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'Hemoglobinuria Paroxysmal Cold' (283)



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Jan
2018

Autoimmune hemolytic anemia (AIHA) is a disease process that involves the destruction of red blood cells mediated by the humoral immune system. It can be characterized as a cold agglutinin syndrome, paroxysmal cold hemoglobinuria, and warm, mixed type, and drug-induced AIHA. Although a well-established relationship exists between the presence of AIHA and lymphoproliferative malignancy, AIHA rarely presents in association with solid malignancies.

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Jun
2017

Paroxysmal cold hemoglobinuria (PCH) is a form of autoimmune hemolytic anemia caused by the Donath-Landsteiner antibody (D-L antibody). In children, this is typically a transient immune-mediated hemolysis that follows a viral illness and does not recur. Recurrent acute or chronic PCH due to D-L antibody is very rare.

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Jul
2017

Complementopathies.

Blood Rev 2017 Jul 6;31(4):213-223. Epub 2017 Feb 6.
Andrea C Baines, Robert A Brodsky
The complement system is an essential part of the innate immune system that requires careful regulation to ensure responses are appropriately directed against harmful pathogens, while preventing collateral damage to normal host cells and tissues. While deficiency in some components of the complement pathway is associated with increased susceptibility to certain infections, it has also become clear that inappropriate activation of complement is an important contributor to human disease. A number of hematologic disorders are driven by complement, and these disorders may be termed "complementopathies".

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Jan
2017

Autoimmune hemolytic anemia (AIHA) in childhood, including paroxysmal cold hemoglobinuria, is an uncommon, potentially life-threatening disorder. AIHA is a recognized complication of several varieties of lymphoproliferative disorders, including high-grade B-cell lymphoma, but it has not been associated with Burkitt lymphoma in people without an underlying immunodeficiency. When AIHA occurs in association with lymphoproliferative disorders, it may precede or accompany the diagnosis of malignant disease or herald relapse.

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Jan
2017

Paroxysmal cold hemoglobinuria (PCH) is a rare form of autoimmune hemolytic anemia caused by a biphasic (Donath-Landsteiner [DL]) immunoglobulin G autoantibody. Estimates of disease frequency after syphilis are lacking and the diagnostic yield of testing for PCH is uncertain. The objectives of this study were: 1) to describe DL testing practices in Canada, 2) to determine how often a biphasic RBC antibody is detected in adults and children, and 3) to evaluate inter-rater reliability of interpretations of positive DL test results in adults.

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Sep
2017

We review the most characteristic clinical and histopathologic findings of the cutaneous manifestations of the occlusive nonvasculitic vasculopathic disorders. Clinically, most of these conditions are characterized by retiform purpura. Histopathologic findings consist of occlusion of the vessel lumina with no vasculitis.

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Apr
2016

The objective of this study was to describe complement activation in hemostatic and pathologic states of coagulation and in the acquired and congenital hemolytic anemias.
We review published and emerging data on the involvement of the classic, alternative and lectin-based complement pathways in coagulation and the hemolytic anemias. The alternative pathway in particular is always "on," at low levels, and is particularly sensitive to hyper-activation in a variety of physiologic and pathologic states including infection, autoimmune disorders, thrombosis and pregnancy, requiring tight control predicated on a variety of soluble and membrane bound regulatory proteins.

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Sep
2015

Role of Complement in Autoimmune Hemolytic Anemia.

Transfus Med Hemother 2015 Sep 7;42(5):303-10. Epub 2015 Sep 7.
Sigbjørn Berentsen
The classification of autoimmune hemolytic anemias and the complement system are reviewed. In autoimmune hemolytic anemia of the warm antibody type, complement-mediated cell lysis is clinically relevant in a proportion of the patients but is hardly essential for hemolysis in most patients. Cold antibody-mediated autoimmune hemolytic anemias (primary cold agglutinin disease, secondary cold agglutinin syndrome and paroxysmal cold hemoglobinuria) are entirely complement-mediated disorders.

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Dec
1969

Complement in hemolytic anemia.

Hematology Am Soc Hematol Educ Program 2015 ;2015:385-91
Robert A Brodsky
Complement is increasingly being recognized as an important driver of human disease, including many hemolytic anemias. Paroxysmal nocturnal hemoglobinuria (PNH) cells are susceptible to hemolysis because of a loss of the complement regulatory proteins CD59 and CD55. Patients with atypical hemolytic uremic syndrome (aHUS) develop a thrombotic microangiopathy (TMA) that in most cases is attributable to mutations that lead to activation of the alternative pathway of complement.

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Nov
2015

Complement in hemolytic anemia.

Blood 2015 Nov 18;126(22):2459-65. Epub 2015 Nov 18.
Robert A Brodsky
Complement is increasingly being recognized as an important driver of human disease, including many hemolytic anemias. Paroxysmal nocturnal hemoglobinuria (PNH) cells are susceptible to hemolysis because of a loss of the complement regulatory proteins CD59 and CD55. Patients with atypical hemolytic uremic syndrome (aHUS) develop a thrombotic microangiopathy (TMA) that in most cases is attributable to mutations that lead to activation of the alternative pathway of complement.

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Nov
2015

[Autoimmune hemolytic anemia in children].

Transfus Clin Biol 2015 Oct-Dec;22(5-6):291-8. Epub 2015 Nov 10.
M Becheur, B Bouslama, H Slama, N E H Toumi
Autoimmune hemolytic anemia is a rare condition in children which differs from the adult form. It is defined by immune-mediated destruction of red blood cells caused by autoantibodies. Characteristics of the autoantibodies are responsible for the various clinical entities.

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Aug
2015

[Hemolytic anemias and vitamin B12 deficieny].

Dtsch Med Wochenschr 2015 Aug 25;140(17):1302-10; quiz 1311-2. Epub 2015 Aug 25.
Hermann Dietzfelbinger, Max Hubmann
Hemolytic anemias consist of corpuscular, immun-hemolytic and toxic hemolytic anemias. Within the group of corpuscular hemolytic anemias, except for the paroxysmal nocturnal hemoglobinuria (PNH), all symptoms are caused by underlying heredetiary disorders within the red blood cell membran (hereditary spherocytosis), deficiencies of red cell enzymes (G6PDH- and pyrovatkinase deficiency) or disorders in the hemoglobin molecule (thalassaemia and sickle cell disease). Immune-hemolytic anemias are acquired hemolytic anemias and hemolysis is caused by auto- or allo-antibodies which are directed against red blood cell antigens.

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Nov
2015

Paroxysmal cold hemoglobinuria (PCH) is an autoimmune hemolytic anemia (AIHA) characterized by the presence of a Donath-Landsteiner (D-L) antibody. PCH occurs most commonly in young children and is associated with acute, often self-limited hemolytic anemia. The D-L antibody is classically a biphasic IgG anti-P autoantibody identified by the D-L test.

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Jun
2015

Paroxysmal cold hemoglobinuria.

Hematol Oncol Clin North Am 2015 Jun 7;29(3):473-8. Epub 2015 Mar 7.
Satish Shanbhag, Jerry Spivak
Paroxysmal cold hemoglobinuria is a rare cause of autoimmune hemolytic anemia predominantly seen as an acute form in young children after viral illnesses and in a chronic form in some hematological malignancies and tertiary syphilis. It is a complement mediated intravascular hemolytic anemia associated with a biphasic antibody against the P antigen on red cells. The antibody attaches to red cells at colder temperatures and causes red cell lysis when blood recirculates to warmer parts of the body.

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Dec
1969

Autoimmune hemolytic anemia (AIHA) is a collective term for several diseases characterized by autoantibody-initiated destruction of red blood cells (RBCs). Exact subclassification is essential. We provide a review of the respective types of AIHA with emphasis on mechanisms of RBC destruction, focusing in particular on complement involvement.

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Dec
1969

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.
We report a case of an 18 year old female who presented in early pregnancy with acute hemolytic anemia and a positive Donath-Landsteiner antibody test.

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Dec
1969

PCH is a rare autoimmune hemolytic anemia (AIHA) but is one of the most common causes of AIAH in children. For the diagnosis, it is important to perform the appropriate methods of serological investigation and show the typical biphasic reaction. This is a case report of a child who presented with features of haemolysis and was diagnosed with PCH of this way.

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Oct
2014

Autoimmune hemolytic anemia (AIHA) is a relatively uncommon disorder caused by autoantibodies directed against self red blood cells. It can be idiopathic or secondary, and classified as warm, cold (cold hemagglutinin disease (CAD) and paroxysmal cold hemoglobinuria) or mixed, according to the thermal range of the autoantibody. AIHA may develop gradually, or have a fulminant onset with life-threatening anemia.

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Dec
1969

Paroxysmal cold hemoglobinuria (PCH) is an acquired hemolytic anemia caused by immunoglobulin G (IgG) antibodies that sensitize red blood cells (RBCs) at cold temperatures by fixing complement to the RBCs causing intravascular hemolysis on rewarming. PCH usually appears in young children as recurrent high fevers, chills, and passage of red-brown urine. The diagnostic test for PCH is the Donath-Landsteiner test, an in vitro assay for biphasic hemolysis.

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Jun
2014

A 28-year-old man presented with a history of intermittent haematuria over the past 10 years usually following fever episodes and requiring blood transfusions during the episodes. History of any thrombotic complications, chest pain or erectile dysfunction was not forthcoming. Examination revealed severe pallor with mild icterus and mild splenomegaly.

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Jul
2014

Autoimmune haemolytic anaemias (AIHAs) are extracorpuscular haemolytic anaemias produced by antierythrocyte autoantibodies which cause a shortened red blood cell life span. There are several reasons why the diagnosis and treatment of AIHAs in children represent a bigger challenge than in adult patients, including the presence of particular AIHA types, the uncertainty of serological tests and the limited clinical experience. All these facts have added up to a poor understanding and management of some topics in childhood AIHA.

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Oct
2013

We report on a 4-year-old boy who developed paroxysmal cold hemoglobinuria (PCH) following the first dose of a seven-valent pneumococcal conjugate vaccine. He was admitted because of dark urine after exposure to cold air. Laboratory tests indicated anemia, increased serum indirect bilirubin and lactate dehydrogenase, and decreased serum haptoglobin.

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Sep
2013

Acquired autoimmune haemolytic anaemia is divided according to the characteristics of immunoglobulin causing haemolysis. The most frequent are haemolytic anaemia with thermal antibodies. They bind to erythrocytes and initiate their destruction in the reticuloendothelial system cells, leading to extravascular haemolysis.

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Jun
2012

PCH is one of the most common causes of acute AIHA in young children, although it affects patients of all ages. In children it is commonly seen following a viral illness or after immunization. Donath Landsteiner test is the diagnostic test.

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Dec
1969

A 15-month-old white male child was admitted to the pediatric intensive care unit with symptoms of upper respiratory tract infection, increased somnolence, pallor, jaundice, fever, and decreased activity level. The purpose of this case study is to report the clinical findings associated with the patient's clinical symptoms and differential laboratory diagnosis.

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Jan
2012

Erythrophagocytosis is a relatively rare observation on blood smears. It has been reported in auto immune hemolytic anemias and sporadically in few other conditions. Here, we report a case of florid erythrophagocytosis with severe anemia following a viral infection in an 18-year-old girl.

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Nov
2012

In children, paroxysmal cold hemoglobinuria (PCH) is generally considered an acute self-limited autoimmune hemolytic anemia caused by an IgG biphasic auto-anti-P antibody identified by the Donath-Landsteiner (D-L) test. We report a case of a 5-year-old female with a chronic hemolytic anemia. The etiology of the hemolysis appears to be an unusual D-L positive, IgM antibody with specificity for the I antigen.

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Feb
2012

Drugs that inhibit complement.

Transfus Apher Sci 2012 Feb 13;46(1):87-92. Epub 2011 Dec 13.
Hubert Schrezenmeier, Britta Höchsmann
The complement system is an important part of the innate immune system. Complement plays a crucial role in the pathophysiology of many disorders. Despite the pivotal role of the complement system, an approved targeted inhibitor of a complement factor became available only recently.

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Aug
2011

Erythrophagocytosis by neutrophils is a rare morphological phenomenon described in patients with clonal malignancies of haematopoiesis with myelodysplasia and in some haemolytic conditions including paroxysmal cold haemoglobinuria, haemolysis caused by snake-bite, sickle cell anaemia and other defects of red cells. We describe a female patient who presented with acquired haemolytic anaemia. Erythrophagocytosis was found in around 35% of neutrophils of the peripheral blood.

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Jan
2011

Hemolysis and hemoglobinuria after direct exposure to cold has rarely been reported in paroxysmal cold hemoglobinuria (PCH). The authors describe a 2.5-year-old boy with PCH (Donath-Landsteiner autoimmune hemolytic anemia), in whom 16 days after presentation, the hemoglobinuria and hemolysis recurred, when he was subjected to physical cooling, as a means to control fever associated with hospital-acquired croup.

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May
2010

Autoimmune hemolytic Anemia (AIHA) is a uncommon but potentially lethal disorder requiring prompt diagnosis and treatment. Diagnosis is based on clinical and laboratory signs of hemolysis and a positive direct antiglobulin test (DAT). AIHA is classified according to the clinical context (primary or secondary) and the characteristics of the auto-antibody (warm vs.

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Jun
2010

New linguistic coinage can signify new practices and fresh perceptions in science: descriptors therefore are not trivial. Here, we consider the shifting valence of 'allergic' and 'autoimmune' in conceptions of experimental encephalomyelitis (EE). Ehrlich's dismissal of the relevance to disease of autoimmunity resulted in its 'long struggle for recognition' notwithstanding the convincing attribution in 1904 of the hemolysis of paroxysmal cold hemoglobinuria.

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Mar
2010

Consideration on autoimmunity began, as did immunology itself, around year 1900, first with Ehrlich's doctrine of 'horror autotoxicus', then interpreted as 'autoimmunity cannot happen'. Yet by 1904 the antibody nature of the autohemolysin responsible for cold hemoglobinuria was described, and soon confirmed, but without generating any durable concept on autoimmunization as a cause of disease. Reasons included Ehrlich's doctrine, the particular directions that immunology was to take after the initial advances, and a greater preoccupation with bodily responses to extrinsic rather than autologous substances.

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Jul
2009

The aetiology of haemolytic disease is diverse and the diagnosis often relies on laboratory testing. We describe a case of intravascular haemolysis, which illustrates that significant intravascular haemolysis can occur in the absence of any abnormal haematological findings. Despite gross haemoglobinuria at presentation, the haemoglobin and reticulocyte counts were both within reference limits and a normal blood film was observed.

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Mar
2009

We present an example of a patient with confirmed cold agglutinin disease who underwent cardiac surgery in hypothermia to illustrate a known fact that, when exposed to cold, cold agglutinins induce haemolysis of erythrocytes and that cryoglobulins and cryofibrinogens may, upon exposition to cold during a surgery under hypothermia, precipitate or gelify and thus increase plasma viscosity and damage microcirculation. Detailed immunological and haematological investigations in all patients awaiting cardiac surgery with a risk of developing hypothermia is not advantageous considering the low number of patients with clinical and laboratory signs of cold agglutinin disease, autoimmune haemolytic anaemia or paroxysmal cold haemoglobinuria and considering that these investigations, in addition, might not detect cryoglobulinaemia and cryofibrinogenemia. Identification of in-risk patients from the warning signs in the medical history, physical or basal laboratory testing who would subsequently undergo confirmatory investigations to verify the presence of these entities and define them accurately might be a potential solution to this clinical issue.

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Dec
1969

Paroxysmal nocturnal hemoglobinuria: an historical overview.

Hematology Am Soc Hematol Educ Program 2008 :93-103
Charles J Parker
The clinical hallmark of paroxysmal nocturnal hemoglobinuria (PNH) is episodic hemoglobinuria, and it was this feature that captured the attention of European physicians in the latter half of the 19th century, resulting in careful observational studies that established PNH as an entity distinct from paroxysmal cold hemoglobinuria and march hemoglobinuria. Curiosity about the etiology of the nocturnal aspects of the hemoglobinuria led the German physician Paul Strübing to develop the prescient hypothesis that the erythrocytes of PNH are abnormally sensitive to hemolysis when the plasma is acidified during sleep because of accumulation of carbon dioxide and lactic acid as a result of slowing of the circulation. Investigation of the intricate pathophysiology that underlies the abnormal sensitivity of PNH erythrocytes to hemolysis in acidified serum produced a number of remarkable scientific achievements that involved discovery of the alternative pathway of complement, identification of the membrane proteins that regulate complement, discovery of a novel mechanism for attachment of proteins to the cell surface, and identification of the genetic basis of the disease.

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Dec
2008

Autoimmune hemolytic anemia (AIHA) is a relatively uncommon cause of anemia. Classifications of AIHA include warm AIHA, cold AIHA (including mainly chronic cold agglutinin disease and paroxysmal cold hemoglobinuria), mixed-type AIHA and drug-induced AIHA. AIHA may also be further subdivided on the basis of etiology.

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