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Case Reports
. 2016 Nov;10(11):ED19-ED20.
doi: 10.7860/JCDR/2016/20740.8926. Epub 2016 Nov 1.

Congenital Acute Myeloid Leukaemia with Pseudo-Chediak-Higashi Like Granules: A Case Report

Vandana Puri  1 , Sandip Barman  2 , Pooja Sharma  2 , Meera Sikka  3
Affiliations
Case Reports

Congenital Acute Myeloid Leukaemia with Pseudo-Chediak-Higashi Like Granules: A Case Report

Vandana Puri et al. J Clin Diagn Res. 2016 Nov.

Abstract

Congenital leukaemia is a very rare entity comprising 0.8% of all childhood leukaemias. Pseudo-Chediak-Higashi Anomaly (PCHA) in acute leukaemia is a rarely described entity. However, co-existence of congenital myeloid leukaemia with PCHA is a very rare entity and to the best of our knowledge has not been described in literature till date. A full term new-born presented on the 27th day of life with severe gastroenteritis. Complete blood counts and peripheral smear examination revealed leucocytosis with presence of 76% blast cells. Approximately 15% of these blast cells showed presence of pseudo-Chediak-Higashi like granules. The diagnosis of acute myeloid leukaemia was confirmed by flow cytometry. The case report is presented due to its rarity and to highlight the differential diagnosis and clinical implications of this entity.

Keywords: Coarse granules; Leukaemia; New born; Prognosis.

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Figures

[Table/Fig-1]:
[Table/Fig-1]:
Wright stained peripheral smear showing blasts with pseudo-Chediak-Higashi granules (×400). Inset showing higher magnification of single blast (×1000).
[Table/Fig-2a]:
[Table/Fig-2a]:
On flow cytometry, gated blasts revealed positivity for CD34, CD117, HLA DR and CD33.
[Table/Fig-2b]:
[Table/Fig-2b]:
Flow cytometry revealing positivity for cMPO.
See this image and copyright information in PMC

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