Nosebleeds lead to doctor's inquiry
When 35-year-old Vincent Hui (real name withheld for patient confidentiality reasons) experienced his first nosebleed, an alarming torrent of blood poured from his nose, soaking his shirt.
The first nosebleed was soon followed by another, then another. While nosebleeds usually cease within 20 minutes, Hui took up to two hours to staunch each bleeding.
Nosebleeds are relatively common and usually not serious. One in seven people may experience a nosebleed at some time in their lives. But the copious amount and extended duration of bleeding in Hui's case, plus the frequent occurrence, alerted him - and his doctors - that something more serious was afoot.
Hui consulted Dr Raymond Liang, director of the Comprehensive Oncology Centre at the Hong Kong Sanatorium and Hospital. Liang noticed that Hui's limbs suffered unusual bruising, which, together with the excessive bleeding and frequent nosebleeds, indicated that Hui had a bleeding tendency.
A bleeding tendency means something in the body's natural ability to clot has gone awry.
The body stops bleeding in three steps - by constricting injured blood vessels, clumping platelets and forming blood clots with the clotting proteins produced in the liver.
To diagnose Hui's condition, Liang had to investigate which of these functions had failed.
As Hui was young and otherwise healthy, and not on medication such as steroids or aspirin, Liang quickly ruled out drugs, liver disease, kidney failure, leukaemia and haemophilia as potential causes of abnormal bleeding.
He ran a blood test on Hui and found that his clot-forming platelets - thrombocytes - were at a critically low level. This finding pointed to immune thrombocytopenia as the likely culprit.
Immune thrombocytopenia is a condition where the body's immune system - for unknown reasons - attacks platelets, drastically reducing their numbers.
Healthy people have 150,000 to 450,000 platelets per microlitre of blood. When the number of platelets drops below 50,000 per microlitre, bleeding problems can occur.
When platelet numbers drop to dangerously low levels of fewer than 20,000, spontaneous bleeding, including that in the brain, becomes a potentially deadly threat.
Hui had only 10,000 platelets per microlitre of blood.
However, as immune thrombocytopenia - like most autoimmune diseases - tend to affect women, Liang could not be certain at first that Hui had the condition, as he did not fit the profile. His medical team swiftly conducted a bone marrow biopsy to see if the low platelet count was a result of reduced platelet production in Hui's bone marrow, but results showed it was normal.
Hui was given an emergency treatment of intravenous immunoglobulin, an antibody-rich blood product, to stave off the suspected immune destruction in his blood.
Within 24 hours of its administration, Hui's platelet count started to climb to normal levels, and the nosebleeds stopped.
His positive response to the stop-gap treatment (its effects wear off within one to two weeks) confirmed Liang's suspicions of immune thrombocytopenia, as it is the only disease that responds to intravenous immunoglobulin treatment.
With a confirmed diagnosis, Hui was started on a long-term treatment of steroids to suppress his immune system to reduce the destruction of the platelets.
However, the steroids gave Hui severe stomach discomfort that required copious amounts of anti-ulcer treatments to relieve. Yet, when the steroids were reduced, Hui's platelet count dropped.
Liang needed to order repeated treatments of intravenous immunoglobulin to help stabilise Hui's condition.
The doctor observed that it is common for men to respond more poorly to steroid treatment than women. After four months of unsuccessful steroid treatment, Liang proposed a splenectomy.
The spleen is a fist-sized organ in the left side of the abdomen that removes old red blood cells and plays a role in the immune system. But platelets are also trapped and destroyed in the spleen, which Liang describes as the 'platelet graveyard'.
Removing the spleen would slow down the destruction of the platelets but also expose Hui to a higher risk of infection.
However, Liang says the infection risk in adults such as Hui can easily be managed, with fewer side effects than the steroid treatment. Hui would have to be vigilant about infections and keep a steady supply of antibiotics handy, taking them at the first sign of fever.
Reluctant to continue grappling with the severe side effects of steroids, Hui agreed to the splenectomy.
To prepare for the operation to remove one of his immune-protective organs, he was given a battery of vaccinations to shore up his body's defences.
The splenectomy was a success, and immediately after the operation, Hui's platelet count rose to a respectable 100,000 per microlitre.
However, although the procedure greatly retarded the rate of platelet destruction, Hui's immune system was still destroying platelets faster than normal, and his body's ability to stem bleeding was still weak. So he must still take extra care to avoid any kind of injury.