It was the Monday night before Easter. The last patient on my schedule was my 2-year-old godson. I assumed he had an upper respiratory track infection like most of the patients I had been seeing but the chart indicated the chief complaint was “bruising”. As I entered the room, there was palpable tension from the parents who were appropriately concerned…regardless of the fact that we were personal friends. I learned that he had a runny nose and cough but no fever, and was active and playful. He had good facial color, not appearing pale or anemic. The dark bruises were all over his lower legs.
The parents reported that they noticed bruises on his legs as early as Friday, but he roughhoused with his older brother and they didn’t think much of it initially. Picking him up from pre-school they were terribly worried as he was wearing short pants, and the bruises were glaringly obvious. Mom had time at work to do a Google search. She kept coming across diagnoses such as leukemia which prompted the late evening visit.
I examined him thoroughly and was relieved to find that he had stable vital signs. He did not appear anemic nor did he have any lymph nodes or enlarged liver or spleen. This was reassuring. He did, however, have dark and large bruises all over his legs with more on his back and abdomen. Trying not to overly worry the parents I calmly said, “I want you to go right to the hospital for some labs, just to be sure that this is NOT something serious. I will call you with the results immediately. Above all, do NOT panic, and drive carefully.”
Needless to say I was quite concerned. The hour to get the labs seemed like an eternity. I was paged for a “critical value” and held my breath when they said the platelet count was 5,000 (normal platelets are 150,000 to 350,000). Platelets are circulating in the blood and allow the body to form clots and prevent bleeding.
Then they read me the rest of the blood count and I was relieved to learn that everything else was normal. His white blood count was normal and he was not anemic. This meant that he most likely had idiopathic thrombocytopenic purpura (ITP), which is also known as immune thrombocytopenic purpura. I called the Hematologist and arranged to have him admitted to the hospital for treatment. The anxious parents were worried that he had to be admitted, but after I explained what this all meant, they were a bit relieved.
ITP is a condition that occurs in both children and adults. In children, over 80% of cases will resolve spontaneously within six months. In adults, it tends to be more chronic and can be difficult to treat. In ITP, there are antibodies that form against the platelets which cause the platelets to be destroyed by the spleen. This often follows a viral infection of some sort in children. When the platelet count drops below 50,000, there is usually noticeable bruising, particularly of the extremities of an active child. The lower the platelet count the more obvious is the bruising. When the platelet count gets below 10,000 there may be bleeding from the gums or nose bleeds. If it gets below 5,000 there is real concern about more serious bleeding in the brain (an intra-cranial hemorrhage) or in the gastro-intestinal tract.
In ITP, if one were to do a bone marrow exam they would find giant megakaryocytes which are the precursors of the platelets. This would confirm that the platelets were being destroyed in the blood stream. The body was trying to compensate for this by “cranking out” more platelets. Luckily, children do not often need a bone marrow exam in the typical presentation of ITP. This was the case with my patient. Patients with platelet counts around 20-50,000 may not need to be admitted.
It is reported in the hematology literature that 50% of ITP cases will resolve by 4 weeks, 80% by 6 months, and 90% by 1 year.
The treatment options for ITP in children can be steroids, IV immunoglobulins (IVIG) or other newer drugs that can increase platelet production or decrease their destruction in the blood stream. In our patient, he received three daily infusions of IVIG. He was able to go home on the third day with a platelet count of 126,000. His bruising had dramatically improved. Needless to say, this made for a peaceful Easter for their family.
In addition to keeping him from being overly active (lots a luck), it is important to avoid ANYTHING that contains aspirin or anything that has Ibuprofen such as Advil or Motrin. These can impair platelet function.
I recently had a patient that had a blood count for an elective surgery. It was noted that the platelet count was 100,000. I discussed this with the Hematologist and he told me that ITP probably occurs far more often than we realize. We just never test for it and many cases go unnoticed. I repeated the platelet count weekly and it did gradually come up to normal values.
The history of ITP is interesting. It was first described in 1735 by a German physician named Paul Gottlieb Werlhof. He described purpuric lesions (bruises) of the extremities. Nobody knew anything about platelets until the early 1800’s.After that the association between a low platelet count and purpura was made. Realizing that the platelets were being destroyed in the spleen led to the first splenectomy (removal of the spleen) in 1916.This was the first line of treatment until steroids were successfully used in the 1950’s. We have come a long way!