Clinico-epidemiological study of extramedullary disease manifestations in childhood acute lymphoblastic leukaemia in a tertiary care centre

Suchi Acharya, Dipshikha Maiti, Supratim Datta


Background: Data on extramedullary manifestation of acute lymphoblastic leukemia (ALL) in Indian children are very limited, authors analyzed the clinical, epidemiological and immunophenotype profile of these manifestations in our pediatric patients.

Methods: This was a prospective observational study in which data of 46 children with ALL, aged 0 to 14 years were analysed.

Results: Among the total 46 patients of ALL admitted 86.95% presented with extra medullary features. Definite male preponderance (67.5%) was observed, 80% patients were aged between one to ten years. Common extra medullary features included hepatomegaly (95%), followed by splenomegaly (77%), lymphadenopathy (58%), arthritis (17.5%), and CNS involvement in 5%.  Mediastinal mass was seen in 5 %. Majority of them (67.5%) had White blood cell (WBC) count less than 20,000/mcL. 92.5% were Pre B ALL, while T ALL was seen in three (7.5%) patients. Out of these three T cell ALL two presented with mediastinal mass and one with CNS leukemia.

Conclusions: The clinicians must be aware of the constellation of different extramedullary features to diagnose ALL. Early diagnosis and prompt treatment may improve the clinical outcome in these patients. Authors suggest larger multi-centric studies to understand the clinical and laboratory profile of extramedullary disease in ALL for better risk stratification to help in risk-adopted therapy.


Acute lymphoblastic leukemia, B-ALL, Immunophenotype, T-ALL

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Gurney JG, Severson RK, Davis S, Robinson L. Incidence of cancer in children in the United States. Cancer. 1995;75(8):2186-95.

Young J, Gloeckler RL, Silverberg E, Horm J, Miller R. Cancer incidence, survival and mortality for children younger than age 15 years. Cancer. 1986;58(S2):598-602.

Arora RS, Eden TO, Kapoor G. Epidemiology of childhood cancer in India. Indian J Cancer. 2009;46(4):264.

Chaudhuri K, Sinha A, Hati GC, Karmakar R, Banerjee A. Childhood malignancies at BS Medical College: a ten year study. Indian J Pathol Microbiol. 2003;46(2):194-6.

Arora RS, Arora B. Acute leukemia in children: A review of the current Indian data. South Asian J Cancer. 2016;5(3):155-60.

Reaman GH. A national approach to evaluating delays in diagnosis and treatment: Potential implications for age related and geo‐political differences in access to care and outcome in children and adolescents with cancer. Pediatr Blood Cancer. 2008;51(4):447-8.

Klein-Geltink JE, Pogany LM, Barr RD, Greenberg ML, Mery LS. Waiting times for cancer care in Canadian children: Impact of distance, clinical, and demographic factors. Pediatr Blood Cancer. 2005;44:318-27.

Marwaha RK, Kulkarni KP. Childhood acute lymphoblastic leukemia: Need of a national population based registry. Indian Pediatr. 2011;48:821.

Khalid S, Moiz B, Adil SN, Khurshid M. Retrospective review of pediatric patients with acute lymphoblastic leukemia: A single center experience. Indian J Pathol Microbiol. 2010;53:704-10.

Arya LS, Padmanjali KS, Sazawal S, Saxena R, Bhargava M, Kulkarni KP, et al. Childhood T-lineage acute lymphoblastic leukemia: management and outcome at a tertiary care center in North India. Indian Pediatr. 2011;48(10):785.

Padhi S, Sarangi R, Mohanty P, Das R, Chakravarty S, Mohanty R, Jacob RT. Cytogenetic profile of pediatric acute lymphoblastic leukemia (ALL): analysis of 31 cases with review of literature. Caryologia. 2011;64(1):33-41.

Kulkarni KP, Marwaha RK, Trehan A, Bansal D. Survival outcome in childhood ALL: experience from a tertiary care centre in North India. Pediatr Blood Cancer. 2009;53(2):168-73.

Biswas S, Chakrabarti S, Chakraborty J, Paul PC, Konar A, Das S. Childhood acute leukemia in West Bengal, India with an emphasis on uncommon clinical features. Asian Pac J Cancer Prev. 2009;10:903-6.

Settin A, Al Haggar M, Al Dosoky T, Al Baz R, Abdelrazik N, Fouda M, et al. Prognostic cytogenetic marker in childhood acute lymphoblastic leukemia. Indian J Pediatr. 2007;74(3):255-63.

Ramyar A, Shafiei M, Rezaei N, Asgarian-Omran H, Esfahani SA, Moazzami K, et al. Cytologic phenotypes of B-cell acute lymphoblastic leukemia-a single center study. Iran J Allergy Asthma Immunol. 2009;8(2):99-106.

Lanzkowsky P. Leukemias in ‘Manual of Pediatric Hematology and Oncology’ 4th ed. Elsevier Academic Press, Burlington; 2005:415-52.