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 five cases of cytomegalovirus infection in immunocompromised patients which were detected by either cytomegalovirus antigenemia assay or in situ hybridization. Four cases had leukemia and the other had chronic renal failure. All the three BMT recipients suffered from GvHD. Interestingly, there was an unique case of CMV disease without a history of BMT, which reminded us that CMV could attack immunocompromised patients who had not undergone transplantation, too. Four out of five cases died. We think that cytomegalovirus infection or disease should not be regarded as a minor problem in post-transplantation infection in Korea.
Adolescent ; Adult ; Antigens, Viral/*blood ; *Bone Marrow Transplantation ; Case Report ; Cytomegalovirus/*immunology ; Cytomegalovirus Infections/complications/*diagnosis ; Fatal Outcome ; Graft vs Host Disease/complications ; Human ; Immunocompromised Host ; In Situ Hybridization ; Kidney Failure, Chronic/complications ; Kidney Transplantation ; Leukemia/*complications/therapy ; Leukemia, Lymphocytic, Acute, L2/complications/therapy ; Leukemia, Myelocytic, Acute/complications/therapy ; Leukemia, Myeloid, Chronic/complications/therapy ; Male ; Viremia/*diagnosis

We report a case of reversible encephalopathy syndrome in a 16-year-old girl with acute myelogenous leukemia (AML), who is undergoing during consolidation chemotherapy composed of BH-AC (N4-behenoyl-1-beta-D-arabinofuranosyl cytosine) and idarubicin. On the 6th day of chemotherapy, she was in a drowsy state following generalized tonic clonic seizure lasting 20 minutes. MR images revealed extensive cortical and subcortical white matter brain edema. Alertness returned over the 24 hr following by the discontinuation of BH-AC and intravenous administration of diphenylhydantoin, although she complained of intermittent headaches and visual disturbance. She gradually recovered from these symptoms during subsequent 7 days. Previously noted abnormal signal intensities have nearly disappreared on follow-up MRI obtained on the 22nd day after the first seizure. She was discharged without any neurologic sequela. This case suggests that BH-AC, a derivative of cytosine arabinoside (1-beta-D-arabinofuranosylcytosine) could be a cause of reversible encephalopathy syndrome.
Adolescence ; Antineoplastic Agents/therapeutic use ; Antineoplastic Agents/adverse effects* ; Brain/radiography ; Case Report ; Cytarabine/therapeutic use ; Cytarabine/analogs & derivatives* ; Cytarabine/adverse effects ; Female ; Human ; Leukemia, Myelocytic, Acute/drug therapy ; Leukemia, Myelocytic, Acute/complications* ; Magnetic Resonance Imaging ; Seizures/radiography* ; Seizures/chemically induced

BACKGROUND: Infection is still a frequent cause of morbidity and mortality in acute myelogenous leukemia (AML) patients receiving chemotherapy. Recently the main cause of infection has changed from gram-negative to gram-positive bacteria and the resistance to antibiotics has increased. This study aimed to access the effectiveness of antimicrobial prophylaxis (AP) with orally absorbable antibiotics. METHODS: Ninety-five AML patients receiving chemotherapy at Catholic Hemopoietic Stem Cell Transplantation Center from March 1999 to July 1999 were randomly divided into the AP group (250 mg ciprofloxacin twice a day, 150 mg roxithromycin twice a day, 50 mg fluconazole once a day) and the control group for a prospective analysis. RESULTS: The incidence of fever was 82.6% in the AP group and 91.6% in the control group (p=0.15). Though classification and sites of infections showed no difference between the two groups, the catheter associated infection occurred more frequently in the AP group in significance. The time interval between initiation of chemotherapy and onset of fever, white blood cell (WBC) count at the onset of fever, duration of leukopenia (WBC < 1,000/mm ), duration of systemic antibiotic therapy, mortality due to infection and hospitalization period from the data starting chemotherapy showed no differences between the two groups. Infections due to gram negative bacteria decreased to 33.3% in the AP group (vs. 92% in the control group), but infections due to gram positive bacteria increased to 66.7% (vs. 8% in the control group). Gram negative bacteria showed 100% resistance to ciprofloxacin in the AP group and gram-positive bacteria showed 90-100% resistance to erythromycin, regardless of the presence of AP. CONCLUSION: The AP could not reduce the occurrence of infection or infection associated death in AML patients receiving chemotherapy. On considering increased gram-positive infection and resistance to fluoroquinolone and macrolide, routine prescription of AP should be reconsidered. Further studies that assess the effectiveness of AP in other malignancies, aplastic anemia and bone marrow transplantation are required.
Adult ; Anti-Infective Agents, Fluoroquinolone/*therapeutic use ; *Antibiotic Prophylaxis ; Bacterial Infections/epidemiology/etiology/*prevention & control ; Ciprofloxacin/*therapeutic use ; Drug Therapy, Combination ; Female ; Fever/epidemiology/etiology ; Fluconazole/therapeutic use ; Human ; Incidence ; Leukemia, Myelocytic, Acute/*complications/drug therapy ; Male ; Middle Age ; Neutropenia/chemically induced/*complications ; Prospective Studies ; Roxithromycin/therapeutic use ; Treatment Outcome