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A rare case of congenital acute lymphoblastic leukemia presenting as Leukemia Cutis and generalized lymphadenopathy

Authors:

Sangeetha Wickramaratne ,

Lady Ridgeway Hospital for Children-Colombo, LK
About Sangeetha
Senior Registrar in Neonatology
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Nipunika Senadheera,

Lady Ridgeway Hospital for Children-Colombo, LK
About Nipunika
Consultant Haematologist
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Sanjeeva Gunasekera,

National Cancer Institute, LK
About Sanjeeva
Consultant Paediatric Oncologist
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Shyama Basnayake

Lady Ridgeway Hospital for Children-Colombo, LK
About Shyama
Consultant Neonatologist
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Abstract

Congenital leukemia is rare and possesses much poor prognosis with higher relapse rate even with intensive chemotherapy. Acute lymphoblastic leukemia (ALL) is even rarer with poorer outcome.

 

This is a case of congenital leukemia detected at birth in a non-syndromic term baby with petechiae, ecchymoses, and splenomegaly. There was leukemia cutis and generalized lymphadenopathy which are considered as uncommon manifestations in congenital ALL. Diagnosis was made with peripheral blood having hyperleukocytosis with 95% lymphoblasts, thrombocytopenia and anaemia with flowcytometry findings suggestive of B type ALL. There was significant pancreatic enlargement detected ultrasonically with normal enzyme levels which is an unusual association in congenital ALL.

 

Despite having extremely high leucocyte count, high Lactate Dehydrogenase with some biochemical features such as hypocalcemia, hyper-phosphatemia and hyperuricemia to suggest tumour lysis, baby was managed successfully with hyperhydration, intravenous steroids and oral Allopurinol during the first 02 weeks of life until baby became stable for initiation of chemotherapy.

 

There was early response to steroid treatment indicating better prognosis while having early age at diagnosis, significant leukocytosis at presentation and CD10 negativity as predictors of poor outcome.

How to Cite: Wickramaratne S, Senadheera N, Gunasekera S, Basnayake S. A rare case of congenital acute lymphoblastic leukemia presenting as Leukemia Cutis and generalized lymphadenopathy. Sri Lanka Journal of Perinatal Medicine. 2021;2(2):19–26. DOI: http://doi.org/10.4038/sljpm.v2i2.43
Published on 13 Dec 2021.
Peer Reviewed

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