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Titolo:
Hyperfibrinolysis in a case of myelodysplastic syndrome with leukemic spread of mast cells
Autore:
Wimazal, F; Sperr, WR; Horny, HP; Carroll, V; Binder, BR; Fonatsch, C; Walchshofer, S; Fodinger, M; Schwarzinger, I; Samorapoompichit, P; Chott, A; Dvorak, AM; Lechner, K; Valent, P;
Indirizzi:
Univ Vienna, Dept Internal Med 1, Div Hematol, Vienna, Austria Univ Vienna Vienna Austria Internal Med 1, Div Hematol, Vienna, Austria Univ Tubingen, Dept Pathol, Tubingen, Germany Univ Tubingen Tubingen Germany Tubingen, Dept Pathol, Tubingen, Germany Univ Vienna, Dept Vasc Biol & Thrombosis Res, Vienna, Austria Univ ViennaVienna Austria Vasc Biol & Thrombosis Res, Vienna, Austria Univ Vienna, Inst Med Biol, Vienna, Austria Univ Vienna Vienna AustriaUniv Vienna, Inst Med Biol, Vienna, Austria Univ Vienna, Inst Lab Med, Vienna, Austria Univ Vienna Vienna AustriaUniv Vienna, Inst Lab Med, Vienna, Austria Univ Vienna, Inst Histol, Vienna, Austria Univ Vienna Vienna AustriaUniv Vienna, Inst Histol, Vienna, Austria Univ Vienna, Inst Clin Pathol, Vienna, Austria Univ Vienna Vienna Austria iv Vienna, Inst Clin Pathol, Vienna, Austria Beth Israel Deaconess Med Ctr, Dept Pathol, Boston, MA USA Beth Israel Deaconess Med Ctr Boston MA USA Dept Pathol, Boston, MA USA
Titolo Testata:
AMERICAN JOURNAL OF HEMATOLOGY
fascicolo: 1, volume: 61, anno: 1999,
pagine: 66 - 77
SICI:
0361-8609(199905)61:1<66:HIACOM>2.0.ZU;2-P
Fonte:
ISI
Lingua:
ENG
Soggetto:
BLOOD MONONUCLEAR-CELLS; PROTOONCOGENE C-KIT; BONE-MARROW; IMMUNOPHENOTYPIC ANALYSIS; SIDEROBLASTIC ANEMIA; TISSUE-SECTIONS; HUMAN BASOPHILS; MASTOCYTOSIS; TRYPTASE; DISEASE;
Keywords:
mast cells; myelodysplastic syndromes; mastocytosis; fibrinolysis; tryptase;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
54
Recensione:
Indirizzi per estratti:
Indirizzo: Valent, P Dept090ternal Med 1, Div Hematol & Hemostaseol, Wahringer Gurtel18-20, A-1 Dept Internal Med 1 Wahringer Gurtel 18-20 Vienna Austria A-1090
Citazione:
F. Wimazal et al., "Hyperfibrinolysis in a case of myelodysplastic syndrome with leukemic spread of mast cells", AM J HEMAT, 61(1), 1999, pp. 66-77

Abstract

Mast cells (MC) are multipotent hemopoietic effector cells producing diverse mediators like histamine, heparin, or tissue type plasminogen activator. We report a 75-year-old male patient with myelodysplastic syndrome (MDS) of recent onset (3 months' history) associated with a massive leukemic spread of immature tryptase(+) MC (tentative term: myelomastocytic leukemia). The patient presented with pancytopenia, bleeding, hypofibrinogenemia, and anincreased cellular tryptase level. Moreover, an excessive elevation of plasmin-antiplasmin complexes (9,200 ng/ml; normal range: 10-150), an elevatedD-dimer, and an increase in thrombin-antithrombin III complexes were found. The identity of the circulating MC was confirmed by immunophenotyping (CD117/c-kit(+), CD123/IL-3R alpha(-), CD11b/C3biR(-)), biochemical analysis (cellular ratio I:ng:ngl of tryptase to histamine >1), and electron microscopy. Bone marrow (bm) examination showed trilineage dysplasia (17% blasts), 30% diffusely scattered MC, and a complex karyotype. No dense, compact MC infiltrates (mastocytosis) were detectable in bm sections. Despite hyperfibrinolysis and mediator syndrome (flushing, headache), the patient received remission induction polychemotherapy (DAV) followed by two cycles of consolidation with intermediate dose ARA-C (2 x 1 g/m(2)/day on days 1, 3, and 5). He entered complete remission after the first chemotherapy cycle without evidence of recurring MDS. Moreover, in response to chemotherapy, the hyperfibrinolysis and mediator syndrome resolved, and the circulating c-kit(+) MCdisappeared. We suggest consideration of polychemotherapy as a therapeuticoption in patients with high-risk MDS of recent onset, even in the case ofMC lineage involvement. (C) 1999 Wiley-Liss, Inc.

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Documento generato il 21/09/20 alle ore 09:41:25