Lennert lymphoma
Lennert lymphoma | |
---|---|
Synonyms | Lymphoepithelioid cell variant of peripheral T-cell lymphoma |
Pronounce | N/A |
Specialty | Hematology, Oncology |
Symptoms | Lymphadenopathy, fever, weight loss, night sweats |
Complications | N/A |
Onset | Typically in adulthood |
Duration | Chronic |
Types | N/A |
Causes | Unknown |
Risks | Possible genetic predisposition, immunosuppression |
Diagnosis | Biopsy, immunohistochemistry |
Differential diagnosis | Hodgkin lymphoma, Non-Hodgkin lymphoma |
Prevention | N/A |
Treatment | Chemotherapy, radiation therapy, stem cell transplant |
Medication | N/A |
Prognosis | Variable, depends on stage and response to treatment |
Frequency | Rare |
Deaths | N/A |
Lennert lymphoma is a rare subtype of T-cell lymphoma, characterized by the presence of lymphoepithelioid cells (Lennert cells) in the lymph nodes. It was first described by Karl Lennert and his colleagues in 1975.
Epidemiology
Lennert lymphoma is extremely rare, accounting for less than 1% of all non-Hodgkin lymphomas. It is more common in adults than in children, and there is no clear gender predominance.
Pathophysiology
The pathophysiology of Lennert lymphoma is not well understood. It is thought to arise from mature T cells, but the exact mechanism of transformation is unknown. The disease is characterized by the presence of large numbers of lymphoepithelioid cells, which are a type of histiocyte that has been transformed into a lymphocyte-like cell.
Clinical Features
Patients with Lennert lymphoma typically present with lymphadenopathy, fever, weight loss, and night sweats. The disease is often advanced at the time of diagnosis, with involvement of the bone marrow, liver, and spleen.
Diagnosis
The diagnosis of Lennert lymphoma is based on the histological examination of a lymph node biopsy. The characteristic finding is the presence of large numbers of lymphoepithelioid cells, which have a distinctive appearance under the microscope.
Treatment
The treatment of Lennert lymphoma is similar to that of other T-cell lymphomas. It typically involves chemotherapy, with or without radiation therapy. The prognosis is generally poor, with a median survival of less than 2 years.
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