Myelodysplastic syndrome is a closely related group of acquired bone marrow disorders characterized by ineffective and dysplastic hematopoiesis. These clonal disorders frequently progress to acute leukemia. Acute myelomonocytic leukemia with eosinophilia is characterized by an increase in abnormal eosinophils in the bone marrow, relatively good clinical course and inv (16) chromosomal abnormality. We experienced one case of refractory anemia with excess blasts which progressed to refractory anemia with excess blasts in transformation and finally to acute myelomonocytic leukemia with eosinophilia showing peculiar chromosomal abnormalities of der (1;7).
Adult ; Anemia/pathology ; Anemia/genetics ; Anemia/etiology ; Bone Marrow/pathology ; Case Report ; Chromosomes, Human, Pair 16* ; Disease Progression ; Eosinophilia/pathology ; Eosinophilia/genetics* ; Eosinophilia/etiology ; Human ; Inversion (Genetics)* ; Karyotyping ; Leukemia, Myelocytic, Acute/pathology ; Leukemia, Myelocytic, Acute/genetics* ; Leukemia, Myelocytic, Acute/etiology ; Male ; Myelodysplastic Syndromes/pathology ; Myelodysplastic Syndromes/genetics* ; Myelodysplastic Syndromes/complications

Lymphadenopathy is a relatively uncommon finding of minimally differentiated acute myelogenous leukemia (AML-MO). We experienced a case of AML-MO in a 57-year-old man initially presented with multiple cervical lymphadenopathy. Bone marrow aspiration revealed myeloblasts, which were negative for myeloperoxidase, Sudan black B, Periodic acid-Schiff, non-specific esterase and double esterase reaction. In cell surface marker studies, CD13, CD14, CD33, CD34, CD45 and HLA-DR were present. CT scan of neck demonstrated multiple lymphadenopathy at both internal jugular chains, spinal accessory chains and submandibular area. He died about two weeks after diagnosis without specific treatment.
Case Report ; Fatal Outcome ; Human ; Leukemia, Myelocytic, Acute/*complications/pathology ; Lymphatic Diseases/*complications/pathology ; Male ; Middle Age ; Neck ; Tomography Scanners, X-Ray Computed

Shwachman-Diamond syndrome (SDS) is a rare genetic disorder of unknown pathogenesis involving exocrine pancreatic insufficiency and hematological and skeletal abnormalities. About 25% of patients develop hematopoietic malignancies. We report on a case of acute myeloid leukemia (M2) in a 21-year-old woman affected by SDS. She was treated with conventional chemotherapy (idarubicin plus cytarabine) and reached complete remission of leukemia. After induction chemotherapy, she underwent allogeneic bone marrow transplantation (BMT). The BMT preparative regimen consisted of total body irradation (TBI) followed by cyclophosphamide. Cyclosporin A and short term methotrexate were used for graft-versus-host disease prophylaxis. After a follow-up of 12 months, she is alive leukemia free off any immunosuppressive agent. Although experience in this field is scarce, we speculate that bone marrow failure in SDS is an indication for BMT which is the only curative trentment option.
Adult ; *Bone Marrow Transplantation ; Case Report ; *Cell Transformation, Neoplastic ; Female ; Human ; Leukemia, Myelocytic, Acute/*pathology/*therapy ; Myelodysplastic Syndromes/*complications/*therapy ; Pancreatic Insufficiency/complications/therapy ; Syndrome ; Transplantation, Homologous

Granulocytic sarcoma is a rare extramedullary tumor composed of myeloid progenitor cells. Primary involvement of the biliary tract without evidence of leukemia is exceedingly rare. Here, we report an isolated biliary granulocytic sarcoma in a 30-yr-old man who presented with jaundice, fever, and chill without any evidence of leukemia. However, five months after the diagnosis, he developed acute myelogenous leukemia with multilineage dysplasia and chromosomal abnormality. A rare possibility of biliary granulocytic sarcoma should be considered as a differential diagnosis in patients with obstructive jaundice. A histologic evaluation by aggressive diagnostic intervention is important and may improve prognosis.
Tomography, X-Ray Computed ; Sarcoma, Granulocytic/*complications/*pathology ; Prognosis ; Male ; Leukemia, Myelocytic, Acute/*diagnosis/*pathology ; Karyotyping ; Humans ; Cell Lineage ; Bile Ducts/metabolism/pathology ; Bile Duct Neoplasms/*complications/*pathology ; Adult