Successful treatment of systemic amyloidosis by combination chemotherapy and plasmapheresis. Effect on plasma IL6 and serum amyloid protein A.
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Biomedical subjects
Publications and source records attributed to I Katayama.
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BACKGROUND: The eyelid dermatitis seen in elderly patients is a relatively heterogenous and troublesome disease. Most cases seem to be idiopathic and show resistance to standard dermatologic therapy. METHODS: Fifty-two patients with primary definite Sjögren's syndrome (4 men, 48 women, mean age 54 years) were enrolled in this study, and the prevalence of eyelid dermatitis was investigated. Diagnostic criteria for Sjögren's syndrome were based on the criteria proposed by the Japanese Ministry of Health and Welfare. Patch test was performed using ICDRG-European standard allergens and/or eye drops, hair dye, and cosmetics. RESULTS: Of the 52 patients, 22 showed eyelid dermatitis. These changes were much more frequent in elderly patients and showed a good correlation with the presence of ocular dry sensation. No significant difference was observed in clinical and other laboratory findings between patients with or without eyelid dermatitis. Although 8 of the 13 patients showed positive patch test reaction to various allergens, no close relationship existed between the use of a suspected substance and the onset or severity of eyelid dermatitis. CONCLUSIONS: These results suggest that the presence of rubbing dermatitis of the eyelid may be one of the cutaneous manifestations of Sjögren's syndrome.
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Clinical and immunopathological analysis was performed on 24 cases of Sjögren syndrome with annular erythema (AESjS). AESjS predominantly appears on the cheek of the face where skin temperature is relatively low in comparison with other sites. VCAM-1 and ICAM-1 were strongly expressed on endothelial cells of AESjS, while epidermal expression of ICAM-1 was focal and weak. VCAM-1 mRNA expression was also much more intense compared to systemic lupus erythematosus. The lymphocyte response to staphylococcal enterotoxin B was higher in AESjS than that of controls, and cells positive for T cell receptor V beta 6,9,12 were expanded after the culture. Superantigen-driven endothelial-cell-dependent T cell infiltration to the skin plays a crucial role in AESjS.
The cell surface expression of multiple immunoglobulin heavy chain (IGH) isotypes, preferentially observed in hairy cell leukemia (HCL), is incompatible with the deletion model of IGH class switching, and alternative models involving RNA-splicing have been proposed. To help discriminate between these possibilities we have examined the configuration of the IGH locus by DNA blot in 38 cases of HCL. Deletion of at least one allele of C mu/C delta was seen in 14 cases (37%). Of 12 cases in which IgG and/or IgA were expressed, three exhibited biallelic deletion of C mu/C delta compatible with deletional class-switching whereas the remaining 9 cases retained both alleles of C mu/C delta. These data indicate that both DNA deletion and RNA-splicing mechanisms of class-switching may operate in HCL. In a further 17 cases, other unexpected abnormalities of the IGH locus mapping to the JH-C mu intron in one of two patterns were observed. First, discordance between JH and C mu in BamHI DNA digests was found in 12 cases. Similar patterns were observed in only 4/91 cases of B-cell chronic lymphocytic leukemia and 0/15 of B-cell prolymphocytic leukemia. To preclude chromosomal translocations involving the JH-C mu intron, rearrangements of BCL1, BCL2, BCL3, and MYC were sought in 23 cases but none of the cases showed oncogene rearrangement to the intron. Second, abnormal-sized C mu fragments arising as a consequence of abnormalities of the JH-C mu intron were detected in HindIII, BgIII, and/or Xbal digests in 10/23 cases.(ABSTRACT TRUNCATED AT 250 WORDS)
We investigated the effects of stromal cells, obtained from lymph nodes with various lymphoid disorders and characterized immunocytochemically as fibroblasts, on the maturation of a cell line "HN" established from an atypical PLL (prolymphocytic leukemia) having phenotypic characteristics of both CLL (chronic lymphocytic leukemia) and HCL (hairy cell leukemia). Coculture with stromal cells, irrespective of the kind of lymphoid disease affecting the lymph node, induced a plasmacytoid cytology in HN cells, an increase of cIg gamma, and decreases of sIg gamma, CD5, CD21, HC2, and B-ly7. In contrast, coculture with the stromal cells produced no marked effects on hairy cells from two HCL patients. Similarly, coculture with stromal cell supernatant or with various cytokines shown to be produced by bone marrow stromal cells produced no effects on HN cells. We propose that lymph node stromal cells play an important role in the differentiation of B cells through direct contact and that HN cells will be useful for investigating the differentiation pathway of chronic B cell leukemia cells.
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We reported the treatment outcome of Protocol 8704T, which included repeated L-asparaginase, for childhood T cell malignancies. Fifteen cases of acute lymphoblastic leukemia (T-ALL) and 11 cases of non-Hodgkin's lymphoma (T-NHL), aged 3 to 14 yrs (median 6 yrs), were enrolled. Twelve T-ALL had mediastinal mass. Murphy's stages of T-NHL were 6 with III and 5 with IV. Types of histology consisted of 8 lymphoblastic and 3 large cell. Treatment was performed for 2 years. Observation periods were from 14 months to 78 months (median 42 months). Twenty-three achieved remission and 6 of them were transplanted with bone marrow or peripheral stem cells in the first remission. The protocol was continued in 17 cases. Fourteen of them remain in first remission, but one died of measles and 2 died of relapse. The 5-year event-free survival was 76.1% for ALL and 65.5% for NHL. In terms of histology, it was 87.5% for lymphoblastic NHL and 33.3% for large cell NHL (p = 0.19). In terms of phenotypes in ALL, it was 88.7% for ALL positive to CD2, 5 and 7, while 2 ALL positive to CD7 alone both failed. Therefore, it was shown that this treatment protocol is very effective for T-lymphoblastic leukemia and lymphoma.
Adult T cell leukemia/lymphoma (ATLL) shares overlapping clinicopathological features with cutaneous T cell lymphoma (CTCL), requiring detection of monoclonal integration of proviral DNA of type 1 human T cell leukemia virus for its differential diagnosis from the latter. We applied in situ hybridization (ISH) and polymerase chain reaction (PCR) to paraffin sections from 63 Japanese autopsy cases that had been diagnosed as CTCL in earlier years when ATLL was still not widely known. Eleven and two cases with confirmed diagnoses of ATLL and CTCL served as positive and negative controls, respectively. It was found that ISH was positive in 7 of 63 test cases and 10 of 11 positive controls, whereas PCR was positive in none of the test cases and eight of the positive control cases. Two negative controls were negative for both ISH and PCR. We conclude that ISH is superior to PCR for detecting type 1 human T cell leukemia virus proviral DNA on paraffin sections and that the ISH method is useful for differentiating CTCL from the cutaneous form of ATLL.
BACKGROUND: Epipodophyllotoxins, etoposide and teniposide, have been shown to be implicated in the development, of acute myelogenous leukemia in patients treated for solid tumors or acute lymphoblastic leukemia. Etoposide has been shown to be an effective agent against Langerhans cell histiocytosis (LCH) and has gained wider use recently for first-line and salvage chemotherapy in cases of systemic LCH. METHODS: The authors report two patients with secondary acute promyelocytic leukemia (APL) with a t(15;17) abnormality after chemotherapy that included etoposide for the treatment of LCH. RESULTS: Patient 1, a 6-year-old girl, had APL develop 11 months after cessation of therapy that included vinblastine, prednisolone, and etoposide (9600 mg/m2 in total dose) for LCH. Patient 2, a 3-year-old girl, had APL develop 9 months after cessation of therapy that included vincristine, methotrexate, prednisolone, cyclophosphamide (10,800 mg/m2), and etoposide (4800 mg/m2) for LCH. CONCLUSIONS: The authors have experience with four patients treated with etoposide for LCH and suggest that there is a predisposition to secondary APL with t(15;17) for patients with LCH treated with etoposide. The authors warn against the imprudent use of etoposide as a first-line therapy for LCH.
The first enzyme in the formation of GDP-L-fucose from GDP-D-mannose, which forms a GDP-4-keto sugar intermediate, was purified to homogeneity from cell extracts of Klebsiella pneumoniae. During purification, the enzyme was found to be highly activated by NADP. It was proven that the pyridine nucleotide coenzyme of the enzyme was NADP, not NAD, which differs from previously accepted information. NAD had no effect on enzyme activity. The product of the enzyme reaction with NADP as coenzyme was separated from other nucleotides by high-performance liquid chromatography, and using ion spray liquid chromatography/mass spectrometry the mass was determined for the first time, as 587, which is same as the calculated mass of GDP-4-keto-6-deoxy-D-mannose.
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Biopsied specimens from skin lesions of SLE were studied for expression of 70 KD heat shock protein (HSP70). The pattern of HSP70 expression in SLE was diffuse in whole epidermis, hair follicles, and sweat gland cells and rather more intense than that in other control groups or normal skin. No significant differences in HSP70 expression were observed between sun-exposed and protected areas of SLE skin lesions. Unlike SLE, reduced or no expression of HSP70 was observed in skin lesions of DLE. In tissue culture, UVB radiation in vitro induced relatively intense expression of HSP70 in the nuclear area of keratinocytes. A few gamma delta T cell receptor positive cells which might respond to HSP70 expressing cells were detected in the basal layer of skin lesions of diseases. These studies suggest that aberrant expression of HSP70 in skin lesions of SLE might contribute to both skin lesions and antibody formation in SLE.
Two Japanese women developed well-demarcated ichthyosiform plaques on the lateral aspect of their lower legs. Deposition of mucin was demonstrated throughout the papillary dermis, unlike the site of mucin deposition seen in pretibial myxedema. Their thyroid function was normal. The condition of both women was complicated by Sjögren's syndrome. One of them who presented with positive anti-microsomal and anti-thyroglobulin antibodies had goiter, suggesting that her malady was also complicated by Hashimoto's thyroiditis. Their skin manifestations differed from those described in cutaneous mucinosis including pretibial myxedema, specifically with regard to the well-demarcated ichthyosiform appearance, the mucin deposition in the papillary dermis, and the association with Sjögren's syndrome. To the best of our knowledge, our cases may thus be considered to be a previously undescribed form of cutaneous mucinosis associated with Sjögren's syndrome.
Two cases of Sjögren syndrome who developed a giant mucocele on the floor of the oral cavity are reported. Histological analysis revealed the dense infiltration of lymph-plasmacytic infiltration around the dilated salivary duct. Similar findings were observed in the biopsied specimens of the labial salivary gland which were consistent with the grade 4 score proposed by Chisholm and Mason's criteria. Mucocele is a common mucocutaneous disease of unknown etiology except for traumatic origin and Sjögren syndrome might be one of the important underlying disease which causes giant mucocele, especially in elderly female patients.
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We report a Japanese patient with skin-coloured keratotic papules on the hands and feet, and pronounced hyperkeratosis of the palms and soles. There was no evidence of fragmentation of dermal elastic tissue on histology. Freckle-like pigmentation was also present on the dorsa of the hands and feet, and this appeared to be closely related to the keratotic disorder. The mode of inheritance was autosomal dominant. We consider our case to be a new variant of familial punctate acrokeratoderma, associated with a pigmentary disorder.
A 61-year-old man presented with a skin tumour in the right axilla of 2 months' duration. An excision biopsy revealed a malignant lymphoma, of diffuse mixed small and large cell type, morphologically suggestive of T-cell lymphoma, but shown to be a T-cell-rich B-cell lymphoma by subsequent immunohistochemical staining. A review of the literature revealed 47 cases of T-cell-rich B-cell lymphoma with descriptions of primary sites, 35 nodal and 12 extranodal, of which only one was a cutaneous primary.