Spontaneous Remission Of Angioimmunoblastic T-cell Lymphoma In A Child With Ataxia-telangiectasia: A Case Report
Abla, D., Al-Battashi, A., Albiroty, K., Abu Qasida, K., Al-Rahbi, N., & Al-Awaidy, S. (2023). Spontaneous remission of angioimmunoblastic T-cell lymphoma in a child with ataxia-telangiectasia: a case report. Journal of medical case reports, 17(1), 347. https://doi.org/10.1186/s13256-023-04050-5
View Original Source →Abstract
BACKGROUND: Angioimmunoblastic T-cell lymphoma is an uncommon subtype of peripheral T-cell lymphoma in children with fewer than 20 cases reported in literature. CASE PRESENTATION: A 3-year-old Omani boy was diagnosed with ataxia-talengectasia presenting with fever and generalized lymphadenopathy. His biopsy revealed atypical lymphocytic infiltrate consistent with the diagnosis of angioimmunoblastic T-cell lymphoma. Within 3 weeks from the initial presentation and without any neoadjuvant therapy, he showed complete recovery of symptoms with absence of fever and regression of all previously affected lymph nodes. He has remained in remission ever since. CONCLUSION: This is the first report of spontaneous improvement of angioimmunoblastic T-cell lymphoma in a patient with ataxia-telangiectasia who was 3 years old at presentation. Owing to the paucity of similar cases, this report adds valuable diagnostic, therapeutic, and monitoring data.
Case Details
Disease Location
Lymph nodes
Personal Characteristics
3-year-old omani male. Parents were first-degree relatives
Clinical Characteristics
Unstable gait and recurrent upper respiratory infections. The diagnosis of ataxia–telangiectasia was confirmed by identifying a novel homozygous mutation in the atm gene. He was started on treatment with monthly and lifelong intravenous immunoglobulin at a prophylactic dose of 0.4 milligrams per kilogram. He presented with 1-week history of fever and cough. Physical examination revealed submandibular, cervical, axillary, and inguinal enlarged lymph nodes (largest was 3 × 3 cm in the right axilla) blood test revealed severe neutropenia, anemia. The patient was started on general supportive care with intravenous hydration, antipyretics (paracetamol 15 milligrams per kilogram intravenously) and intravenous ceftriaxone at a dose of 50 milligrams per kilogram twice a day for 5 days. The patient underwent a right axillary lymph node excisional biopsy. Microscopic analysis demonstrated infiltration with atypical lymphoid cells having small, medium to large nuclei and clear cytoplasm localized around high endothelial venules. Immunohistochemical stains showed positivity of the atypical lymphoid cells for CD3, CD4, CD10, bcl6, and pd1. He was diagnosed with angioimmunoblastic t‐cell lymphoma (atcl). Bone marrow examination did not show any abnormal cellular infiltration.
Remission Characteristics
The patient went into complete resolution of lymphoma symptoms with remittance of fever and resolution of the generalized lymphadenopathy within 3 weeks
Treatment & Mechanisms
Clinical Treatment
Biopsy immunoglobulin IV fluids and paracetamol. Ceftriaxone. Biopsy bone marrow aspiration
Non-Clinical Treatment
None reported