# Gamma-Delta Hepatosplenic T-Cell Lymphoma in a Two-Year-Old: A Case Report **Authors:** Hibah Mohammed, Adib Khan, Fiona Lin, Amia Mourad, Madhura Butala PMC · DOI: 10.7759/cureus.102138 · 2026-01-23 ## TL;DR A rare and aggressive form of T-cell lymphoma in a young child is described, highlighting diagnostic challenges and treatment resistance. ## Contribution This case report adds to the limited literature on γδ hepatosplenic T-cell lymphoma in young children and emphasizes the need for early diagnosis and advanced treatment strategies. ## Key findings - γδ hepatosplenic T-cell lymphoma presented in a 26-month-old with nonspecific symptoms and poor response to therapies. - Diagnosis was confirmed via bone marrow and liver biopsy, revealing clonal γδ T-cell infiltration. - Persistent minimal residual disease required escalated treatment and highlights the aggressive nature of this lymphoma in children. ## Abstract Gamma-delta (γδ) hepatosplenic T-cell lymphoma (HSTCL) is a rare, aggressive malignancy that is exceptionally uncommon in young children. It often presents with nonspecific symptoms that delay diagnosis. We describe a previously healthy 26-month-old girl who developed daily fevers, weight loss, decreased appetite, and progressive lower-extremity weakness leading to loss of ambulation. Examination revealed marked hepatosplenomegaly without lymphadenopathy, and laboratory studies showed lymphocytic leukocytosis with evolving cytopenias. Bone marrow evaluation identified a clonal γδ T-cell population, and liver biopsy confirmed sinusoidal infiltration, establishing the diagnosis of γδ HSTCL. Her clinical course was characterized by poor response to multiple induction, consolidation, and targeted therapies. She presented with persistent minimal residual disease (MRD), requiring repeated treatment escalation and substantial supportive care. This case illustrates the diagnostic difficulty and therapeutic resistance of pediatric γδ HSTCL. Early recognition, molecular characterization, and timely consideration of possible stem cell transplant are essential to improving outcomes in this rare and highly aggressive pediatric lymphoma. ## Full-text entities - **Genes:** CD4 (CD4 molecule) [NCBI Gene 920] {aka CD4mut, IMD79, Leu-3, OKT4D, T4}, SETD2 (SET domain containing 2, histone lysine methyltransferase) [NCBI Gene 29072] {aka HBP231, HIF-1, HIP-1, HSPC069, HYPB, KMT3A}, CD5 (CD5 molecule) [NCBI Gene 921] {aka LEU1, T1}, INO80 (INO80 complex ATPase subunit) [NCBI Gene 54617] {aka INO80A, INOC1}, CRP (C-reactive protein) [NCBI Gene 1401] {aka PTX1}, TRBC1 (T cell receptor beta constant 1) [NCBI Gene 28639] {aka BV05S1J2.2, TCRB, TCRBC1}, CD7 (CD7 molecule) [NCBI Gene 924] {aka GP40, LEU-9, TP41, Tp40}, CD8A (CD8 subunit alpha) [NCBI Gene 925] {aka CD8, CD8alpha, IMD116, Leu2, p32}, STAT5B (signal transducer and activator of transcription 5B) [NCBI Gene 6777] {aka GHISID2, STAT5}, PTEN (phosphatase and tensin homolog) [NCBI Gene 5728] {aka 10q23del, BZS, CWS1, DEC, GLM2, MHAM}, TRBV20OR9-2 (T cell receptor beta variable 20/OR9-2 (non-functional)) [NCBI Gene 6962] {aka CDR3, TCRBV20S2, TCRBV2O, TCRBV2S2O}, STAT3 (signal transducer and activator of transcription 3) [NCBI Gene 6774] {aka ADMIO, ADMIO1, APRF, HIES}, APP (amyloid beta precursor protein) [NCBI Gene 351] {aka AAA, ABETA, ABPP, AD1, APPI, CTFgamma}, PTPRC (protein tyrosine phosphatase receptor type C) [NCBI Gene 5788] {aka B220, CD45, CD45R, GP180, IMD105, L-CA}, CD2 (CD2 molecule) [NCBI Gene 914] {aka LFA-2, SRBC, T11}, NCAM1 (neural cell adhesion molecule 1) [NCBI Gene 4684] {aka CD56, MSK39, NCAM}, CD38 (CD38 molecule) [NCBI Gene 952] {aka ADPRC 1, ADPRC1, cADPR1}, ARID1B (AT-rich interaction domain 1B) [NCBI Gene 57492] {aka 6A3-5, BAF250B, BRIGHT, CSS1, DAN15, ELD/OSA1} - **Diseases:** abdominal distension (MESH:D000007), lymphoid malignancies (MESH:D008223), cellulitis (MESH:D002481), constipation (MESH:D003248), associated (MESH:D018886), decreased appetite (MESH:D001068), lymphocytosis (MESH:D008218), fungal infection (MESH:D009181), lower-extremity weakness (MESH:D020335), infectious (MESH:D003141), febrile neutropenia (MESH:D064147), hematochezia (MESH:D006471), HSTCL (MESH:D016399), organomegaly (MESH:D016878), ascites (MESH:D001201), viral or (MESH:D014777), Hepatomegaly (MESH:D006529), Cytopenias (MESH:D006402), hypertension (MESH:D006973), bacteremia (MESH:D016470), ALL (MESH:D054198), thrombocytopenia (MESH:D013921), leukocytosis (MESH:D007964), nodal (MESH:D013611), Escherichia coli bacteremia (MESH:D004927), weight loss (MESH:D015431), toxicities (MESH:D064420), ED (MESH:D004630), autoimmune process (MESH:D001327), Splenomegaly (MESH:D013163), lymphadenopathy (MESH:D008206), knee pain (MESH:D046788), cytomegalovirus viremia (MESH:D014766), Paget's disease (MESH:C537701), fevers (MESH:D005334), hematologic malignancy (MESH:D019337), acute leukemia (MESH:D015470), loss of ambulation (MESH:D051346), gastrointestinal symptoms (MESH:D012817), Inflammatory (MESH:D007249), primary cutaneous gammadelta T-cell lymphoma (MESH:D016410), Neoplasms (MESH:D009369), multiple myeloma (MESH:D009101), hepatosplenomegaly (MESH:C535727), peripheral T-cell neoplasm (MESH:D016411), Gallbladder Edema (MESH:D004487), abdominal pain (MESH:D015746) - **Chemicals:** daratumumab (MESH:C556306), steroid (MESH:D013256), cytarabine (MESH:D003561), ifosfamide (MESH:D007069), ICE (MESH:D007053), azacitidine (MESH:D001374), IVAC (-), carboplatin (MESH:D016190), nelarabine (MESH:C104457), 6-mercaptopurine (MESH:D015122), etoposide (MESH:D005047), methotrexate (MESH:D008727), lactulose (MESH:D007792), cyclophosphamide (MESH:D003520), platinum (MESH:D010984), anthracycline (MESH:D018943), polyethylene glycol (MESH:D011092), venetoclax (MESH:C579720) - **Species:** Staphylococcus epidermidis (species) [taxon 1282], Homo sapiens (human, species) [taxon 9606] ## Figures 2 figures with captions in the complete paper: https://tomesphere.com/paper/PMC12925368/full.md --- Source: https://tomesphere.com/paper/PMC12925368