ALK+ large B-cell lymphoma
- ALK+ Large B-Cell Lymphoma
ALK+ Large B-Cell Lymphoma is a rare subtype of diffuse large B-cell lymphoma (DLBCL), characterized by the expression of the anaplastic lymphoma kinase (ALK) protein. This lymphoma is distinct from other types of DLBCL due to its unique genetic and clinical features.
Pathophysiology[edit | edit source]
ALK+ Large B-Cell Lymphoma is defined by the presence of a chromosomal translocation involving the ALK gene, most commonly t(2;17)(p23;q23), which results in the expression of a chimeric protein with constitutive kinase activity. This aberrant kinase activity leads to uncontrolled cell proliferation and survival, contributing to the oncogenic process.
The ALK protein is normally expressed in neural tissues during development, but its expression in lymphoid tissues is abnormal and indicative of malignancy. The presence of ALK protein can be detected by immunohistochemistry, which is a key diagnostic tool for this lymphoma.
Clinical Presentation[edit | edit source]
Patients with ALK+ Large B-Cell Lymphoma typically present with systemic symptoms such as fever, night sweats, and weight loss, along with lymphadenopathy. The disease can affect both nodal and extranodal sites, including the skin, bone, and soft tissues.
Diagnosis[edit | edit source]
Diagnosis of ALK+ Large B-Cell Lymphoma involves a combination of histopathological examination, immunophenotyping, and genetic studies. The hallmark of this lymphoma is the expression of the ALK protein, which can be detected using immunohistochemical staining. Additionally, cytogenetic analysis can identify the characteristic chromosomal translocations.
Treatment[edit | edit source]
The treatment of ALK+ Large B-Cell Lymphoma typically involves chemotherapy regimens similar to those used for other types of DLBCL, such as R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone). However, due to the rarity of this lymphoma, there is limited data on the optimal treatment approach.
Targeted therapies that inhibit ALK kinase activity, such as crizotinib, have shown promise in treating ALK+ lymphomas, although their use in ALK+ Large B-Cell Lymphoma specifically is still under investigation.
Prognosis[edit | edit source]
The prognosis for patients with ALK+ Large B-Cell Lymphoma is generally poor compared to other types of DLBCL, partly due to its aggressive nature and the lack of established targeted therapies. However, early diagnosis and treatment can improve outcomes.
Also see[edit | edit source]
- Diffuse large B-cell lymphoma
- Anaplastic large cell lymphoma
- Non-Hodgkin lymphoma
- Immunohistochemistry
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Contributors: Prab R. Tumpati, MD
