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Paroxysmal Cold Hemoglobinuria

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2. Paroxysmal Cold Hemoglobinuria

Paroxysmal Cold Hemoglobinuria Paroxysmal Cold Hemoglobinuria Toggle navigation Brain Head & Neck Chest Endocrine Abdomen Musculoskeletal Skin Infectious Disease Hematology & Oncology Cohorts Diagnostics Emergency Findings Procedures Prevention & Management Pharmacy Resuscitation Trauma Emergency Procedures Ultrasound Cardiovascular Emergencies Lung Emergencies Infectious Disease Pediatrics Neurologic Emergencies Skin Exposure Miscellaneous Abuse Cancer Administration 4 Paroxysmal Cold (...) Hemoglobinuria Paroxysmal Cold Hemoglobinuria Aka: Paroxysmal Cold Hemoglobinuria , Donath-Landsteiner Hemolytic Anemia From Related Chapters II. Pathophysiology Rare Caused by complement fixing IgG autoantibody Activates classic complement pathway III. Causes Idiopathic Acute Viral Infection IV. Symptoms Exposure to cold classically precedes symptoms Back pain and Abdominal cramps and and chills V. Signs Transient Pale fingers, toes, and tip of nose VI. Labs in fresh urine No urine RBCs present Weakly

2018 FP Notebook

3. Paroxysmal cold hemoglobinuria: a difficult diagnosis in adult patients. (PubMed)

Paroxysmal cold hemoglobinuria: a difficult diagnosis in adult patients. 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

2016 Transfusion

4. Case report: paroxysmal cold hemoglobinuria presenting during pregnancy. (PubMed)

Case report: paroxysmal cold hemoglobinuria presenting during pregnancy. 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. She was diagnosed with paroxysmal cold (...) hemoglobinuria and treated supportively. Her hemolysis resolved within 6 weeks. Because maternal IgG autoantibodies can cross the placenta, the patient was monitored closely throughout her pregnancy for recurrence. The outcome of the pregnancy was successful, with no evidence of neonatal anemia or hemolysis.This patient had a classic presentation of paroxysmal cold hemoglobinuria with rapid onset of hemolytic anemia that resolved spontaneously. To our knowledge, this is the first report of paroxysmal cold

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2015 BMC Hematology

5. Paroxysmal Nocturnal Hemoglobinuria

Paroxysmal Nocturnal Hemoglobinuria Paroxysmal Nocturnal Hemoglobinuria Toggle navigation Brain Head & Neck Chest Endocrine Abdomen Musculoskeletal Skin Infectious Disease Hematology & Oncology Cohorts Diagnostics Emergency Findings Procedures Prevention & Management Pharmacy Resuscitation Trauma Emergency Procedures Ultrasound Cardiovascular Emergencies Lung Emergencies Infectious Disease Pediatrics Neurologic Emergencies Skin Exposure Miscellaneous Abuse Cancer Administration 4 Paroxysmal (...) Nocturnal Hemoglobinuria Paroxysmal Nocturnal Hemoglobinuria Aka: Paroxysmal Nocturnal Hemoglobinuria , PNH From Related Chapters II. Pathophysiology Rare intrinsic RBC membrane defect Increased RBC sensitivity to complement damage III. Symptoms and signs Chronic Retrosternal pain Lumbar back pain Superficial migratory Nocturnal IV. Labs Test Negative Hypochromasia (Chronic urinary iron loss) Urine may be present Hemosiderin more often present or consistent with Hemosiderin s and Urine Positive

2018 FP Notebook

6. Study to Assess Safety, Efficacy, Pharmacokinetics, and Pharmacodynamics of RO7112689 in Healthy Volunteers and Participants With Paroxysmal Nocturnal Hemoglobinuria

Study to Assess Safety, Efficacy, Pharmacokinetics, and Pharmacodynamics of RO7112689 in Healthy Volunteers and Participants With Paroxysmal Nocturnal Hemoglobinuria Study to Assess Safety, Efficacy, Pharmacokinetics, and Pharmacodynamics of RO7112689 in Healthy Volunteers and Participants With Paroxysmal Nocturnal Hemoglobinuria - Full Text View - ClinicalTrials.gov Hide glossary Glossary Study record managers: refer to the if submitting registration or results information. Search for terms x (...) × Study Record Detail Saved Studies Save this study Warning You have reached the maximum number of saved studies (100). Please remove one or more studies before adding more. Study to Assess Safety, Efficacy, Pharmacokinetics, and Pharmacodynamics of RO7112689 in Healthy Volunteers and Participants With Paroxysmal Nocturnal Hemoglobinuria The safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Listing a study does not mean it has been evaluated

2016 Clinical Trials

7. Hemoglobinuria, Paroxysmal Cold (Treatment)

Hemoglobinuria, Paroxysmal Cold (Treatment) Paroxysmal Cold Hemoglobinuria Treatment & Management: Medical Care, Surgical Care, Consultations Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMjAwOTQ3LXRyZWF0bWVudA (...) == processing > Paroxysmal Cold Hemoglobinuria Treatment & Management Updated: Mar 13, 2018 Author: Neetu Radhakrishnan, MD; Chief Editor: Emmanuel C Besa, MD Share Email Print Feedback Close Sections Sections Paroxysmal Cold Hemoglobinuria Treatment Medical Care The mainstay of treatment for paroxysmal cold hemoglobinuria is supportive care and the avoidance of cold exposure. Once hemolysis is suspected, folic acid 1 mg/d orally should be instituted to help with erythropoiesis. Folic acid is lost via

2014 eMedicine.com

8. Hemoglobinuria, Paroxysmal Cold (Overview)

Hemoglobinuria, Paroxysmal Cold (Overview) Paroxysmal Cold Hemoglobinuria: Practice Essentials, Background, Pathophysiology Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMjAwOTQ3LW92ZXJ2aWV3 processing (...) > Paroxysmal Cold Hemoglobinuria Updated: Mar 13, 2018 Author: Neetu Radhakrishnan, MD; Chief Editor: Emmanuel C Besa, MD Share Email Print Feedback Close Sections Sections Paroxysmal Cold Hemoglobinuria Overview Practice Essentials Paroxysmal cold hemoglobinuria (PCH) is a form of autoimmune (AIHA) that, while rare, is nevertheless one of the most common causes of acute AIHA in young children. In PCH, the red blood cells are targeted by an autoantibody, the Donath-Landsteiner antibody, whose formation

2014 eMedicine.com

9. Hemoglobinuria, Paroxysmal Cold (Follow-up)

Hemoglobinuria, Paroxysmal Cold (Follow-up) Paroxysmal Cold Hemoglobinuria Follow-up: Further Outpatient Care, Further Inpatient Care, Deterrence/Prevention Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache (...) =aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMjAwOTQ3LWZvbGxvd3Vw processing > Paroxysmal Cold Hemoglobinuria Follow-up Updated: Mar 13, 2018 Author: Neetu Radhakrishnan, MD; Chief Editor: Emmanuel C Besa, MD Share Email Print Feedback Close Sections Sections Paroxysmal Cold Hemoglobinuria Follow-up Further Outpatient Care When the acute phase of paroxysmal cold hemoglobinuria is over, several follow-up visits for assessment of blood counts to ensure recovery may be all that is necessary, with instructions to patients to avoid cold exposure. Confirming

2014 eMedicine.com

10. Paroxysmal Cold Hemoglobinuria (Diagnosis)

Paroxysmal Cold Hemoglobinuria (Diagnosis) Donath-Landsteiner Hemolytic Anemia: Practice Essentials, Pathophysiology, Etiology Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvOTU1MTc2LW92ZXJ2aWV3 processing (...) ). In the majority of patients, the antibody is directed specifically against the P or I antigen on the red blood cell (RBC) surface. Most cases are due to polyclonal IgG, but IgM-induced DLHA has been described. [ , , ] (Whipple et al reported a case of autoimmune hemolytic anemia in which paroxysmal cold hemoglobinuria was caused by an IgA Donath-Landsteiner [D-L] antibody. [ ] ) In contrast to DLHA, cold agglutinin disease is always due to a cold-reacting IgM antibody. Cold agglutinin disease is described

2014 eMedicine Pediatrics

11. Paroxysmal Cold Hemoglobinuria (Follow-up)

Paroxysmal Cold Hemoglobinuria (Follow-up) Donath-Landsteiner Hemolytic Anemia Treatment & Management: Approach Considerations, Treatment of Severe Disease, Supportive Care Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache (...) evaluations until the values are normal and stable: Clinical examination CBC count Reticulocyte count Total bilirubin Lactate dehydrogenase Microscopic urinalysis Direct antiglobulin test (DAT) Repeat the above testing if the patient presents with similar signs or symptoms. Have a low threshold to evaluate the child for recurrent disease. Previous References Hayashi H, Yasutomi M, Hayashi T, et al. Paroxysmal cold hemoglobinuria caused by an IgM-class Donath-Landsteiner antibody. Pediatr Int . 2013 Oct

2014 eMedicine Pediatrics

12. Paroxysmal Cold Hemoglobinuria (Overview)

Paroxysmal Cold Hemoglobinuria (Overview) Donath-Landsteiner Hemolytic Anemia: Practice Essentials, Pathophysiology, Etiology Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvOTU1MTc2LW92ZXJ2aWV3 processing > Donath (...) of patients, the antibody is directed specifically against the P or I antigen on the red blood cell (RBC) surface. Most cases are due to polyclonal IgG, but IgM-induced DLHA has been described. [ , , ] (Whipple et al reported a case of autoimmune hemolytic anemia in which paroxysmal cold hemoglobinuria was caused by an IgA Donath-Landsteiner [D-L] antibody. [ ] ) In contrast to DLHA, cold agglutinin disease is always due to a cold-reacting IgM antibody. Cold agglutinin disease is described in a separate

2014 eMedicine Pediatrics

13. Paroxysmal Cold Hemoglobinuria (Treatment)

Paroxysmal Cold Hemoglobinuria (Treatment) Donath-Landsteiner Hemolytic Anemia Treatment & Management: Approach Considerations, Treatment of Severe Disease, Supportive Care Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache (...) evaluations until the values are normal and stable: Clinical examination CBC count Reticulocyte count Total bilirubin Lactate dehydrogenase Microscopic urinalysis Direct antiglobulin test (DAT) Repeat the above testing if the patient presents with similar signs or symptoms. Have a low threshold to evaluate the child for recurrent disease. Previous References Hayashi H, Yasutomi M, Hayashi T, et al. Paroxysmal cold hemoglobinuria caused by an IgM-class Donath-Landsteiner antibody. Pediatr Int . 2013 Oct

2014 eMedicine Pediatrics

14. Hemoglobinuria, Paroxysmal Cold (Diagnosis)

Hemoglobinuria, Paroxysmal Cold (Diagnosis) Paroxysmal Cold Hemoglobinuria: Practice Essentials, Background, Pathophysiology Edition: No Results No Results Please confirm that you would like to log out of Medscape. If you log out, you will be required to enter your username and password the next time you visit. https://profreg.medscape.com/px/getpracticeprofile.do?method=getProfessionalProfile&urlCache=aHR0cHM6Ly9lbWVkaWNpbmUubWVkc2NhcGUuY29tL2FydGljbGUvMjAwOTQ3LW92ZXJ2aWV3 processing (...) > Paroxysmal Cold Hemoglobinuria Updated: Mar 13, 2018 Author: Neetu Radhakrishnan, MD; Chief Editor: Emmanuel C Besa, MD Share Email Print Feedback Close Sections Sections Paroxysmal Cold Hemoglobinuria Overview Practice Essentials Paroxysmal cold hemoglobinuria (PCH) is a form of autoimmune (AIHA) that, while rare, is nevertheless one of the most common causes of acute AIHA in young children. In PCH, the red blood cells are targeted by an autoantibody, the Donath-Landsteiner antibody, whose formation

2014 eMedicine.com

15. Paroxysmal Cold Hemoglobinuria

Paroxysmal Cold Hemoglobinuria Paroxysmal Cold Hemoglobinuria Toggle navigation Brain Head & Neck Chest Endocrine Abdomen Musculoskeletal Skin Infectious Disease Hematology & Oncology Cohorts Diagnostics Emergency Findings Procedures Prevention & Management Pharmacy Resuscitation Trauma Emergency Procedures Ultrasound Cardiovascular Emergencies Lung Emergencies Infectious Disease Pediatrics Neurologic Emergencies Skin Exposure Miscellaneous Abuse Cancer Administration 4 Paroxysmal Cold (...) Hemoglobinuria Paroxysmal Cold Hemoglobinuria Aka: Paroxysmal Cold Hemoglobinuria , Donath-Landsteiner Hemolytic Anemia From Related Chapters II. Pathophysiology Rare Caused by complement fixing IgG autoantibody Activates classic complement pathway III. Causes Idiopathic Acute Viral Infection IV. Symptoms Exposure to cold classically precedes symptoms Back pain and Abdominal cramps and and chills V. Signs Transient Pale fingers, toes, and tip of nose VI. Labs in fresh urine No urine RBCs present Weakly

2015 FP Notebook

16. THE MECHANISM OF HEMOLYSIS IN PAROXYSMAL COLD HEMOGLOBINURIA. I. THE ROLE OF COMPLEMENT AND ITS COMPONENTS IN THE DONATH-LANDSTEINER REACTION (PubMed)

THE MECHANISM OF HEMOLYSIS IN PAROXYSMAL COLD HEMOGLOBINURIA. I. THE ROLE OF COMPLEMENT AND ITS COMPONENTS IN THE DONATH-LANDSTEINER REACTION 14803552 2004 02 15 2018 12 01 0021-9738 30 1 1951 Jan The Journal of clinical investigation J. Clin. Invest. The mechanism of hemolysis in paroxysmal cold hemoglobinuria. I. The role of complement and its components in the Donath-Landsteiner reaction. 11-21 JORDAN W S WS Jr PILLEMER L L DINGLE J H JH eng Journal Article United States J Clin Invest (...) 7802877 0021-9738 9007-36-7 Complement System Proteins OM Cell Death Complement System Proteins Hemoglobinuria Hemoglobinuria, Paroxysmal Hemolysis Humans 5120:34731:427:428 HEMOGLOBINURIA PAROXYSMAL HEMOLYSIS 1951 1 1 1951 1 1 0 1 1951 1 1 0 0 ppublish 14803552 10.1172/JCI102410 PMC436222 J Clin Invest. 1951 Jan;30(1):22-30 14803553 Science. 1947 Sep 26;106(2752):293-4 17840741 J Clin Invest. 1929 Apr;7(1):27-43 16693852 Science. 1941 Nov 7;94(2445):437 17758314

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1951 Journal of Clinical Investigation

17. THE MECHANISM OF HEMOLYSIS IN PAROXYSMAL COLD HEMOGLOBINURIA. II. OBSERVATIONS ON THE BEHAVIOR AND NATURE OF THE ANTIBODY (PubMed)

THE MECHANISM OF HEMOLYSIS IN PAROXYSMAL COLD HEMOGLOBINURIA. II. OBSERVATIONS ON THE BEHAVIOR AND NATURE OF THE ANTIBODY 14803553 2004 02 15 2018 12 01 0021-9738 30 1 1951 Jan The Journal of clinical investigation J. Clin. Invest. The mechanism of hemolysis in paroxysmal cold hemoglobinuria. II. Observations on the behavior and nature of the antibody. 22-30 JORDAN W S WS Jr PILLEMER L L DINGLE J H JH eng Journal Article United States J Clin Invest 7802877 0021-9738 0 Antibodies 0 (...) Immunoglobulins OM Antibodies Cell Death Hemoglobinuria Hemoglobinuria, Paroxysmal Hemolysis Humans Immunoglobulins 5120:34732:427:428 HEMOGLOBINURIA PAROXYSMAL HEMOLYSIS 1951 1 1 1951 1 1 0 1 1951 1 1 0 0 ppublish 14803553 10.1172/JCI102412 PMC436223 J Clin Invest. 1951 Jan;30(1):11-21 14803552 Science. 1947 Sep 26;106(2752):293-4 17840741 J Clin Invest. 1929 Apr;7(1):27-43 16693852

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1951 Journal of Clinical Investigation

18. Visceral leishmaniasis presenting with paroxysmal cold haemoglobinuria (PubMed)

Visceral leishmaniasis presenting with paroxysmal cold haemoglobinuria 23867185 2014 11 12 2019 01 08 1723-2007 12 Suppl 1 2014 Jan Blood transfusion = Trasfusione del sangue Blood Transfus Visceral leishmaniasis presenting with paroxysmal cold haemoglobinuria. s141-3 10.2450/2013.0034-13 D'Angiò Mariella M Department of Cellular Biotechnologies and Haematology, Division of Haematology, "Sapienza" University, Rome, Italy. Ceglie Teresa T Department of Cellular Biotechnologies and Haematology (...) Hemoglobinuria, Paroxysmal etiology Humans Leishmania infantum isolation & purification Leishmaniasis, Visceral complications diagnosis parasitology Parasitemia complications diagnosis parasitology Pharyngitis complications microbiology Splenomegaly etiology Streptococcal Infections complications Streptococcus pyogenes 2013 01 30 2013 04 09 2013 7 23 6 0 2013 7 23 6 0 2014 11 13 6 0 ppublish 23867185 2013.0034-13 10.2450/2013.0034-13 PMC3934238 Rev Infect Dis. 1990 May-Jun;12(3):518-9 2193355 Bull World

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2014 Blood Transfusion

19. Paroxysmal Nocturnal Hemoglobinuria

Paroxysmal Nocturnal Hemoglobinuria Paroxysmal Nocturnal Hemoglobinuria Toggle navigation Brain Head & Neck Chest Endocrine Abdomen Musculoskeletal Skin Infectious Disease Hematology & Oncology Cohorts Diagnostics Emergency Findings Procedures Prevention & Management Pharmacy Resuscitation Trauma Emergency Procedures Ultrasound Cardiovascular Emergencies Lung Emergencies Infectious Disease Pediatrics Neurologic Emergencies Skin Exposure Miscellaneous Abuse Cancer Administration 4 Paroxysmal (...) Nocturnal Hemoglobinuria Paroxysmal Nocturnal Hemoglobinuria Aka: Paroxysmal Nocturnal Hemoglobinuria , PNH From Related Chapters II. Pathophysiology Rare intrinsic RBC membrane defect Increased RBC sensitivity to complement damage III. Symptoms and signs Chronic Retrosternal pain Lumbar back pain Superficial migratory Nocturnal IV. Labs Test Negative Hypochromasia (Chronic urinary iron loss) Urine may be present Hemosiderin more often present or consistent with Hemosiderin s and Urine Positive

2015 FP Notebook

20. Mechanisms of Immune Lysis of the Red Cells in Hereditary Erythroblastic Multinuclearity with a Positive Acidified Serum Test and Paroxysmal Nocturnal Hemoglobinuria (PubMed)

Mechanisms of Immune Lysis of the Red Cells in Hereditary Erythroblastic Multinuclearity with a Positive Acidified Serum Test and Paroxysmal Nocturnal Hemoglobinuria The red cells of patients with hereditary erythroblastic multinuclearity with a positive acidified serum test (HEMPAS), a form of congenital dyserythropoietic anemia, and the cells of patients with paroxysmal nocturnal hemoglobinuria (PNH) are lysed more readily than normal cells by certain antibodies, notably cold agglutinins

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1974 Journal of Clinical Investigation

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