Acute Philadelphia Chromosome Positive Biphenotypic Leukemia Presenting with Bilateral Orbital Chloroma: A Rare Case Report

Introduction: Chloromas are characteristically formed by the extramedullary soft tissue infiltration by the immature myeloid malignant cells. Such extramedullary masses are most commonly seen in acute myeloid leukemia usually in the M2, M4, M5 subtypes of the AML FAB classification. However, it has been reported to rarely present only in pediatric patients with acute lymphoblastic leukemia.Presentation of the case: We encountered an unusual case of a young male, who presented with proptosis of both eyes followed by fever and fatigue. On evaluation, he was diagnosed to have bilateral orbital chloroma which was due to infiltration by leukemic cells of acute leukemia. Flowcytometry revealed features confirming an acute biphenotypic leukemia. Subsequently, cytogenetic evaluation revealed the leukemic cells to be Philadelphia chromosome positive.Conclusion: To our knowledge, this is the first case of bilateral orbital choloroma due to Philadelphia positive biphenotypic acute lymphoblastic leukemia

Head and Neck Lymphomas: Tip of the Iceberg?

ABSTRACT Background: Lymphomas comprise around 5% of all head and neck neoplasms and is the second most common extra nodal non hodgkin's lymphoma (NHL). However there is sporadic data on this entity from the subcontinent and hence we undertook this study. Methodology: This retrospective observational study was conducted at a tertiary care oncology center in India on diagnosed cases of NHL between January 2007 and December 2013. All patients were diagnosed based on histopathology and immunohistochemistry. Staging work up was done in all patients. Patients were considered as primary Head and Neck lymphomas if there was head and neck as the predominant site with or without regional lymph node involvement. Results: A total of 39 patients were studied. The age at presentation ranged from 29 to 78 years. The most common site of presentation was oral cavity (26%; n=10), followed by parotid and thyroid (18% each; n=7), eye (12%, n=5), maxilla (8%; n=3), paranasal sinuses (8%; n-=3) cheek (8%, n=3), and nasal cavity (2%, n=1). 41% (n=16) cases were in stage I, 43% (n=17) in stage II, 3% (n=1) in stage III, and 13% (n=5) were in stage IV. Most common histology was DLBCL (71%; n=28), followed by plasmablastic (10%; n=4), marginal zone (8%, n=3), mantle cell (3%; n=1), follicular lymphomas (5%; n=2), and NK/T cell lymphoma (3%; n=1). Most of the patients were of low risk (67%; n=26), followed by intermediate (23%; n=9), and high risk (10%; n=4). Patients were treated with anthracycline based chemotherapy +/-radiotherapy. In this study, stage I and stage II patients had a better prognosis and overall survival, median OS 28 months and 11 months, respectively. In stage III and IV, it was 7 and 3 months, respectively. According to site, the best median overall survival was seen with parotid (27 m), paranasal sinus (26m), and oral cavity (23 m), followed by thyroid (18 m) nasal cavity (17 m), maxilla (11 m), eye (8 m), and cheek (7 m)