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Paroxysmal nocturnal hemoglobinuria is a rare disease in which red blood cells break down earlier than normal.
Alternative NamesPNH
Causes, incidence, and risk factorsPersons with this disease have blood cells that are missing a gene called PIG-A. This gene allows a substance called glycosyl-phosphatidylinositol (GPI) to help certain proteins stick to cells.
Without PIG-A, important proteins cannot connect to the cell surface and protect the cell from substances in the blood called complement. As a result, red blood cells break down too early. The red cells leak hemoglobin into the blood, which can pass into the urine. This can happen at any time, but is more likely to occur during the night or early morning.
The disease can affect people of any age. It may lead to aplastic anemia, myelodysplastic syndrome, or acute myelogenous leukemia.
Risk factors, except for prior aplastic anemia, are not known.
Symptoms- Abdominal pain
- Back pain
- Blood clots -- may form in some people
- Dark urine -- comes and goes
- Easy bruising or bleeding
- Headache
- Shortness of breath
Red and white blood cell counts and platelet counts may be low.
Red or brown urine signals the breakdown of red blood cells and that hemoglobin is being released into the body's circulation and eventually into the urine.
Tests that may be done to diagnose this condition may include:
- Complete blood count (CBC)
- Coombs' test
- Flow cytometry to measure certain proteins
- Ham's (acid hemolysin) test
- Serum hemoglobin and haptoglobin
- Sucrose hemolysis test
- Urinalysis
- Urine hemosiderin
Steroids or other drugs that suppress the immune system may help slow the break down of red blood cells. Blood transfusions may be needed. Supplemental iron and folic acid are provided. Blood thinners may also be needed to prevent clot formation.
Soliris (eculizumab) is a drug used to treat PNH. It blocks the breakdown of red blood cells.
Bone marrow transplantation can cure this disease.
All patients with PNH should receive vaccinations against certain types of bacteria to prevent infection. Ask your doctor which ones are right for you.
Expectations (prognosis)The outcome varies. Most people survive greater than 10 years after their diagnosis. Death can result from complications such as blood clot formation (thrombosis) or bleeding.
In rare cases, the abnormal cells may decrease over time.
Complications- Acute myelogenous leukemia
- Aplastic anemia
- Blood clots
- Death
- Hemolytic anemia
- Iron deficiency anemia
- Myelodysplasia
Call your health care provider if you find blood in your urine, if symptoms worsen or do not improve with treatment or if new symptoms develop.
PreventionThere is no known way to prevent this disorder.
ReferencesUS Food and Drug Administration. FDA Approves First-of-its-Kind Drug to Treat Rare Blood Disorder. Rockville, MD: National Press Office; March 16, 2007. Release P07-47.
Schwartz RS. Autoimmune and intravascular hemolytic anemias. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 164.
Paroxysmal nocturnal hemoglobinuria is a rare disease in which red blood cells break down earlier than normal.
Alternative NamesPNH
Causes, incidence, and risk factorsPersons with this disease have blood cells that are missing a gene called PIG-A. This gene allows a substance called glycosyl-phosphatidylinositol (GPI) to help certain proteins stick to cells.
Without PIG-A, important proteins cannot connect to the cell surface and protect the cell from substances in the blood called complement. As a result, red blood cells break down too early. The red cells leak hemoglobin into the blood, which can pass into the urine. This can happen at any time, but is more likely to occur during the night or early morning.
The disease can affect people of any age. It may lead to aplastic anemia, myelodysplastic syndrome, or acute myelogenous leukemia.
Risk factors, except for prior aplastic anemia, are not known.
Symptoms- Abdominal pain
- Back pain
- Blood clots -- may form in some people
- Dark urine -- comes and goes
- Easy bruising or bleeding
- Headache
- Shortness of breath
Red and white blood cell counts and platelet counts may be low.
Red or brown urine signals the breakdown of red blood cells and that hemoglobin is being released into the body's circulation and eventually into the urine.
Tests that may be done to diagnose this condition may include:
- Complete blood count (CBC)
- Coombs' test
- Flow cytometry to measure certain proteins
- Ham's (acid hemolysin) test
- Serum hemoglobin and haptoglobin
- Sucrose hemolysis test
- Urinalysis
- Urine hemosiderin
Steroids or other drugs that suppress the immune system may help slow the break down of red blood cells. Blood transfusions may be needed. Supplemental iron and folic acid are provided. Blood thinners may also be needed to prevent clot formation.
Soliris (eculizumab) is a drug used to treat PNH. It blocks the breakdown of red blood cells.
Bone marrow transplantation can cure this disease.
All patients with PNH should receive vaccinations against certain types of bacteria to prevent infection. Ask your doctor which ones are right for you.
Expectations (prognosis)The outcome varies. Most people survive greater than 10 years after their diagnosis. Death can result from complications such as blood clot formation (thrombosis) or bleeding.
In rare cases, the abnormal cells may decrease over time.
Complications- Acute myelogenous leukemia
- Aplastic anemia
- Blood clots
- Death
- Hemolytic anemia
- Iron deficiency anemia
- Myelodysplasia
Call your health care provider if you find blood in your urine, if symptoms worsen or do not improve with treatment or if new symptoms develop.
PreventionThere is no known way to prevent this disorder.
ReferencesUS Food and Drug Administration. FDA Approves First-of-its-Kind Drug to Treat Rare Blood Disorder. Rockville, MD: National Press Office; March 16, 2007. Release P07-47.
Schwartz RS. Autoimmune and intravascular hemolytic anemias. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 164.
Reviewed ByReview Date: 03/28/2010
David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and James R. Mason, MD, Oncologist, Director, Blood and Marrow Transplantation Program and Stem Cell Processing Lab, Scripps Clinic, Torrey Pines, California. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
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What does the medical abbreviation PNH mean?
Paroxysmal Nocturnal Hemoglobinuria
What is the airport code for Phnom Penh International Airport?
The airport code for Phnom Penh International Airport is PNH.
Sugar-water hemolysis test?
DefinitionThe sugar-water hemolysis test is a blood test to detect fragile red blood cells by testing their ability to withstand swelling in a low-salt solution.Alternative NamesSucrose hemolysis testHow the test is performedBlood is typically drawn from a vein, usually from the inside of the elbow or the back of the hand. The site is cleaned with germ-killing medicine (antiseptic). The health care provider wraps an elastic band around the upper arm to apply pressure to the area and make the vein swell with blood.Next, the health care provider gently inserts a needle into the vein. The blood collects into an airtight vial or tube attached to the needle. The elastic band is removed from your arm.Once the blood has been collected, the needle is removed, and the puncture site is covered to stop any bleeding.In infants or young children, a sharp tool called a lancet may be used to puncture the skin and make it bleed. The blood collects into a small glass tube called a pipette, or onto a slide or test strip. A bandage may be placed over the area if there is any bleeding.The blood sample is sent to a laboratory to be tested. When a low-salt solution containing sucrose (sugar) is added to certain fragile cells, a part of the body's defense mechanism called complement is activated, binds to the cells, and bursts them.How to prepare for the testThere is no special preparation needed for this test.How the test will feelWhen the needle is inserted to draw blood, some people feel moderate pain, while others feel only a prick or stinging sensation. Afterward, there may be some throbbing.Why the test is performedYour doctor may order this test if you have signs or symptoms of paroxysmal nocturnal hemoglobinuria (PNH) or hemolytic anemia of unknown cause. PNH red blood cells are very likely to be harmed by the body's complement system.Normal ValuesNegative: less than 5% red blood cell breakdown (hemolysis)Positive: more than 10% hemolysisNote: Normal value ranges may vary slightly among different laboratories. Talk to your doctor about the meaning of your specific test results.What abnormal results meanParoxysmal nocturnal hemoglobinuria (PNH)Autoimmune hemolytic anemias and leukemia may give false positive resultWhat the risks areThere is very little risk involved with having your blood taken. Veins and arteries vary in size from one patient to another and from one side of the body to the other. Taking blood from some people may be more difficult than from others.Other risks associated with having blood drawn are slight but may include:Excessive bleedingFainting or feeling light-headedHematoma (blood accumulating under the skin)Infection (a slight risk any time the skin is broken)Special considerationsA negative test does not rule out PNH. False-negative results may occur if the fluid part of blood (serum) lacks complement.ReferencesSchwartz R. Autoimmune and intravascular hemolytic anemias. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 164.Brodsky RA. Paroxysmal nocturnal hemoglobinuria. In: Hoffman R, Benz EJ, Shattil SS, et al, eds. Hematology: Basic Principles and Practice. 5th ed. Philadelphia, Pa: Elsevier Churchill Livingstone; 2008:chap 30.Reviewed ByReview Date: 03/21/2010Linda J. Vorvick, MD, Medical Director, MEDEX Northwest Division of Physician Assistant Studies, University of Washington, School of Medicine; James R. Mason, MD, Oncologist, Director, Blood and Marrow Transplantation Program and Stem Cell Processing Lab, Scripps Clinic, Torrey Pines, California. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
What is Flight time from Hong Kong to phnum penh?
2hr 35min Hong Kong (HKG) to Phnom Penh (PNH) by a nonstop flight operated by Dragonair.
What is the Flight time from kuala lumpur to phnom penh?
A random search for flights from KUL to PNH shows: OPTION 1: 1hr 50min by a nonstop flight operated by Malaysia Airlines.
Most expensive medicine?
Zolgensma, a gene therapy drug used to treat spinal muscular atrophy in infants, is currently one of the most expensive medicines in the world at a cost of around $2.1 million per treatment. It is considered one of the most expensive drugs due to its high efficacy and rarity.
How long does it take to get to phnom phen from Perth?
A random search for flights from Phnom Penh (PNH) to Perth (PER) shows:OPTION 1: 9hr 40min via Bangkok (BKK)PNH - BKK Thai Airways Flt 585 Dep 09:10pm 20Nov,FriBKK - PER Thai Airways Flt 485 Dep 11:55pm 20Nov,FriFlight Duration: 8hr 00min; Layover Time: 1hr 40min; Total Trip Time: 9hr 40minOPTION 1: 12hr 30min via Kuala Lumpur (KUL) & SingaporePNH - KUL Malaysia Airlines Flt 755 Dep 11:10am 20Nov,FriKUL - SIN Malaysia Airlines Flt 613 Dep 05:30pm 20Nov,FriSIN - PER Singapore Airlines Flt 215 Dep 07:35pm 20Nov,FriFlight Duration: 7hr 50min; Layover Time: 4hr 40min; Total Trip Time: 12hr 30min
What is the flight time between Shanghai China and Krabi Thailand?
The flight time from Shanghai in China to Krabi in Thailand is approximately 4 hours and 32 minutes. The total distance is approximately 3478.34 km or 2161 miles. This flight time is approximate. Factors such as weather conditions could affect the flight time.
How long does it take to fly from Singapore to phnom penh?
The flight times varies according to the speed of the flight. If the flight is flying at a faster speed then the times may change. We are providing you with standard flight timings. The flight time for the above mentioned trip is.1 hours 50 mins
Ham test?
DefinitionThe Ham test is done to diagnose paroxysmal nocturnal hemoglobinuria (PNH). The test checks whether red blood cells become more fragile when they are placed in mild acid.Alternative NamesAcid hemolysin testHow the test is performedBlood is typically drawn from a vein, usually from the inside of the elbow or the back of the hand. The site is cleaned with germ-killing medicine (antiseptic). The health care provider wraps an elastic band around the upper arm to apply pressure to the area and make the vein swell with blood.Next, the health care provider gently inserts a needle into the vein. The blood collects into an airtight vial or tube attached to the needle. The elastic band is removed from your arm.Once the blood has been collected, the needle is removed, and the puncture site is covered to stop any bleeding.In infants or young children, a sharp tool called a lancet may be used to puncture the skin and make it bleed. The blood collects into a small glass tube called a pipette, or onto a slide or test strip. A bandage may be placed over the area if there is any bleeding.How to prepare for the testThere is no special preparation needed for this test.How the test will feelWhen the needle is inserted to draw blood, some people feel moderate pain, while others feel only a prick or stinging sensation. Afterward, there may be some throbbing.Why the test is performedA positive test can confirm the diagnosis of PNH.The Ham test can also be used to diagnose another rare disorder called congenital dyserythropoietic anemia.Normal ValuesA negative test is normal.What abnormal results meanAbnormal results may be due to:Paroxysmal nocturnal hemoglobinuriaCongenital dyserythropoietic anemiaWhat the risks areThere is very little risk involved with having your blood taken. Veins and arteries vary in size from one patient to another and from one side of the body to the other. Taking blood from some people may be more difficult than from others.Other risks associated with having blood drawn are slight but may include:Excessive bleedingFainting or feeling light-headedHematoma (blood accumulating under the skin)Infection (a slight risk any time the skin is broken)Special considerationsThe HAM test is increasingly being replaced by a newer test called flow cytometry.ReferencesElghetany M, Banki K. Erythrocytic disorders. In: McPherson R, Pincus M, eds. Henry's Clinical Diagnosis and Management by Laboratory Methods. 21st ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 31.Schwartz R. Autoimmune and intravascular hemolytic anemias. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 164.
What is the name of the main airport in Cambodia?
The main airport in Cambodia is the Phnom Penh International Airport and is located in the country's capital city, Phnom Penh. The IATA code is PNH, its ICAO code is VDPP The airport used to be called the Pochentong International Airport. The second largest airport is the Siem Reap-Angkor International Airport. It serves the most international flights entering and leaving Cambodia. Its IATA code is REP, and its ICAO code is VDSR
Serum hemoglobin?
Serum hemoglobin is a measure of the amount of hemoglobin, a protein in red blood cells that carries oxygen, in the liquid component of blood. It is often used as a marker to assess an individual's iron status and diagnose anemia. Normal levels vary by age, sex, and other factors.
