Journal of Cytology
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Year : 2020  |  Volume : 37  |  Issue : 2  |  Page : 114-115
Unusual presentation of chronic myeloid leukemia in chronic phase as multiple soft tissue chloromas


Department of Pathology, Dr Baba Saheb Medical College and Hospital, Rohini Sector 6, New Delhi, India

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Date of Submission08-Nov-2019
Date of Acceptance02-Feb-2020
Date of Web Publication02-Apr-2020
 

How to cite this article:
Misra S, Gupta K, Mathur P, Rani P. Unusual presentation of chronic myeloid leukemia in chronic phase as multiple soft tissue chloromas. J Cytol 2020;37:114-5

How to cite this URL:
Misra S, Gupta K, Mathur P, Rani P. Unusual presentation of chronic myeloid leukemia in chronic phase as multiple soft tissue chloromas. J Cytol [serial online] 2020 [cited 2020 Aug 3];37:114-5. Available from: http://www.jcytol.org/text.asp?2020/37/2/114/281790




Sir,

A 32-year-old woman with swelling over left gluteal region and posterior aspect of both legs was referred from the dermatology OPD with clinical diagnosis of erythema nodosum. The swellings were noted 3 months back and gradually increased in size. She also gave history of weakness since last 5 months. On examination, the gluteal swelling measured 4 × 3 × 2 cm; right and left leg swellings measured 3 × 3 × 1 cm and 4 × 3 × 1 cm, respectively [Figure 1]a. They were subcutaneous with ill-defined margins and firm to feel. The overlying skin was discolored; there was no history of trauma. Fine-needle aspiration cytology (FNAC) was performed from all three lesions and revealed similar findings. The smears were cellular and showed sheets of immature granulocytic precursors with occasional myeloblasts [Figure 1]b. Based on the cytology findings, a diagnosis of multiple soft tissue chloromas was rendered and patient was reviewed. She was found to have massive splenomegaly. Hemogram revealed an elevated total leucocyte count (TLC) of 2.5 × 105/μl, hemoglobin of 8.2 g/dl, and platelet count of 80 × 103/μl. The peripheral smear showed prominent myeloid bulge with increase in granulocytic precurors and 5% myeloblasts in differential count. A final diagnosis of chronic myeloid leukemia in chronic phase (CML-CP) was given. Bone marrow performed confirmed the diagnosis. The tumor was found to be positive for BCR-ABL1 (p210) by RT-PCR. She was put on imatinib mesylate (400 mg/day). Four months posttreatment, resolution of skin lesions and normalization of TLC (6400/μl) was noted.
Figure 1: 1a: Left leg indurated swelling with greenish-brown discoloration; 1b: Cellular cyto-smear from the leg lesion showing sheets of immature granulocytic precursors with occasional myeloblasts (Giemsa, 400x)

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Chloromas/granulocytic sarcomas (GS) are rare extramedullary tumors composed of immature myeloid cells which are usually associated with acute myeloid leukemia (AML).[1] They were first described in 1811[2] while the term cholorma was coined in 1853;[3] their association with leukemia was established in 1893 by Rappaport who gave the term GS.[4] These tumors often display greenish discoloration due to enzymatic action of myeloperoxidase enzyme.[3] Most common sites of involvement are skin, lymphnode, bones of facial skeleton, and gums. In a study by Bangerter et al., most cases of chloromas were secondary to AML or CML in blast crisis.[5] With tyrosine kinase inhibitors becoming the standard therapy, risk of blastic transformation of CML-CP and progression to choloromas has greatly reduced. However, multiple soft tissue chloromas as the initial presentation of CML-CP is very rare with only few cases reported in the literature.[6]

Cytomorphological differential diagnoses include hematologic disorders like non-Hodgkin's lymphoma, Hodgkin's lymphoma, leukemoid reaction; undifferentiated carcinomas and Ewings sarcoma.[6] Evidence of myeloid differentiation on FNAC and complete hemogram followed by molecular studies (FISH/RT-PCR) would help clinch the diagnosis of CML. Choloromas are supposed to be a poor prognostic factor in AML, while in CML, they are thought to be indicative of an impending blast crisis. Our case presented with multiple soft tissue chloromas which were diagnosed on FNAC, following which a complete hematologic work-up revealed final diagnosis of CML-CP. Thus, FNAC is a rapid and accurate means of diagnosis of soft tissue choloromas which prompts urgent hematologic workup so that a definitive diagnosis can be reached and early treatment can be initiated.

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.



 
   References Top

1.
Madhumathi DS, Raman RN, Lakshmi DV, Gayathri DM, Rani AS. Skin lesions in malignancy: Case 1. Chronic myeloid leukemia in lymphoid blast crisis presenting as multiple cutaneous masses. J Clin Oncol 2001;19:2098-102.  Back to cited text no. 1
    
2.
Burns A. Observations of Surgical Anatomy: Head and Neck. Edinburgh, Scotland: Thomas Royce; 1811. p. 364-6.  Back to cited text no. 2
    
3.
King A. A case of chloroma. Monthly J Med 1853;17:97.  Back to cited text no. 3
    
4.
Rappaport H. Tumors of the Hematopoietic System. Atlas of Tumor Pathology, Section 111, Fascicle 8. Washington, DC: Armed Forces Institute of Pathology; 1966. p. 241-3.  Back to cited text no. 4
    
5.
Bangerter M, Hildebrand A, Waidmann O, Griesshammer M. Diagnosis of granulocytic sarcoma by fine-needle aspiration cytology. Acta Haematol 2000;103:102-8.  Back to cited text no. 5
    
6.
Nagarajarao HS, Akhtar I, Heard K, Baliga M. Unusual presentation of chronic myelogenous leukemia as multiple skin chloromas: Report of a case with clinical and cytologic correlation. Acta Cytol 2009;53:235-8.  Back to cited text no. 6
    

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Correspondence Address:
Dr. Kusum Gupta
Department of Pathology, Dr Baba Saheb Ambedkar Hospital, Rohini Sector 6, New Delhi- 110085
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/JOC.JOC_142_19

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