Myeloablative cord blood transplantation for adults with hematological malignancies using tacrolimus and short-term methotrexate for graft-versus-host disease prophylaxis: single-institution analysis.

We studied clinical outcomes of 25 adult patients with hematological malignancies who underwent cord blood transplantation (CBT) after a myeloablative conditioning regimen, including high-dose cytosine arabinoside (CA) (8 g/m(2)), cyclophosphamide (CY) (120 mg/kg), and total-body irradiation (TBI) (12 Gy). For graft-versus-host disease (GVHD) prophylaxis, all patients received a combination of tacrolimus and short-term methotrexate (sMTX). Neutrophil engraftment was achieved in 20 of 25 patients. Of the 22 evaluable patients, 2 and 7 had grades I and II acute GVHD, respectively, and only 1 developed grade III acute GVHD after discontinuation of tacrolimus due to encephalopathy. Chronic GVHD developed in 13 of 19 evaluable patients, including 4 with the extensive type. However, the Karnofsky scores of survivors at 1 year after CBT were 90% or 100%. Eight of 25 patients died of nonrelapse causes (n = 4) and relapse/progressive disease (n = 4); 17 patients are currently alive with 15 free of disease at the present time (median follow-up, 24 months). The probability of disease-free survival at 2 years among patients with standard risk was 89% and that of high-risk patients was 30%. Transplantation-related mortality within 100 days was 12%. These results suggested that the CA/CY/TBI combination is a promising conditioning regimen for myeloablative CBT. Furthermore, tacrolimus and sMTX seemed to have suppressed severe acute GVHD and chronic GVHD, which may also contribute to the favorable results.

Clonal evolution from trisomy into tetrasomy of chromosome 8 associated with the development of acute myeloid leukemia from myelodysplastic syndrome.

Tetrasomy 8, though rare, is usually associated with trisomy 8, a far more common chromosomal abnormality in acute myeloid leukemia (AML). Yet the clonal relationship between trisomy 8 and tetrasomy 8 in the cases with these chromosomal abnormalities has been unclear. Here, we report a case of a 17-year-old male, diagnosed as having a myelodysplastic syndrome (MDS). Chromosome analysis showed the presence of trisomy 8. Five years later, he developed overt AML exhibiting tetrasomy 8 only. After chemotherapy, the blast cells in the bone marrow decreased to 3.4%, and the karyotype showed trisomy 8 alone. Fluorescence in situ hybridization using a probe specific for chromosome 8 showed that the percentages of cells exhibiting 2/ 3 /4 signals were 7.8/89.2/2.0 at the MDS stage, 20.5/36.1/41.0 when overt AML developed and 24.0/72.1/2.4 after chemotherapy. These results suggested that tetrasomy 8 is derived from the AML clone, possibly evolved from the MDS clone with trisomy 8. To our knowledge, this is the first detailed case report of clonal evolution from trisomy 8 into tetrasomy 8 associated with the development of AML from MDS.

A cutaneous agranular CD2- CD4+ CD56+ "lymphoma": report of two cases and review of the literature.

We report 2 cases of agranular CD2- CD4+ CD56+ non-Hodgkin lymphoma in which skin seemed to be the primary site. A 21-year-old woman's initial symptom was a skin nodule on the right cheek. She also had tumors in the nasopharynx, and the bone marrow subsequently became involved. No lymphadenopathy was present. She experienced complete remission after dose-intensified therapy with cyclophosphamide, hydroxydaunomycin, vincristine [Oncovin], and prednisone (CHOP), but the disease relapsed in the central nervous system 6 months later. An 81-year-old man experienced an 11-month history of skin nodules in the left forearm. On admission, he had a bone marrow infiltration of lymphoma cells. He died of pneumonia during chemotherapy. The malignant cells of the 2 patients had similar morphologic features, with a monocytoid nucleus and no cytoplasmic granules. The cells in both cases showed a unique phenotype: CD2-, CD3-, CD4+, CD8-, CD13-, CD14-, CD34-, CD16-, CD56+, CD57-, HLA-DR-positive. Staining for peroxidase and alpha-naphthyl butyrate esterase was negative. The T-cell receptor beta, gamma, delta, IgH, kappa, lambda genes were of germ line configurations. The DNA of Epstein-Barr virus was not detected from the bone marrow cells by polymerase chain reaction. Only 3 other cases with similar phenotypes have been reported; all had skin lesions. Although the origin of these cells remains unknown, we propose that this is a distinct clinicopathologic entity.

Inhibitory effect of bacterial attachment on candidal growth due to adherence with mannose-sensitive pili.

A bacterial strain with affinity to Candida albicans was successfully obtained from a natural environment. An uncovered Petri dish containing a suspension of heat-killed C. albicans cells was allowed to stand in a laboratory for several days. Some bacteria which had adhered to the candidal cells were tested for their ability to agglutinate the cells. A bacterial strain, designated later as CAB-1, was found to agglutinate candidal cells through bridging by mannose-sensitive pili. CAB-1 showed similar bacteriological characteristics to those of Citrobacter freundii by ID test. The adherence of CAB-1 to candidal cell was precisely presented by scanning electron microscopy. The inhibitory effect of CAB-1 attachment to candidal cells on the growth of Candida was also preliminarily confirmed.

[An improved phagocytic plaque method using gram-negative bacilli].

The phagocytic plaque method developed for evaluation of the phagocytic function of leukocytes is not only very useful but convenient for both basic and clinical investigations. So far, only Staphylococcus aureus has been demonstrated to give clear phagocytic plaques. In this study, we successfully obtained clear phagocytic plaques with gram-negative bacilli (Pseudomonas aeruginosa and Escherichia coli). The plaques given by E. coli were morphologically nearly the same as those by S. aureus, whereas those of P. aeruginosa were elongated.

[A case of giant hepatocellular carcinoma successfully treated by arterial administration of SMANCS].

A 68-year-old male was referred to our hospital for the precise examination of a giant hepatic tumor detected in a mass survey. The lesion occupied most of the right hepatic lobe, further advancing to the medial segment of the left lobe. However, hepatic functions were well preserved (ICG K = 0.141). Considering the characteristic images of the lesion with positive anti-HCV and high titer of PIVKA II (0.860 AU/ml), the diagnosis was hepatocellular carcinoma. First arterial administration of SMANCS was performed on May in 1994, followed by 6 successive procedures with an interval of about 2 months (total dosage 36 mg), resulting in remarkable tumor shrinkage and tumor marker normalization. On January in 1995, a metastatic lesion to the right rib was controlled by 2 mg of SMANCS administered to the intercostal artery combined with radiation therapy (60 Gray). Frequent administrations of SMANCS caused no serious complications, and tumor feeders were well preserved. Therefore, arterial administration of SMANCS is thought to be one choice for the therapy of giant HCC with good functional reserve.

[Eighteen cases of tortuosity of the internal carotid--usefulness of MR-angiography in diagnosis].

There have been few reports on tortuosity of the internal carotid exhibiting pulsation or swelling in the pharyngeal wall. Because this disease carries the risk of causing massive hemorrhage at the time of incision of a peritonsillar abscess, surgical treatment of the adenoids, or tonsillectomy, otolaryngologists should be aware of the existence of this disorder as a risk factor. We encountered tortuosity of the internal carotid in the pharynx in 18 cases over an approximately two year period. Females (15 cases) outnumbered males (3 cases), and the mean age was 68 years at the time of diagnosis. There was no right-to-left difference in terms of the incidence of the disease. The complication of hypertension was noted in 10 cases. MR-Angiography (MRA) was performed in 13 cases, and very clear images were obtained; this technique was useful for diagnosing this disorder. For the most part, contrast angiography has been employed for the diagnosis of this disorder, but various complications, including deaths, have been reported. As the incidence of tortuosity of the internal carotid is high in the elderly, it is thought that any complication which may occur has the potential to become serious. Non-invasive diagnostic techniques are thus recommended. MRA is absolutely non-invasive, and we therefore believe that this technique should be the modality of first choice for diagnosing tortuosity of the internal carotid.

Diagnosis of carcinoma of the papilla of Vater by duodenofiberscopy: simultaneous attempt on endoscopic observation, aspiration cytology, retrograde pancreatocholangiography and biopsy.

Clinical features and validity of a duodenofiberscopic examination for the diagnosis of carcinoma of the papilla of Vater were evaluated on 13 cases preoperatively diagnosed endoscopically. Followings are the conclusions derived. 1) Average age was 59.8 years. Incidence was equal among men and women. 2) As initial symptoms, general fatigue and easy fatigability or symptoms of cholangitis should be emphasized. 3) Other important symptoms include jaundice, fever with chills and weight loss. Abdominal pain, when present, was noted as one of symptoms of cholangitis in most cases. 4) Important laboratory findings include elevated serum bilirubin and alkaline phosphatase, elevated erythrocyte sedimentation rate, positive occult blood in stool. 5) Carcinomas of the papilla of Vater were divided into 3 types (type I, II and III) according to endoscopical and pathological findings. Clinical features and laboratory findings were discussed in relation to the type of lesions. Characteristic endoscopic findings of each type of the lesions were described. Validity of aspiration cytology, pancreatocholangiography and biopsy under duodeno-fiberscopic observation was also discussed. As a result, duodenofiberscopy was considered to be the most useful method for the diagnosis of carcinoma of the papilla of Vater because it provides us with an opportunity to perform simultaneously an endoscopic observation, aspiration cytology, pancreatocholangiography and biopsy.