scholarly journals Clonal studies of CD3- lymphoproliferative disease of granular lymphocytes

Blood ◽  
1993 ◽  
Vol 81 (9) ◽  
pp. 2363-2368 ◽  
Author(s):  
R Nash ◽  
P McSweeney ◽  
R Zambello ◽  
G Semenzato ◽  
TP Jr Loughran
Keyword(s):  
Coagulation Factor ◽  
Normal Activity ◽  
Lymphoproliferative Disease ◽  
Clear Correlation ◽  
Unique Role ◽  
Delayed Bleeding ◽  
History Of ◽  
Granular Lymphocytes ◽  
Pai 1

A 63-year-old man was evaluated for a lifelong history of bleeding commencing with frequent epistaxis as a child; all previous routine coagulation parameters were within the normal range. The patient's hemorrhagic disorder is characterized predominantly by delayed bleeding at surgical sites. In the resting state, there was no clinical or laboratory evidence of excessive fibrin(ogen)olysis. Bleeding was not caused by disseminated intravascular coagulation, factor XIII deficiency, alpha 2-antiplasmin deficiency, or dysfibrinogenemia. It was found that the patient was deficient in plasma PAI-1 antigen and activity but with approximately half normal antigen and normal activity of platelet PAI-1. The low concentration of plasma PAI-1 was insufficient to neutralize circulating t-PA, resulting in high t-PA activity with normal antigen and causing the hyperfibrinolytic activity observed. Studies on seven family members of the proband indicated autosomal inheritance of plasma PAI-1 deficiency. Studies on this patient emphasize a clear correlation between decreased plasma PAI-1 activity and hyperfibrinolytic bleeding and also emphasize the unique role of plasma PAI-1 in the balance between the coagulation and fibrinolytic mechanisms

scholarly journals Clonal studies of CD3- lymphoproliferative disease of granular lymphocytes

Blood ◽  
1993 ◽  
Vol 81 (9) ◽  
pp. 2363-2368 ◽  
Author(s):  
R Nash ◽  
P McSweeney ◽  
R Zambello ◽  
G Semenzato ◽  
TP Jr Loughran
Keyword(s):  
Coagulation Factor ◽  
Normal Activity ◽  
Lymphoproliferative Disease ◽  
Clear Correlation ◽  
Unique Role ◽  
Delayed Bleeding ◽  
History Of ◽  
Granular Lymphocytes ◽  
Pai 1

Abstract A 63-year-old man was evaluated for a lifelong history of bleeding commencing with frequent epistaxis as a child; all previous routine coagulation parameters were within the normal range. The patient's hemorrhagic disorder is characterized predominantly by delayed bleeding at surgical sites. In the resting state, there was no clinical or laboratory evidence of excessive fibrin(ogen)olysis. Bleeding was not caused by disseminated intravascular coagulation, factor XIII deficiency, alpha 2-antiplasmin deficiency, or dysfibrinogenemia. It was found that the patient was deficient in plasma PAI-1 antigen and activity but with approximately half normal antigen and normal activity of platelet PAI-1. The low concentration of plasma PAI-1 was insufficient to neutralize circulating t-PA, resulting in high t-PA activity with normal antigen and causing the hyperfibrinolytic activity observed. Studies on seven family members of the proband indicated autosomal inheritance of plasma PAI-1 deficiency. Studies on this patient emphasize a clear correlation between decreased plasma PAI-1 activity and hyperfibrinolytic bleeding and also emphasize the unique role of plasma PAI-1 in the balance between the coagulation and fibrinolytic mechanisms

scholarly journals Deficiency of plasma plasminogen activator inhibitor 1 results in hyperfibrinolytic bleeding

Blood ◽  
1993 ◽  
Vol 81 (9) ◽  
pp. 2357-2362 ◽  
Author(s):  
MH Lee ◽  
E Vosburgh ◽  
K Anderson ◽  
J McDonagh
Keyword(s):  
Coagulation Factor ◽  
Plasminogen Activator Inhibitor ◽  
Normal Activity ◽  
Clear Correlation ◽  
Plasminogen Activator Inhibitor 1 ◽  
Unique Role ◽  
Delayed Bleeding ◽  
History Of ◽  
Pai 1

A 63-year-old man was evaluated for a lifelong history of bleeding commencing with frequent epistaxis as a child; all previous routine coagulation parameters were within the normal range. The patient's hemorrhagic disorder is characterized predominantly by delayed bleeding at surgical sites. In the resting state, there was no clinical or laboratory evidence of excessive fibrin(ogen)olysis. Bleeding was not caused by disseminated intravascular coagulation, factor XIII deficiency, alpha 2-antiplasmin deficiency, or dysfibrinogenemia. It was found that the patient was deficient in plasma PAI-1 antigen and activity but with approximately half normal antigen and normal activity of platelet PAI-1. The low concentration of plasma PAI-1 was insufficient to neutralize circulating t-PA, resulting in high t-PA activity with normal antigen and causing the hyperfibrinolytic activity observed. Studies on seven family members of the proband indicated autosomal inheritance of plasma PAI-1 deficiency. Studies on this patient emphasize a clear correlation between decreased plasma PAI-1 activity and hyperfibrinolytic bleeding and also emphasize the unique role of plasma PAI-1 in the balance between the coagulation and fibrinolytic mechanisms.

scholarly journals Deficiency of plasma plasminogen activator inhibitor 1 results in hyperfibrinolytic bleeding

Blood ◽  
1993 ◽  
Vol 81 (9) ◽  
pp. 2357-2362 ◽  
Author(s):  
MH Lee ◽  
E Vosburgh ◽  
K Anderson ◽  
J McDonagh
Keyword(s):  
Coagulation Factor ◽  
Plasminogen Activator Inhibitor ◽  
Normal Activity ◽  
Clear Correlation ◽  
Plasminogen Activator Inhibitor 1 ◽  
Unique Role ◽  
Delayed Bleeding ◽  
History Of ◽  
Pai 1

Abstract A 63-year-old man was evaluated for a lifelong history of bleeding commencing with frequent epistaxis as a child; all previous routine coagulation parameters were within the normal range. The patient's hemorrhagic disorder is characterized predominantly by delayed bleeding at surgical sites. In the resting state, there was no clinical or laboratory evidence of excessive fibrin(ogen)olysis. Bleeding was not caused by disseminated intravascular coagulation, factor XIII deficiency, alpha 2-antiplasmin deficiency, or dysfibrinogenemia. It was found that the patient was deficient in plasma PAI-1 antigen and activity but with approximately half normal antigen and normal activity of platelet PAI-1. The low concentration of plasma PAI-1 was insufficient to neutralize circulating t-PA, resulting in high t-PA activity with normal antigen and causing the hyperfibrinolytic activity observed. Studies on seven family members of the proband indicated autosomal inheritance of plasma PAI-1 deficiency. Studies on this patient emphasize a clear correlation between decreased plasma PAI-1 activity and hyperfibrinolytic bleeding and also emphasize the unique role of plasma PAI-1 in the balance between the coagulation and fibrinolytic mechanisms.

Spontaneous chronic subdural hematomas in young adults with a deficiency of coagulation factor XIII

2005 ◽  
Vol 102 (6) ◽  
pp. 1130-1132 ◽  
Author(s):  
Alessio Albanese ◽  
Antonio Tuttolomondo ◽  
Carmelo Anile ◽  
Giovanni Sabatino ◽  
Angelo Pompucci ◽  
...  
Keyword(s):  
Young Adults ◽  
Factor Xiii ◽  
Coagulation Factor ◽  
Content Type ◽  
Coagulation Factor Xiii ◽  
Subdural Hematomas ◽  
History Of ◽  
Bridging Veins ◽  
Fine Print

✓ Chronic subdural hematomas (SDHs) generally occur in elderly patients. Its pathogenesis is usually related to head trauma with tearing and rupture of the bridging veins, although in some cases a history of trauma is not recognizable. There are many reports regarding the association between spontaneous chronic SDHs and an alteration in coagulative parameters. A coagulative disorder should be suspected when an unexplained hemorrhage occurs, especially in a young patient. The authors report on three young men with a deficiency in coagulation factor XIII (FXIII) who underwent surgery for chronic SDHs. The role of FXIII in the pathogenesis of chronic SDH is emphasized. In patients with unexplained chronic SDH all coagulation parameters and factors should be screened to identify an eventual coagulative disorder.

scholarly journals The Role of TPA I/D and PAI-1 4G/5G Polymorphisms in Multiple Sclerosis

2014 ◽  
Vol 2014 ◽  
pp. 1-8 ◽  
Author(s):  
Maja Živković ◽  
Nada Starčević Čizmarević ◽  
Luca Lovrečić ◽  
Inge Klupka-Sarić ◽  
Aleksandra Stanković ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Proteolytic Enzymes ◽  
Harmful Effect ◽  
Geographic Location ◽  
Tissue Type ◽  
Type Plasminogen Activator ◽  
Pcr Rflp ◽  
History Of ◽  
Pai 1

Background. Previous studies have shown impaired fibrinolysis in multiple sclerosis (MS) and implicated extracellular proteolytic enzymes as important factors in demyelinating neuroinflammatory disorders. Tissue-type plasminogen activator (t-PA) and its inhibitor (PAI-1) are key molecules in both fibrinolysis and extracellular proteolysis. In the present study, an association of the TPA Alu I/D and PAI-1 4G/5G polymorphisms with MS was analyzed within the Genomic Network for Multiple Sclerosis (GENoMS).Methods. The GENoMS includes four populations (Croatian, Slovenian, Serbian, and Bosnian and Herzegovinian) sharing the same geographic location and a similar ethnic background. A total of 885 patients and 656 ethnically matched healthy blood donors with no history of MS in their families were genotyped using PCR-RFLP.Results. TPA DD homozygosity was protective (OR = 0.79, 95% CI 0.63–0.99,P=0.037) and PAI 5G5G was a risk factor for MS (OR = 1.30, 95% CI 1.01–1.66,P=0.038). A significant effect of the genotype/carrier combination was detected in 5G5G/I carriers (OR = 1.39 95% CI 1.06–1.82,P=0.017).Conclusions. We found a significantly harmful effect of the combination of the PAI-1 5G/5G genotype and TPA I allele on MS susceptibility, which indicates the importance of gene-gene interactions in complex diseases such as MS.

scholarly journals Cell membrane expression and functional role of the p75 subunit of interleukin-2 receptor in lymphoproliferative disease of granular lymphocytes

Blood ◽  
1990 ◽  
Vol 76 (10) ◽  
pp. 2080-2085 ◽  
Author(s):  
R Zambello ◽  
L Trentin ◽  
G Pizzolo ◽  
P Bulian ◽  
M Masciarelli ◽  
...  
Keyword(s):  
T Cell ◽  
Functional Role ◽  
Interleukin 2 ◽  
Cell Receptor ◽  
Lymphoproliferative Disease ◽  
Gamma Delta ◽  
Membrane Expression ◽  
Alpha Beta ◽  
Granular Lymphocytes

Abstract The cell membrane expression and functional role of the interleukin-2 receptor (IL-2R) was analyzed in nine patients with lymphoproliferative disease of granular lymphocytes (LDGL) using monoclonal antibodies (MoAbs) specific for the p75 (TU27) and the p55 (anti-Tac) subunits of IL-2R. Four patients were characterized by the proliferation of CD3+CD8+ granular lymphocytes (GL) expressing the alpha/beta T-cell receptor (T alpha beta) and one case by the proliferation of CD3+CD4- CD8- GL expressing the gamma/delta T-cell receptor (T gamma delta); in four additional cases proliferating cells were CD3 negative GL. Consistent with data observed on normal GL, phenotypic analysis demonstrated that patients' GL lack the expression of the p55 IL-2R, whereas the p75 subunit is constitutionally expressed by expanding GL of both T-cell (either T alpha beta and T gamma delta) and natural killer (NK) origin in variable proportions (11% to 94% of cells). The analysis of the cytotoxic and proliferative activity demonstrated that the anti-p55 MoAb failed to inhibit IL-2-mediated activation, whereas a marked inhibition of both cytotoxicity and proliferation were obtained using the anti-p75 chain specific MoAb. These data indicate that the p75 chain of IL-2R is responsible for IL-2 signal transduction in both CD3+ and CD3- LDGL patients' GL.

Analysis of NK cell/DC interaction in NK-type lymphoproliferative disease of granular lymphocytes (LDGL): role of DNAM-1 and NKp30

2009 ◽  
Vol 37 (10) ◽  
pp. 1167-1175 ◽  
Author(s):  
Mirna Balsamo ◽  
Renato Zambello ◽  
Antonella Teramo ◽  
Marco Pedrazzi ◽  
Bianca Sparatore ◽  
...  
Keyword(s):  
Nk Cell ◽  
Lymphoproliferative Disease ◽  
Granular Lymphocytes

Phenotypic and functional analyses of dendritic cells in patients with lymphoproliferative disease of granular lymphocytes (LDGL)

Blood ◽  
2005 ◽  
Vol 106 (12) ◽  
pp. 3926-3931 ◽  
Author(s):  
Renato Zambello ◽  
Tamara Berno ◽  
Giovanna Cannas ◽  
Ilenia Baesso ◽  
Gianni Binotto ◽  
...  
Keyword(s):  
Dendritic Cells ◽  
Specific Antigen ◽  
Cell Types ◽  
Lymphoproliferative Disease ◽  
Topographic Organization ◽  
Functional Analyses ◽  
Granular Lymphocytes

We investigated whether dendritic cells (DCs) play a role in favoring granular lymphocyte (GL) proliferation in patients with lymphoproliferative disease of granular lymphocytes (LDGL). The presence of in vivo circulating DCs was studied in 11 patients (5 CD3+ and 6 CD3- LDGL). Autologous immature (iDCs) and mature (mDCs) DCs generated in vitro were studied for stimulatory activity on cell proliferation of CD3+ and CD3- GLs. The topographic organization of GLs and DCs was also studied in bone marrow (BM) biopsies. Peripheral blood (PB) CD3- GLs from patients showed significant proliferative activity in the presence of iDCs and mDCs. Conversely, monoclonal CD3+ GLs were unresponsive to autologous and allogeneic PB DCs. Analysis of BM biopsies demonstrated a topographic distribution of DCs and GLs that indicates contact between the 2 cell types. On functional assays, DCs obtained from BM were more efficient than PB DCs in stimulating CD3- GLs, and surprisingly, a low but definite stimulatory effect was demonstrated also on CD3+ GLs. The putative contact between DCs and GLs in the BM and, more crucial, the proliferative response of discrete GL populations to DC stimulation suggest the presence of a specific antigen within BM DCs, providing evidence for a role of DCs in the pathogenesis of LDGL.

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