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LETTER TO EDITOR
Year : 2017  |  Volume : 10  |  Issue : 5  |  Page : 500-501  

Chloroma in a child with T-cell acute lymphoblastic leukemia


1 Department of Pediatrics, Sitaram Bhartia Institute of Science and Research, New Delhi, India
2 Department of Pediatrics, Chacha Nehru Bal Chikitsalaya, New Delhi, India
3 Department of Pediatrics, All Institute of Medical Sciences, New Delhi, India

Date of Web Publication14-Nov-2017

Correspondence Address:
Rachna Seth
Department of Pediatrics, 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/MJDRDYPU.MJDRDYPU_7_17

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How to cite this article:
Mandal A, Singh A, Gnanaguru V, Seth R. Chloroma in a child with T-cell acute lymphoblastic leukemia. Med J DY Patil Univ 2017;10:500-1

How to cite this URL:
Mandal A, Singh A, Gnanaguru V, Seth R. Chloroma in a child with T-cell acute lymphoblastic leukemia. Med J DY Patil Univ [serial online] 2017 [cited 2019 May 24];10:500-1. Available from: http://www.mjdrdypu.org/text.asp?2017/10/5/500/218201



Sir,

A 6-year-old, premorbidly asymptomatic boy presented with fever, generalized weakness, left eye swelling, and right scrotal swelling for about 25 days. Fever was intermittent and was responding to medication. Left eye swelling was involving the lower eyelid only, painless, gradually progressive and was not associated with difficulty in vision. There was no history of preceding trauma to the eye or scrotum. There was no bone pain, nodular swelling over the body, abdominal distention, bleeding from any site, jaundice, or history of blood transfusion. His past medical and surgical history was unremarkable. On examination, he was hemodynamically stable. There was mild pallor but no jaundice. There was a painless, diffuse swelling over the left lower eyelid with no redness or chemosis and his vision was preserved [Figure 1]a. Another eye was normal. Right testis was larger than the left one, painless with no other signs of inflammation [Figure 1]b. There was bilateral cervical and left inguinal lymphadenopathy [Figure 1]a and [Figure 1]b. The liver was palpable 4 cm below the right costal margin with a span of 11 cm, and the spleen was palpable 5 cm below the left costal margin; both were nontender and firm in consistency. Other systemic examination was essentially normal. He was admitted and initial investigations revealed pancytopenia with hemoglobin 8.3 g/dl, total leukocyte count 2700/mm 3, and platelets 25,000/mm 3. The renal and liver functions were within normal limits. Peripheral smear showed 32% MPO-negative blasts. Flow cytometry from the peripheral blood revealed blasts which were cCD3+, CD7+, CD5+, cd11b+, CD34−, CD4−, CD8−, CD2−, sCD3−, CD1a−, cMPO−, cCD79a−, CD117−, CD13−, CD33−, and CD19−; suggestive of T-cell acute lymphoblastic leukemia (ALL). Noncontrast computed tomography (CT) scan of orbit revealed a hypodense lesion in the lower eyelid [Figure 1]c. Subsequently, fine-needle aspiration cytology of the orbital mass region revealed blast cells, confirming the diagnosis of chloroma. Chest X-ray showed mediastinal mass [Figure 2]a, which was further confirmed with CT scan of the chest [Figure 2]b. He was started on chemotherapy for ALL, and after 1 year, his disease is currently in remission.
Figure 1: (a) A six-year-old male child with left lower eyelid painless swelling with no signs of inflammation (black arrow) and cervical lymphadenopathy (blue arrow); (b) right testicular painless swelling with no signs of inflammation (black arrow) and left inguinal lymphadenopathy (blue arrow); (c) contrast-enhanced computed tomography head showing hypodense collection in preseptal space (red arrow)

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Figure 2: (a) Chest X-ray posteroanterior view showing anterior mediastinal mass; (b) contrast-enhanced computed tomography chest, mediastinal window showing anterior mediastinal mass lesion with contrast enhancement and no evidence of airway compression

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Acute lymphoblastic leukemia (ALL) is the most common malignancy in childhood. It can be either of B-cell or T-cell lineages. Common manifestations of ALL are fever, anemia, bleeding, bone pain, lymphadenopathy, hepatosplenomegaly, etc.[1] Chloroma, also known as myeloid sarcoma, granulocytic sarcoma, myeloblastoma, and extramedullary myeloid cell tumor, is one of the subgroups of myeloid neoplasms in the WHO classification. It is mostly found in associated with acute myelogenous leukemia, less commonly with other myeloproliferative neoplasm, myelodysplastic syndromes, hypereosinophilic syndrome, or polycythemia vera, and rarely as an isolated entity.[2]

Chloroma in ALL has been extremely rare, limited to a few case reports only; they were mostly found as an intracranial mass lesion either at presentation [3] or after relapse of ALL [4] of B-cell lineage. In the absence of an established diagnosis of leukemia, the differentials to be considered for such an ocular swelling are rhabdomyosarcoma, metastatic neuroblastoma, Langerhans cell histiocytosis, and orbital cellulitis.

Testicular involvement is a known feature of relapsed ALL; however again, overt testicular disease has been rarely described with lymphoblastic leukemia [5] and is associated with high-risk features and poor prognosis.

Among the other extramedullary sites to get involved in cases of childhood, ALL at presentation or at the time of relapse includes [6] the central nervous system, heart, lungs, ovaries, pancreas, urinary bladder, gastrointestinal tract, kidneys,[7] skin and subcutaneous tissue,[8] breast,[9] maxillary sinus,[10] parotid gland,[11] and bone and joints including vertebrae.[12] Extramedullary manifestations pose diagnostic difficulties and are known to delay in treatment in childhood leukemias. This is probably the first case of ALL presenting with chloroma and testicular involvement at diagnosis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
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Bhojwani D, Yang JJ, Pui CH. Biology of childhood acute lymphoblastic leukemia. Pediatr Clin North Am 2015;62:47-60.  Back to cited text no. 1
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Ünal S, Kuskonmaz B, Balci YI, Cengiz B, Tuncer M, Gürgey A, et al. An unusual presentation of pediatric acute lymphoblastic leukemia with parotid gland involvement and dactylitis. Turk J Haematol 2010;27:117-9.  Back to cited text no. 11
    
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Agarwal BR, Currimbhoy ZC. Isolated vertebral body relapse of acute lymphoblastic leukemia. Indian Pediatr 1994;31:579-81.  Back to cited text no. 12
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