Renal Presentation in Pediatric Acute Leukemia

Abstract

Renal enlargement at time of diagnosis of acute leukemia is very unusual. We here in report 2 pediatric cases of acute leukemia who had their renal affection as the first presenting symptom with no evidences of blast cells in blood smear and none of classical presentation of acute leukemia.The first case is a 4-year-old girl who presented with pallor and abdominal enlargement. Magnetic resonance imaging showed bilateral symmetrical homogenous enlarged kidneys suggestive of infiltration. Complete blood picture (CBC) revealed white blood count 11 × 109/L, hemoglobin 8.7 g/dL and platelet count 197 × 109/L. Bone marrow aspiration was performed, and diagnosed precursor B-cell ALL was made. The child had an excellent response to modified CCG 1991 standard risk protocol of chemotherapy with sustained remission, but unfortunately relapsed 11 month after the end of therapy.The second child was 13-month old, presented with pallor, vomiting, abdominal enlargement, and oliguria 2 days before admission. Initial CBC showed bicytopenia, elevated blood urea, creatinine, and serum uric acid, while abdominal ultrasonography revealed bilateral renal enlargement. Bone marrow examination was done and showed 92% blast of biphenotypic nature. So, biphynotypic leukemia with bilateral renal enlargement and acute renal failure was subsequently diagnosed. The patients admitted to ICU and received supportive care and prednisolone. Renal function normalized and chemotherapy was started. The child achieved complete remission with marked reduction of kidney size but, unfortunately she died from sepsis in consolidation phase of therapy. This case demonstrates an unusual early renal enlargement in childhood acute leukemia.Renal involvement of acute leukemia should be considered in child presenting with unexplained bilateral renal enlargement with or without renal function abnormalities and bone marrow examination should be included in the workup.

Accurate detection of renal leukemic involvement in children using 3‐D computed tomography modeling

“…In the present cohort, nine of 25 patients (36%) had renal involvement. Most of them had bilateral mass lesions and only one patient had diffuse nephromegaly alone without mass lesions, consistent with past reports …”

“…In the present limited series, almost all cases of renal involvement were detected as nodules, in which half were accompanied by nephromegaly consisting of small piled‐up nodules. True diffuse nephromegaly, however, was also identified in one of the present patients (case 2, Table ), which could presented only as enlarged kidneys without any nodule formations, and have poor prognosis …”

“…The correlation between renal involvement and extramedullary lesions has not been identified in large clinical trials, but some cases of childhood ALL with renal involvement and other concurrent extramedullary involvements such as CNS, testes and pancreas have been reported to be associated with poor prognosis. 4,5,23,37 In the present study, one patient with renal involvement had repeated extramedullary relapse, including renal mass lesions, CNS and bone marrow nodular lesions. The relationship between adhesion molecules and extramedullary involvement has been intensively studied, although no specific markers have been identified so far.…”

“…In the present study, patients with renal involvement had significant extramedullary involvement other than CNS involvement. The correlation between renal involvement and extramedullary lesions has not been identified in large clinical trials, but some cases of childhood ALL with renal involvement and other concurrent extramedullary involvements such as CNS, testes and pancreas have been reported to be associated with poor prognosis . In the present study, one patient with renal involvement had repeated extramedullary relapse, including renal mass lesions, CNS and bone marrow nodular lesions.…”

“…Most of them had bilateral mass lesions and only one patient had diffuse nephromegaly alone without mass lesions, consistent with past reports. [21][22][23] Conventionally, renal involvement in pediatric ALL has been diagnosed on ultrasonography or i.v. pyelogram according to the definition of unilateral or bilateral nephromegaly, based on the length of the major or minor axis (>2 SD), diffuse abnormal findings, or mass lesions.…”