Unusual Presentation of Chronic Myeloid Leukemia in Chronic Phase as Multiple Soft Tissue Chloromas.

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 1a]. 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 1b]. 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)

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.

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