Overview
Primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL-LT) is a rare and aggressive form of non-Hodgkin lymphoma that originates in the skin, classified among the primary cutaneous B-cell lymphomas. Despite its name, while it most commonly presents on the lower legs (particularly in elderly women), it can also occur on other body sites. The disease typically manifests as rapidly growing, red-to-bluish nodules or tumors on one or both legs. These lesions may ulcerate and can spread to involve lymph nodes, bone marrow, and other organs if not treated promptly. The tumor cells are large B-lymphocytes that express markers such as BCL2, MUM1/IRF4, and FOXP1, which help distinguish this entity from other cutaneous B-cell lymphomas. It is associated with a more aggressive clinical course and poorer prognosis compared to other primary cutaneous B-cell lymphomas. PCDLBCL-LT predominantly affects elderly individuals, with a median age of onset typically in the seventh to eighth decade of life, and shows a female predominance. The disease is classified under the WHO-EORTC classification of cutaneous lymphomas and is recognized as a distinct clinicopathologic entity. Diagnosis requires skin biopsy with immunohistochemical analysis, and staging workup including imaging studies is essential to rule out systemic involvement. Treatment typically involves R-CHOP (rituximab combined with cyclophosphamide, doxorubicin, vincristine, and prednisone) chemotherapy, which is the standard first-line regimen. Radiotherapy may be used for localized disease or as an adjunct to chemotherapy. Despite treatment, the disease has a relatively high relapse rate and a 5-year survival rate that is significantly lower than other primary cutaneous B-cell lymphomas, estimated at approximately 50-60%. Novel targeted therapies and immunotherapies are being investigated to improve outcomes for patients with this condition.
Also known as:
Sporadic
Usually appears on its own, not inherited from a parent
Late onset
Begins later in life, typically after age 50
Treatments
No FDA-approved treatments are currently listed for Primary cutaneous diffuse large B-cell lymphoma, leg type.
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Specialists
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Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Primary cutaneous diffuse large B-cell lymphoma, leg type.
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Common questions about Primary cutaneous diffuse large B-cell lymphoma, leg type
What is Primary cutaneous diffuse large B-cell lymphoma, leg type?
Primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL-LT) is a rare and aggressive form of non-Hodgkin lymphoma that originates in the skin, classified among the primary cutaneous B-cell lymphomas. Despite its name, while it most commonly presents on the lower legs (particularly in elderly women), it can also occur on other body sites. The disease typically manifests as rapidly growing, red-to-bluish nodules or tumors on one or both legs. These lesions may ulcerate and can spread to involve lymph nodes, bone marrow, and other organs if not treated promptly. The tumor cells are lar
How is Primary cutaneous diffuse large B-cell lymphoma, leg type inherited?
Primary cutaneous diffuse large B-cell lymphoma, leg type follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Primary cutaneous diffuse large B-cell lymphoma, leg type typically begin?
Typical onset of Primary cutaneous diffuse large B-cell lymphoma, leg type is late onset. Age of onset can vary across affected individuals.