Human gd T lymphocytes represent a minor subset of T cells in the peripheral blood, which exhibit a limited diversity and a tissue-restricted repertoire in contrast to their broad specificity. Most postthymic neoplasms that arise from this T-cell subpopulation belong to the hepatosplenic gd lymphoma entity. Only a few cases of nonhepatosplenic gd lymphomas have been described in detail previous...

Kojima et al recently reported 20 patients with Down’s syndrome and acute leukemia.’ Of these 20 patients, 14 who were 3 years old and less were diagnosed as having acute megakaryoblastic leukemia (AMKL). They were characterized by the presence of bone marrow fibrosis, a history of myelodysplastic syndrome (MDS), and a poor response to chemotherapy. Ten of the 14 patients were male, and platele...

Dear Editor The authors claim that they identified a patient with AML (sub-type; acute megakaryocytic leukemia, AMKL) who presented with a peripheral blast count of 47% but an elevated platelet count of 519 × 10 9 /L. The blasts were myeloperoxidase (MPO)+and contained Auer rods (not described in the literature). In Fig. 1 these cells displayed typical agranular blasts and a rare granular blast...

Human platelet glycoprotein Ib beta (GPIb beta) (M(r) 22,000) is part of the GPIb-V-IX system that constitutes the receptor for von Willebrand factor and mediates platelet adhesion in the arterial circulation. The four members of the receptor (GPs Ib alpha, Ib beta, V, and IX) share structural and functional features. Individually, GPIb beta contributes to surface expression of the receptor and...

cytoplasmic inclusions were first noted by May almost 100 years ago.7 FTNS is distinguished by the additional clinical features of high-tone sensorineural deafness, cataracts, and nephritis. APSM is similar to FTNS except that Döhle-like inclusions have not been described and it is distinguished from the classical X-linked form of Alport syndrome in that COL4A5 gene mutations are not present. F...

Platelets perform a central role in haemostasis and thrombosis. They adhere to subendothelial collagens exposed at sites of blood vessel injury via the glycoprotein (GP) Ib-V-IX receptor complex, GPVI and integrin alpha(2)beta(1). These receptors perform distinct functions in the regulation of cell signalling involving non-receptor tyrosine kinases (e.g. Src, Fyn, Lyn, Syk and Btk), adaptor pro...

Glycoprotein V (GPV), a subunit of the platelet GPIb-V-IX receptor for von Willebrand factor and thrombin, is specifically found in platelets and mature megakaryocytes. Studies of the GPV gene can therefore provide insight into the mechanisms governing megakaryocyte differentiation. The human GPV promoter was isolated, and elements important for its tissue specific transcriptional activity were...

Adhesion of platelets to extracellular matrix via von Willebrand factor (vWF) and activation of platelets by thrombin are critical steps in hemostasis. Glycoprotein (GP) V is a component of the GPIb-V-IX complex, the platelet receptor for vWF. GPV is also cleaved by thrombin. Deficiency of GPIb or GPIX results in Bernard-Soulier syndrome (BSS), a bleeding disorder in which platelets are giant a...

Glycoprotein V (GPV) is a subunit of the platelet GPIb-V-IX receptor for von Willebrand factor and thrombin. GPV is cleaved from the platelet surface during activation by thrombin, but its role in hemostasis is still unknown. It is reported that GPV knockout mice had a decreased tendency to form arterial occluding thrombi in an intravital thrombosis model and abnormal platelet interaction with ...

OBJECTIVE The platelet glycoprotein (GP)Ib-V-IX complex is a receptor required for normal hemostasis deficient in the Bernard-Soulier bleeding disorder. To evaluate the consequences of GPIb-V-IX deficiency in thrombosis we generated mouse models of the disease by targeting the GPIb beta subunit. METHODS AND RESULTS Complete deletion (GPIb beta-/-) or an intracellular truncation (GPIb beta del...