[Angioimmunoblastic lymphadenopathy with dysproteinemia terminating in peripheral T cell lymphoma with cytotoxic/suppressor phenotype: a case report].
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Biomedical subjects
Publications and source records attributed to M Kikuchi.
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The effects of diltiazem hydrochloride on insulin secretion and glucose tolerance were studied in diabetic subjects treated with dietary management alone for their glycemic control. Sixteen diabetic subjects with heart complaints and/or abnormal electrocardiographic findings which were compatible with diagnosis of ischemic heart disease were given diltiazem hydrochloride 90 mg daily for a mean of 9.3 +/- 1.0 (SE) months and oral 100 g glucose tolerance tests (o-GTT) were performed before and after the administration of diltiazem hydrochloride. As a control group, 14 diabetic patients with roughly the same level of fasting blood glucose and the same degree of glucose intolerance were selected, and in these patients oral 100 g glucose tolerance tests were also performed before and after a mean follow up period of 12.6 +/- 1.5 months. Although improvement in glucose tolerance was observed at the end of the trial in the group of patients with diltiazem hydrochloride, control group also showed improved glucose tolerance and there was no significant difference in the change of glucose tolerance between both groups. No significant change of IRI response in oral 100 g glucose tolerance test was observed in both groups. We could, therefore, conclude from these observations that diltiazem hydrochloride with usual clinical dosage dose not exert unfavourable effects on glucose tolerance and insulin secretion in the mild diabetic patients.
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The retrovirus-associated adult T-cell leukemia/lymphoma (ATL) has not been previously documented in Taiwan. Five cases identified recently by the authors are reported. Three of the patients were women, and their ages ranged from 36 to 60 years. The most important diagnostic clue was the observation of polylobated lymphoid cells in the peripheral blood. Other variably observed significant features included hypercalcemia, cutaneous eruptions, osteolytic bone lesion, hepatomegaly, and lymphadenopathy. Surface marker studies revealed that the leukemic or lymphoma cells were T-helper cells. Histopathologic examination revealed one case of pleomorphic type and three cases of medium-sized cell type. No tissue was available for study in one case. The diagnosis of ATL was confirmed by the indirect immunofluorescence test on MT-1 cell for antibodies to adult T-cell leukemia virus-associated antigen (ATLA). Three patients were dead within 6 months, and two patients had been in clinical remission for 7 and 10 months, respectively. These two latter cases were similar to the so-called smoldering type of ATL. Two descendents among nine relatives of the patients were also positive for anti-ATLA (22%). Two husbands were negative. Four of the five patients lived in the same county in northeastern coastal Taiwan, which suggested a possible clustering of ATL in that region.
A serological survey of antibodies to the adult T-cell leukemia virus-associated antigen (HTLV-A) conducted in Taiwan revealed II seropositive individuals among 2,565 serum donors tested, which included 1,187 hospital or free clinic patients, 1,065 blood donors and 313 aborigines. The overall prevalence rate was 0.43% and was 0.90% (9/995) for adults aged 40 years or over, which was similar to the rates observed in the non-endemic areas in Japan. No highly prevalent area was demonstrated. The II seropositive individuals identified were 8 hospital or clinic patients, 1 blood donor and 2 aborigines. Among 108 various leukemia/lymphoma patients also studied, 7 were positive. These 7 anti-HTLV-A-positive patients also had clinicopathologic features of adult T-cell leukemia/lymphoma (ATL). Among 21 relatives of 3 seropositive individuals in the epidemiologic study, 2 were positive; and among 25 relatives of 5 ATL patients, 6 were positive. Our data support the contention that horizontal familial transmission of human T-cell leukemia virus (HTLV-I) is an important route and HTLV-I infection can occur on a different genetic background. Furthermore, the antibody titer did not correlate with the manifestation or the state of the disease.
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Chromosome and pathologic studies were performed on two patients (a 12-year-old boy and a 62-year-old woman) with malignant histiocytosis (MH). Both patients had chromosome abnormalities in their neoplastic cells: the boy's karyotype was 45,Xp+,-Y,9p+,18q-, and the woman's 48,XX,+16, inv(1),mar(5),6p-,10p+,12q+,i(18q),+i(18q). The boy had typical clinical and pathologic findings of MH, and died without achieving remission by chemotherapy. At the initial stage the woman had the clinical and hematologic findings of MH. Chemotherapy was given, but had no beneficial effects. At the terminal stage the bone marrow (BM) biopsy and aspirate, and the autopsy findings, were consistent with those of non-Hodgkin lymphoma, diffuse large cell type, although some histiocytes or abnormal cells in monocyte-macrophage lineage remained in the BM and the autopsied lymph nodes. This study and a review of data on six other cases have failed to establish any specific chromosome changes in MH.
The image diagnoses of a case of so-called "nonfunctioning" adrenal adenoma, weak mineralocorticoids producing adrenal carcinoma, congenital adrenogenital syndrome due to 21-hydroxylase deficiency--simple virilizing form--, and 5 cases of pheochromocytoma were studied. In a patient with so-called "nonfunctioning" adrenal adenoma (2.3 X 3.0 X 3.3 cm), in which steroids biosynthesis was confirmed, computed tomography (CT) delineated the tumor shadow with extremely low density, and ultrasonography (US) demonstrated the round tumor echo with homogenous and low echogenicity at the superior region of the right renal pole. Adrenal scintigraphy also showed the tumor image. A weak mineralocorticoids-producing left adrenal carcinoma (3.5 X 3.5 X 3.0 cm) was shown as a heterogenous round tumor at the left lateral portion of the vertebra by CT. On adrenal scintigraphy under dexamethasone pretreatment, there was good uptake in the tumor and disappearance of the contralateral. Both bilateral adrenal images on CT in a patient with congenital adrenogenital syndrome were linear-shaped and markedly enlarged. The enlarged right adrenal was clearly demonstrated by US with an electronic sector scanner but not with an electronic linear scanner, although the left one was hardly shown by either US instruments. Three of 4 patients with pheochromocytomas examined by US were correctly detected, while in the remaining one the tumor image was judged to be a retroperitoneal tumor. CT also correctly demonstrated the former 3 pheochromocytomas, but misjudged the latter one as a pancreatic cancer. Good uptake of Adosterol by bilateral adrenals was shown in a case of extra-adrenal pheochromocytoma. Three of 4 cases of adrenal pheochromocytoma showed the isotope uptake of the contralateral normal adrenal alone. In another case of right adrenal pheochromocytoma, isotope accumulation in the colon obscured whether the isotope uptake in the right adrenal was shown or not.
Neighboring proteins in rat liver 60 S ribosomal subunits were investigated by two kinds of cross-linking techniques: treatment of 60 S subunits with 1) hydrogen peroxide, which promotes the formation of protein-protein disulfide linkages and 2) a disulfide-bridged bifunctional reagent dithiobis(succinimidyl propionate). The cross-linked protein complexes formed were separated by two-dimensional polyacrylamide gel electrophoresis in a basic-sodium dodecyl sulfate gel system under nonreducing conditions. Each complex in the gel was labeled with 125I and extracted under reducing conditions. The protein components of the complex were analyzed by two kinds of two-dimensional polyacrylamide gel electrophoresis, followed by autoradiography. Closely neighboring pairs disulfide-linked by hydrogen peroxide were identified as L4-L6, L4-L29, L6-L29, L18a-L29, and L29-L32; more distant pairs cross-linked with dithiobis(succinimidyl propionate) were identified as L3-L5, L3-L24, L3-L37a, L4-L14, L4-L18a, L5-L10, L5-L11, L7/L7a-L27, L7/L7a-L36, L13-L35, and L13a-L14.
Rat liver 60 S ribosomal subunits were modified with 2-iminothiolane. After treatment with hydrogen peroxide, the cross-linked proteins were extracted and then separated into 24 fractions by chromatography on carboxymethylcellulose. Each protein fraction was then analyzed by diagonal polyacrylamide-sodium dodecyl sulfate gel electrophoresis (Sommer, A., and Traut, R.R. (1974) Proc. Natl. Acad. Sci. U. S. A. 71, 3946-3950). The pieces of gel containing cross-linked protein spots that were shifted from the diagonal line were labeled with 125I. The labeled protein was extracted from the gel and identified by three kinds of two-dimensional gel electrophoresis, followed by autoradiography. Fifty-three cross-linked protein pairs involving 35 protein species containing two acidic proteins were identified. From these and previous results, a preliminary model of the protein topography of the 60 S ribosomal subunit was constructed and discussed in relation to other functional data on 60 S ribosomal proteins.
Comparative study of image diagnosis of ultrasonography (US) by linear electronic scanner, computed tomography (CT), and adrenal scintigraphy was performed in 14 patients with Cushing's syndrome. Adrenal imaging by scintigraphy was performed at the 5th and 6th day or further 7th day following the injection of 1 mCi of Adosterol. Cushing's disease (11 cases) US failed to detect the adrenals in 4 cases examined. Measurement of the adrenals on CT film demonstrated the enlargement of adrenals (greater than mean + 2SD) in 6 of 7 cases (85.7%). Scintiscanning showed the increased uptake of bilateral adrenals in 4 of 10 cases (40%). Adrenal scintigraphy with dexamethasone pretreatment (DP) still demonstrated the isotope uptake of bilateral adrenals in all of those 4 cases tested, although the other 6 cases were not studied with DP. From these findings, it was suggested that the measurement of adrenal size by CT was useful for the additional image diagnosis of Cushing's disease, and the adrenal scintigraphy with DP was also available for complementary study of Cushing's disease. Cushing's syndrome due to adrenocortical adenoma (3 cases) In one case examined by US, which had the smallest adenoma (0.6 X 1.0 X 2.0 cm) in this syndrome, the adenoma was not detected. All of 3 adrenal adenomas (2.6 X 2.6 X 2.2 cm to 0.6 X 1.0 X 2.0 cm) were detected by CT. Adrenal scintigraphy demonstrated good uptake by adrenal adenoma but no visualization of the contralateral adrenal in every case.
The shape and size of the adrenals in control subjects without adrenal disorders were studied by computed tomography (CT), and a comparative assessment of diagnostic values of ultrasonography (US) by electronic linear scanner, CT, and adrenal scintigraphy was made on 9 patients with primary aldosteronism. Adrenal imaging by scintigraphy was performed on the 5th and 6th day, or further on the 7th day after the injection of 1 mCi of Adosterol. CT findings of the adrenals in control subjects: Eighty-two % of 100 control right adrenals, and 89% of 100 control left adrenals were detected by CT. Seventy-seven % of the right adrenals were in linear-shape, and the others were in V-shape. The shape of the left adrenals could be classified into triangular-shape (54%), Y-shape (28%) and V-shape (18%). The mean width and thickness of the right adrenals were 28.6 +/- 7.5 mm (M +/- SD) and 3.8 +/- 1.4 mm, respectively. Those of the left ones were 19.4 +/- 5.5 mm and 5.3 +/- 1.8 mm. Image diagnosis of primary aldosteronism: In 2 out of 3 patients examined by US, aldosteronomas were detected. In these 2 patients, one had 2 adenomas 2.8 X 1.7 X 1.2 cm and 1.0 X 1.0 X 2.0 cm in size, and the other had one adenoma 0.8 X 1.0 X 2.0 cm in size. On adrenal scintiscanning under dexamethasone pretreatment (DP), the isotope uptake of aldosteronoma was still seen with the disappearance of the contralateral adrenal in 7 out of 9 cases. In these 7 cases, the laterality of the tumor was confirmed. In one of the remaining 2 cases, the bilateral adrenal images were obtained regardless of DP. In the other case, of which aldosteronoma was the smallest (0.6 X 0.6 X 0.8 cm), the image of the affected adrenal with adenoma as well as the contralateral adrenal disappeared under DP. CT delineated all aldosteronomas in 8 cases examined including 2 cases in which adrenal scintiscanning failed to elucidate the localization of aldosteronoma. These results indicated that the combination of these 3 new image diagnostic methods was available for the detection of aldosteronomas of various sizes.
Two cases of hyperplastic lymphoid lesion of the stomach with cytoplasmic immunoglobulin of monotypic pattern are presented. Both patients were young, and the postgastrectomy course was uneventful for 38 and 76 months, respectively. The lesion had been diagnosed as pseudolymphoma based on the presence of hyperplastic follicles with germinal center and mixed infiltration of plasma cells and mature lymphocytes with no significant cytologic atypia. However, the immunoperoxidase method showed monotypic cytoplasmic immunoglobulin; lambda/IgM in one case and lambda/IgG in the other. The staining pattern of germinal centers was also monotypic in one, but polytypic in the other. These cases suggest the presence of monoclonal but reactive lymphoid hyperplasia, i.e., monoclonal-type pseudolymphoma in the stomach. On the other hand, this type of lesion has to be carefully followed for the possible development of malignant lymphoma.
A total of 56 cases of malignant lymphoma presumed to be of peripheral T-cell origin were investigated with regard to histological and immunohistochemical features. The goal of the study was to determine whether virus-associated T-cell lymphomas can be morphologically or immunohistochemically distinguished from presumably virus-negative T-cell lymphomas. The cases came from endemic and non-endemic regions of Japan, the United Kingdom (including 4 Caribbean cases) and the Federal Republic of Germany. Sera of all Japanese and Caribbean patients and 8 German patients were tested for antibodies to adult T-cell leukaemia virus-associated antigen HTLV-A. In all cases sections were examined blind by 5 well-trained histopathologists. In most cases cryostat sections could be prepared from fresh tissue specimens and stained with a large panel of monoclonal antibodies. All HTLV-A-positive cases were morphologically classifiable as the pleomorphic type of T-cell lymphoma. Approximately 70% of the tested cases of pleomorphic T-cell lymphoma, however, showed a positive serum reaction for HTLV-A. All other types of peripheral T-cell lymphoma (T-immunoblastic lymphoma, chronic lymphocytic leukaemia of T type, T-zone lymphoma, "AILD type" and lymphoepithelioid cell lymphoma) were HTLV-A-negative and mostly observed in European patients. Thus virus-associated T-cell lymphomas appear to be invariably of the pleomorphic type; but pleomorphism is not specific to HTLV-A-positive cases. This was also evident from the results of an experiment in which 2 Japanese histopathologists attempted to recognize HTLV-A positivity in a blind study of pleomorphic T-cell lymphomas. A maximum of about 80% of cases were correctly identified, with about 10% false-positive diagnoses (in HTLV-A-negative or presumably negative cases) and 10% false-negative diagnoses. The immunohistochemical analysis revealed not only many common features but also 2 distinct differences between HTLV-A-positive and -negative T-cell lymphomas. All but one of the HTLV-A-positive cases showed reactivity with anti-Tac and all cases in the virus-positive group were negative for TU14. All other cases were Tac-negative and approximately 65% of these cases exhibited reactivity with TU14. Preliminary cytogenetic observations suggest that there are also differences in specific chromosome aberrations.