Bilateral massive nephromegaly-A rare presentation of t-cell acute lymphoblastic leukemia

双侧巨大肾肿大——T细胞急性淋巴细胞白血病的一种罕见表现

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Abstract

INTRODUCTION: Renal infiltration by leukemia causing massive bilateral nephromegaly is an extremely rare presentation of T-cell acute lymphoblastic leukemia(T-ALL). CASE REPORT: 18-month-old female toddler presented with fever and progressive abdominal distension of 4-6 weeks duration. Imaging revealed bilateral massively enlarged kidneys with normal excretion. Peripheral blood counts and smear examination was unremarkable and immunophenotypic evaluation of marrow was consistent with T-ALL. Chest imaging was unremarkable. She was started on modified Indian Childhood Collaborative Leukemia Group (ICiCLe) ALL protocol. Even with the best anti-tumor lysis syndrome (TLS) prophylaxis the child required two sessions of hemodialysis. An end-induction morphological remission & end-consolidation negative minimal residual disease (MRD) could be achieved. CONCLUSION: Bilateral massive nephromegaly is an extremely rare presentation of T-ALL. This case emphasizes the unusual presentation, need for prompt remediation of TLS, and most importantly the use of early intensification with four drug anthracycline & dexamethasone-based therapy for the treatment of T-ALL in children.

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