Clinical significance of hemophagocytosis in BM clot sections during the peri-engraftment period following allogeneic hematopoietic SCT.

The effects of macrophage activation on the outcome of allogeneic hematopoietic SCT (allo-HSCT) have yet to be fully examined. A total of 70 adult patients who received a first allo-HSCT for hematological diseases were studied. We counted the number of hemophagocytic cells in BM clot sections on day +14±7, and analyzed its impact on subsequent outcome. In all, 23 patients were diagnosed as having increased numbers of hemophagocytic cells (HP group), whereas 47 were not (non-HP group). The HP group was not associated with an increased incidence of acute or chronic GVHD, but was associated with worse hematopoietic recovery than the non-HP group. The 2-year OS for the HP group and the non-HP group was 30 and 65% (P<0.01), respectively, and 2-year non-relapse mortality was 48% and 27% (P<0.01), respectively. Multivariate analysis confirmed that the HP group was associated with a lower OS (hazard ratio (HR)=2.3; 95% confidence interval (CI), 1.0-5.4; P=0.048) and higher non-relapse mortality (HR=4.0; 95% CI, 1.6-9.9; P<0.01). The HP group had higher incidences of death due to graft failure (P<0.01) and endothelial complications, such as sinusoidal obstruction syndrome and transplant-associated microangiopathy (P=0.01). Macrophage activation is a previously unrecognized complication with negative impact on outcome of allo-HSCT.

[A case of Wegener's granulomatosis with episcleritis, mediastinal lesions, and pleural effusion and without serum anti-neutrophil cytoplasmic antibodies].

We report a 63-year-old male case of Wegener's granulomatosis. His complaints were cough and fever, and chest X-ray showed subpleural infiltrative opacities in the bilateral lungs and small nodular lesions in the middle portion of the right lung. He had been treated as bacterial pneumonia at another hospital without any improvement and was referred to this hospital. Chest CT scan disclosed several small nodular lesions in the subpleural region of the right lung, consolidation in the posterior mediastinum, and the left pleural effusion. Transbronchial lung biopsy revealed inflammatory granulation with predominantly lymphocytes and micro-necrosis in the peribronchiolar region. Although these findings suggested Wegener's granulomatosis, open lung biopsy was performed because both antineutrophil cytoplasmic antibodies (ANCA) and antibodies to proteinase-3 were negative and ear, nose, and throat abnormalities were not found. Pathologic findings consisted mainly of granulomatous inflammation with lymphocytes, histiocytes, and multinucleated giant cells, and granulomatous vasculitis with micro-necrosis. Furthermore, the patient was diagnosed as having bilateral episcleritis by an ophthalmologist. No renal involvement was found. Standard treatment with cyclophosphamide and glucocorticoid resulted in marked improvement within a couple of weeks, and the patient was in complete remission eight months after initiation of therapy. This case is of interest in that the distribution of lesions was not typical and ANCA was negative.

[Diffuse large cell lymphoma with spontaneous regression in the lung and lymph nodes. Case report].

An 84-year-old woman was admitted to our hospital because of swelling of the cervical lymph nodes and multiple tumorous lesions observed on radiographic studies. Transcutaneous lung biopsy was performed, but necrosis of the tissue was too marked to make a diagnosis. The diagnosis of diffuse large-cell lymphoma was made based on a biopsy of the pretracheal lymph node. During the first two months after admission, the left cervical lymph nodes and most of the pulmonary lesions regressed not withstanding of special treatment for lymphoma. The patient eventually died of generalized peritonitis. At autopsy, metastasis of systemic organs by malignant lymphoma was observed. Most of the lung regions were cicatrized, but clusters of atypical lymphocytes were observed in the necrotic tissue. The tumor in the mucosa of the small intestine showed necrosis, which accounted for the intestinal perforation. The total clinical course after admission was about six months. Spontaneous regression of diffuse large cell lymphoma is rare, and this is the second reported case in Japan.

[A case of primary antiphospholipid syndrome with fever, pulmonary thromboembolism and endocardial lesion].

A previously healthy 16-year-old girl complaining of fever, hemosputum, chest pain and dyspnea was hospitalized. On admission, physical examination revealed mental confusion, holosystolic heart murmur, and swelling of the left foot. Laboratory investigations showed anemia, leukocytosis, thrombocytopenia, activation of inflammatory reactions, prolongation of PT and APTT, and hypoxia. Antinuclear antibody test was negative. There were no other findings suggestive of collagen diseases such as SLE. Chest X-ray showed consolidation in the left lower lung field and pleural effusion. Echocardiography disclosed a mass lesion in the left atrium in contact with the mitral valve, and mitral regurgitation. No findings indicative of an infectious etiology were present. The patient rapidly improved with high dose corticosteroid and anticoagulant therapy. A venogram of the lower extremity disclosed deep venous thrombosis. A lung ventilation-perfusion scan revealed multiple pulmonary thromboemboli. Elevation of anticardiolipin antibody was noted. Based on these findings, the diagnosis of primary antiphospholipid syndrome was made. Further administration of steroid and anticoagulant resulted in decrease of the titer of anticardiolipin antibody. This is the second report of primary antiphospholipid syndrome in Japan. The clinical significance of this disease is also discussed.

[Study on postoperative perforation in patients with chronic empyema--reoperated cases].

In 18 cases undergone reoperation because of perforation after the initial operation for primary and secondary chronic empyema during the period from 1974 through August 1986, we evaluated findings of fistula, procedures and causes of failure of the initial operation, and procedures and results of reoperation. Subjects consisted of 16 cases of chronic empyema complicated with pulmonary tuberculosis, 1 case of group III nontuberculous mycobacteriosis, and 1 case of chronic empyema secondary to pulmonary fibrosis. All patients had fistula at the initial operation. The initial operation was performed by Kinchu method in 8 cases, pulmonary detachment in 6 cases, and thoracic cavity reduction chiefly by a modification of Grow's method in 4 cases. The initial operation failed because of incomplete closure of the fistula in 11 cases (61%) and appearance of new fistula in 7 cases (39%). In reoperation, the fistula was closed by pedicle muscle plombage in 1 case undergone the initial operation by Kinchu method, while thoracic cavity reduction chiefly by a modification of Grow's method in all of the other 17 cases. After the operation, 15 patients (83%) were cured, 16 patients (89%) could be socially rehabilitated and 2 patients (11%) had recurrence of empyema. Two recurrent patients died from hepatitis and exacerbation of nontuberculous mycobacteriosis, respectively. Out of 10 patients undergone pedicle muscle plombage, 8 patients (80%) were cured. From these findings, we consider that the results of one stage operation can be improved by applying pedicle muscle plombage for closure of fistula after the initial operation following treatment with effective antibiotics.(ABSTRACT TRUNCATED AT 250 WORDS)

[A study on selection of operative technique for chronic empyema based on findings obtained from reoperated cases].

In 13 patients undergone reoperation for chronic empyema during 9 years period from 1975 to 1983, the operative technique employed for the initial treatment, number of operations, findings of fistula and residual cavity and complications associated with each technique were reviewed with following results. Among these reoperated patients, the initial operation was thoracic cavity reduction in 5 cases, pulmonary detachment in 6 cases and air-plombage (Kinchu method) in 2 cases. Reoperation was carried out in a total of 27 times. There were 6 cases (46%) which were operated more than 3 times. These 6 cases included none of the cases which underwent air plombage. At the time of the first reoperation, fistula was observed in 11 out of 13 cases. At the final operation the residual cavity was accompanied with fistula in 7 cases and empyema in 5 cases. As the final operation, chest wall plombage chiefly by a modification of Grow's method combined with pedicle muscle plombage was employed. In cases indicated for more than two times of operations of thoracic cavity reduction, chest wall plombage was useful for reoperation. On the basis of these results, indication of each operation to improve results of surgical treatment of chronic empyema was discussed by taking into consideration advantages and complications of each operative technique.

[Two cases of Hermansky-Pudlak syndrome with interstitial pneumonia].

Two cases of Hermansky-Pudlak syndrome with interstitial pneumonia were reported. Both patients had evidence of pulmonary involvement characterized by diffuse bilateral infiltrates. They had oculocutaneous albinism. Case 1 was a 55 year-old female who had a history of easy bruising. Her two sisters were albino and had died of pulmonary fibrosis. One of them was diagnosed as Hermansky-Pudlak syndrome on autopsy. Bone marrow aspirate disclosed typical macrophages with ceroid-like pigment. Transbronchial lung biopsy showed alveolar wall thickening. Lumi-aggregometer showed a decrease of platelet aggregation and an absence of ATP release. Case 2 was a 43 year-old female and had a bleeding tendency during a surgical procedure. Lumi-aggregometer showed normal platelet aggregation but an absence of ATP release. BALF analysis did not disclose macrophages with ceroid-like pigment.

[A case of primary pulmonary sporotrichosis].

We report the first case of primary pulmonary sporotrichosis in Japan. A 53-year-old man was admitted to our hospital for further examination of the abnormal shadows on chest X-ray film. Six months before admission, he was admitted to another hospital because of alcoholic liver disease and diabetes mellitus. Since the initial chest film showed cavities with infiltration in the left upper lung field, he was treated with antituberculous drugs despite negative sputum cultures for mycobacterium. In spite of the medication, his chest X-ray film revealed another cavitary lesion, so he was referred to our hospital. He had been asymptomatic during this period. Chest X-ray on admission disclosed multiple cavities in the left upper lobe and a cavity in the right lower lobe. Repeated sputum specimens, bronchial washings and brushings for cytology and cultures were all negative. In an attempt to clarify the pathogen, percutaneous lung aspiration (PLA) was performed. The PLA sample yielded a positive culture of Sporothrix shenckii. After the diagnosis, S. schenckii was also cultured from sputa. A sporothrix skin test and yeast agglutination test for S. schenckii were positive. In the absence of a history for skin lesion, the patient was diagnosed as a primary pulmonary sporotrichosis. As iodide therapy was ineffective, he was started on a regimen of intravenous amphotericin B. However his renal function progressively deteriorated, so amphotericin B was discontinued. Now he receives miconazole intravenously and is still under careful observation. As far as we know, this is the first report of primary pulmonary sporotrichosis in Japan. The possibility of sporotrichosis should be considered in any cases of undiagnosed cavitary lung diseases.