behavior of lymphoid, hematopoietic, and related tissue
Corresponding ICD-10-CM Codes (U.S. only)
D47.Z9 Other specified neoplasms of uncertain behavior of lymphoid, hematopoietic and related tissue (effective October 01, 2015)
Epidemiology and Mortality
Sources
WHO Classification of Tumours Editorial Board. Haematolymphoid tumours. Lyon (France): International Agency for Research on Cancer; 2024. (WHO classification of tumours series, 5th ed.; vol. 11). https://publications.iarc.who.int/637.
Section: Mature T-cell and NK-cell neoplasms
Pages: 401-402
Section: Mature T-cell and NK-cell neoplasms
Pages: 401-402
International Classification of Diseases for Oncology, 3rd edition (including revisions). Geneva: World Health Organization, 2020.
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577
PDQ® Adult Treatment Editorial Board. PDQ Mycosis Fungoides and Other Cutaneous T-Cell Lymphomas Treatment. Bethesda, MD: National Cancer Institute. Updated <02/19/2025>. Available at: https://www.cancer.gov/types/lymphoma/hp/mycosis-fungoides-treatment-pdq. Accessed <03/31/2025>. [PMID: 2638928
Section: Mycosis Fungoides and Other Cutaneous T-Cell Lymphomas Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/lymphoma/hp/mycosis-fungoides-treatment-pdq
Section: Mycosis Fungoides and Other Cutaneous T-Cell Lymphomas Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/lymphoma/hp/mycosis-fungoides-treatment-pdq
