Transition Between Diffuse Large B-Cell Lymphoma and Classical Hodgkin Lymphoma– Our Histopathological and Clinical Experience With Patients With Intermediate Lymphoma

Abstract

Even though information about the pathophysiology and clinical features of grey-zone lymphoma, an entity intermediate between classical Hodgkin lymphoma and diffuse large B-cell lymphoma, is growing, there are still a number of unanswered questions. The disease has no easily reproducible diagnostic criteria, which makes identification challenging. Uncommon, mixed histological picture and unusual clinical presentation should raise suspicion for grey-zone lymphoma. In this retrospective analysis we present 9 gray zone lymphoma patients, who were diagnosed in our institute between 2008 and 2018. The histological diagnoses was oftentime challenging, we asked for a revision in three cases due to the unusual clinical behavior and in other three cases only the relapse of the disease proved to be grey-zone lymphoma. Based on the initial histopathological diagnoses we applied adriablastine-bleomycine-vinblastine and procarbasine or cyclophosphamide-vincristine-adriablastine and prednisolon as first line chemotherapy regime with additional rituximab in six cases and brentuximab-vedotine in one patient. In six of the nine patients due to the primary refractory disease we used rituximab plus cisplatine, cytosine-arabinoside, prednisolone salvage treatment and five of these patients responded well enough to become eligible for autologous stem cell transplantation. One young male patient was refractory for various treatments and died due to the progression of his lymphoma. As a rare disease grey-zone lymphoma has no existing diagnostic criteria or guiedlines for its standard of care, which makes the everyday practice rather challenging for the clinicians, and emphasize the importance of unique decision making in every case and the repeated consultation between the pathologist and hematologist.

Mediastinal gray zone lymphoma in a pregnant woman presenting with cardiac tamponade

“…Cancer staging and treatment are also challenging in pregnancy because of the risk that threatens the mother and fetus (9). Currently, the R-CHOP regimen is the treatment of choice for gray zone lymphoma (10). chemotherapy without Rituximab was started for the patient because the evidence showed CHOP regimen is safe beyond the rst trimester (11).…”

Gray zone lymphoma effectively treated with cyclophosphamide, doxorubicin, vincristine, prednisolone, and rituximab chemotherapy: A case report

“…If the tumor cells are CD20+, the addition of rituximab to the chemotherapy regimen should be considered[ 22 ]. R-CHOP or dose-adjusted etoposide, prednisolone, vincristine, cyclophosphamide, doxorubicin, and rituximab (DA-EPOCH-R) regimens are recommended[ 23 - 25 ].…”