Acute lymphoblastic leukemia: An unusual cause of nephromegaly in infancy

Abstract

Sir,We present here an infant who had acute lymphoblastic leukemia with massively enlarged kidneys due to leukemic infiltrates. A 7-month-old girl child presented with complaints of low grade fever and coryza for 6 days. The mother noticed angular deviation of mouth and tongue to right side for the past 4 days and decreased movements of right upper limb for same duration. On examination, the child had pallor and few petechie over the trunk. The vitals were HR 122/min, pulses well palpable, RR 48/min; blood pressure was 134/94 mm Hg. Her anthropometry was: weight 6.6 Kg (10th centile), length 65 cm (25 th centile), and head circumference was 41.5cm (25 th centile). Central nervous system examination revealed normal sensorium. There was left facial lower motor neuron palsy, right hypoglossal palsy and right sided hemiparesis (upper motor neuron type). There were no meningeal or cerebellar signs. She had a soft hepatomegaly (2.5 cm below costal margin) and splenomegaly (6 cm below costal margins). Bilateral kidneys were palpable and occupied most of the abdomen, their surface was smooth.The mother had undergone ultrasonography during the antenatal period and no abnormality of fetal kidneys was reported. Initial investigations revealed hemoglobin 5.8g%, TLC 7500/µl, polymorphs 35, lymphocytes 65 and Platelet count of 26X10 9 /L. The peripheral smear had normocytic normochromic to mildly hypochromic red blood cells, platelets were diminished and 3% blasts were present. The biochemistry showed blood urea 45mg/dL, serum creatinine 0.8 mg/dL, serum ALT 24 IU/L, AST 25 IU/L. albumin 4.1 gm/dL, SAP 240 IU/L. The serum sodium was 133 mEq/L, K 4.0 mEq/L, calcium 8.2 mg/ dL, and phosphates 5.4 mg/dL. A venous blood gas had a pH of 7.35, bicarbonate 21 mEq/L, base excess -5 mEq/ L. A lumbar puncture examination done showed 80 cells, polymorphs 45 and lymphocytes 25 and presence of abnormal cells; CSF sugar was 87 mg/dL and protein 62 mg/dL. Glomerular filtration rate as calculated by the Schwartz method was 45 ml/min/1.73m².An ultrasonogram of the abdomen revealed liver of normal size and echotexture. The spleen was enlarged 9.2 cm, right kidney (10.7X 5.0 cm); left kidney (10.2 X 5.5 cm), bilateral grossly enlarged kidneys with smooth outline extending from epigastrium to pelvic inlet compressing midline retroperitoneal vessels. Multiple dilated tubular structures were seen in cortex and medulla. A computed tomography of the abdomen showed enlarged smooth Acute Lymphoblastic Leukemia: An Unusual cause of Nephromegaly in Infancy kidneys with no cystic dilatation with maintained corticomedullary differentiation. An MRI of the brain was suggestive of generalized brain atrophy. A bone marrow aspirate showed presence of 21% lymphobalsts (PAS & myeloperoxidase negative blasts) in the marrow. A diagnosis of acute lymphoblastic leukemia with renal and CNS infiltrations was made. A renal biopsy was not done in view of persistent thrombocytopenia.The child was started on chemotherapy for standard risk: ALL (prednisolone, L-asparginase...

Renal Presentation in Pediatric Acute Leukemia

“… 2 , 5 , 9 , 10 In contrast, bilateral symmetrical renal enlargement at the time of diagnosis of ALL is an infrequent finding, being present in 3% to 5% of cases. 11 A potential mechanism in the trafficking of leukemia cells is the interaction of the chemokine receptor CXCR4, which is expressed on ALL cells, and its ligand stromal cell-derived factor-1 (SDF-1), produced by stromal cells in bone marrow and extramedullary organs. 12 As high expression of the chemokine receptor CXCR4 predicts extramedullary organ infiltration in ALL, Crazzolara et al 12 report that CXCR4 and its ligand play an important role in extramedullary invasion.…”

Acute lymphoblastic leukemia presenting as nephromegaly in a child: A rare case report

“…Extensive literature search revealed around 9 cases of pediatric ALL with clinically palpable nephromegaly reported in English language to date (Table I). [4][5][6][7][8][9][10][11][12] The possible causes of nephromegaly in ALL can be due to leukemic infiltration or may be simple hypertrophy or hyperplasia. Leukemic infiltration of the liver, spleen, lymph nodes, and mediastinum is more common at diagnosis.…”

“…There are a few case reports of children having ALL with clinically palpable nephromegaly to date (Table I). [4][5][6][7][8][9][10][11][12] We report a 2 years old child who presented with bilateral nephromegaly and was eventually diagnosed with ALL on bone marrow examination.…”

Clinical, laboratory, and morphological characteristics of kidney damage in lymphoproliferative disorders

“…При острых лейкозах повреждение почек вызвано совокупностью факторов, связанных с рас-стройством микроциркуляции вследствие массивной лейкемической инфильтрации паренхимы почек, а также токсическим действием лизоцима и цитоки-нов, продуцируемых или активируемых бластными клетками. Лейкемическая инфильтрация паренхимы почек выявляется преимущественно в продвинутых стадиях лейкоза, приблизительно в 42 % случаев, а в период манифестации значительно реже -в 3-5 % [2,3]. При неходжкинских лимфомах (НХЛ) пораже-ние почек обнаруживается в основном при прогрес-сировании или рецидиве заболевания [4].…”