| Article Access Statistics|
| Viewed||1414 |
| Printed||34 |
| Emailed||0 |
| PDF Downloaded||16 |
| Comments ||[Add] |
Click on image for details.
|Year : 2017 | Volume
| Issue : 4 | Page : 271-272
Precursor B-cell acute lymphoblastic leukemia: An unusual cause of bilateral nephromegaly in an infant
D Ramadoss1, S Karande1, M Muranjan1, P Wagle2
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 Publication||11-Oct-2017|
Department of Pediatrics, Seth GS Medical College and KEM Hospital, Parel, Mumbai, Maharashtra
Source of Support: None, Conflict of Interest: None
|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. Renal involvement in leukemia is well known, however, presentation in the form of nephromegaly is very rare (only 3%–5% of ALL cases). Furthermore, only 2% cases of childhood leukemia occur in the age group of <1 year and are termed as infant leukemia. ALL in an infant presenting as bilateral nephromegaly has been rarely documented.,,
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|
Click here to view
|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)|
Click here to view
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%), infant leukemia has a poor prognosis which worsens with decreasing age.,, Renal involvement and kidney size usually do not affect the outcome in afflicted older children, but its significance in infant leukemia is uncertain. 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., 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
Conflicts of interest
Dr. Sunil Karande is the Editor of the Journal of Postgraduate Medicine.
| :: References|| |
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.
Mantan M, Singhal KK, Sethi GR. Acute lymphoblastic leukemia: An unusual cause of nephromegaly in infancy. Indian J Pediatr 2010;77:583.
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.
Balogun TM, Salisu MA. Infant acute lymphoblastic leukemia with bilateral nephromegaly: Report of an unusual combination. ARC J Hematol 2016;1:6-9.
Kulkarni KP, Arya LS. Infantile acute lymphoblastic leukemia and nephromegaly. Indian J Pediatr 2010;77:1199-200.
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.
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.
Pui CH, Evans WE. Acute lymphoblastic leukemia in infants. J Clin Oncol 1999;17:438-40.
[Figure 1], [Figure 2]