Kids, colds, and complement: paroxysmal cold hemoglobinuria
نویسندگان
چکیده
منابع مشابه
Complement C3dg-mediated erythrophagocytosis: implications for paroxysmal nocturnal hemoglobinuria.
The clinical management of paroxysmal nocturnal hemoglobinuria (PNH), a rare but life-threatening hematologic disease, has fundamentally improved with the introduction of a therapeutic that prevents complement-mediated intravascular hemolysis. However, a considerable fraction of PNH patients show insufficient treatment response and remain transfusion dependent. Because the current treatment onl...
متن کاملCharacterization of the complement sensitivity of paroxysmal nocturnal hemoglobinuria erythrocytes.
The affected erythrocytes of paroxysmal nocturnal hemoglobinuria (PNH II and PNH III cells) are abnormally sensitive to complement-mediated lysis. Normal human erythrocytes chemically modified by treatment with 2-amino-ethylisothiouronium bromide (AET) have been used as models for PNH cells inasmuch as they also exhibit an enhanced susceptibility to complement. To investigate the bases for the ...
متن کاملComplement-mediated granulocyte dysfunction in paroxysmal nocturnal hemoglobinuria.
In paroxysmal nocturnal hemoglobinuria (PNH), infection, both viral and bacterial, disproportionate to the mild neutropenia seen in many such patients is responsible for significant morbidity. We report impaired granulocyte chemotaxis efficiency which may contribute to the problems induced by bacterial infections. PNH (but not normal) granulocytes, after exposure to very small concentrations of...
متن کاملCase report: paroxysmal cold hemoglobinuria presenting during pregnancy
BACKGROUND Paroxysmal cold hemoglobinuria is caused by a biphasic IgG autoantibody that triggers complement-mediated intravascular hemolysis. Paroxysmal cold hemoglobinuria has not previously been reported to occur in association with pregnancy. CASE PRESENTATION We report a case of an 18 year old female who presented in early pregnancy with acute hemolytic anemia and a positive Donath-Landst...
متن کاملComplement sensitivity of paroxysmal nocturnal hemoglobinuria bone marrow cells.
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired disorder in which erythrocytes, granulocytes, and platelets are defective, as shown by increased susceptibility of RBCs, WBCs, and platelets to complement-mediated lysis in vitro. The purpose of this study is to determine the sensitivity to complement lysis of PNH and non-PNH erythroid and myeloid precursors using the release of 59Fe and ...
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ژورنال
عنوان ژورنال: Transfusion
سال: 2017
ISSN: 0041-1132
DOI: 10.1111/trf.14128