Grey zone lymphoma
This is an entity that has features of non-Hodgkin’s and Hodgkin’s lymphoma. This is a subtype of B-cell non-Hodgkin lymphoma which has features of diffuse large B-cell lymphoma and classical Hodgkin Lymphoma. The NCCN guidelines recommend treating gray zone lymphomas in a manner similar to diffuse large B-cell lymphoma. There is no evidence supporting the use of the regimen requested as a treatment for mediastinal gray zone lymphoma. Notably, this lymphoma is only dimly positive for CD30. This treatment is experimental/investigational since a lack of NCCN endorsement indicates that the prevailing opinion among experts is that more
studies would be needed to support the efficacy of this treatment.
Gray zone lymphoma is a rare type of lymphoma cancer of a part of the immune system called the lymph system. It is called “gray zone” lymphoma because it has features intermediate between classical Hodgkin lymphoma and diffuse large B-cell lymphoma (DLBCL), but cannot be assigned specifically to either type.
The standard first-line treatment for grey zone lymphoma (GZL) is still under consideration as researchers are still learning more about the condition. Most specialists will assess whether the GZL is acting more like Hodgkin lymphoma (HL) or more like primary mediastinal B-cell lymphoma (PMBCL). The NCCN guidelines recommend treating gray zone lymphomas in a manner similar to diffuse large B-cell lymphoma. . Off of a clinical trial, usual treatment is R-CHOP (rituximab, cyclophosphamide, doxorubicin, oncovin, prednisolone) or dose-adjusted EPOCH-R (etoposide, prednisolone, oncovin, cyclophosphamide, doxorubicin, rituximab) for the frontline treatment of GZL.
Grey zone lymphoma is an aggressive disease that can be difficult to treat, however, it is potentially curable with standard treatment that includes combination chemotherapy and immunotherapy (chemoimmunotherapy), sometimes with radiotherapy and stem cell transplant.
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