Precursor T-lymphoblastic lymphoma

Precursor T-lymphoblastic lymphoma (T-LBL) is a rare type of non-Hodgkin lymphoma that primarily affects children and young adults. It is characterized by the rapid growth of immature white blood cells known as T-lymphocytes, in the lymph nodes, thymus, and other tissues.

Etiology

The exact cause of T-LBL is unknown. However, it is believed to be associated with genetic abnormalities, such as translocations and deletions, that lead to the uncontrolled proliferation of immature T-lymphocytes. Exposure to certain environmental factors, such as radiation and certain chemicals, may also increase the risk of developing this disease.

Clinical Presentation

Patients with T-LBL often present with symptoms related to the mass effect of the tumor, such as dyspnea, cough, and chest pain. Other common symptoms include fever, weight loss, and night sweats. In some cases, patients may also have lymphadenopathy and hepatosplenomegaly.

Diagnosis

The diagnosis of T-LBL is based on the histological examination of the tumor tissue, which typically shows a diffuse proliferation of small to medium-sized lymphoid cells with scant cytoplasm and round to irregular nuclei. Immunohistochemical staining is used to confirm the T-cell lineage of the tumor cells. Other diagnostic tests include complete blood count, liver function tests, and imaging studies such as computed tomography and positron emission tomography.

Treatment

The treatment of T-LBL typically involves intensive chemotherapy to kill the cancer cells, followed by radiation therapy to the chest if there is a mediastinal mass. In some cases, stem cell transplantation may be considered.

Prognosis

The prognosis of T-LBL is generally poor, with a 5-year survival rate of less than 50%. However, early detection and aggressive treatment can significantly improve the survival rate.

File:Precursor T-lymphoblastic lymphoma - intermed mag.jpg

Histopathological image of precursor T-lymphoblastic lymphoma. Hematoxylin & eosin stain.

See Also

• Acute lymphoblastic leukemia
• Non-Hodgkin lymphoma
• T-cell lymphoma