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Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder
Other Resources UpToDate PubMed

Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder

Contributors: Kishan Pandya BS, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder (PCSM-TCLPD) is classified as a T-cell lymphoproliferative disorder rather than a true lymphoma in the 2016 World Health Organization (WHO) classification, given its indolent behavior. It can occur at any age, but it most commonly presents in the sixth and seventh decade of life, and it presents equally in men and women.

PCSM-TCLPD typically presents as a single papule, nodule, or plaque on the head, neck, or trunk. In around 25% of cases, more than one lesion is seen. Typically, this is a slow-growing lesion, but there have been cases reported where the lesion can enlarge rapidly. Disease is localized to the skin, follows a benign course, and carries an excellent prognosis.

Codes

ICD10CM:
D47.9 – Neoplasm of uncertain behavior of lymphoid, hematopoietic and related tissue, unspecified

SNOMEDCT:
788674000 – Primary cutaneous CD4 positive small/medium T-cell lymphoproliferative disorder

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Differential Diagnosis & Pitfalls

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Therapy

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References

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Last Reviewed:01/30/2022
Last Updated:02/23/2022
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Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder
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A medical illustration showing key findings of Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder : Neck, Trunk
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