Primary cutaneous CD30 positive anaplastic large cell lymphoma

Signs and symptoms

Primary cutaneous anaplastic large cell lymphoma typically manifests as multifocal, solitary, or grouped nodules on the upper limbs that last for three to four weeks.

Causes

The cause is complex and multifaceted. Only a small proportion of patients have CD30+ cells in normal lymphoid tissue. These are the cells that are thought to be the precursors of systemic anaplastic large cell lymphoma. It is believed that CD30's interaction with its ligand, CD30L, mediates the disease's proliferative and anti-proliferative phases. The chemokine receptors CCR8 and CCR10, which are implicated in skin localization, are also assumed to mediate cutaneous presentation through their expression. Another theory is that Langerhans cell-mediated antigen presentation chronically activates T cells, leading to cutaneous T cell lymphomas.

Diagnosis

Primary cutaneous anaplastic large cell lymphoma is characterized histologically by large, irregular polygonal cells distributed in sheets throughout the dermis and occasionally the subcutis.

Treatment

Complete surgical resection and/or radiation therapy are used to treat primary cutaneous anaplastic large cell lymphoma as a single lesion; the majority of patients experience total remission. While radiation of the primary lesion and the surrounding lymph nodes has been advised for patients whose local lymph nodes are involved, adding radiation to surgical excision does not seem to improve localized disease.

References

References

1. (2013). "Primary cutaneous anaplastic large-cell lymphoma — case report". FapUNIFESP (SciELO).
2. (2011). "Perils and Pitfalls Regarding Differential Diagnosis and Treatment of Primary Cutaneous Anaplastic Large-Cell Lymphoma". Hindawi Limited.
3. (June 23, 2009). "Differential diagnosis and treatment of primary, cutaneous, anaplastic large cell lymphoma: not always an easy task". Springer Science and Business Media LLC.
4. (2017). "Primary cutaneous anaplastic large cell lymphoma". Journal of Cutaneous Pathology.
5. (June 26, 2019). "Primary Cutaneous Anaplastic Large-Cell Lymphoma". MDPI AG.
6. (1995). "CD30/Ki-1-positive lymphoproliferative disorders of the skin--clinicopathologic correlation and statistical analysis of 86 cases: a multicentric study from the European Organization for Research and Treatment of Cancer Cutaneous Lymphoma Project Group.". American Society of Clinical Oncology (ASCO).
7. (August 14, 2023). "Primary Cutaneous Anaplastic Large Cell Lymphoma—A Review of Clinical, Morphological, Immunohistochemical, and Molecular Features". MDPI AG.
8. (2003). "Cd30+ cutaneous lymphoproliferative disorders: The stanford experience in lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma". Elsevier BV.