Primary Granulocytic sarcoma of the ileum, preceding de novo acute myelogenous leukemia

Atanas Stanchev, Kamelia Alexandrova, Svetlana Christova, Dora Popova, Milena Velizarova, Georgi Balatzenko, Zoja Spasova, Evgueniy Hadjiev

Résultats de recherche: Contribution à un journalArticleRevue par des pairs

Résumé

Primary granulocytic sarcomas (GS) are tumor masses composed of immature cells of granulocytic series in extramedullary sites, diagnosed more than 30 days before the appearance of overt leukemia. We report a case of a 34-year old male who had a previous history of acute myelomonocytic leukemia (FAB-M4) diagnosed in 1993. He was in complete remission for 10 years. In 2003, he was admitted in the clinic of gastroenterology with diffuse abdominal pain, nausea and vomiting. Radiography of ileum and CT-scan of abdomen revealed data suggestive of Crohn's disease and treatment with corticosteroids and 5-aminosalycilates was started. Five months later, the patient was referred to the clinic of haematology because of peripheral blood leukocytosis with blast cells. Bone marrow phenotyping was consistent with TdT+ acute myelomonocytic leukemia; Cytogenetic abnormalities were not detected; complete remission was achieved following 2 induction cycles with Idarubicin and Cytosine- arabinoside. Four months later, the patient was admitted with bone marrow relapse and a tumor mass in the ileocaecal region. The CT-scan showed an eccentric infiltrate narrowing the ileal lumen and spreading to the ileocaecal region. The patient died after 3 cycles with Cytosine-arabinoside, Mitoxantrone and Etoposide without clinical response. A histologically confirmed massive leukemic infiltration of the ileum was revealed during the autopsy with positive immunostains for MPO, CD34. This is a rare case of GS preceding relapse of acute myelomonocytic leukemia, determining poor prognosis, shorter extent of the complete remission and overall survival.

langue originaleAnglais
Pages (de - à)97-100
Nombre de pages4
journalHAEMA
Volume10
Numéro de publication1
étatPublié - 1 janv. 2007
Modification externeOui

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