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Background Data on management of gray zone lymphoma (GZL) in children and adolescents are scarce. Procedure This retrospective study assessed clinical characteristics and outcome in six Austrian patients with GZL less than 18 years of age (male‐to‐female ratio: 1:1; median age: 15.8 years). Results Two patients each had a classical Hodgkin lymphoma (cHL)‐like and composite GZL subtype, and one patient each had a large B‐cell non‐Hodgkin lymphoma (LBCL)‐like and sequential GZL subtype. All had advanced disease with mediastinal and extranodal involvement. Five patients received an LBCL‐ and one patient a cHL‐directed polychemotherapy ± radiotherapy. Out of the former patients, three survived, including two who relapsed and underwent high‐dose chemotherapy with autologous stem cell rescue. The latter patient survived. Conclusions GZL remains a diagnostic and therapeutic challenge, necessitating the development of novel treatment concepts performed in a prospective setting.

The development of a second malignancy after diagnosis of childhood acute lymphoblastic leukemia (ALL) is a rare event. Certain second malignancies have been linked with specific elements of leukemia therapy, yet the etiology of most second neoplasms remains obscure and their optimal management strategies are unclear. This is a first comprehensive report of non-Hodgkin lymphomas (NHL) following pediatric ALL therapy, excluding stem-cell transplantation. We analyzed data of patients enrolled in 12 collaborative pediatric ALL trials between 1980-2018, who developed NHL following ALL diagnosis. Eighty-five patients developed NHL, with mature B-cell lymphoproliferations as the dominant subtype (56/85). Forty-six of these 56 cases (82%) occurred during or within six months of maintenance therapy. The majority (65%) exhibited histopathological characteristics associated with immunodeficiency, predominantly evidence of Epstein-Barr virus (EBV)-driven lymphoproliferation. We investigated 66 cases of post-ALL immunodeficiency-associated lymphoid neoplasms, 52 from our study and 14 additional cases from a literature search. With a median follow-up of 4.9 years, five-year overall survival for the 66 patients with immunodeficiency-associated lymphoid neoplasms was 67.4% (95% CI, 56-81). Five-year cumulative risks of lymphoid neoplasm- and leukemia-related mortality were 20% (95% CI, 10.2-30) and 12.4% (95% CI, 2.7- 22), respectively. Concurrent hemophagocytic lymphohistiocytosis was associated with increased mortality (HR, 7.32; 95% CI, 1.62 to 32.98; p=0.01). A large proportion of post-ALL lymphoid neoplasms are associated with an immunodeficient state, likely precipitated by ALL maintenance therapy. Awareness of this underrecognized entity and pertinent diagnostic tests are crucial for early diagnosis and optimal therapy.

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