A case of granulocytic sarcoma involving the uterine cerivx as the primary manifestation, before the peripheral blood and bone marrow showed evidences of overt leukemia, is presented. Six weeks after the onset of the genital tract symptom the patient developed acute myelogenous leukemia. The uterine tumor was initially believed to be a histiocytic lymphoma. The diagnosis of granulocytic sarcoma was confirmed by the naphthol AS-D chloracetate stain for esterase, which was performed on the uterine cervix and obturator lymphnodes taken by hysterectomy and pelvic node dissection. The literatrue was reviewed with emphasis on the differential diagnosis of granulocytic sarcoma and histiocytic lymphoma, and the clinical and pathological problems that arise when the tumor presents at an unusual location and without peripheral blood manifestation of leukemia.
Adult ; Cervix Neoplasms/diagnosis* ; Diagnosis, Differential ; Female ; Human ; Leukemia, Myelocytic, Acute/diagnosis* ; Leukemia, Myelocytic, Acute/pathology ; Leukemia, Myeloid/diagnosis* ; Lymphoma/diagnosis

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

We report an unusual case of acute myelogenous leukemia in a patient who showed an extramedullary relapse in her uterus, without bone marrow recurrence, two years after an allogeneic bone marrow transplant. She complained of irregular vaginal spotting, and magnetic resonance imaging demonstrated a uterine mass. A biopsy revealed a massive infiltration of immature myeloid cells. A variable number of tandem repeats (VNTR) based on an examination of peripheral blood cells showed full donor chimerism. After receiving chemotherapy, her uterine mass had completely resolved. She has remained in complete remission for more than 6 months. This case suggests that physicians should be aware of the possibility of a uterine relapse in female bone marrow transplant recipients with acute myelogenous leukemia.
Adult ; Female ; Hematopoietic Stem Cell Transplantation/*adverse effects ; Human ; Leukemia, Myelocytic, Acute/*pathology/*therapy ; Neoplasm Recurrence, Local ; Sarcoma, Granulocytic/etiology/*pathology ; Uterine Neoplasms/etiology/*pathology

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

We report a case of granulocytic sarcoma presented as a recurrent breast tumor in a 42-year-old woman with no history of leukemia. The case was initially diagnosed as malignant lymphoma on a previous biopsy specimen and she refused chemotherapy. At the time of recurrence of the breast tumor, the patient showed full-blown features of leukemia. This case of rare tumor suggests that differential diagnosis should be considered when malignant lymphoma of the breast is detected.
Adult ; Breast Neoplasms/surgery ; Breast Neoplasms/pathology ; Breast Neoplasms/diagnosis+ACo- ; Carcinoma, Lobular/diagnosis ; Case Report ; Cell Nucleus/ultrastructure ; Diagnosis, Differential ; Diagnostic Errors+ACo- ; Female ; Human ; Leukemia, Myelocytic, Acute/pathology ; Leukemia, Myelocytic, Acute/diagnosis+ACo- ; Lymphoma, Large-Cell, Diffuse/diagnosis+ACo- ; Neoplasm Recurrence, Local/pathology

We report a case of granulocytic sarcoma presented as a recurrent breast tumor in a 42-year-old woman with no history of leukemia. The case was initially diagnosed as malignant lymphoma on a previous biopsy specimen and she refused chemotherapy. At the time of recurrence of the breast tumor, the patient showed full-blown features of leukemia. This case of rare tumor suggests that differential diagnosis should be considered when malignant lymphoma of the breast is detected.
Adult ; Breast Neoplasms/surgery ; Breast Neoplasms/pathology ; Breast Neoplasms/diagnosis+ACo- ; Carcinoma, Lobular/diagnosis ; Case Report ; Cell Nucleus/ultrastructure ; Diagnosis, Differential ; Diagnostic Errors+ACo- ; Female ; Human ; Leukemia, Myelocytic, Acute/pathology ; Leukemia, Myelocytic, Acute/diagnosis+ACo- ; Lymphoma, Large-Cell, Diffuse/diagnosis+ACo- ; Neoplasm Recurrence, Local/pathology

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

We describe a case with acute myelogenous leukemia (AML; M2) who developed prolonged marrow hypoplasia with residual leukemic blasts and recurrent infections after induction chemotherapy. He was treated successfully with a sequential treatment of recombinant human granulocyte-macrophage colony-stimulating factor (rhGM-CSF) and low-dose cytosine arabinoside (LD AraC). To the best of our knowledge this is the first reported case of a successful treatment of a patient with AML, who showed prolonged markedly hypocellular bone marrow with significant residual leukemic cells after induction chemotherapy, with a sequential treatment of GM-CSF and LD AraC.
Bone Marrow Diseases/chemically induced/*drug therapy ; Case Report ; Cytarabine/*administration & dosage ; Granulocyte-Macrophage Colony-Stimulating Factor/*therapeutic use ; Human ; Leukemia, Myelocytic, Acute/*drug therapy/pathology ; Male ; Middle Age

Stem cell factor (SCF), a c-kit ligand, has a preferential effect on the proliferation of several classes of immature hematopoietic progenitor cells in combination with GM-CSF or IL-3. To analyze the costimulatory role of SCF in leukemic growth, we investigated the effect of SCF in the presence of GM-CSF and/or IL-3 on isolated CD34-positive (CD34+) leukemic blasts from 15 patients with acute myelogenous leukemia (AML). Cultures of CD34+ cells from normal bone marrow were used as controls. When the proliferation of CD34+ AML blasts in the presence of GM-CSF and/or IL-3 were evaluated in vitro for the effects of SCF, two patterns emerged. In one pattern, CD34+ AML blasts responded with a significant increase in DNA synthesis and/or colony formation when SCF was used with GM-CSF and/or IL-3 relative to the growth with SCF alone; This result is consistent with those CD34+ bone marrow cells from normal donors. Six patients (40%) were included in this category. The addition of SCF as a single factor resulted in colony formation in all six of these cases. In the other pattern, nine of the patients (60%) had CD34+ leukemic cells whose growth with SCF plus either GM-CSF, IL-3, or GM-CSF+IL-3, was not significantly different from the growth noted in the presence of SCF alone. Among them seven cases that did not form colonies in response to SCF alone, and one case showing autocrine, background growth were included. In the six cases in which the costimulating effects of SCF were documented, CD34+ c-kit+ blasts comprised 50.5 +/- 18.7% of the CD34+ leukemic blasts-higher than 21.8 +/- 19.4% of cases in which the costimulating effect of SCF was not documented. In the cases showing high c-kit antigen expression(> or = 40%), SCF had a costimulatory effect in 71% (5/7) of the patients. In conclusion, our data indicate that CD34+ leukemic blasts from a good proportion of patients with AML did not respond to the costimulating effects of SCF in the presence of GM-CSF adn/or IL-3, in contrast to those CD34+ bone marrow cells from normal donors. The possible use of SCF for acute leukemia must await further cytogenetic and molecular studies, which should clarify the preferential costimulating role of SCF in normal hematopoiesis.
Antigens, CD/*analysis ; Antigens, CD34 ; Granulocyte-Macrophage Colony-Stimulating Factor/*pharmacology ; Hematopoietic Cell Growth Factors/*pharmacology ; Human ; Interleukin-3/*pharmacology ; Leukemia, Myelocytic, Acute/*immunology/pathology ; Stem Cell Factor ; Support, Non-U.S. Gov't ; Tumor Markers, Biological