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A Case of Extramedullary Acute Myeloid Leukemia Presenting With Pulmonary Infiltrates FREE TO VIEW

Shine Raju, MD; Kavitha Bagavathy, MD; Ranjit Joseph, MD; Prashant Grover, MD
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University of Connecticut Health Center, Farmington, CT

Chest. 2013;144(4_MeetingAbstracts):626A. doi:10.1378/chest.1705362
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SESSION TITLE: Cancer Student/Resident Case Report Posters I

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM

INTRODUCTION: Extramedullary acute myeloid leukemia (AML) or myeloid sarcoma (MS) is a rare occurance. We describe a case of MS with predominantly pulmonary symptoms.

CASE PRESENTATION: A 20-year-old female was admitted with 9/10 right-sided pleuritic chest pain and exertional dyspnea of 2-days duration. On recent admissions, she was found to be pancytopenic with negative workup. Family history was significant for leukemia in her mother and lupus in her aunt. Laboratory data revealed pancytopenia, low reticulocyte count and an ANA titer of 1:5120 with a speckled pattern. All other serologies were negative. Computerized tomography of the chest showed ‘fluffy infiltrates’ in the right middle lobe and bilateral bases. She was diagnosed with pneumonia and started on intravenous Moxifloxacin. Over the next 72 hours, her respiratory status progressively worsened and she was intubated for acute hypoxic respiratory failure. She suffered a cardiac arrest and expired, despite all resuscitative efforts. An autopsy was performed which showed myeloid sarcoma (MS) extensively involving bilateral lungs, kidneys, stomach, esophagus and cardiac muscle. Multifocal lymphatic and splenic involvement was also noted. There was no evidence of pathologic involvement of the bone marrow or peripheral blood.

DISCUSSION: Myeloid sarcoma (MS) also called extramedullary AML, granulocytic sarcoma or chloroma is a rare manifestation characterized by the occurrence of 1 or more tumor myeloid masses occurring at an extramedullary site. The incidence of myeloid sarcoma is less than 1% among all cases of AML [1]. It most commonly involves the bone, skin or lymphatic tissue, although any part of the body can be affected [2]. MS is mostly comprised of myeloblasts that may partially or completely efface the tissue architecture. MS may develop de novo or in conjunction with Acute myeloid leukemia (AML), myeloproliferative neoplasm (MPN) or myelodysplastic syndrome (MDS). The definitive diagnosis involves identifying the tumor cells as myeloid with the use of myeloperoxidase or lysozyme staining, flow cytometry, or immunophenotyping from tissue sections [3].

CONCLUSIONS: Myeloid sarcoma can present with a myriad of symptoms involving nearly all organ systems. Pulmonary involvement is rare, but should always be considered in patients with a family history of hematological malignancies presenting with pulmonary infiltrates showing minimal response to antibiotics. In such cases, prompt tissue diagnosis may prevent fatal complications.

Reference #1: Acute leukemia incidence and patient survival among children and adults in the United States, 2001-2007.Dores GM, Devesa SS, Curtis RE; Blood. 2012;119(1):34.

Reference #2: Granulocytic sarcoma: a clinicopathologic study of 61 biopsied cases.Neiman RS, Barcos M, Berard C; Cancer. 1981;48(6):1426

Reference #3: Granulocytic sarcoma: 32 cases and review of the literature.Paydas S, Zorludemir S, Ergin MS; Leuk Lymphoma. 2006;47(12):2527

DISCLOSURE: The following authors have nothing to disclose: Shine Raju, Kavitha Bagavathy, Ranjit Joseph, Prashant Grover

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