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176 Case Report

Acute Renal Failure Associated with Bilateral


Enlargement of the Kidneys: A Rare Manifestation
of Acute Lymphoblastic Leukemia (ALL)

Authors

H. Escobar1, K. Hner1, M. Pohl1, H. Hopfer2, O. Determann3, M. Lauten1, U. Kontny1

Aliations

Aliation addresses are listed at the end of the article

Key words
childhood

acute lymphoblastic

leukemia
renal enlargement

renal failure

Abstract
&

Zusammenfassung
&

We report a 6-year-old patient who presented


with acute renal failure resolving after vigorous intravenous hydration. Renal biopsy was
taken because of unexplained enlargement of
both kidneys. Histological workup showed infiltration by lymphoblasts while blood counts
showed a normal dierential. Subsequent bone
marrow aspiration revealed 34 % lymphoblasts
of T-lineage origin, leading to the diagnosis of TALL. This case underlines that malignant hematologic infiltration should be considered in patients presenting with unexplained renal failure
and enlarged kidneys.

Wir berichten ber die ungewhnliche Manifestation einer ALL bei einem Mdchen, das initial
wegen eines akuten Nierenversagens behandelt
wurde und bei dem wegen eines sekundrem
Creatininanstieg eine Nierenbiopsie durchgefhrt wurde. Nierenbiopsie und nachfolgende
Knochenmarkpunktion fhrten zur Diagnose einer ALL. In diesem Fall war die Durchfhrung der
Nierenbiopsie entscheidend, da das Blutbild mit
Ausnahme einer leichten Anmie keine Aulligkeiten zeigte.

Introduction
&

Case report
&

Acute renal failure (ARF) occurs in 23 % of children admitted to pediatric special care centers.
Beyond the neonatal age group, it is mostly
caused by renal disease, especially hemolyticuremic syndrome and glomerulonephritis [4].
Kidney biopsy is indicated whenever the etiology
of renal involvement is unclear.
Acute lymphoblastic leukemia (ALL) is the most
common malignancy in children. It accounts for
one-fourth of all childhood cancers and approximately 75 % of all cases of childhood leukemia
[10]. Extramedullary involvement of parenchymatous organs such as liver and spleen leading to
organomegaly is frequently observed. Renal
enlargement is less common, with a frequency of
around 30 % and is usually not associated with
elevated serum creatinine levels [6, 7].
Here, we present the case of a 6-year-old girl with
ALL who presented with ARF associated with
bilateral enlargement of the kidneys and a normal peripheral blood count except of moderate
anemia.

The 6-year-old girl presented to a local hospital


with a one-month history of fatigue, anorexia
and abdominal pain, followed by vomiting, diarrhea and oliguria. Physical examination showed
pallor, hypertension and dehydration (clinically
about 5 %), whereas no enlargement of lymph
nodes, liver or spleen was noted. Initial laboratory findings revealed a blood count with hemoglobin 9.6 g/dl, platelets 401 G/l and leukocytes
9.3 G/l with a normal dierential (lymphocytes
26 %, granulocytes 60 %, monocytes 7 %, eosinophils 3 %, basophils 1 %, no blasts). Serum lactate dehydrogenase was 335 U/l (normal: 104
311 U/l), uric acid 11.5 mg/dl (normal: 2.0
6.0 mg/dl), creatinine 8.8 mg/dl (normal: 0.3
1.0 mg/dl), blood urea nitrogen 115 mg/dl (normal up to 45 mg/dl), potassium 7.0 mmol/l
(normal: 3.25.4 mmol/l). Blood gas analysis
showed a pH of 7.25 with a base excess
of 13 mmol/l. The diagnosis of ARF of prerenal
origin secondary to dehydration due to gastroin-

Schlsselwrter
Kindheit

akute Lymphatische

leukmie
Nierenvergrerung

Nierenversagen

Bibliography
DOI 10.1055/s-0029-1216365
Klin Padiatr 2009; 221: 176178
Georg Thieme Verlag KG
Stuttgart New York
ISSN 0300-8630
Correspondence
Prof. U. Kontny
Universittsklinikum Freiburg
ZKJ
Klinik IV: Pdiatrische
Hmatologie und Onkologie
Mathildenstrae 1
79106 Freiburg
Germany
Tel.: + 49/761/270 4628
Fax: + 49/761/270 4518
udo.kontny@uniklinik-freiburg.
de

Escobar H et al. Acute Renal Failure Associated with Bilateral Klin Padiatr 2009; 221: 176178

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Akutes Nierenversagen mit bilateraler Nephromegalie: Eine seltene


Manifestation der akuten lymphatischen Leukmie (ALL)

Case Report 177

13

18

24

10
8
6
4
2
0
-2

14

28

Days

Days

testinal infection was suspected and intravenous hydration was


started. After ten days, creatinine had dropped to 1.0 mg/dl, but
Fig. 1a). Ultrasubsequently increased again to 5.2 mg/dl (
sonography (US) of the kidneys still showed bilateral non-obstructive enlargement of the kidneys, with hyperechogenic parenchyma on both sides. Magnetic resonance imaging confirmed
Fig. 2), but no additional abdominal or retrothe US findings (
peritoneal abnormalities were seen.
The patient was transferred to our hospital and renal biopsy was
performed revealing extensive interstitial infiltration by atypical
Fig. 3a, b). On
monomorphic medium-sized lymphatic cells (
immunohistochemistry, the cells were positive for CD3 and TdT,
negative for CD10, CD34 and PAX5, leading to the suspicion of
precursor T-cell ALL.
Subsequently, a bone marrow aspirate was done which showed
Fig. 3c). Cytochemistry was positive
34 % mononuclear blasts (
for PAS and acid phosphatase. On immunophenotyping the
blasts expressed TdT, CD7, cyCD3, CD3, CD5, CD2 and CD1a in a
significant proportion of the cells indicating precursor T-cell
ALL.
Cytogenetic analysis of the bone marrow showed a normal karyotype without clonal alterations. Molecular genetic testing for the
fusion products of TEL-AML1, BCR-ABL and MLL-AF4 was negative.
Examination of the cerebrospinal fluid (CSF) surprisingly revealed
massive leukemic infiltration of the central nervous system with
454 cells/l, even though the patient had never complained about
headache, and vomiting had stopped under hydration therapy.
Chemotherapy according to the ALL-Berlin Frankfurt Mnster
(BFM) 2000 protocol was started. The girl had a good response to
induction treatment, being a prednisone good responder ( < 1 000
blasts/mm3 on day 8 of treatment), having a M1 bone marrow on
days 15 and 33 and no evidence of blasts in the CSF at day 18.
After 29 days of chemotherapy serum creatinine and blood urea
Fig. 1b). On day 8, renal
nitrogen values had normalized (

dimensions had been reduced to 55 % of their initial size, and on


day 29 normal dimensions were documented by US.

Discussion
&
ARF in children with leukemia is usually treatment-related and
caused by hyperphosphatemia and hyperuricemia. Rarely, it is
due to ureteral obstruction by enlarged lymph nodes. The occurrence of ARF due to leukemic infiltration as the initial presenting
sign of ALL, such as in our case, has only occasionally been described [1, 2]. Whereas in the other cases reported, abnormalities of the peripheral blood count hinted to the diagnosis of
Fig. 2 Magnetic
resonance imaging
before renal biopsy
showing bilateral
enlargement of the
kidneys without focal
lesions.

Fig. 3 a) Renal biopsy showing dense infiltration by monomorphic atypical medium-sized lymphatic cells (H&E 200 ) which already can be detected on a
touch preparation (b) (10 60); c) Bone marrow aspiration with an increased proportion of monomorphic blasts of lymphoid origin (10 40).

Escobar H et al. Acute Renal Failure Associated with Bilateral Klin Padiatr 2009; 221: 176178

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10
8
6
4
2
0

Fig. 1 Creatinine values before the diagnosis (a)


and after start of BFM therapy (b) the arrow shows
lower creatinine values after vigorous intravenous
hydration.

Evolution of Creatinine
Creatinine mg/dl

Creatinine mg/dl

Evolution of Creatinine

178 Case Report

Conclusion
&
We report on an unusual clinical manifestation of ALL in a child,
who was initially treated for prerenal ARF and underwent renal
biopsy for a secondary increase of creatinine levels. Renal biopsy
and subsequent bone marrow aspiration revealed the diagnosis
of ALL. In this case, renal biopsy was crucial, because blood
counts and dierentiation of leukocytes were entirely normal
except of moderate anemia and did not point towards a hematologic malignancy.

Aliations
1
University Medical Center, Department of Pediatrics and Adolescent
Medicine, Division of Pediatric Hematology and Oncology, Freiburg,
Germany
2
Institute for Pathology, University Hospital Basel, Switzerland
3
University Medical Center Schleswig-Holstein, Campus Kiel, Institute
for Pathology, Section for Hematopathology and Lymph Node Registry,
Kiel, Germany

References
1 Ali SH et al. Acute lymphoblastic leukemia presenting as bilateral renal
enlargement in a child. Med Princ Pract 2008; 17: 504506
2 Boueva A, Bouvier R. Precursor B-cell lymphoblastic leukemia as a
cause of a bilateral nephromegaly. Pediatr Nephrol 2005; 20: 679
682
3 DAngelo P et al. Prognostic value of nephromegaly at diagnosis of
childhood acute lymphoblastic leukemia. Acta Haematol 1995; 94:
8489
4 Flynn JT. Causes, management approaches, and outcome of acute renal
failure in children. Curr Opin Pediatr 1998; 10: 184189
5 Frei E, III et al. Renal and hepatic enlargement in acute leukemia. Cancer 1963; 16: 10891092
6 Hann IM et al. Renal size as a prognostic factor in childhood acute
lymphoblastic leukemia. Cancer 1981; 48: 207209
7 Hilmes MA et al. Pediatric renal leukemia: spectrum of CT imaging
findings. Pediatr Radiol 2008; 38: 424430
8 Neglia JP et al. Kidney size at diagnosis of childhood acute lymphocytic leukemia: lack of prognostic significance for outcome. Am J Pediatr
Hematol.Oncol 1988; 10: 296300
9 Obrador GT et al. Acute renal failure due to lymphomatous infiltration
of the kidneys. J Am Soc Nephrol 1997; 8: 13481354
10 Pui CH. Acute lymphoblastic leukemia in children. Curr Opin Oncol
2000; 12: 312

Conflict of interest: The authors have no conflict of interest to


disclose.

Escobar H et al. Acute Renal Failure Associated with Bilateral Klin Padiatr 2009; 221: 176178

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acute leukemia, blood smear as well as blood counts of our patient was normal except of moderate anemia. In addition, renal
enlargement in acute leukemia usually occurs in association
with hepatosplenomegaly, which was not the case in our patient
[5].
Renal involvement without elevation of serum creatinine in ALL
is not uncommon. Two larger studies using intravenous pyelograpy have shown renal enlargement in 24 % and 30 % of cases
[3, 6]. On contrast-enhanced CT, renal leukemic involvement
may present with a variety of imaging findings such as solitary
or multiple low attenuation masses either uni- or bilaterally [7].
Reduction of renal dimensions under induction of chemotherapy is usually seen [9]. In a retrospective study on 142 children
with ALL, no influence of renal enlargement on survival was
noted [8].