PELGEROID-LIKE ANOMALOUS CELLS IN THE DIAGNOSIS OF NEUTROPHILIC DERMATOSIS ASSOCIATED WITH MYELODYSPLASTIC SYNDROME

Neutrophilic dermatoses (ND), with or without accompanying myelodysplastic syndrome (MDS), were examined in terms of nuclear abnormality like pelgeroid anomaly of infiltrating cells into skin lesions. Six ND accompanying MDS showed 1.0 to 13.5% of such anomalous cells among infiltrating cells. In contrast, ND without accompanying myeloproliferative disorders rarely had such anomalous cells. Our findings suggest that pelgeroid-like anomalous cells infiltrating into ND are probably a good marker of underlying MDS.     

Cystic lymphangioma of the gall-bladder: A case report

Intra-abdominal cystic lymphangiomas are rare lesions that can be difficult to diagnose. We present a report of a patient with a giant multilocular cystic lesion in the abdomen. Ultrasonography and computed tomography scans of the abdomen revealed that the cyst had originated in the gallbladder fossa. There was some calcification and thickening of the cyst wall. Endoscopic retrograde cholangiopancreatography demonstrated a medially deviated common bile duct, an elongated cystic duct and an inferior compressed gallbladder. There was no apparent communication between the cyst and the biliary tract; however, an abdominal angiogram revealed that the lesion was supplied by a branch of the cystic artery. Histological findings obtained intra-operatively were consistent with a cystic lymphangioma. Its characteristic histology was observed in the subserous layer of the gall-bladder. This case is a rare instance of a cystic lymphangioma originating in the gall-bladder.     

Cyclophosphamide therapy for pure red cell aplasia associated with granular lymphocyte-proliferative disorders

Granular lymphocytes have been characterized as cells with azurophilic granules in the cytoplasm. Patients with increased numbers of granular lymphocytes are designated as granular lymphocyte-proliferative disorders (GLPDs). A variety of haematological abnormalities are associated with T-cell-lineage GLPD. Among these, pure red cell aplasia is frequent, and adequate therapy is required. Seven patients with pure red cell aplasia, or a related condition complicating T-cell-lineage GLPD, were entered into this study. Cyclophosphamide was initiated at a daily oral dose of 100 mg. After 2 weeks the dose was reduced to 50 mg/d, and maintained at that dose. Cyclophosphamide was administered until the lymphocyte count was <1 ×10⁹ l and T-cell receptor-β gene analysis was used to monitor the response to treatment. All the patients were successfully treated, irrespective of their former treatment. Clinical remission was associated with the disappearance of the abnormal granular lymphocyte clone, as detected by Southern blot hybridization analysis. Therapeutic responses began after 8 weeks, and clinical complete remissions were obtained after 6 months. Oral cyclophosphamide monotherapy can successfully treat the pure red cell aplasia associated with T-cell-lineage GLPD.     

Cutting Balloon Angioplasty for a Restenosis Lesion at the Distal Anastomosis of a Saphenous Vein Graft

We successfully performed cutting balloon angioplasty for a restenotic lesion involving the distal saphenous vein graft (SVG) anastomosis that had developed within 1 month after conventional balloon angioplasty. We considered that cutting balloon angioplasty, which has been considered to produce less vascular injury, could be a viable choice for the treatment of a lesion at the distal anastomosis of an SVG.     

Haemophagocytic syndrome associated with Epstein-Barr virus infection and promoted by tuberculosis reactivation in a patient undergoing chronic haemodialysis

SUMMARY: A 73-year-old man who had been undergoing chronic haemodialysis (CHD) for 3 years developed haemophagocytic syndrome (HPS) that might have been triggered by Epstein-Barr virus (EBV) infection. the patient finally died of miliary tuberculosis (TB) reactivation that promoted the progression of HPS. Immunological abnormalities in patients undergoing CHD may be notable. the early diagnosis of TB reactivation may be important for reducing the mortality in cases of HPS, as a high incidence of TB is encountered in patients undergoing CHD. In contrast, the simultaneous occurrence of an EBER-positive hybridization signal with T cell-specific immunolabelling of CD45RO cells was well detected in the spleen and lymph nodes, and interferon gamma was elevated in the serum. These findings coincide with the reported preferential expansion of T cells rather than B cells in EBV infection, and support the hypothesis that systemic hypercytokinaemia caused by the proliferation of EBV-infected T cells may play a crucial role in the development of HPS.     

There is No Correlation Between Function and Lymphokine Production of HBs-Antigen-Specific Human CD4+-Cloned T Cells

The question whether antigen-specific human CD4+ T cells can be classified on the basis of appropriate and fixed lymphokine production patterns and their corresponding functions still remains to be elucidated. We generated ten CD4+ T-cell clones specific for HBsAg from HBsAb-positive but HBsAg-negative individuals. Seven of these clones exhibited helper activity for HBsAb response, while the three other clones did not. Both helper- and non-helper-type T-cell clones produced interleukin 4 (IL-4) after antigenic stimulation. By stimulation with phytohaemagglutinin (PHA) plus phorbol myristate acetate (PMA), three of the seven helper-type clones produced interleukin 2 (IL-2) in addition to IL-4. However, the other four helper-type clones did not produce IL-2 by such stimulation, although they continued the production of IL-4. All non-helper-type T-cell clones produced a large amount of IL-2, and some of them completely became an IL-2 producer after certain stimulation. These results suggested that both helper- and non-helper-type CD4+ T-cell clones specific for HBsAg might have no strict pattern of lymphokine production as in the TH1/TH2 dichotomy of murine CD4+ T cells. The data also revealed that lymphokine-producing capacity of individual cloned T cells is changeable depending upon the sort of activation.     

Systemic disorders of calcium dynamics in rats with adenine-induced renal failure: Implication for chronic kidney disease-related complications

Aim:   Chronic kidney disease (CKD) causes the dysregulation of systemic mineral metabolism. A major issue in CKD patients is the emergence of ectopic calcification in soft tissues, presumably due to increased levels of calcium (Ca) or inorganic phosphorus (Pi); however, the precise mechanisms have not been fully elucidated. Therefore, this study aims to evaluate Ca dynamics in an animal model of CKD.
Methods:   Renal failure was produced in rats by feeding an adenine-containing diet for 4 weeks, and time-course changes in biochemical parameters, including Ca, Pi, creatinine (Cr), blood urea nitrogen (BUN), parathyroid hormone (PTH), 1,25-dihydroxyvitamin D₃, and N-telopeptide and cross-linked collagen type I (NTx), were monitored once a week during the feeding period. Intestinal absorption, tissue contents, and urinary excretion of Ca were monitored using radioisotope (RI) ⁴⁵Ca.
Results:   Adenine-fed rats exhibited renal failure, ectopic calcification and altered serum parameters, including elevated levels of serum Pi, Cr, PTH and BUN. Serum Ca levels were not increased in rats with renal failure. RI-based experiments revealed that abnormal Ca dynamics including attenuated intestinal absorption, increased incorporation into soft tissues, particularly aortic tissue, in which it was increased threefold, and enhanced urinary excretion occurred in renal failure rats.
Conclusion:   Rats with renal failure induced by an adenine diet exhibited severe abnormality of Ca dynamics, including Ca shortage and ectopic accumulation of Ca. These findings would provide useful information to research CKD-related complications.     

Varicella-zoster virus replication site in internal organs of an otherwise healthy child with varicella and sudden death

Pathological findings of an otherwise healthy 17 month old boy who was exposed to Varicella-zoster virus (VZV) in his family and unexpectedly died 3 days after onset of varicella are reported. They showed a disseminated VZV infection with involvement of skin, lung, liver, spleen, gastrointestinal tract and other organs where VZV antigen was detected by the enzyme-immunoassay with monoclonal antibodies to VZV. Since the subject was the full-term product of an uncomplicated pregnancy, who grew and developed normally, and had no symptoms or laboratory findings suggestive of immunodeficiency until his death, these findings suggest that many organs are involved as major internal sites of viral replication before or during infection of skin with VZV in the immunocompetent host.