Edit concept Question Editor Create issue ticket

Hemoglobinuria

Haemoglobinuria


Presentation

  • Case presentation An 18 year old female patient presented with acute symptoms of abdominal and flank pain, nausea, dark red urine, fevers and chills.[bmchematol.biomedcentral.com]
  • Hemoglobinuria results in the production of classically dark urine during the night and in the morning (about 25 % of patients), and patients may present with renal insufficiency. Jaundice may be present.[orpha.net]
Common Cold
  • When his hemoglobin drops below a 7 he starts to become short of breath with the slightest activity and a common cold could take triple the amount of time to recover from. There are only two treatment options for those with this disease.[globalgenes.org]
  • Pathophysiology Paroxysmal cold hemoglobinuria shares similar antibody thermal activity range as cold hemagglutinin disease (CHD), the more common cold variant of autoimmune hemolytic anemia.[emedicine.medscape.com]
Abdominal Tenderness
  • On physical exam, her skin is jaundiced, her liver edge is palpable, and she has abdominal tenderness to palpation. Her physicians are suspicious of Budd-Chiari syndrome.[step1.medbullets.com]
Rectal Bleeding
  • bleeding (mesenteric vein thrombosis) headache, vomiting, and seizures (cerebral venous sinous thrombosis) fever, jaundice, hepatomegaly (Budd-Chiari syndrome) renal impairment Evaluation Flow cytometry – most accurate test CD55/59-negative RBCs Labs[medbullets.com]
Purpura
  • Causes 2 Diagnosis 3 See also 4 References 5 External links Causes [ edit ] Acute glomerulonephritis Burns Renal cancer Malaria Paroxysmal nocturnal hemoglobinuria Microangiopathies, e.g. hemolytic-uremic syndrome (HUS), thrombotic thrombocytopenic purpura[en.wikipedia.org]
  • Infections may produce fever and purpura may be apparent. The Budd-Chiari syndrome will produce hepatomegaly and ascites. There may be splenomegaly too. Ischaemia of the gut produces absent bowel sounds.[patient.info]
  • […] called acute glomerulonephritis Burns Crushing injury Hemolytic uremic syndrome (HUS) Kidney infection Kidney tumor Malaria Paroxysmal nocturnal hemoglobinuria Paroxysmal cold hemoglobinuria Sickle cell anemia Thalassemia Thrombotic thrombocytopenic purpura[mountsinai.org]
  • Crushing injury Hemoglobin C disease Hemoglobin SC disease Hemolytic uremic syndrome (HUS) Kidney infection Kidney tumor Malaria Paroxysmal nocturnal hemoglobinuria Paroxysmal cold hemoglobinuria Sickle cell anemia Thalassemia Thrombotic thrombocytopenic purpura[ucsfhealth.org]
  • Acute glomerulonephritis Burns Crushing injury Hemoglobin C disease Hemoglobin SC disease Hemolytic uremic syndrome (HUS) Kidney infection Kidney tumor Malaria Paroxysmal nocturnal hemoglobinuria Sickle cell anemia Thalassemia Thrombotic thromocytic purpura[ucsfbenioffchildrens.org]
Red Urine
  • With clinical disease, rapid intravascular hemolysis leads to severe anemia, tachycardia, weakness, hemoglobinuria with dark brown or red urine, and pallor over several days. Milk production drops rapidly.[msdvetmanual.com]
  • The clinical features of PNH are shown in Table 1 .[ 3, 4 ] The most common are fatigue (typically out of proportion to the degree of anemia), shortness of breath, and hemoglobinuria (red urine), especially in the first morning sample ( Figure 1 ).[bcmj.org]
Nocturia
  • 10-CM D59.6 Hemoglobinuria due to hemolysis from other external causes Or: 2015/16 ICD-10-CM D59.8 Other acquired hemolytic anemias Approximate Synonyms Extrinsic hemoglobinuria Hemoglobinuria - external causation Hemoglobinuria, extrinsic Paroxsymal nocturia[icd9data.com]
  • "Eine neue form intermittierender haemoglobinurie (Haemoglobinuria paroxysmalis nocturia)". Klin Wochenschr (in German). 7 (43): 2045–2047. doi : 10.1007/BF01846778 . External links [ edit ][en.wikipedia.org]
  • […] neoplasms ( C00-D49 ) symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified ( R00 - R94 ) Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism Approximate Synonyms Paroxsymal nocturia[icd10data.com]
Microscopic Hematuria
  • hematuria ) White blood cells Eosinophiluria Proteinuria Albuminuria / Microalbuminuria Myoglobinuria Hemoglobinuria Small molecules Glycosuria Ketonuria Bilirubinuria Hyperuricosuria / Hypouricosuria Aminoaciduria Pathogens Bacteriuria Other Chyluria[en.wikipedia.org]

Workup

  • A subsequent workup failed to reveal any cardiac or pulmonary cause for her symptoms, and abdominal imaging was also unremarkable.[hematologyandoncology.net]
  • The laboratory workup revealed a white blood cell count 4.6 k/uL, RBC count of 3.8 k/uL, hemoglobin of 11.8 g/dL, hematocrit 35%, MCV 91 fl, platelets 327 k/uL, reticulocytes 24.5%, PTT 27.8, INR 1.7, D dimer 2.5 ug/mL, bilirubin 1.3 mg/dL, bilirubin[f1000research.com]
  • These differentials were kept in mind prior to ordering the workup. His laboratory data at admission are outlined in table 1 . A previous renal function test was done in 2007 which showed 97.24 µmol/l serum creatinine and 4.99 mmol/l BUN.[karger.com]
Albuminuria
  • Urobilinogen Bilirubin Creatinine RBC WBC Urinary casts Chemical properties Urine specific gravity Urine osmolality Urine pH Urine anion gap Abnormal findings Red blood cells Hematuria ( Microscopic hematuria ) White blood cells Eosinophiluria Proteinuria Albuminuria[en.wikipedia.org]
  • Proteinuria 1.3 g/day with albuminuria 170 mg/day and non-selective aminoaciduria, thus indicating PTD. The acid-base status, phosphate and uric acid were normal.[revistanefrologia.com]
Microalbuminuria
  • Bilirubin Creatinine RBC WBC Urinary casts Chemical properties Urine specific gravity Urine osmolality Urine pH Urine anion gap Abnormal findings Red blood cells Hematuria ( Microscopic hematuria ) White blood cells Eosinophiluria Proteinuria Albuminuria / Microalbuminuria[en.wikipedia.org]
Ketonuria
  • […] gravity Urine osmolality Urine pH Urine anion gap Abnormal findings Red blood cells Hematuria ( Microscopic hematuria ) White blood cells Eosinophiluria Proteinuria Albuminuria / Microalbuminuria Myoglobinuria Hemoglobinuria Small molecules Glycosuria Ketonuria[en.wikipedia.org]
Hyperuricosuria
  • Urine pH Urine anion gap Abnormal findings Red blood cells Hematuria ( Microscopic hematuria ) White blood cells Eosinophiluria Proteinuria Albuminuria / Microalbuminuria Myoglobinuria Hemoglobinuria Small molecules Glycosuria Ketonuria Bilirubinuria Hyperuricosuria[en.wikipedia.org]
Glycosuria
  • […] specific gravity Urine osmolality Urine pH Urine anion gap Abnormal findings Red blood cells Hematuria ( Microscopic hematuria ) White blood cells Eosinophiluria Proteinuria Albuminuria / Microalbuminuria Myoglobinuria Hemoglobinuria Small molecules Glycosuria[en.wikipedia.org]

Treatment

  • Treatment The treatment of PNH is directed at the specific symptoms that are present in each individual and includes a variety of different therapeutic options. In 2007, the U.S.[rarediseases.org]
  • ; an increase in serum creatinine by 25% or more compared to baseline or nadir during Soliris treatment; or, an increase in serum LDH by 25% or more over baseline or nadir during Soliris treatment.[soliris.net]

Prognosis

  • Prognosis after immunosuppressive therapy (IST) in the good prognosis and the poor prognosis group. (A) Overall survival. (B) Failure-free survival. (C) Transplantation-free survival.[haematologica.org]
  • , Prevention, and Complications Prognosis improved with use of eculizumab Complications renal insufficiency thromboembolism 5% develop myelodysplasia 2.5% develop acute leukemias Please rate topic.[medbullets.com]
  • Treatment and prognosis blood transfusions anticoagulation bone marrow transplant 1 eculizumab 1 Without therapy approximately 50% of patients die as a direct result of the disease. Many others are transfusion dependent for decades 3.[radiopaedia.org]

Etiology

  • Artesunate was stopped suspecting it to be the etiological agent of hemolysis and hemoglobinuria.[atmph.org]
  • Etiology: —The condition most commonly results from the direct influence of poisons, such as carbonic acid gas, naphtha and other petroleum vapors, or from phosphorus, arseniuretted hydrogen, potassium chlorate, pyrogallic acid and turpentine, and from[henriettes-herb.com]
  • Pathogenesis Etiology Erythrocyte lysis within urine Hematuria Hematuria. Intravascular hemolysis Hemoglobin filtered by glomeruli. Intravascular hemolysis is more likely to result in hemoglobinuria than is extravascular hemolysis. Idiopathic.[vetstream.com]
  • Etiology PNH is caused by somatic mutations in the PIGA gene (Xp22.1), encoding a protein involved in the biosynthesis of the glycosylphosphatidylinositol (GPI) anchor.[orpha.net]

Epidemiology

  • Summary Epidemiology Although PNH has been described worldwide, exact prevalence data are not available. It is estimated at 1/80,000 in France.[orpha.net]
  • […] granulocytes have thrombosis within 10 years after diagnosis Flow cytometry testing – monitor response to monoclonal antibody therapy directed against C5 Therapy success associated with increased ratios of erythrocyte clones to granulocyte clones Background Epidemiology[arupconsult.com]
  • Epidemiology This is a haematopoietic stem cell mutation defect of the PIGA gene in the X chromosome [ 2 ]. It is not familial and sex distribution is equal. It is rare but may be slightly less rare in Southeast Asian communities.[patient.info]
Sex distribution
Age distribution

Pathophysiology

  • Over the last 20 years, we have seen a dramatic unraveling of the pathophysiology of this rare and complex disorder.[dovepress.com]
  • PNH is rarely investigated in stroke patients and its contribution to the pathophysiology of ESUS is unknown.[clinicaltrials.gov]
  • Pathophysiology Hemoglobinuria may result from either of two main sources: True hemoglobinuria results when hemoglobin is filtered by the glomeruli. Hemoglobinuria also results from hematuria when erythrocytes are lysed within the urine.[vetstream.com]

Prevention

  • Prevention There is no known way to prevent this disorder. References Brodsky RA. Paroxysmal nocturnal hemoglobinuria. In: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Basic Principles and Practice . 7th ed.[mountsinai.org]
  • Correction of mineral deficiencies and elimination of plant toxins from the diet may help prevent recurrence. 2018 Merck Sharp & Dohme Corp., a subsidiary of Merck & Co., Inc., Kenilworth, NJ, USA)[msdvetmanual.com]
  • Blood thinners may also be needed to prevent clots from forming. Soliris (eculizumab) is a drug used to treat PNH. It blocks the breakdown of red blood cells. Bone marrow transplantation can cure this disease.[nlm.nih.gov]
  • Absence of PIG-A function in a cell prevents synthesis of the glycosylphosphatidylinositol (GPI) moiety, which anchors many different types of proteins to the cell membrane.[clinicaltrials.gov]

Ask Question

5000 Characters left Format the text using: # Heading, **bold**, _italic_. HTML code is not allowed.
By publishing this question you agree to the TOS and Privacy policy.
• Use a precise title for your question.
• Ask a specific question and provide age, sex, symptoms, type and duration of treatment.
• Respect your own and other people's privacy, never post full names or contact information.
• Inappropriate questions will be deleted.
• In urgent cases contact a physician, visit a hospital or call an emergency service!