1/11. Leukaemic presentation of small cell variant anaplastic large cell lymphoma: report of four cases.We report four cases of a rare subtype of CD30-positive anaplastic large cell lymphoma (ALCL) with a predominant small cell component (small cell variant of ALCL) presenting with a leukaemic feature. Lymph node biopsy showed malignant cells of varying size with a predominant population of small to medium-sized malignant cells associated with large anaplastic cells strongly positive for CD30 and epithelial membrane antigen (EMA). Both large and small cells were reactive with antibody ALK1, which recognizes the chimaeric NPM-ALK protein associated with the t(2;5)(p23;q35). All patients presented with hyperleucocytosis with atypical small lymphocytes. Bone marrow involvement was detected on both aspirate and bone marrow trephine where scattered malignant cells were only demonstrated by immunostaining for CD30 and ALK protein. Atypical cells in peripheral blood, lymph node and skin biopsies showed a T or null cell phenotype. cytogenetic analysis of blood, bone marrow and/or lymph node revealed the t(2:5)(p23;q35) characteristic of ALCL. The patients responded to chemotherapy but showed early relapse without abnormal cells in peripheral blood. This report shows that the small cell variant of ALCL may have a leukaemic presentation with peripheral blood involvement by atypical lymphocytes and provides evidence that, in the small cell variant of ALCL, the small cell component is a part of the malignant clone.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/11. adult acute disseminated encephalomyelitis associated with poststreptococcal infection.Acute disseminated encephalomyelitis (ADEM) is a monophasic illness that is thought to develop from antigenic mimicry with antibodies having cross-reactivity to host epitopes in the nervous system. The disorder typically follows an exanthematous or recent viral infection. In contrast, complications from bacterial poststreptococcal infections more commonly give rise to disorders in the pediatric population including Sydenham's chorea, pediatric autoimmune neuropsychiatric disorders, and ADEM. We present the novel case of documented streptococcal pharyngitis and elevated antideoxyribonuclease B (ADNB) titers in an adult giving rise to ADEM. Furthermore, the absence of basal ganglia abnormalities on MRI and the degree of leukocytosis in the CSF distinguish the adult form of ADEM from childhood ADEM and adult viral demyelinating diseases.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
3/11. Megakaryoblastic transformation of chronic granulocytic leukemia.Three cases of megakaryoblastic transformation of chronic granulocytic leukemia (CGL) are reported. In each case, the leukemic transformation had morphologic features suggesting megakaryocytic differentiation. This was confirmed by positive immunostaining with a monoclonal antibody (HP1-1D) specific for platelet and megakaryocyte glycoprotein IIb/IIIa antigen, which was expressed by the majority of the leukemic blasts in all three cases. Cases with evidence of multilineage differentiation of the leukemic transformation were excluded. A striking feature in two patients was the manifestation of lytic bone lesions and soft tissue masses at presentation. A biopsy of a lytic bone lesion and soft tissue mass in one patient revealed a megakaryoblastic leukemic infiltrate, which by immunocytochemical staining was positive for the megakaryocytic markers, glycoprotein IIb/IIIa antigen, and factor viii (von willebrand factor) antigen. In contrast to granulocytic sarcomas, the megakaryoblastic sarcoma did not stain cytochemically for chloroacetate esterase. The mean survival after acute transformation was 5.3 months. The three cases of megakaryoblastic transformation represented a significant proportion of all CGL blastic transformation cases (ten cases) evaluated by bone marrow examination in our institution during a 13-month period. Megakaryoblastic transformation of CGL may occur more frequently than has been appreciated, and can present as lytic bone lesions or as soft tissue megakaryoblastic sarcomas.- - - - - - - - - - ranking = 3keywords = antigen (Clic here for more details about this article) |
4/11. Human leukocyte antigen in Sweet's syndrome and its relationship to Behcet's disease.A 41-year-old man with Sweet's syndrome (SS) had symptoms similar to Behcet's disease (BD). To study the relationship of the two diseases, human leukocyte antigen (HLA) typing was performed on 28 patients with SS and 49 patients with BD. Of the 28 patients with SS, seven had BD symptoms. The frequencies of both HLA-B51 and -DQw3 were significantly higher in patients with BD. However, the frequencies of the two hla antigens in the 28 patients with SS and the 21 patients with SS without BD symptoms were not significantly different from the controls. The frequency of HLA-Bw54 was significantly increased in both groups of patients with SS. Taken together, these data indicate that SS is a genetically distinct disease entity from BD, although their symptoms are similar and the incidence of SS among patients with BD is high in japan.- - - - - - - - - - ranking = 6keywords = antigen (Clic here for more details about this article) |
5/11. Acquired hypogammaglobulinemia following infectious mononucleosis.The case of a 17-year-old male who developed hypogammaglobulinemia following infectious mononucleosis is presented. The family history revealed that a male sibling had died some years earlier with encephalitis also following infectious mononucleosis. Laboratory investigations revealed adequate numbers of T and B cells and normal proportions of helper and suppressor T lymphocytes but poor in vitro responses to mitogens and an absence of hypersensitivity to skin test antigens. Serial serological examinations for Epstein-Barr virus antibodies indicated a primary immune response to this virus in a hypogammaglobulinemic individual. The patient probably represents a case of the X-linked lymphoproliferative syndrome. The unique feature of the present case is the demonstration of Epstein-Barr virus-specific T-cell memory. The significance of this finding and the variable expression of this syndrome in two members of the same family are discussed.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
6/11. hypersensitivity lung disease presumptively due to Cephalosporium in homes contaminated by sewage flooding or by humidifier water.Two cases of hypersensitivity lung disease apparently caused by home environment contamination with Cephalosporium have been identified. In one case repeated flooding of the home with sewer water appeared to be important in maintaining an environment contaminated with Cephalosporium, and in the other case a contaminated humidifier appeared to be the source. Both patients had chronic respiratory symptoms remitting during absence from the home, and both presented problems in diagnosis. In both cases cultures of the home environment or humidifier and serum antibody studies indicated Cephalosporium as the antigenic source responsible for the airway disease. An inhalation challenge in one case was positive, adding further evidence for Cephalosporium as the antigenic source and etiologic agent. In both cases complete remission followed moving from the home. Cephalosporium is reported as an apparent antigenic source for home contamination resulting in significant chronic pulmonary morbidity.- - - - - - - - - - ranking = 3keywords = antigen (Clic here for more details about this article) |
7/11. Isolation and characterization of a new strain of ehrlichia chaffeensis from a patient with nearly fatal monocytic ehrlichiosis.ehrlichia chaffeensis is the causative agent of human monocytic ehrlichiosis, a disease that ranges in severity from asymptomatic infection to death. Only one isolate of E. chaffeensis has been made, the arkansas strain, upon which all characterizations of the agent of human monocytic ehrlichiosis have been based. We report the isolation and characterization of a new strain of E. chaffeensis, the 91HE17 strain, which was cultivated from a patient with a nearly fatal illness. The new isolate grows best in culture with careful control of pH. The two isolates are nearly identical as determined by light and electron microscopy and have significant antigenic identity in fluorescent-antibody and immunoblot assays using polyclonal antisera and the E. chaffeensis-specific monoclonal antibody 1A9. Isolate 91HE17 had 99.9% nucleotide sequence identity with the arkansas strain in the 16S rRNA gene. Parts of the escherichia coli GroE operon homologs had identical restriction enzyme digestion patterns, and a 425-bp region of the GroEL gene had at least 99.8% sequence identity between the E. chaffeensis arkansas and 91HE17 strains. Isolate 91HE17 lacked an epitope identified in E. chaffeensis arkansas by the monoclonal antibody 6A1. This new E. chaffeensis isolate is very similar to the arkansas strain and provides the opportunity to substantiate the existence of diversity among ehrlichiae which infect humans. Specific factors which differ among strains may then be compared to assess their potential contributions toward cellular pathogenicity and ultimately toward the development of disease in humans.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
8/11. Sweet's syndrome: unique local response to streptococcal antigen.Although hypersensitivity has been postulated, the etiology of Sweet's syndrome has never been elucidated. In the present case intradermal tesing with viridans streptococcus led to an immediate reaction and reproduced a lesion clinically and histologically identical to that of the primary disease. Other bacterial and fungal antigens failed to elicit any response. These findings provide further support for the role of hypersensitivity in the pathogenesis of this disorder. It would appear that immediate or humoral immunit- - - - - - - - - - ranking = 5keywords = antigen (Clic here for more details about this article) |
9/11. New cell line from hairy-cell leukaemia producing interleukin-6 after Epstein-Barr virus immortalization.A hairy-cell leukaemia (HCL) cell line, HCL-O, was established from the peripheral blood of a 62-year-old Japanese patient with a unique variant of HCL strongly expressing CD21, the receptor for the Epstein-Barr virus (EBV). The HCL-O cells expressed antigens similar and dissimilar to those expressed with the original hairy cells. The HCL-O cells were more mature than the original cells in their degree of B-cell differentiation, as indicated by a decrease of CD19 and surface immunoglobulin (sIg) expression together with the appearance of CD38 and cytoplasmic Ig (cIg). In addition, the cells expressed CD11c recognized by Leu-M5, a monoclonal antibody usually positive for HCL. Their karyotype and Ig gene rearrangement pattern were identical to those of the original cells. The EBV genome was detected in the HCL-O cells but not in the original cells. The HCL-O cells spontaneously produced a large quantity of interleukin-6 (IL-6) in the conditioned medium, whereas IL-6 serum level was not so high. These findings indicate that the HCL-O cell line is derived from the leukaemic hairy cells and possibly, in vitro EBV infection took place easily in the original hairy cells through their CD21, resulting in subsequent immortalization. IL-6 production by HCL-O cells may be induced or enhanced by EBV, and the secreted IL-6 might play a role in their own growth or differentiation.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
10/11. Transient myeloproliferative disorder in a Down's neonate with rearranged T-cell receptor beta gene and evidence of in vivo maturation demonstrated by dual-colour flow cytometric dna ploidy analysis.Neonates with Down's syndrome may develop a transient myeloproliferative disorder (TMPD) which on presentation is indistinguishable from acute leukemia, with the difference manifest only on follow-up. The clinical course is one of spontaneous remission in TMPD and relentless progression in leukemia. We describe a Down's neonate presenting with hyperleucocytosis and circulating blasts which were positive for CD34, myeloid (CD33), megakaryocytic (CD41, CD42b, CD61), and T-lineage (CD3, CD7), but not B-lineage, associated antigens. Clonal rearrangement of the T-cell receptor beta (TCR beta) gene was found, with the immunoglobulin heavy chain gene in germline configuration, showing the disease to be a clonal proliferation of a multipotential stem cell involving the myeloid and T lineages. Dual-colour flow cytometric dna ploidy analysis of CD41 positive blasts showed initially a predominant 2N population, but polyploidization to 6N and 8N cells was found on follow-up, concomitant with a progressive decrease in circulating blasts, suggesting maturation of the abnormal clone and a provisional diagnosis of TMPD. This was shown by the eventual resumption of normal haemopoiesis with the disappearance of blasts and the clonally rearranged TCR beta gene.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
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