اختلالات غیر بدخیم در هماتولوژی

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ee Toxic Granulation Toxic Granulation/Neutrophil 

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9 Pelger Huet Anomaly Pelger-Huet 

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آنومالى الرر-ريلى ا ات۱۰۱۵ اتوزومی مغلوب لا سا ال 

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Poe Chediak Higashi Anomaly ‏*اتوزومال متلوب‎ ۱ ‏مز‎ 

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Phagolysosome or extracellular space 

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Fig. 1: the vascular NADPH oxidase complex may contain Nox! or Nox4 as substitutes for the catalytic p91 phox subunit of the phagocytic oxidase. Superoxide (IO 2°) is produced intracellularly. The Nox subunit is bound to p22phox in the plasma membrane, and they stabilise each other. The cytosolic subunits shown may also be required for full and sustained activation of the complex in vascular cells, although there is evidence that alternative subunits may be involved for Nox! activation 

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Integrin activation ‘Migration through eee ‘Stable adhesion Rolling ‘endothelium Sialyl-Lewss K-modfied glycoprctein Integrin (low attinty state) لود معنا وه عدوا لومم 1 دوا 3 وه وی « و ‎chemokines‏ ‎Ss Macrophage‏ ‎‘with mierobos‏ Fionn ana toronesth, (euracotular mars) شرت ها مک کی 

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CalDAG GEFs 

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Inflammatory conditions Infections ‘Tuberculosis Bacterial endocarditis Fever of unknown origin SyPhili Other Systemic lupus erythematosus Rheumatoid arthritis ‘Temporal arteritis Polyarterii Ulcerative colitis Sarcoidos Myositis Malignant disorders Acute myeloid leukaemia Hodgkin disease Non-Hodgkin lympho: Histiocytoses Carcinomas Myelodysplas: syndrome Miscellaneous Cyclic neutropenia Chronic idiopathic neutrope Kostmann syndrome Post splenectomy 

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Tay-Sachs disease | Hexosaminidase A: Cer—Glc—Gal(NeuAc|—GalNAc ‘ Mental retardation, blindness, muscular weakness, ۱ Gyp Ganglioside Fabry disease‘ u-Galactosidase Cor—Glc—Gal--Cal Skin rash, kidney failure full symptoms only in i Globotriaosylceramide —_' males; X-linked recessive), Metachromatic ‏ام‎ ‘Cor Gal-050; Mental retardation and psychologic diturbances in 3Sulfo jelacosyleramide | adults; ‘demyelination, Mental retardation; myelin almost absent +n, erosion of long bones, infants Enlarged liver and sf mental retardation Niemann disease —choline Enlarged iverand spleen, menial retardation, fatalin in Sphingomyelin early life. Farber's disease: Ceramidase ‏وکام‎ Hoarseness, dermatitis, skeletal deformation, mental * Ceramide retardation; fatal in early life. "NeuAc, N-acetylneuraminic acid; Cer, ceramide; Glc, glucose: Gal, galactose, + site of deficient enzyme reaction, 

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