Down-regulation of α-globin synthesis causes α-thalassemia with underproduction of fetal (HbF, α 2 γ 2 ) and adult (HbA, α 2 β 2 ) hemoglobin. This article focuses on the…
The inherited disorders of hemoglobin, including the thalassemias, are by far the commonest monogenic diseases. Although several factors are responsible for their very high frequency, the major mechanism seems to…
The thalassemias are attributable to the defective production of the α- and β-globin polypeptides of hemoglobin. Significant discoveries have illuminated the pathophysiology and enhanced the prevention and treatment of the…
Fig. 5.1 Focal nodular hyperplasia. (a) Mass magin is clear and the fibrous scar can be seen in the center of the lesion; (b) The color of the mass is…
Ligand Functions Copies/platelet Integrins αIIbβ3 (GPIIb-IIIa) Fibrinogen Aggregation, adhesion ~80,000 α2β1 (GPIa-IIa) Collagen Adhesion 3000–5000 α5β1 (VLA-5) Fibronectin Adhesion 1000 α6β1 (VLA-6) Laminin Adhesion 1000 αvβ3 Vitronectin Adhesion 100…
Study No. of patients Age at ITP diagnosis (year) Duration before rituximab (month) Age at rituximab treatment (year) Times of injection Duration of observation (month) Rate of initial response (CR…
Decreased platelet production (hypoplastic thrombocytopenia) Aplastic anemia (AA) Amegakaryocytic thrombocytopenia Acute leukemia Chemotherapy-induced thrombocytopenia (CIT) Myelodysplastic syndrome (MDS) Malignancy with bone marrow infiltration or suppression (e.g., lymphoma, leukemia, solid tumors) Increased…
Fig. 1 Megakaryopoiesis and thrombopoiesis. Differentiation from hematopoietic stem cells (HSCs) to platelets proceeds by a number of intermediate stages. Megakaryopoiesis contains stages from HSC to mature MKs in the…
Platelet size Disease OMIM Inheritance Gene Features Small Wiskott-Aldrich syndrome 301000 X WAS Immunodeficiency X-linked thrombocytopenia 313900 X WAS No immunodeficiency FYB thrombocytopenia na AR FYB Activated platelets Normal ANKRD26…
