Abstract:
:A 27-year-old male patient with ataxia telangiectasia (AT) developed atypical chronic lymphocytic leukemia with increasing bone marrow infiltration in the absence of organomegaly. One-third of the leukemia cells expressed a mature suppressor/cytotoxic T cell phenotype (T3+ T4- T6- T8+ T10-), two-thirds demonstrated additional helper/inducer T cell-associated antigens (T3+ T4+ T6- T8+ T10-), and a small fraction reacted with a natural killer (NK) cell-specific monoclonal antibody (Leu 11+). The proliferative response to stimulation in vitro with lectins and various monoclonal antibodies resembled the proliferation pattern of mature thymocytes: The cells responded to phytohemagglutinin (PHA), concanavalin A (ConA), stimulation of the T3-Ti receptor complex with Sepharose-bound anti-T3, and stimulation of the sheep erythrocyte receptor protein with anti-T11(2) and anti-T11(3) in conjunction with exogenous interleukin-2 (IL 2); they failed, however, to proliferate after stimulation with anti-T11(2) and anti-T11(3) alone. There was no response in the mixed lymphocyte reaction (MLR) and no suppression of the MLR between two healthy donors. Antibody-dependent cell-mediated cytotoxicity and NK activity could not be demonstrated. Cytogenetic analysis revealed complex clonal aberrations, including an interstitial deletion of the long arm of chromosome 14 concerning bands q21-31, loss of chromosome 20, and loss of the Y chromosome. Cytostatic chemotherapy was of little use and caused serious side effects, whereas leukapheresis proved effective in reducing the tumor load. The clinical data and laboratory findings in this case correspond to three previously described patients with AT who developed chronic T cell leukemia. Thus, in adult patients with AT, malignant proliferation of cytogenetically marked and phenotypically heterogeneous mature T cells seems to be a frequent complication.
journal_name
Bloodjournal_title
Bloodauthors
Dührsen U,Uppenkamp M,Uppenkamp I,Becher R,Engelhard M,König E,Meusers P,Meuer S,Brittinger Gsubject
Has Abstractpub_date
1986-08-01 00:00:00pages
577-85issue
2eissn
0006-4971issn
1528-0020journal_volume
68pub_type
杂志文章相关文献
BLOOD文献大全abstract::Transfusions purportedly induce dysfunction of cell-mediated immunity in sickle cell anemia (SCA). We studied hematologic and lymphocytic indices in 173 human immunodeficiency virus (HIV)-negative subjects with SCA and 131 black controls. Children aged 1 to 7 years with SCA had leukocyte counts and percentages of gran...
journal_title:Blood
pub_type: 杂志文章,多中心研究
doi:
更新日期:1995-04-15 00:00:00
abstract::Sixty-five patients with hairy cell leukemia underwent splenectomy; 27 had a complete remission as defined by a return in WBC, RBC, and platelet counts to a defined level, and 38 had a partial remission with a return of only one or two of these parameters to the defined level. The 5-yr actuarial survival for all patie...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1983-02-01 00:00:00
abstract::Currently availabel staging systems for non-Hodgkin lymphomas are not useful for clinical staging classification of most primary cutaneous lymphomas. The tumor, node, metastases (TNM) system used for mycosis fungoides (MF) and Sézary syndrome (SS) is not appropriate for other primary cutaneous lymphomas. A usable, uni...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2006-10-054601
更新日期:2007-07-15 00:00:00
abstract::B-cell chronic lymphocytic leukemia (CLL) consists of 2 prognostic entities where cases with mutated immunoglobulin V(H) genes have better outcome than unmutated cases. V(H)-mutated CLLs display longer telomeres compared with unmutated cases and telomere length has been indicated to predict outcome, although the progn...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2004-11-4394
更新日期:2005-06-15 00:00:00
abstract::In vitro agar culture patterns of bone marrow cells in acute myeloid leukemia may show several growth patterns, including cultures where no colonies or clusters develop, cultures with varying numbers of clusters and no colonies, or colony and cluster formation with an extremely high ratio of clusters to colonies. Twel...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1979-08-01 00:00:00
abstract::In 21 patients with lymphoproliferative disease of granular lymphocytes (LDGL), we investigated the expression and the function of molecules belonging to TNF-receptor and TNF-ligand superfamilies (CD30/CD30L; CD40/CD40L; CD27/CD70; Fas [CD95]/FasL[CD95L]). Fourteen patients were characterized by a proliferation of gra...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:2000-07-15 00:00:00
abstract::We have used a single intensive chemotherapy regimen in the treatment of young patients with diffuse, aggressive, malignant lymphomas. There were two major histologic types of lymphoma in our series: lymphoblastic lymphomas, which presented most often with mediastinal tumor (64%), and undifferentiated lymphomas (mostl...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1984-05-01 00:00:00
abstract::An immunosuppressant Rapamycin (Rap) has been reported to cause G1 arrest by inhibiting p70 S6 kinase and G1 cyclin/cdks kinase activities when added to quiescent cells with mitogens. However, antiproliferative effects of Rap on exponentially growing cells have been poorly investigated. We examined the intracellular e...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1998-01-15 00:00:00
abstract::All-trans retinoic acid (tRA) and arsenic trioxide (As(2)O(3)) induce non-cross-resistant complete clinical remission in patients with acute promyelocytic leukemia with t(15;17) translocation and target PML-RARalpha, the leukemogenic protein, by different pathways suggesting a possible therapeutic synergism. To evalua...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood.v97.1.264
更新日期:2001-01-01 00:00:00
abstract::Previous studies have suggested that, in patients with AL amyloidosis treated with high-dose melphalan and autologous stem-cell transplantation (HDM/SCT), the greatest benefit is seen in those patients achieving a hematologic complete response (CR). We analyzed a series of 421 consecutive patients treated with HDM/SCT...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2011-01-330738
更新日期:2011-10-20 00:00:00
abstract::We have recently shown that interleukin-9 (IL-9) strongly stimulates the proliferation of mouse thymic lymphomas in vitro. Here we report that this factor is also one of the most potent inhibitors of apoptosis induced by dexamethasone (DEX) in such cell lines, even if they do not depend on exogenous factors for growth...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1995-03-01 00:00:00
abstract::Adult-onset hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening disease of immune hyperactivation. Unlike pediatric HLH, adult HLH is rarely driven by germline genetic variants. Although numerous precipitating etiologies have been identified, the reason that HLH occurs in only a subset of individuals ...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood.2020008206
更新日期:2020-12-24 00:00:00
abstract::Tumor cells from six patients with immunoglobulin G (IgG) multiple myeloma were analyzed for surface antigens, cytoplasmic paraprotein, morphology, and response to various culture conditions. The tumor marker was the paraprotein idiotype. Low numbers of tumor cells were found in the blood of most of the patients. In s...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1989-05-15 00:00:00
abstract::Monoclonal antibodies were raised after injecting mice with isolated human dense granules. Several of these monoclonals were found to recognize a 40-Kd dense granule membrane protein. Western blot and immunofluorescent analysis confirmed the dense-granule specificity. After thrombin activation, the protein was found i...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1991-01-01 00:00:00
abstract::Several patients with clinical features of chronic myeloid leukemia (CML) have fusion of the TEL (ETV6) gene on 12p13 with ABL on 9q34 and express a chimeric Tel-Abl protein that contains the same portion of the Abl tyrosine kinase fused to Tel, an Ets family transcription factor, rather than Bcr. In a murine retrovir...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2001-12-0244
更新日期:2002-06-15 00:00:00
abstract::To migrate efficiently through the interstitium, dendritic cells (DCs) constantly adapt their shape to the given structure of the extracellular matrix and follow the path of least resistance. It is known that this amoeboid migration of DCs requires Cdc42, yet the upstream regulators critical for localization and activ...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2012-01-407098
更新日期:2012-05-10 00:00:00
abstract::The response to intravenous challenge with sheep erythrocytes was determined in Sprague-Dawley rats following autotransplantation of splenic tissue into the subcutaneous tissue, peritoneal cavity, or a surgically created omental pouch. There was a marked rise in heterophil antibody titer following intravenous challeng...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1977-05-01 00:00:00
abstract::Liver injury is a frequent, serious complication of allogeneic hematopoietic cell transplantation (HCT) following myeloablative preparative regimens. We sought to determine the frequency and severity of hepatic injury after nonmyeloablative conditioning and its relationship to outcomes. One hundred ninety-three consec...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2003-04-1311
更新日期:2004-01-01 00:00:00
abstract::A male neonate with Down's syndrome and congenital myeloproliferative disorder was studied. His blood picture showed the unique coexistence of leukocytosis with matured cells and a large number of blast cells. The in vitro proliferation and differentiation of blast cells into various lineages in the presence of phytoh...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1987-02-01 00:00:00
abstract::Atypical hemolytic uremic syndrome (aHUS) is associated with complement system dysregulation, and more than 25% of pediatric aHUS cases are linked to mutations in complement factor H (CFH) or CFH autoantibodies. The observation of thrombocytopenia and platelet-rich thrombi in the glomerular microvasculature indicates ...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2009-03-205096
更新日期:2009-11-12 00:00:00
abstract::Despite increasing knowledge on the mechanisms of invariant natural killer T (iNKT)-cell development in the thymus, the function of recent thymic emigrant (RTE) iNKT cells remains largely unexplored, principally because of a lack of bona fide markers to distinguish RTE from long-lived iNKT cells. Whether the recently ...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2011-01-329268
更新日期:2011-09-15 00:00:00
abstract::Uniformly adopted response criteria are essential for assessment of therapies incorporating conventional chemotherapy and chemoimmunotherapy regimens. Recently, immunomodulatory agents, such as immune checkpoint inhibitors, have demonstrated impressive activity in a broad range of lymphoma histologies. However, these ...
journal_title:Blood
pub_type: 杂志文章,评审
doi:10.1182/blood-2016-05-718528
更新日期:2016-11-24 00:00:00
abstract::We studied eight patients with intermittent bleeding episodes usually following trauma and associated with the ingestion of medicine known to interfere with platelet function. All patients had a normal or minimally prolonged baseline bleeding time. All had a normal platelet count, glass bead retention test, and platel...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1978-07-01 00:00:00
abstract::Dendritic cells (DC) are the main stimulators of primary T-cell responses and, thus, probably play a role in the immune reactions after stem cell transplantation. Very little is known about DC in cord blood (CB) and about their potential involvement in the low incidence and severity of acute graft-versus-host disease ...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1999-04-01 00:00:00
abstract::Apoptotic cells were shown to induce dendritic cell immune tolerance. We applied a proteomic approach to identify molecules that are secreted from apoptotic monocytes, and thus may mediate engulfment and immune suppression. Supernatants of monocytes undergoing apoptosis were collected and compared using sodium dodecyl...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2006-03-013334
更新日期:2006-11-15 00:00:00
abstract::It is widely accepted that the serum of healthy individuals contains natural antibodies only against those blood group A or B antigens that are not expressed on the individual's red blood cells. The mechanisms involved in tolerance to autologous blood group antigens remain unclear. In the present study, we show that I...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1999-06-15 00:00:00
abstract::The association of fibrinogen with washed human platelets was examined by immunocytochemistry during aggregation induced by adenosine diphosphate (ADP) and during deaggregation. The platelets were suspended either in a medium containing 2 mmol/L Ca2+ or in a medium containing no added Ca2+ (20 mumol/L Ca2+). Platelets...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1988-04-01 00:00:00
abstract::The primate erythrocyte (E) complement receptor, CR1, is a transmembrane glycoprotein located in clusters on the surface of E. In vivo studies have demonstrated that during processing and clearance of complement-opsonized immune complexes, large amounts of immunoglobulin G (IgG) can be bound to primate E via CR1 with ...
journal_title:Blood
pub_type: 杂志文章
doi:
更新日期:1997-02-01 00:00:00
abstract::RANTES (CCL5) is a chemokine implicated in many human diseases. We previously showed that the transcription factor Kruppel-like factor 13 (KLF13) controls the late (3-5 days after activation) expression of RANTES in T lymphocytes and that KLF13 itself is translationally regulated through the 5'-untranslated region of ...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2012-03-415968
更新日期:2012-08-23 00:00:00
abstract::Protein C is activated by thrombin with a value of k(cat)/K(m) = 0.11mM(-1)s(-1) that increases 1700-fold in the presence of the cofactor thrombomodulin. The molecular origin of this effect triggering an important feedback loop in the coagulation cascade remains elusive. Acidic residues in the activation domain of pro...
journal_title:Blood
pub_type: 杂志文章
doi:10.1182/blood-2012-03-415323
更新日期:2012-07-19 00:00:00