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

The prognostic significance of multidrug resistance (MDR) gene expression is controversial. We investigated whether multidrug resistance gene 1 (MDR1), multidrug resistance-related protein (MRP) and lung resistance protein (LRP) mRNA expression are associated with outcomes in acute leukemia patients. At diagnosis we examined MDR1, MRP and LRP mRNA expression in bone marrow samples from 71 acute leukemia patients (39 myeloid, 32 lymphoblastic) using nested RT-PCR. The expression of each of these genes was then expressed as a ratio in relation to beta-actin gene expression, and the three genes were categorized as being either 0, 1+, 2+ or 3+. MDR1, MRP and LRP mRNA expression was detected in 23.9%, 83.1% and 45.1 %, respectively. LRP mRNA expression was significantly associated with resistance to induction chemotherapy in acute leukemia patients, and in the AML proportion (p=0.02 and p=0.03, respectively). MRP and high MDR1 mRNA expression was associated with poorer 2-yr survival (p=0.049 and p=0.04, respectively). Patients expressing both MRP and LRP mRNA had poorer outcomes and had worse 2-yr survival. The present data suggest that MDR expression affects complete remission and survival rates in acute leukemia patients. Thus, determination of MDR gene expression at diagnosis appears likely to provide useful prognostic information for acute leukemia patients.
Vault Ribonucleoprotein Particles/*genetics ; Survival Rate ; RNA, Neoplasm/genetics ; RNA, Messenger/genetics ; Prognosis ; Neoplasm Proteins/*genetics ; Multidrug Resistance-Associated Proteins/*genetics ; Middle Aged ; Male ; Leukemia, Myelocytic, Acute/drug therapy/genetics/mortality ; Leukemia, Lymphocytic, Acute/drug therapy/genetics/mortality ; Leukemia/drug therapy/*genetics/mortality ; Infant ; Humans ; *Genes, MDR ; Gene Expression ; Female ; Child, Preschool ; Child ; Base Sequence ; Aged ; Adult ; Adolescent

Intense myelofibrosis is rarely associated with de novo acute myeloid leukaemia (AML) except in acute megakaryoblastic leukaemia (AML-M7) where there is diffuse marrow fibrosis as a consequence of proliferation of neoplastic myeloid cells. AML associated with significant myelofibrosis developing both de novo or secondary to primary (idiopathic) myelofibrosis is characterised by a fulminant course and extremely poor prognosis, primarily due to treatment-resistant disease. The prognostic value of degree of marrow fibrosis in de novo AML has been poorly investigated. We describe a case of extensive myelofibrosis associated with acute erythroblastic leukaemia (AML-M6) that responded to induction therapy of the leukaemia.
Myelofibrosis ; Acute ; Leukemia, Myelocytic, Acute ; therapeutic aspects ; prognostic

We retrospectively reviewed the medical records of 284 patients with neutropenic fever following chemotherapy for acute leukemia at the Catholic Hematopoietic Stem Cell Transplantation Center from January 1998 to December 1999, to identify prognostic factors for infection related mortality. Twenty-eight patients died of infections. There was no difference in median age, gender ratio, or underlying disease between the dying and surviving groups. Bacteria were the main pathogens following chemotherapy, and Gram positive organisms predominated in the dying group. Pneumonia and sepsis were the main causes of death. There were 72 cases of invasive fungal infection and their mortality was 27.8%. Invasive fungal infection and previous history of fungal infection were independent prognostic factors for outcome. Recovery from neutropenia was the significant protective factor for mortality. In conclusion, the prognostic factors identified in this study could be useful for deciding on more intensive treatment for those patients at greater risk of death. To our knowledge, this is the first Korean study delineating prognostic factors in acute leukemic patients with infectious complications.
Adolescent ; Adult ; Aged ; Bacterial Infections/complications/*mortality ; Cause of Death ; Female ; Humans ; Korea ; Leukemia ; Leukemia, Lymphocytic, Acute/complications/*microbiology/*mortality ; Leukemia, Myelocytic, Acute/complications/*microbiology/*mortality ; Male ; Middle Aged ; Morbidity ; Multivariate Analysis ; Mycoses/complications/mortality ; Neutropenia ; Pneumonia/complications/mortality ; Prognosis ; Retrospective Studies ; Sepsis/complications/mortality ; Survival Rate

Muscle involvement in acute febrile neutrophilic dermatosis is uncommon. Herein, we report a case of acute febrile neutrophilic myositis, without cutaneous involvement, as the first manifestation of acute myeloid leukemia. The patient was a 35-year-old male, referred due to painful swelling of the left upper arm and fever. The overlying skin looked normal, and a muscle biopsy revealed dense infiltrates, predominantly composed of mature neutrophils, edema and tissue necrosis. All culture reports were negative, and he was finally diagnosed as having acute febrile neutrophilic myositis, associated with acute myeloid leukemia. Corticosteroid treatment resulted in the progressive regression of the fever, myalgia and swelling.
Neutrophils/*metabolism ; Myositis/*etiology/metabolism ; Male ; Leukemia, Myelocytic, Acute/*diagnosis ; Humans ; Fever/etiology ; Adult

BACKGROUND: Haploidentical transplantation has become a popular modality of treatment for acute myeloid leukemia (AML) patients lacking donors with matching HLA. We attempted to assess the success rate and ramifications of full haplotype mismatch transplantation. METHODS: Four patients received stem cell transplantation from their full haplotype mismatched family donors. The conditioning regimen included total-body irradiation, intravenous busulfan, antithymocyte globulin, and fludarabine. Megadose CD34+ stem cell transplants were performed, in a dosage range between 10.9 X 10 (6) /kg and 20.6 X 10 (6) /kg. Neither GvHD prophylaxis nor post-transplant G-CSF were administered. We monitored patients' bone marrow cellularity and peripheral blood chimerism using real-time PCR. RESULTS: All patients evidenced stable engraftment. The most frequent side effect was severe mucositis, but all patients recovered successfully, without early death. No patients exhibited acute GvHD. Two refractory patients relapsed soon after transplantation. The other 2 patients have remained in good clinical condition, with a follow-up duration of 1~4 months. CONCLUSION: Using a newly-developed conditioning regimen, we were able to circumvent GvHD and graft failure, which are the main limitations associated with full haplotype mismatch transplantation. According to our analysis of the relevant literature, it appears that this is the first report of such a conditioning regimen.
Adolescent ; Adult ; Antigens, CD34/*analysis ; Female ; Graft vs Host Disease/prevention & control ; Histocompatibility ; Humans ; Leukemia, Myelocytic, Acute/*therapy ; Male ; Pilot Projects ; Stem Cell Transplantation/*methods ; Transplantation Conditioning/methods

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

Telomerase activity and the expression of telomerase subunits (for example, telomerase reverse transcriptase and telomerase associated protein 1 and telomerase RNA component) of peripheral white blood cells were detected in the patients with acute myelogenous leukaemia (AML) and the correlation between telomerase activity and the expression of telomerase subunits was observed. In 94 peripheral white blood cells from 18 healthy volunteers and 76 patients with AML, including 31 AML at initial presentation, 24 at relapse and 21 at complete remission, the telomerase activity and telomerase subunits mRNA or RNA were detected by PCR-ELISA and RT-PCR respectively. The results showed that the positive rate of telomerase from patients with AML at initial presentation, at relapse and at complete remission was 74.1%, 79.2% and 4.8% respectively. The positive rate of telomerase reverse transcriptase mRNA from healthy volunteers, AML at initial presentation, AML at relapse and AML at complete remission was 5.6%, 80.6%, 83.3% and 9.5% respectively. The positive rate of telomerase associated protein 1 mRNA and telomerase RNA component in all samples were 100%. It was suggested that the up-regulation of telomerase activity and the expression of telomerase reverse transcriptase is correlated closely with the occurrence and relapse of AML, so telomerase activity and the expression of telomerase reverse transcriptase may be used to estimate the curative effect and predict relapse of AML. Moreover, the up-regulation of telomerase activity is correlated with the expression of telomerase reverse transcriptase significantly.
Carrier Proteins/*metabolism ; DNA-Binding Proteins ; Leukemia, Myelocytic, Acute/*enzymology ; RNA/metabolism ; RNA, Messenger/metabolism ; Reverse Transcriptase Polymerase Chain Reaction ; Telomerase/*metabolism

Reported here is a case of microsporidiasis that occurred in an acute myeloblastic leukemia (AML) -M3 patient who underwent chemotherapy. Fever, cough, expectorate and dyspnea were observed during the therapy. Since this case was considered as adult respiratory distress syndrome due to the chest X-ray and arterial blood gas findings, the male patient was bounded to a mechanical ventilator. As coagulation tests showed compatible findings with disseminate intravascular coagulation (DIC), it was thought to be a case of sepsis originating from the lungs and DIC. Pseudomonas aeruginosa and Staphylococcus aureus were found in the sputum of the patient. Although he was given combined antibiotic therapy, there was no reduction in the fever. A bronchoalveolar lavage (BAL) sample was taken and Microsporidia sp. was found upon staining with Giemsa. The patient died due to sepsis and DIC just before receiving therapy for microsporidiasis. Pulmonary infection with Microsporidia, although classically occurring in patients with HIV infection, may occur rarely in leukemia patients, especially if previously treated with systemic immune suppression. This case reinforces the need to consider Microsporidia as a possible pathogen in immunocompromised patients with pulmonary infections.
Diagnostic Errors ; Fatal Outcome ; Human ; Immunocompromised Host ; Leukemia, Myelocytic, Acute/*complications ; Lung Diseases, Parasitic/*complications/diagnosis ; Male ; Microsporidiosis/*complications/diagnosis ; Middle Aged

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