Journal of Leukemia
Open Access

Acute Leukemic form of Blastic Plasmacytoid Dendritic Cell Neoplasm in Children

Stefano Carugo^{* *}Correspondence: Stefano Carugo, Department of Clinical Science and Oncology, University of Milan, Milano MI, Italy, Email:

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About the Study

A previously healthy 2-year-old girl had a fever and cough for 2 days. With 76% blasts, peripheral blood showed marked leukocytosis (white blood cells, 75000/mL) (Figure 1).

Figure 1: Blastic plasmacytoid dendritic cell neoplasm in Children.

Blastic plasmacytoid dendritic cell neoplasm in children

Panels A-D: The blasts varied in size from medium to large, with agranular paleblue cytoplasm. The nuclei were irregular, folded or formed like flowers. A prominent nucleolus was seen in some of the specimens. Blasts were found in 87% of the cerebrospinal fluid [1].

Panel E: 47, X, der(X)t(X;8)(q24;q24.1), add(5)(p13), -6, del(6) (q13q27), +8, der(8), t(X;8), add(8) (q24.1) × 2, del(9)(q12q31), del(9)(q13q22), der(10;13)(q10;q10), add(12)(p13), -14, +3mar (20), del(9)(q12q31), del(9)(q13q22), der(10;13)(q10;q10), add(12)(p13),-14 MYC rearrangement t(X;8) (q24;q24.1) is confirmed by Fluorescence In Situ Hybridization (FISH).

FISH (Fluorescence In Situ Hybridization) showed a single deletion of the ETV6 gene but no MLL gene rearrangement, BCR/ABL1 translocation, or ETV6/RUNX1 translocation. The patient was diagnosed with blastic plasmacytoid dendritic cell neoplasm and was in complete remission 4 weeks after starting high-risk acute lymphoblastic leukaemia therapy, according to a bone marrow biopsy. The recognized distinguish this case: young age, negative CD⁵⁶, acute leukaemia without skin lesions, and central nervous system involvement [2].

Panels F-H: The lactate dehydrogenase level in the patient's blood was 3759 IU/L. The patient's lymphadenopathy was significant, but she had no skin lesions or hepatosplenomegaly. The blasts were positive for CD⁴, CD⁷, CD³⁶, CD³⁸, dim CD⁴⁵, CD¹²³, and HLA-DR by flow cytometry, but negative for the tested lineage markers CD², CD³ (surface and cytoplasmic), CD⁵, CD¹⁴, CD¹⁹, CD²⁰, CD³³, CD³⁴, CD⁴¹, CD⁵⁶, CD⁶⁴, CD¹¹⁷, TdT, and myeloperoxidase by flow cytometry [3].

Panel I: The blasts tested positive for T-cell leukaemia 1 by immunohistochemistry.

References

1. Mullighan CG, Miller CB, Radtke I, Letha A Phillips, James Dalton, Jing Ma, et al. BCR–ABL1 lymphoblastic leukaemia is characterized by the deletion of Ikaros. Nature. 2008;453(7191):110-114.

   [CrossRef] [Google Scholar] [PubMed]

2. Kosmider O, Gelsi-Boyer V, Cheok M, Sophie Grabar, Véronique Della-Valle, Françoise Picard, et al. TET2 mutation is an independent favorable prognostic factor in myelodysplastic syndromes (MDSs). Blood. 2009;114(15):3285-3291.

   [CrossRef] [Google Scholar] [PubMed]

3. Tefferi A, Pardanani A, Lim KH, O Abdel-Wahab, T L Lasho, J Patel, et al. TET2 mutations and their clinical correlates in polycythemia vera, essential thrombocythemia and myelofibrosis. Leukemia. 2009;23(5):905-911.

   [CrossRef] [Google Scholar] [PubMed]