Fatal tracheal aspergillosis during rituximab combined chemotherapy for diffuse large B-cell lymphoma that developed after lung transplantation.

Invasive tracheal aspergillosis (ITA) is an infection that is unique to patients who have undergone lung transplantation (LT). Although the activity of this disease often appears on imaging, we encountered a case of ITA that became exacerbated, despite few computed tomography (CT) findings, during rituximab combined chemotherapy for diffuse large B-cell lymphoma. ITA developed during immunosuppressive therapy after LT. Because CT findings may show false-negative results, bronchoscopy is recommended for such cases.

Real-time coverage monitoring of initial oxidation processes on Si(001) by means of surface differential reflectance.

Initial oxidation processes on Si(001) have been studied by means of surface differential reflectance (SDR). The time courses of the SDR spectra measured during thermal oxidation at 820 and 920 K allowed two different growth modes, Langmuir-type adsorption and two-dimensional island growth, to be distinguished. No photon energy dependence was observed in the time course of the SDR intensity at either temperature. On the other hand, different uptake curves were observed at different photon energies for oxidation at 300 K. The difference between the oxidation mechanisms at 300 K and at high temperatures was qualitatively apparent from SDR results, because significant photon energy dependence was observed only at 300 K. Possible assignments of the spectral components in the SDR spectra are discussed.

IRTA1 and IRTA2, novel immunoglobulin superfamily receptors expressed in B cells and involved in chromosome 1q21 abnormalities in B cell malignancy.

Abnormalities of chromosome 1q21 are common in B cell malignancies, but their target genes are largely unknown. By cloning the breakpoints of a (1;14) (q21;q32) chromosomal translocation in a myeloma cell line, we have identified two novel genes, IRTA1 and IRTA2, encoding cell surface receptors homologous to the Fc and inhibitory receptor families. Both genes are selectively expressed in mature B cells: IRTA1 in marginal zone B cells and IRTA2 in centrocytes, marginal zone B cells, and immunoblasts. As a result of the t(1;14), IRTA1 is fused to the immunoglobulin Calpha domain to produce a chimeric IRTA1/Calpha fusion protein. In tumor cell lines with 1q21 abnormalities, IRTA2 expression is deregulated. Thus, IRTA1 and IRTA2 are novel immunoreceptors implicated in B cell development and lymphomagenesis.

5q- syndrome presenting chronic myeloproliferative disorders-like manifestation: a case report.

A 28-year-old Japanese woman with suspected essential thrombocythemia (ET) had marked thrombocytosis, mild leukocytosis with normal neutrophil alkaline phosphatase activity, and no anemia. She was monitored without being given any medication. Eleven years later, complete blood counts showed no remarkable changes but some non-lobulated mononuclear megakaryocytes were found in the bone marrow. Cytogenetic analysis revealed deletion of the long arm of chromosome 5 (5q-). Subsequently, hemoglobin and platelet counts decreased gradually, splenomegaly appeared and progressed, after which myelofibrosis developed. Acute leukemia developed 16 years after the first documentation of thrombocytosis. 5q- syndrome is known to be a myelodysplastic syndrome (MDS) with unique clinical features and cases with this syndrome presenting with thrombocytosis of more than 1,000 x 10(9)/L but without anemia are rare. Furthermore, it is noteworthy that in this patient transition to acute leukemia occurred following development of myelofibrosis and marked splenomegaly, which are generally observed in blastic crises resulting from chronic myeloproliferative disorders (CMPD). The patient showed features indicative of CMPD rather than of MDS in spite of presenting with 5q- chromosomal abnormality. This case supports the concept of "mixed myelodysplastic and myeloproliferative syndromes" and suggests the possibility of the appearance of CMPD-like manifestations in 5q- syndrome.

[Pulmonary pseudallescheriasis in a patient with diabetes mellitus and alcoholic liver cirrhosis].

A 62-year-old man with diabetes mellitus and alcoholic liver cirrhosis was admitted to the hospital because of hemoptysis. Chest X-ray films and computed tomograms showed a dense infiltrative lesion and a healed tuberculous cavity with a possible fungus ball in the upper lobe of the right lung. Bronchoscopy revealed that the hemoptysis originated from the right upper-lobe bronchus. The bleeding stopped after thrombin was applied into the bronchus. Filamentous fungi were seen in lavage fluid from the right upper-lobe bronchus. The fungi were identified as Pseudallescheria boydii, and pulmonary pseudallescheriasis was diagnosed. the patient was treated successfully with miconazole (400 mg/day) for 2 months. Pseudallescheriasis should be taken into account in the differential diagnosis of aspergilloma-like lesions.

Expression of the TCL1 gene at 14q32 in B-cell malignancies but not in adult T-cell leukemia.

The TCL1 gene was recently cloned as a candidate target within the 14q32.1 breakpoint cluster region observed in T-cell malignancies. We examined the TCL1 gene expression in 21 patients with adult T-cell leukemia (ATL) and 5 cell lines, because ATL is reported to have frequent chromosome 14 band q32 aberrations. However, 20 of the ATL patients and all 5 cell lines lacked any TCL1 expression on northern blot analysis, and TCL1 transcripts were only very faintly detected in the remaining one patient. Expansion of our analysis to include other types of hematopoietic malignancies revealed strong expression of the TCL1 gene in almost all tumor cells of B-cell lineage except myelomas. However, no TCL1 signals were encountered in cells of T-cell or myeloid lineages. In normal human tissues TCL1 was found to be expressed in the spleen, lymph nodes and B-lymphocytes of peripheral blood. These results indicate that TCL1 is not a major target gene for ATL, but that it may play a role in B-cell differentiation and proliferation.

Relation between smoking status of physicians and their enthusiasm to offer smoking cessation advice.

Are nonsmoking physicians more likely to give smoking cessation advice to their patients? To determine this, we sent a questionnaire individually to physicians in Tokyo. The average age of the 323 respondents was 59.8 +/- 12.9 (mean +/- SD); 84.8% of them were male and 21.1% were smokers. Among the respondents, 88.8% asked their patients about their smoking status, 79.9% advised smoking patients to stop, and 93.5% believed smoking cessation interventions to be necessary. Nonsmoking physicians were more likely to advise patients to stop smoking (85.6%) than smoking physicians (70.1%); the smoking physicians who themselves wished to reduce cigarette consumption or stop smoking were more likely to do so (85.0%) than those who did not wish to reduce or stop (43.5%). Moreover, more nonsmoking physicians seriously felt that smoking cessation interventions are necessary (31.2%) than did smoking physicians (6.5%). In conclusion, the smoking status and attitude towards smoking of physicians influences their enthusiasm to give advice to their patients against smoking.

[Comparison of long-term survival between bone marrow transplantation and maintenance chemotherapy for adult acute lymphoblastic leukemia in first remission].

To clarify the efficacy of allogeneic bone marrow transplantation (BMT) for adult ALL in first remission we retrospectively studied long-term outcomes of adult ALL patients of age between 15 and 44 years who were treated in our institute from 1980 to 1990. In this period thirteen patients with HLA compatible donors were offered allogeneic BMT during the first remission, while 16 patients without HLA-compatible donor were treated with maintenance chemotherapy (Cancer Chemoth Pharmacology 33:359-365, 1994). Patient and disease characteristics (age, leukocyte count at presentation, immunophenotype, Ph1 chromosome, and duration to first remission) in the two groups were not significantly different (chi-square test p > 0.1). As causes of treatment failure, relapse was 90% for chemotherapy while relapse and therapy-related death were 67% and 33%, respectively, for transplantation. The leukemia-free survival (LFS) rates at 10 years were 52 +/- 13% for transplantation and 30 +/- 11% for chemotherapy (P > 0.2, g-Wilcoxon, Logrank). The 10-year-LFS rates of Ph1-negative patients of 15 to 29 year-old were 67 +/- 15% for transplantation (n = 9) and 62 +/- 15% for chemotherapy (n = 8) (P > 0.9). Although the present data are derived from a non randomized retrospective study and a relatively small number of patients, this study revealed no superiority of BMT over chemotherapy for the prolongation of first remission in adult ALL, especially, in a standard risk group such as young patients without Ph1 chromosome.

[Flutropium bromide was effective in relieving symptoms of lymphangioleiomyomatosis--a case report].

A 32-year-old woman was admitted with persistent dyspnea. Chest roentgenogram showed hyperinflation of the lungs, and diffuse reticular shadows in both lung fields. Chest CT showed diffuse cystic lesions and thickened vasculature. Examinations revealed severe hypoxemia, restrictive and obstructive ventilatory impairments, increased residual volume, and decreased carbon monoxide diffusing capacity. Lymphagioleiomyomatosis was strongly suspected, and a diagnosis confirmed histopathologically by lung biopsies. Inhalation of flutropium bromide was remarkably effective in relieving dyspnea and impaired pulmonary functions. It is suggested that flutropium bromide is beneficial for symptomatic improvement in patients with lymphangioleiomyomatosis.

[Effect of on-demand inhalation of dry oxygen on nasal mucociliary clearance].

AIM: To determine whether nasal mucociliary clearance is affected by dry oxygen delivered on demand. METHODS: Saccharin nasal transit time was measured as an index of nasal mucociliary clearance. One group of patients was given dry oxygen via a nasal cannula and a demand delivery system, and another group was given dry oxygen by continuous inhalation. In each group, continuous inhalation of humidified oxygen served as the control. Saccharin transit time was measured before and after 8 hours of oxygen inhalation. SUBJECTS: The subjects were 25 patients with chronic respiratory failure who were receiving home oxygen therapy via nasal cannula. All the subjects were clinically stable (age 72.1, mean +/- SE), %VC 46.6 +/- 3.0. FEV/FVC% 9.0 +/- 1.5, PaO2 55.7 +/- 2.0 Torr. PaCO2 53.9 +/- 1.9 Torr). RESULTS: Saccharin transit time after inhalation of dry oxygen on demand did not differ from that after continuous inhalation of humidified oxygen (15.6 +/- 1.5 vs 14.9 +/- 1.4 min). Saccharin transit time after continuous inhalation of dry oxygen was significantly lower than that after continuous inhalation of humidified oxygen (26.6 +/- 5.4 vs 16.7 +/- 2.6 min. p > 0.05). CONCLUSION: We conclude that using the demand oxygen delivery system with a nasal cannula for 8 hours had no adverse effects on nasal mucociliary clearance.

Identification of MLL and chimeric MLL gene products involved in 11q23 translocation and possible mechanisms of leukemogenesis by MLL truncation.

11q23 chromosome aberrations are frequently observed in infantile as well as therapy-related leukemias. The target gene at 11q23, MLL, is disrupted by the translocation and becomes fused to various translocation partner genes such as AF4/FEL, LTG9/AF9 and LTG19/ENL. The resulting chimeric mRNAs are fused in frame and have been predicted to encode leukemia-specific chimeric proteins. In the present study, we raised antibodies against MLL, LTG9 and LTG19 and demonstrated that MLL and chimeric MLL-LTG9 and MLL-LTG19 products are synthesized in vivo and are localized in the nuclei, using immunofluorescence and cell fractionation studies. The truncated N-terminal portion of the MLL product common to the various types of 11q23 translocation was also localized in the nuclei in a similar fashion. Murine 32Dc13 cells stably expressing the truncated N-terminal MLL protein exhibited an inhibition of differentiation and a growth advantage following stimulation by granulocyte-colony stimulating factor, although the IL-3 dependency was not significantly changed in comparison to the parental cells. These results suggest that the N-terminal portion common to various MLL-chimeric products plays an important role in leukemogenesis.

[Allogenic bone marrow transplantation for Fanconi's anemia with leukemic transformation from an HLA identical father].

We report a case of a 19-year-old male with congenital aplastic anemia and multiple abnormalities; short stature, hypoplastic thumb, skin pigmentation and mental retardation. He was admitted to our hospital because of severe pancytopenia. Bone marrow aspiration showed markedly hypocellular marrow with 42% myeloblasts. He was diagnosed as AML (M2) transformed from Fanconi's anemia and underwent allo-BMT from an HLA-identical father. The conditioning regimen consisted of high dose Ara-C, high dose etoposide and 12Gy fractionated total body irradiation. Severe toxicity associated with the conditioning regimen was not observed. Cyclosporin A and short-term methotrexate were administered for prophylaxis of acute GVHD. Neither acute nor chronic GVHD were observed. He is well and free of disease for 15 months since BMT. Very few cases of Fanconi's anemia with leukemic transformation treated by BMT have been reported. Long-term observation will be necessary to evaluate our conditioning regimen for Fanconi's anemia with leukemic transformation.

Benign hemangioendothelioma of the pancreas with obstructive jaundice.

A 3-year-old boy with benign hemangioendothelioma of the pancreas is reported. He showed the presenting sign of obstructive jaundice. The boy was treated with temporary choledochojejunal bypass without additional treatment. In a 16-month follow-up the child has been well and asymptomatic. Laboratory data showed no abnormality. Biopsied specimen of pancreatic head showed hemangiomatous pattern histologically. Immunohistochemical studies of the biopsy revealed positive reaction to factor VIII-related antigen (FVIII-RAG), and endothelial cell marker, supporting an endothelial origin. Hemangioendothelioma has a favorable prognosis and spontaneous regression can be expected. So the first treatment of the tumor should be conservative.

[Postoperative chemotherapy of perforating appendicitis in children; with special reference to latamoxef therapy].

We employed latamoxef (LMOX) as antibiotic therapy subsequent to the performance of appendectomies in children. Fifteen patients, consisting of 8 cases of catarrhal appendicitis and 7 cases of gangrenous appendicitis, were administered LMOX at 50 mg/kg/day, while 13 patients with peritonitis due to a perforated appendix were treated with a dosage of 90 mg/kg/day. Both of these dosages were administered in 3 equally-divided doses, as one shot intravenous injections. All of the cases of catarrhal and gangrenous appendicitis showed good, steady recovery after the surgery, and there were no instances of complications. With regard to the 13 cases of perforating appendicitis, a clinical efficacy of at least "good" was obtained in 12 patients, for an efficacy rate of 92.3%. In addition, bacteriologically, 35 out of 36 strains that were isolated initially could no longer be detected after the LMOX therapy, indicating an eradication rate of 97.2%. Complications consisted of 1 case of dehiscence of the wound, and 1 case of adhesive ileus. Comparison of the values of the laboratory tests before and after the LMOX therapy revealed no development of abnormalities. Moreover, with regard to side effects, with the exception of 1 case of transient diarrhea, there were no side effects which presented clinical problems. It was found that the causative microbes involved in these cases of appendicitis--especially in the perforative appendicitis cases--were Gram-negative bacilli such as E. coli, and an anaerobic bacterium, B. fragilis.(ABSTRACT TRUNCATED AT 250 WORDS)

Arthus-type nephritis. II. Glomerular clearing system against poorly soluble and insoluble immune complexes.

The uptake and transport of immune complexes (IC) in glomeruli were studied in Arthus-type nephritis induced by ferritin anti-ferritin IC. This experimental nephritis was transient in nature, forming electron dense aggregates and deposits in glomeruli, and was considered to be suitable for the analysis of the glomerular defence against immune complex injury. The IC were taken up and removed from the glomerular circulation first by polymorphonuclear leukocytes and later by macrophages. The IC in the glomerular capillary walls and mesangium were seen to shift slowly from the peripheral part of the glomeruli to the more central and juxtaglomerular area. It is speculated that the glomerular integrity is maintained from the immune complex induced tissue injury through the handling and removal of phlogogenic IC by the blood derived phagocytic system and intrinsic glomerular clearing system.

Accelerated serum sickness in the rabbit. IV. Characteristic endarteritis in the pulmonary artery.

A unique angiitis developing in acute serum sickness in the rabbit was studied by immunofluorescence and light microscopy and by transmission and scanning electron microscopy. The angiitis occurred predominantly in the pulmonary artery and was characterized by a prominent accumulation of monocytes in the subendothelial space. During the early stage, scanning electron microscopy revealed the occurrence of various endothelial alterations, especially the formation of many endothelial holes leading to a subendothelial emigration of monocytes. There was neither emigration of polymorphs nor significant thrombotic lesion, and it is possible that this was due to a paucity in the amount of immune complexes deposited. The monocytes ingested platelets and fibrin, suggesting their preventive role in the development of thrombotic changes. The intimal change resolved within several days, leaving only slight fibrosis. A distinct resemblance in the disease process between this type of angiitis and acute serum sickness nephritis is emphasized.