title: When to Treat Adults Like Children: Optimizing Therapy for Lymphoblastic Lymphoma in Young Adults.
abstract: A 23-year-old man was urgently referred for evaluation of rapidly enlarging cervical lymphadenopathy, progressive dyspnea, fatigue, night sweats, and an unintentional weight loss of 25 pounds. A computed tomography scan of the neck performed 30 days before referral revealed bilateral cervical and supraclavicular lymphadenopathy (6 × 5 cm). A fine-needle aspirate of nasopharyngeal tissue demonstrated fibroadipose tissue. Tissue collected by core needle biopsy of a left internal jugular lymph node demonstrated a reactive lymph node but no malignancy. The patient was admitted to an academic medical center hospital. His physical examination was remarkable for bulky cervical and supraclavicular lymphadenopathy. A testicular examination was normal. The patient's lactate dehydrogenase concentration was 327 U/L (normal range, 118-225 U/L). A positron emission tomography scan revealed bilateral cervical and supraclavicular lymphadenopathy (6 × 5 cm with a standardized uptake value [SUV] of 14), a 1.3-cm subcutaneous nodule in the left thigh (SUV 16), and two 2.7-cm liver lesions (SUV 14). He underwent an excisional lymph node biopsy. The lymph node revealed effacement of the architecture by an interfollicular infiltrate of lymphoid cells (Fig 1). Mitotic figures were abundant (Ki-67 stain 80% to 90% positive) and there were multiple foci of tissue necrosis. The lymphoblasts were examined by flow cytometry and immunohistochemistry and expressed the T-cell markers CD2, CD3, CD4, and terminal deoxynucleotidyl transferase. A subpopulation of T cells was positive for both CD4 and CD8. Polymerase chain reaction studies revealed a clonal rearrangement of the T-cell receptor γ gene. A marrow aspirate and biopsy revealed normal trilineage hematopoiesis with no evidence of lymphoma and a normal male karyotype (46, XY). A lumbar puncture sample did not contain lymphoma cells. The patient's diagnosis was T-lymphoblastic lymphoma.
authors.affiliation: University of Rochester Medical Center, Wilmot Cancer Institute, Rochester, NY kristen_odwyer@urmc.rochester.edu.
authors.forename: Kristen M
authors.initials: KM
authors.lastname: O'Dwyer
authors.affiliation: The Cleveland Clinic, Taussig Cancer Institute, Cleveland, OH.
authors.forename: Anjali S
authors.initials: AS
authors.lastname: Advani
mesh_terms: D015703:Antigens, CD / Q000032:analysis; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000627:therapeutic use*; D001706:Biopsy; D060830:Consolidation Chemotherapy; D015321:Gene Rearrangement; D019675:Genes, T-Cell Receptor gamma / Q000235:genetics; D006801:Humans; D060828:Induction Chemotherapy; D008198:Lymph Nodes / Q000000981:diagnostic imaging / Q000473:pathology*; D060046:Maintenance Chemotherapy; D008297:Male; D049268:Positron-Emission Tomography; D054218:Precursor T-Cell Lymphoblastic Leukemia-Lymphoma / Q000175:diagnosis* / Q000188:drug therapy* / Q000235:genetics; D012074:Remission Induction; D013601:T-Lymphocytes / Q000737:chemistry; D055815:Young Adult
chemical_list: D015703:Antigens, CD