Abstract:
:Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and highly aggressive hematological malignancy derived from precursors of plasmacytoid dendritic cells. This disease typically presents with cutaneous involvement as the first manifestation, with subsequent or simultaneous spread to bone marrow and peripheral blood. It is exceedingly rare to diagnose BPDCN without a cutaneous lesion. Here, we report a 21-year-old male who was diagnosed with BPDCN in the absence of cutaneous symptoms. Clinically, left inguinal nodules were noticed for 4 months. The diagnosis of BPDCN was established based on histological and immunohistochemical study of a lymph node biopsy. The patient was classified as stage IVA with bone marrow involvement and underwent three cycles of hyper-CVAD alternating with high-dose methotrexate and cytarabine. A complete response was established after one cycle; however, the patient relapsed with disseminated cutaneous lesions and bone marrow involvement following a response duration of 10 months. This case is significant for BPDCN presenting with lymph node and bone marrow involvement in the absence of characteristic cutaneous manifestations.
journal_name
Acta Haematoljournal_title
Acta haematologicaauthors
Wang H,Cao J,Hong Xdoi
10.1159/000334703subject
Has Abstractpub_date
2012-01-01 00:00:00pages
124-7issue
2eissn
0001-5792issn
1421-9662pii
000334703journal_volume
127pub_type
杂志文章,评审abstract::This study was undertaken to define to which platelet components 75Se-methionine is bound after its injection to normal rats, and to study the curves of specific radioactivity of each labelled fraction. It has been shown that, for a part, platelet labelling is due to adsorption of plasma proteins (albumin and fibrinog...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207633
更新日期:1979-01-01 00:00:00
abstract::Twenty-four infants and children suffering from glucose-6-phosphate dehydrogenase (G6PD) deficiency during hemolytic crisis were included in this study. Their ages ranged between 3 and 36 months with a median of 10 months. 22 were males and 2 were females. Fourteen out of them received a single bolus dose of desferrio...
journal_title:Acta haematologica
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1159/000205320
更新日期:1989-01-01 00:00:00
abstract::The first thrombocytopenia cases related to the human immunodeficiency virus (HIV) were described even before its isolation in 1983. Subsequently, multiple mechanisms have been proposed to elucidate the etiology of thrombocytopenia. In addition to other types of cytopenia affecting patients with HIV, thrombocytopenia ...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000313782
更新日期:2010-01-01 00:00:00
abstract::The case history of a 71-year-old woman with three episodes of a microangiopathic hemolytic anemia over a 22-year span is detailed. During the last episode a possible response of her thrombotic thrombocytopenic purpura (TTP)-like syndrome to the administration of intravenous immunoglobulin is documented. In retrospect...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000205377
更新日期:1989-01-01 00:00:00
abstract::Patients with sickle cell disease have an increased tendency to develop frequent and severe pyogenic infections. Lymphocyte subpopulations were studied in 14 patients with homozygous sickle cell anaemia (SCA) using the OKT monoclonal antibody. The mean absolute lymphocyte counts observed in patients with SCA were simi...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206155
更新日期:1985-01-01 00:00:00
abstract::Significant DNA polymorphisms have been reported in the beta-globin gene cluster of epsilon-G gamma-A gamma-psi beta-delta-beta-gene region, in normal (Hb AA) individuals and in patients with sickle cell anaemia (SCA). Investigations of the extent of the DNA polymorphisms in the beta A- and beta S-globin gene cluster ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204653
更新日期:1992-01-01 00:00:00
abstract:OBJECTIVE:To review the diagnostic significance, safety and possible risk factors of splenectomy in fever of unknown origin (FUO) with splenomegaly. METHODS:The records of 54 patients with FUO and splenomegaly who underwent splenectomy in our hospital in the past 20 years were reviewed retrospectively. Pathologic find...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000118632
更新日期:2008-01-01 00:00:00
abstract::Cardiac scintigraphy has been performed in 60 beta-thalassaemia major patients aged 8-35 years who received regular blood transfusions and subcutaneous desferrioxamine (DFX) chelation. Fifty-seven showed no clinical, radiological or electrocardiographic evidence of heart disease and 3 had clinically apparent cardiac f...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205046
更新日期:1990-01-01 00:00:00
abstract::A modified cytochemical method was developed for demonstrating magnesium-activated adenosine triphosphatase in haemic cells. The enzyme was shown in a variety of normal and leukaemic, unfixed cells. The high sensitivity of this enzyme to all fixatives tried in this study hampered further exploration and characterizati...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205983
更新日期:1987-01-01 00:00:00
abstract::The plasma cortisol levels of 108 children aged 1-16 years with various Hb genotypes (AA, AS and SS) were measured. The mean plasma cortisol levels of both the AS and SS groups (during steady state) fell within the normal range but the mean of the SS group is significantly lower than the AA group. In fact, 9 of the si...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205689
更新日期:1988-01-01 00:00:00
abstract::In evaluating hypoimmunoglobulinemia in a patient with nonsecretory myeloma, studies of in vitro immunoglobulin synthesis were performed during a 3-year period. Impaired polyclonal response to mitogen stimulation associated with excessive suppressor activity was demonstrable at diagnosis. Despite successful chemothera...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205622
更新日期:1988-01-01 00:00:00
abstract::A rare case of acute promyelcytic leukemia (APL) is reported in a 7-year-old boy. The patient displayed the typical features of APL including impaction of the marrow with promyelocytes, marked elevation of the serum vitamin B12 and transcobalamin I levels and a hemorrhagic diathesis. The bleeding diathesis in the case...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207589
更新日期:1979-01-01 00:00:00
abstract::The effect of total-body cold exposure on plasma concentrations of von Willebrand factor (vWF), endothelin-1 (ET) and thrombomodulin (TM), all of which are considered to be generated from the endothelium, was studied in systemic lupus erythematosus (SLE) patients with and without Raynaud's phenomenon. The plasma level...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204684
更新日期:1992-01-01 00:00:00
abstract::The expression of the enzyme marker terminal deoxynucleotidyl transferase (TdT) was examined by immunofluorescence assay in the cells from 333 cases with various types and subtypes of leukemia or lymphoma. More than 90% of cALL and T-ALL, 70% of Null-ALL and 80% of pre-B-ALL were TdT-positive. One case in the commonly...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206072
更新日期:1986-01-01 00:00:00
abstract::A new case of IgE myeloma is described. A 77-year-old woman presented with bone pain and fatigue. Serum protein analysis revealed a paraprotein of the IgE kappa type; bone marrow aspirate and immunofluorescence confirmed the diagnosis; ultrastructural examination showed immature plasma cells. Treatment with prednisone...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204851
更新日期:1991-01-01 00:00:00
abstract::A 27-year-old male developed massive generalized lymphadenopathy with chronic myelomonocytic leukemia (CMML) presenting as extramedullary blast crisis mimicking a lymphocytic lymphoma. On presentation, a consistent chromosomal abnormality involving chromosomes 8 and 13, i.e. 46,XY,t(8;13) (q11;p11), was present in lym...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204687
更新日期:1992-01-01 00:00:00
abstract::The frequency of chronic lymphocytic leukemia (CLL) varies a great deal from one population to another. We have undertaken to compare the aspects of CLL in Maghreb and Europe through two series of comparable importance, studied during the same period and under similar conditions in Algiers and Amiens. This comparison ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206896
更新日期:1983-01-01 00:00:00
abstract::A patient developed a drug rash and neutropenia while receiving tobramycin, ticarcillin and flucloxacillin intravenously for osteomyelitis. Incorporation of these antibiotics into in vitro cultures of bone marrow granulocyte macrophage precursors (CFU-C) showed no inhibition of the patient's marrow or normal marrow by...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206794
更新日期:1983-01-01 00:00:00
abstract::The objective of this study was to assess the pharmacokinetics of rhG-CSF after a single intraperitoneal injection 2 h post-TBI in B6D2F1 lethally irradiated mice and to analyze the effect of rhG-CSF on the endogenous response of interleukin-3 (IL-3), interleukin-6 (IL-6) and granulocyte-macrophage colony-stimulating ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000203604
更新日期:1997-01-01 00:00:00
abstract::Agranulocytosis is a rare side effect of phenytoin treatment. We describe the case of an elderly man who developed agranulocytosis 2 weeks following initiation of phenytoin treatment, on no cytotoxic drugs or any other medications except decadron. The white blood cell count was 300/mm3 with absent granulocytes. The li...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204838
更新日期:1991-01-01 00:00:00
abstract::It is unusual to find microorganisms in peripheral blood smears, and their presence is frequently associated with overwhelming sepsis and consequently a poor prognosis. In this report, we demonstrate 4 cases with bacteria in blood smears. Two of them had a fatal outcome, but the other 2 were caused by a contamination ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000058318
更新日期:2002-01-01 00:00:00
abstract::A case of systemic mastocytosis with unusual clinical manifestations, appearing as an isolated splenohepatomegaly, is described. The proliferative character is evident from the cytological characteristics of immaturity and the presence of a moderate percentage of mast cells in the peripheral blood. These cells make up...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207846
更新日期:1977-01-01 00:00:00
abstract::The plasminogen activator content of a vein wall has been measured by estimating the radio-activity released from a 125I-tagged fibrin clot by incubating that clot with a known weight of the vein wall. This method could be used in the investigation of the fibrinolytic system of the body in its normal distribution and ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207720
更新日期:1978-01-01 00:00:00
abstract::Inferferon alfa-2b (IFN) plays a major role in the current management of previously untreated patients with chronic myelogenous leukemia (CML) as well as patients with CML who have relapsed after bone marrow transplantation. Hydroxyurea (HU) is the best conventional drug for treatment of CML in the chronic phase. Ten ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000203859
更新日期:1996-01-01 00:00:00
abstract::Thymopoietin (TP) was originally isolated as a 5-kD 49-aa protein from bovine thymus and was subsequently observed to affect T-cell differentiation and function. We report here the molecular cloning of a murine TP cDNA. The 2,514 bp fragment contains a 630 bp open reading frame that encodes for 210 aa, highly homologo...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000203673
更新日期:1997-01-01 00:00:00
abstract::Mesenteric panniculitis is a rare idiopathic inflammatory disorder that can lead to sclerosis. We describe a patient with mesenteric panniculitis presenting with abdominal symptoms and autoimmune haemolytic anaemia. The symptoms remitted after splenectomy and gradual steroid taper. This may suggest an autoimmune compo...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000046627
更新日期:2002-01-01 00:00:00
abstract::Janus kinases are critical components of signaling pathways that regulate hematopoiesis. Mutations of the non-receptor tyrosine kinase JAK2 are found in many BCR-ABL-negative myeloproliferative neoplasms. Preclinical results support that JAK2 inhibitors could show efficacy in treating chronic myeloproliferative neopla...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000356784
更新日期:2014-01-01 00:00:00
abstract::In pregnant mice. 55Fe-labeled iron-dextran (Imferon) is transferred across the placenta. It was detected in the bone marrow, liver, spleen and peripheral blood of the pregnant animal, as well as in the embryonic liver erythroid precursors and peripheral blood. Uptake by liver and peripheral blood cells of pregnant an...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000208195
更新日期:1975-01-01 00:00:00
abstract::A 28-year-old woman with Bence-Jones multiple myeloma (MM) presented with several osteolytic lesions and a massive bone marrow infiltration with mature plasmocytes. After 6 cycles of chemotherapy with melphalan and prednisone, the patient, in apparent clinical remission, underwent allogeneic bone marrow transplantatio...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205969
更新日期:1987-01-01 00:00:00
abstract::The cutaneous inflammatory response in patients with Hodgkin's and non-Hodgkin's lymphomas was investigated by light and scanning electron microscopy and compared with that of healthy subjects at 24 and 48 h. Both groups of patients showed a reduction in overall cellularity and statistically significant differences in...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207253
更新日期:1980-01-01 00:00:00