TY - JOUR
T1 - Primary Granulocytic sarcoma of the ileum, preceding de novo acute myelogenous leukemia
AU - Stanchev, Atanas
AU - Alexandrova, Kamelia
AU - Christova, Svetlana
AU - Popova, Dora
AU - Velizarova, Milena
AU - Balatzenko, Georgi
AU - Spasova, Zoja
AU - Hadjiev, Evgueniy
PY - 2007/1/1
Y1 - 2007/1/1
N2 - 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.
AB - 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.
KW - Crohn disease
KW - De novo acute myeloid leukemia
KW - Granulocytic sarcoma
KW - Small intestine
UR - http://www.scopus.com/inward/record.url?scp=84865345425&partnerID=8YFLogxK
M3 - Article
AN - SCOPUS:84865345425
SN - 1108-2682
VL - 10
SP - 97
EP - 100
JO - HAEMA
JF - HAEMA
IS - 1
ER -