The presence of the free hemoglobin in the blood in abnormal quantities is known as hemoglobinuria. There are many blood related syndromes that present with hemoglobinuria as a main clinical symptom. It is usually manifested as one of the following disorders:

  • Paroxysmal nocturnal hemoglobinuria

  • Cold hemoglobinuria


In most of cases, there is lysis of the red blood cells so some clinicians also term the excessive RBCs lysis as hemoglobinuria as it almost always raises the hemoglobin levels in the blood and urine.


Following symptoms are usually seen in case of hemoglobinuria:

  • Back pain

  • Dark smoky urine

  • Blood clots in the urine.

  • Easy bruising or bleeding

  • Headache

  • Shortness of breath


The causes of hemoglobinuria have not been established well yet and are still being studied. However, it is thought that genetic factors (absence of the PIG-A gene) are responsible for causing this problem. Bacterial and viral infections can also the cause for this disease.  Any disease that causes the breakdown of red blood cells like sickle cell anemia, also results in hemoglobinuria.


Lab tests are used to diagnose this problem. Following things are to be done for assessment of hemoglobinuria:

  • CBC (there will be low RBCs count)

  • Urine analysis (there will be blood in urine)

  • Urine and serum hemosiderin test (it will be raise)

  • Ham’s acid hemolysin test


By curing the underlying cause, hemoglobinuria can be treated.

  • Antibiotics are given to combat the bacterial infections like haemophilus influenzae, assumed to cause this problem.

  • Antiviral drugs are given to treat conditions like mumps that are also the hidden reason for appearance of this problem.

  • Steroids are administered to suppress immune processes and slow down the breakdown of RBCs.

  • Curing the underlying blood related disorders that may be affecting and lysing the red blood cells.