Rare non‐Hodgkin lymphoma of childhood and adolescence: A consensus diagnostic and therapeutic approach to pediatric‐type follicular lymphoma,marginal zone lymphoma,and nonanaplastic peripheral T‐cell lymphoma

Pediatric‐type follicular (PTFL), marginal zone (MZL), and peripheral T‐cell lymphoma (PTCL) account each for <2% of childhood non‐Hodgkin lymphoma. We present clinical and histopathological features of PTFL, MZL, and few subtypes of PTCL and provide treatment recommendations. For localized PTFL and MZL, watchful waiting after complete resection is the therapy of choice. For PTCL, therapy is subtype‐dependent and ranges from a block‐like anaplastic large cell lymphoma (ALCL)‐derived and, alternatively, leukemia‐derived therapy in PTCL not otherwise specified and subcutaneous panniculitis‐like T‐cell lymphoma to a block‐like mature B‐NHL‐derived or, preferentially, ALCL‐derived treatment followed by hematopoietic stem cell transplantation in first remission in hepatosplenic and angioimmunoblastic T‐cell lymphoma.     

CD56‐negative extranodal nasal type NK/T‐cell lymphoma

Extranodal natural killer (NK)/T‐cell lymphoma, nasal type, is a rare lymphoma that occurs predominantly in Asian adults. In this report, we describe the clinical and pathologic features of an unusual aggressive lymphoid neoplasm in a child and review the literature on NK/T‐cell lymphoma in children. The patient was a 4‐year‐old Native American male with facial swelling, lymphadenopathy, and fevers. Biopsy demonstrated neoplastic lymphoid cells that expressed CD3, CD8, TIA‐1, and EBV‐encoded RNA without CD56. The patient failed multiagent chemotherapy and died of therapy‐related complications. This case represents an extranodal NK/T‐cell lymphoma, nasal type, with an unusual lack of CD56. Pediatr Blood Cancer 2010;55:186–189. © 2010 Wiley‐Liss, Inc.     

Ongoing remission after intensive All‐type chemotherapy in pediatric intestinal T‐cell lymphoma

A rare case of primary intestinal T‐cell lymphoma (ITL) of an 8‐year‐old boy is reported. Medium‐ to large‐sized tumor cells were βF1+, CD3+, CD8+. TIA‐1+, but CD4?, CD5?, CD30?, CD56?, CD20?, CD79a?, TdT?, consistent with an intraepithelial lymphocyte (IEL) origin. They showed monoclonal rearrangement of the T‐cell receptor γ‐chain and no evidence of EBV infection. No clinical, histologic, laboratory, or genetic evidence of celiac disease was detected. In adults, ITL is often associated with enteropathy and has a very poor outcome. Our patient remains in first remission 30 months after finishing the acute lymphoblastic leukemia protocol COALL‐07‐03 high risk standard. Pediatr Blood Cancer 2010;54:610–612. © 2009 Wiley‐Liss, Inc.     

TIM‐3 deficiency presenting with two clonally unrelated episodes of mesenteric and subcutaneous panniculitis‐like T‐cell lymphoma and hemophagocytic lymphohistiocytosis

This report offers novel clinical and diagnostic aspects of the association between germline mutations in HAVCR2 and subcutaneous panniculitis‐like T‐cell lymphoma (SPTCL). The patient presented with panniculitis‐like T‐cell lymphoma involving mesenteric fatty tissue associated with hemophagocytic lymphohistiocytosis (HLH). Five years later, he developed a clonally unrelated SPTCL and underwent hematopoietic stem cell transplantation. Retrospectively, he was found to carry germline mutations in HAVCR2 associated with reduced T‐cell immunoglobulin mucin‐3 (TIM‐3) expression. We show that mesenteric fatty tissue localization of SPTCL can be the presenting manifestation of TIM‐3 deficiency, that this condition predisposes to recurrent lymphoma, and that flow cytometry is a possible screening tool.     

Sustained first remission in an adolescent with hepatosplenic T‐cell lymphoma treated with T‐cell leukemia induction,nucleoside analog‐based consolidation,and early hematopoietic stem cell transplant

Hepatosplenic T‐cell lymphoma (HTCL) is a rare malignancy. Prognosis is poor with only a few case reports of long‐term survivors. While HTCL universally involves the bone marrow, the condition has been most often treated with multimodal lymphoma specific chemotherapy. We report a durable, sustained first remission in an adolescent treated for HTCL who received induction therapy according to a high risk T‐cell leukemia regimen, a nucleoside analog‐based consolidation, and allogeneic transplantation associated with GVHD. Pediatr Blood Cancer 2009;53:1127–1129. © 2009 Wiley‐Liss, Inc.