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CASE SNIPPET
Year : 2017  |  Volume : 63  |  Issue : 4  |  Page : 271-272

Precursor B-cell acute lymphoblastic leukemia: An unusual cause of bilateral nephromegaly in an infant


1 Department of Pediatrics, Seth GS Medical College and KEM Hospital, Parel, Mumbai, Maharashtra, India
2 Department of Pathology, Tata Memorial Hospital, Parel, Mumbai, Maharashtra, India

Date of Web Publication11-Oct-2017

Correspondence Address:
D Ramadoss
Department of Pediatrics, Seth GS Medical College and KEM Hospital, Parel, Mumbai, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jpgm.JPGM_231_17

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How to cite this article:
Ramadoss D, Karande S, Muranjan M, Wagle P. Precursor B-cell acute lymphoblastic leukemia: An unusual cause of bilateral nephromegaly in an infant. J Postgrad Med 2017;63:271-2

How to cite this URL:
Ramadoss D, Karande S, Muranjan M, Wagle P. Precursor B-cell acute lymphoblastic leukemia: An unusual cause of bilateral nephromegaly in an infant. J Postgrad Med [serial online] 2017 [cited 2020 Jan 23];63:271-2. Available from: http://www.jpgmonline.com/text.asp?2017/63/4/271/216439




Acute lymphoblastic leukemia (ALL) accounts for approximately 77% of all cases of childhood leukemia.[1] Renal involvement in leukemia is well known, however, presentation in the form of nephromegaly is very rare (only 3%–5% of ALL cases).[2] Furthermore, only 2% cases of childhood leukemia occur in the age group of <1 year and are termed as infant leukemia.[1] ALL in an infant presenting as bilateral nephromegaly has been rarely documented.[2],[3],[4]

We report a 2-month-old male child, second by birth order (birth weight 3.5 kg), born of a nonconsanguineous marriage, who presented with increasing pallor, progressive abdominal distension, and mild intermittent fever for 1 month. On examination, the infant had severe pallor, but no icterus, edema, or lymphadenopathy. His weight was 6 kg and length was 56 cm (50–75 per centile and 25–50 per centile on standard WHO growth charts). The liver was palpable 4 cm below right costal margin, firm, nontender with a span of 7 cm. The spleen was palpable 5 cm below left costal margin. Both kidneys were palpable and ballotable. Rest of the physical examination was normal.

Investigations revealed severe anemia (hemoglobin 35 g/L), leukocytosis (white cell count of count 28.4 × 109/L (20% neutrophils and 80% lymphocytes), and thrombocytopenia (platelet count 40 × 109/L). No abnormal cells were seen on peripheral blood smear examination. A peripheral blood flow cytometry did not reveal any cells with aberrant markers suggestive of blasts. Serum aminotransferases (alanine transaminase 530 U/L, aspartate transaminase 289 U/L) and serum lactate dehydrogenase (1522.3 U/L) were elevated. Renal function tests and serum electrolytes were normal. Urine routine examination was normal. Ultrasonography of the abdomen revealed bilateral homogeneously enlarged kidneys [Figure 1]a and [Figure 1]b with preserved corticomedullary junction along with hepatosplenomegaly. Bone marrow biopsy examination revealed the marrow to be diffusely replaced by blast cells [Figure 2]a. Sheets of dyspoietic megakaryocytes were seen. No normal hematopoietic elements were seen. Immunohistochemical staining showed the blasts were TdT and Pax5 positive [Figure 2]b and [Figure 2]c; and CD3, CD20 negative. Few blasts were positive for MPO. A diagnosis of precursor B-cell ALL was confirmed.
Figure 1: (a) Ultrasonography of right kidney measuring 94.88 mm × 48.98 mm; (b) left kidney measuring 49.12 mm × 43.6 mm

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Figure 2: (a) Bone marrow biopsy showing blasts: large cells having high nuclear to cytoplasmic ratio and hyperchromatic nuclei (H and E, ×40); (b) blasts showing strong nuclear immunopositivity for immature/precursor lymphoid cell marker TdT (×40); (c) blasts showing nuclear immunopositivity for pre-B cell marker Pax5 (×40)

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The child was managed symptomatically with packed red cell and platelet concentrate transfusions. The patient was referred to a tertiary care hematooncology unit for chemotherapy. Unfortunately, the child died within 3 weeks of presentation pending initiation of chemotherapy due to financial constraints.

Although ALL in children has a favorable prognosis (overall survival rate being near 80%),[1] infant leukemia has a poor prognosis which worsens with decreasing age.[4],[5],[6] Renal involvement and kidney size usually do not affect the outcome in afflicted older children, but its significance in infant leukemia is uncertain.[7] Known predictors of poor outcome include very young age, high initial leukocyte count, lack of CD10 expression, presence of myeloid-associated antigen expression, presence of 11q23 translocations (or MLL rearrangements), and poor response to initial chemotherapy.[6],[8] Due to financial constraints, further immunophenotypic and molecular characterization of the disease could not be done. The purpose of reporting this case is to highlight that infant with precursor B-cell ALL can rarely develop bilateral nephromegaly. A high degree of clinical suspicion is required for its early diagnosis and initiation of therapy as the disease has a rapid downhill course.

Financial support and sponsorship

Nil.

Conflicts of interest

Dr. Sunil Karande is the Editor of the Journal of Postgraduate Medicine.



 
 :: References Top

1.
Tubergen DG, Bleyer A, Ritchey AK, Friehling E. The leukemias. In: Kliegman RM, Stanton BM, St. Geme J, Schor NF, editors. Nelson Textbook of Pediatrics. 20th ed. Philadelphia: Elsevier; 2016. p. 2437-45.  Back to cited text no. 1
    
2.
Mantan M, Singhal KK, Sethi GR. Acute lymphoblastic leukemia: An unusual cause of nephromegaly in infancy. Indian J Pediatr 2010;77:583.  Back to cited text no. 2
[PUBMED]    
3.
Martins A, Cairoli H, Domínguez P, Martin S, Ortiz C, Potasznik J, et al. Nephromegaly: As unusual presentation of acute lymphoblastic leukemia in an infant. Arch Argent Pediatr 2008;106:263-5.  Back to cited text no. 3
    
4.
Balogun TM, Salisu MA. Infant acute lymphoblastic leukemia with bilateral nephromegaly: Report of an unusual combination. ARC J Hematol 2016;1:6-9.  Back to cited text no. 4
    
5.
Kulkarni KP, Arya LS. Infantile acute lymphoblastic leukemia and nephromegaly. Indian J Pediatr 2010;77:1199-200.  Back to cited text no. 5
[PUBMED]    
6.
Pieters R, Schrappe M, De Lorenzo P, Hann I, De Rossi G, Felice M, et al. A treatment protocol for infants younger than 1 year with acute lymphoblastic leukaemia (Interfant-99): An observational study and a multicentre randomised trial. Lancet 2007;370:240-50.  Back to cited text no. 6
[PUBMED]    
7.
Neglia JP, Day DL, Swanson TV, Ramsay NK, Robison LL, Nesbit ME Jr. Kidney size at diagnosis of childhood acute lymphocytic leukemia: Lack of prognostic significance for outcome. Am J Pediatr Hematol Oncol 1988;10:296-300.  Back to cited text no. 7
[PUBMED]    
8.
Pui CH, Evans WE. Acute lymphoblastic leukemia in infants. J Clin Oncol 1999;17:438-40.  Back to cited text no. 8
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