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Year : 2011  |  Volume : 54  |  Issue : 1  |  Page : 210-211
Acute basophilic leukemia in an infant with proptosis


1 Department of Medical Oncology, Dr. B. R. A. Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi - 110 029, India
2 Department of Laboratory Oncology, Dr. B. R. A. Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi - 110 029, India

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Date of Web Publication7-Mar-2011
 

How to cite this article:
Ghosh I, Bakhshi S, Gupta R. Acute basophilic leukemia in an infant with proptosis. Indian J Pathol Microbiol 2011;54:210-1

How to cite this URL:
Ghosh I, Bakhshi S, Gupta R. Acute basophilic leukemia in an infant with proptosis. Indian J Pathol Microbiol [serial online] 2011 [cited 2019 Dec 11];54:210-1. Available from: http://www.ijpmonline.org/text.asp?2011/54/1/210/77411


Acute basophilic leukemia (ABL) is a rare diagnosis and needs to be differentiated from other related disorders with similar morphology. We report an infant with ABL and outline the differentials.

This 7-month-old male infant presented with irritability and bilateral proptosis of 3 weeks duration. Examination revealed an irritable infant with anemia and bilateral proptosis. The tip of spleen was palpable. There were no petechiae, lymphadenopathy, gum hyperplasia, urticaria, flushing or circulatory disturbance. Hemogram showed hemoglobin of 9.4 g/dl, total leukocyte count of 61 × 10 9 /l and platelet count of 314 × 10 9 /l. Peripheral blood smear showed leukocytosis with 2% promyelocytes, 15% myelocytes, 9% metamyelocytes, 2% monocytes, 10% lymphocytes, 62% neutrophils and an occasional nucleated red blood cell. Contrast-enhanced computed tomography scan of orbits showed diffuse bone lysis with periosteal reaction involving skull base, skull vault and bilateral bony orbits with associated soft tissue masses extending intracranially, consistent with marrow infiltrative disorder. A bone marrow aspirate showed near total replacement of immature myeloid cells with abundant coarse basophilic granules [Figure 1]. Cytochemistry for toluidine blue (TB) was strongly positive [Figure 2], for myeloperoxidase (MPO) was moderately positive, while that for non-specific esterase and acid phosphatase were negative. Flow cytometry findings were CD13+, CD15+, CD33+, MPO+, CD64+, CD123+, CD45+, CD25-, CD34-,CD117-, CD14- and HLA-DR-. B and T cell markers were negative. At this time, basophilic variant of acute promyelocytic leukemia (APL) and ABL were diagnostic considerations. Cytogenetic studies revealed normal male karyotype (46XY). Fluorescence in-situ hybridization using PML/RARA dual color dual fusion probe was negative in all the cells. A final diagnosis of ABL was considered. The family was counseled for starting acute myeloid leukemia (AML) induction therapy but they declined any treatment.
Figure 1: Bone marrow aspirate (Jenner-Giemsa, ×1000) showing myeloblasts with abundant, coarse basophilic granules

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Figure 2: Bone marrow aspirate (TB, ×1000) showing metachromatic staining of myeloblasts

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The term ABL was first coined by Jaochim in 1906. ABL has been included in the recent World Health Organization (WHO) classification as a subtype of AML, not otherwise specified. True ABL is rare and needs to be distinguished from AML with minimal differentiation, AML with increased basophils, APL with basophilic differentiation, acute mast cell leukemia and also from chronic myeloid leukemia (CML) with basophilic differentiation, especially in accelerated or blastic phase. Symptoms attributable to hyperhistaminemia are seen in a fair proportion of patients with ABL and often provide the first clue for the diagnosis. In ABL, presence of coarse basophilic granules and reactivity for MPO and TB are seen in only a proportion of cells. The present case was unusual for the absence of features of hyperhistaminemia, with almost all the cells exhibiting coarse basophilic granules and reactivity for TB. The basophilic variant of APL has been shown to express similar morphological features but reactivity for TB is seen in only a proportion of cells and coagulopathy is prominent.[1] Although metachromasia with TB is seen in almost all of the mast cell leukemia cells, they do not show reactivity for MPO. Ultrastructural demonstration of immature basophilic granules and theta granules may be helpful in identifying basophilic lineage. On immunophenotyping, the blasts may express pan-leukocyte antigen CD45, HLA-DR, myeloid makers like CD13, CD33, CD11b, other markers like CD9, CD25, CDw17, CD88, CD38, CD117 and CD123. CD203 cytoplasmic positivity has also been suggested as highly representative of basophilic lineage. Numerous karyotypic abnormalities have been reported in ABL, like normal karyotype, translocation t(X; 6) (p11; q23), monosomy 7 and trisomy 8. [2],[3],[4] Though no cytogenetic abnormality is characteristic of ABL, cytogenetic and molecular studies are important to rule out the other differentials like CML and APL with basophilic differentiation, as these conditions have different treatment strategies.

There are no uniform guidelines for management of these patients and various protocols have been tried with variable results. Children are reported to have better outcome compared to adults. Surprisingly, all the four infants with ABL reported in literature were in remission 2 years or more after diagnosis [Table 1]. [3],[4],[5] Unfortunately, the family of the child in our report was not willing for treatment.
Table 1: Summary of infants reported with acute basophilic leukemia

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We highlight the fact that though ABL is a rare entity, one should consider it when the blasts show basophilic granules on light microscopy and then undertake a further detailed evaluation to establish the diagnosis.

 
   References Top

1.Invernizzi R, Iannone AM, Bernuzzi S, D'Alessio A, Fiamenghi C, Fenoglio C, et al. Acute promyelocytic leukemia toluidine blue subtype. Leuk Lymphoma 1995;18:57-60.  Back to cited text no. 1
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2.Seth T, Vora A, Bhutani M, Ganessan K, Jain P, Kumar R, et al. Acute basophilic leukemia with t (8; 21). Leuk Lymphoma 2004;45:605-8.   Back to cited text no. 2
    
3.Dastugue N, Duchayne E, Kuhlein E, Rubie H, Demur C, Aurich J, et al. Acute basophilic leukaemia and translocation t(X; 6) (p11; q23). Br J Haematol 1997;98:170-6.  Back to cited text no. 3
    
4.Bernini JC, Timmons CF, Sandler ES. Acute basophilic leukemia in a child. Anaphylactoid reaction and coagulopathy secondary to vincristine-mediated degranulation. Cancer 1995;75:110-4.  Back to cited text no. 4
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5.Kurosawa H, Eguchi M, Sakakibara H, Takahashi H, Furukawa T. Ultrastructural cytochemistry of congenital basophilic leukemia. Am J Pediatr Hematol Oncol 1987;9:27-32.  Back to cited text no. 5
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Correspondence Address:
Ritu Gupta
Department of Laboratory Oncology, Dr. B. R. A. Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi - 110 029
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.77411

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