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Recently, the chromosome 14q11 anomaly has been reported to be specific to adult T-cell leukemia (ATL), and this anomaly has also been confirmed in the preleukemic state of adult T-cell leukemia (pre-ATL) patients. Because the cytogenetic abnormality at the stage of human T-cell leukemia virus type I (HTLV-I) carrier remains uncertain, we performed cytogenetic studies of lymphocytes stimulated with phytohemagglutinin in three HTLV-I carriers and three non-HTLV-I carriers in an ATL family. As a result, in three HTLV-I carriers, four of 311 cells examined (1.3%) had chromosome 14q11 anomaly. However, in three non-HTLV-I carriers, none of 260 cells examined had chromosome 14q11 anomaly. These results suggest that chromosome 14q11 anomaly is already present at the stage of HTLV-I carrier and seems to be an important cytogenetic clue to the pathogenesis of ATL.

A 35-year-old female presented in 1989 with hepatosplenomegaly, but no conclusive diagnosis was established. From 1992, she experienced transient episodes of facial flushing and palpitations. Osteosclerotic change was detected radiologically. Colonoscopy revealed massive polypoid lesions. Mast cells were demonstrated in bone marrow smear and imprinted preparations of colon biopsy specimens by toluidine blue staining. Plasma concentrations of histamine and soluble c-kit were elevated. She was successfully treated with interferon-alpha and prednisolone, resulting in the disappearance of histamine-related attacks and a gradual decrease in tumor size. However, the remission was interferon dose dependent. This case was considered as systemic mastocytosis with massive polypoid colon lesions and showed the importance of maintenance therapy with interferon-alpha.

Plasma concentration of M-CSF was measured in 35 patients with adult T cell leukemia (ATL), using a radioimmunoassay (RIA). ATL patients showed elevated levels of plasma M-CSF concentration when compared with healthy adult volunteers. Higher M-CSF levels were observed in acute ATL patients than in patients with chronic or smouldering ATL (P < 0.0001). There was a significant positive correlation of M-CSF concentration with serum lactic dehydrogenase (LDL) level, a reliable marker for assessing the grade of malignancy in ATL (P = 0.0003). There was, however, no correlation of M-CSF concentration with total counts of peripheral blood ATL cells, neutrophils or monocytes, or with serum calcium levels. Although there was a significant positive correlation of M-CSF concentration with body temperature (P = 0.003), there was not a significant correlation of M-CSF concentration with C-reactive protein (CRP), a protein indicative of the severity of inflammation (P = 0.063). These results indicate that plasma M-CSF concentration reflects the disease activity of ATL, and can thus serve as a marker in the clinical subclassification of ATL patients.

The authors examined peripheral blood samples from patients with adult T-cell leukemia (ATL) using the monoclonal antibody Ki-67 which detects a nuclear antigen present in actively proliferating cells. In patients with chronic ATL, the percentage of Ki-67-positive cells was significantly lower than in acute ATL patients (median values, 3.3% versus 18.9%, P less than 0.001). Furthermore, there was a significant inverse correlation between the percentage of Ki-67-positive cells and the length of survival (P less than 0.001). Serum lactic dehydrogenase (LDH) levels also showed a significant inverse correlation with survival, but this was less strong than that for Ki-67 (0.01 less than P less than 0.02). Thus, Ki-67 positivity appears to indicate the aggressiveness of ATL, and can possibly be used for the clinical classification of ATL patients as well as for the prediction of prognosis.

Recently, the chromosome 14q11 anomaly has been reported to be specific to adult T-cell leukemia (ATL), and this anomaly has also been confirmed in the preleukemic state of adult T-cell leukemia (pre-ATL) patients. Because the cytogenetic abnormality at the stage of human T-cell leukemia virus type I (HTLV-I) carrier remains uncertain, we performed cytogenetic studies of lymphocytes stimulated with phytohemagglutinin in three HTLV-I carriers and three non-HTLV-I carriers in an ATL family. As a result, in three HTLV-I carriers, four of 311 cells examined (1.3%) had chromosome 14q11 anomaly. However, in three non-HTLV-I carriers, none of 260 cells examined had chromosome 14q11 anomaly. These results suggest that chromosome 14q11 anomaly is already present at the stage of HTLV-I carrier and seems to be an important cytogenetic clue to the pathogenesis of ATL.

Various morphological dysplastic changes were observed in patients with chronic myelogenous leukemia, especially in the acute crisis. To clarify their significance, we divided 45 patients in the acute crisis into two groups by our scoring system, the dysplastic group and the nondysplastic group. Five of 25 subjects in the non-dysplastic group entered complete remission. None of 20 subjects in the dysplastic group did so, and the mean survival after the onset of acute crisis is significantly shorter in the dysplastic group than in the non-dysplastic group. Some patients in the dysplastic group had obvious dysplastic changes several months before the acute crisis. These findings suggest that acute crises in some cases may occur with or be preceded by the development of dysplastic clones similar to myelodysplastic syndrome; these patients respond poorly to conventional chemotherapy.

A case of primary pancreatic carcinoma confirmed by postmortem examination in a 15-year-old girl is presented. The tumor was studied by light and electron microscopy and an indirect immunoperoxidase technique. Some of the tumor cells contained eosinophilic, PAS-positive, diastase-resistant granules. Electron microscopy revealed large electron-dense granules resembling zymogen granules. The granules seen in the islet cell tumors were not demonstrated by electron microscopy and immunoperoxidase technique. These findings suggest that the tumor is of duct cell origin with some differentiation toward the acinar cells. The tumor appears to belong to the so-called "pancreatoblastoma".

Chromosomes and surface markers in lymph node cells from 2 patients with angioimmunoblastic lymphadenopathy associated with dysproteinemia (AILD) or immunoblastic lymphadenopathy (IBL) and with IBL-like T-cell lymphoma, respectively, were examined before treatment. In the patient with AILD, a small clone with chromosome abnormality was found in lymph node cells although a surface marker study failed to demonstrate monoclonality. In this patient, the clinical and cytogenetic findings suggested a subtype of lymphoma with a mild clinical course and a benign histological appearance. In the patient with IBL-like T-cell lymphoma, a high percentage of metaphases showed chromosome abnormalities such as ring chromosomes. The clinical, immunological and cytogenetic findings suggested an aggressive type of lymphoma, even though the histologic appearance was similar to that of IBL.

The chromosome 14q11 anomaly has been reported to be specific to adult T-cell leukemia (ATL) and this anomaly has also been confirmed in preleukemic state of ATL (pre-ATL) patients though the frequency is low. In an attempt to clarify if the same chromosome aberrations could be found also at the stage of HTLV-I carrier and if there is any cytogenetic difference from non-HTLV-I carriers, a cytogenetic study of lymphocytes stimulated with phytohemagglutinin in three HTLV-I healthy carriers and three non-HTLV-I carriers in an ATL family was performed. The results were as follows. 1. In three HTLV-I carriers, 7 of 311 cells examined (2.3%) showed chromosome aberrations, and 4 cells (1.3%) had 14q11 anomaly. 2. In three non-HTLV-I carriers, 4 of 260 cells examined (1.5%) showed chromosome aberrations, whereas no cells had 14q11 anomaly. These findings suggest that 14q11 anomaly is already present at the stage of HTLV-I carrier and seems to be an important cytogenetic clue to the pathogenesis of ATL.

One hundred and eighty-two (182) cases of malignant lymphoma registered at the Nagasaki Tumor Registry from 1973 to the end of 1977 were studied in relation to atomic bomb exposure. No significant difference in the incidence of malignant lymphoma was found between the exposed and nonexposed groups. One hundred and thirty-one (131) cases excluding Hodgkin's disease and mycosis fungoides were histologically reviewed and classified according to Lymphoma Study Group (L.S.G.) and Working Formulation (W.F.) Classifications. Using the L.S.G. Classification, the three histological types (diffuse large cell, diffuse pleomorphic, and diffuse medium sized cell) occupied 72.7% and 69.0% of the exposed and nonexposed groups respectively. No significant difference in histological type between the exposed and nonexposed groups could be found with an exception of a slightly higher incidence of the diffuse medium sized cell type in the exposed group than in the nonexposed group. An evaluation of these results was made in comparison with the results in Hiroshima.

A case of primary pancreatic carcinoma confirmed by postmortem examination in a 15-year-old girl is presented. The tumor was studied by light and electron microscopy and an indirect immunoperoxidase technique. Some of the tumor cells contained eosinophilic, PAS-positive, diastase-resistant granules. Electron microscopy revealed large electron-dense granules resembling zymogen granules. The granules seen in the islet cell tumors were not demonstrated by electron microscopy and immunoperoxidase technique. These findings suggest that the tumor is of duct cell origin with some differentiation toward the acinar cells. The tumor appears to belong to the so-called "pancreatoblastoma".

We studied 89 cases of thyroid cancer registered at the Nagasaki Tumor Registry during the period from 1973--1977. The incidence of thyroid cancer tended to be higher in persons exposed to the atomic bomb, especially women, than in the nonexposed population. Compared with nonexposed individuals, the relative risk for thyroid cancer was increased in distally as well as proximally exposed persons, but not in early entrants to the bombed city. In 83 histologically examined patients, papillary carcinomas were highly predominant, they were more prevalent in the exposed than in the nonexposed population.

To prolong the survival of patients with chronic myeloid leukemia (CML), 19 patients were treated with busulfan to keep their leukocyte counts within normal range by controlling bone marrow hyperplasia. The duration of chronic phase in these patients was significantly longer than that in historical controls who were treated conventionally with busulfan. This prolongation was not ascribable to the difference in such prognostic factors between the two therapy groups as splenomegaly, leukocyte count and percentage of peripheral blasts. There was a significant difference again in the duration of chronic phase between the two therapy group even when restricted to each 11 patients with intermediate relative risk (0.7-1.5) according to Sokal et al. Four patients showed thrombocytopenia less than 5 x 10(4)/microliters, but all these patients recovered within 4 months and there was no further critical side effect except subcutaneous bleeding. This study suggests that maintenance of leukocyte count within normal range and suppression of granuloid hyperplasia in bone marrow with busulfan may prolong chronic phase of CML. Probability of clonal evolution may be decreased by reducing the total leukemic cell mass and suppressing cellular turnover of primitive CML stem cells. Another possibility is that prolongation of chronic phase might be dependent on the appearance of normal karyotype clone after long-term bone marrow suppression just like after intensive chemotherapy or alpha-interferon therapy.