2 1/2 yr old newly diagnosed

Hi - My 2 1/2 year old was diagnosed last weekend with ITP. His platelets were at 15000. They gave him prednisone for 4 days. He had his last does this morning. yesterday his platelets were at 95000 already. YAY! My question - are they most likely to drop again now that he is off the prednisone? What is typical? Every doctor I have talked to says this won't last long in children but all the real life stories I am reading seem to say otherwise.

Given his age, has he had any recent vaccinations? They "say" in children it's typically acute, but I'm seeing an awful lot of little kids who've had ITP for years. Given your son's age you might consider food allergies as a possible immune system overload (you can ELISA test for those) and/or vaccines as an immune system response that could have triggered the ITP.

He hasn't had any vaccines recently. Not since Feb. They also asked if he had any illness lately and he didn't. Unless he had a sore throat I was unaware of.
I should check into the food allegies. He also has excema pretty bad. Is that seen commonly in ITP cases?

Eczema is very common in all children and not really seen any more often with ITP than normal.

If you want to do some reading, the latest international guidelines are at bloodjournal.hematologylibrary.org/content/115/2/168.full and the section on childhood ITP is about half way down.

Your child being so young means he has a good chance of recovering quickly. Don't forget that the parents still here are the unlucky ones, the 80% with the acute ITP children don't stay long. Good luck.

You might want to look at this. There is a type of ITP in which eczema is an accompanying symptom. He may not have it, but it's worth checking out.

Wiskott-Aldrich syndrome and X-linked thrombocytopenia, originally described in 1937, affects males almost exclusively and includes moderate to severe thrombocytopenia, immunodeficiency, and eczema. A milder form exists as X-linked thrombocytopenia (XLT) in which eczema and immunodeficiency are not clinically significant. A distinguishing characteristic of these related disorders is the presence of microthrombocytes, with significantly reduced mean platelet volume (MPV 3.5-5.0 fL; normal 7-11 fL) and a stable platelet count of 5000 to 50 000/µL. The combination of reduced platelet size and number results in a combined platelet mass that is about 1% of healthy individuals.

Diagnosis. In any male child with thrombocytopenia or immunodeficiency, the diagnosis of WAS should be considered. The presence of microthrombocytes increases the likelihood of this diagnosis. Affected individuals with classical WAS are generally recognized within 1 year of life due to bruising and bleeding as well as recurrent infections (bacterial more than viral). Diagnosis of patients with XLT may not be made until later in life. Eczema develops after the first year of life, and dermatologic evaluation to assess the severity and treatment of eczema should be undertaken. Confirmatory sequencing of the WAS gene to detect mutations should be performed in families without prior diagnosis. In addition, Western blot or flow cytometry of lymphocytes demonstrates absent/diminished expression of WAS protein. Specialized laboratories can usually detect abnormal lymphocyte proliferation in response to mitogens, HLA, or anti-CD3 antibody as well as impaired class switching from IgM to IgG for classical WAS. In contrast, individuals with XLT may have no defect of lymphocyte function by in vitro testing. Chorionic villus sampling or amniocentesis can also be used to obtain material for prenatal diagnosis when the mother is a known carrier.

Etiology. In 1994, the gene mutated in WAS and XLT (WAS) was identified on the short arm of the X chromosome (Xp11.22).59,60 This gene is composed of 12 exons and encodes a polypeptide of 502 amino acids. The mature WAS protein (WASP) contains numerous protein-interacting domains and appears to provide a critical link between the cellular cytoskeleton and signal transduction pathways. Although it does not encode enzymatic activity, WASP includes subdomains that permit binding to a variety of signaling proteins (SH3-containing proteins, GTPases), membrane phospholipids (Pleckstrin homology domain), polarized cytoskeletal elements (Cofilin and Verprolin domains), and the unique WASP-interacting protein (WIP),61 (for a review, see in Ochs59). Interestingly, the development of microthrombocytes, a distinctive feature of the disease, is not well understood, and MKs from patients with WAS are capable of forming proplatelet processes and platelets of normal size in vitro.62 In the bone marrow of affected individuals, MKs appear to be normal or increased in frequency, suggesting a block during thrombopoiesis. Also, the observation that platelet size and number often improves after splenectomy suggests that the reticuloendothelial system could play a role in platelet removal (although antiplatelet antibodies are not present).

Phenotype/genotype correlation. To a large extent, the phenotypic variation of WAS can be explained by the location and nature of the genetic mutation. Frameshifts, nonsense mutations, and large deletions generally cause classical WAS, whereas single amino acid substitutions, especially of exons 1 to 3, cause the milder XLT (for a review, see Ochs59). When WASP is completely absent, the disease is more severe; when it is present at low-moderate levels, the disease is frequently milder.

Current therapy. Infections are the most common cause of death in patients with WAS. In the past 4 decades, median life expectancy for patients with severe WAS has been extended from less than 5 years to 10 to 20 years with a significant proportion living into the third and fourth decades of life, largely due to the common use of intravenous γ-globulin (IVIG) and broad-spectrum antibiotics. Thrombocytopenia may cause life-threatening bleeding events. Treatment of acute bleeding is principally platelet transfusions, which provide normal increments and survival of circulating platelets. Splenectomy may induce clinically significant increases in platelet counts but must be considered in the context of an increased risk of sepsis or infection. When eczema is severe, it is treated with topical steroids or short courses of systemic steroids. Individuals over 20 years of age have a markedly increased incidence of autoimmune diseases and lymphomas. In contrast, the milder XLT has a substantially lower risk of these late complications. The only curative therapy remains hematopoietic stem cell transplantation, which corrects both thrombocytopenia and immunodeficiency. For classical WAS, stem cell transplantation should be undertaken for all affected individuals with an appropriate donor.

bloodjournal.hematologylibrary.org/content/103/2/390.full

Aside from what Sandi posted - because I know nothing about that - I do know eczema is very frequently a food allergy with dairy seeming to be the biggest offender. We have BTDT with eczema that covered my son's entire body and bled regularly. As soon as we removed all dairy he finally got rid of it. At the very minimum, you've got a couple of ideas you can look up now (including what Sandi posted - very interesting). As well, Feb. is plenty recent enough for vaccinations to be causative. One more avenue to explore (although the entire medical community will swear it's not - you'll have to look elsewhere for information on this issue).

Thanks so much for all of the information. I have never heard of ITP until last friday.
I found out yesterday that my mother-in-law had a problem with brusing easy, to the touch in ninth grade. Her mother took her to the doctor for it. She is now deceased so unfortunately I can't ask her about it. Her friend from highschool was the one that told us about it. So can ITP be hereditary?

There are hereditary types of ITP (such as WAS), but ITP itself is not hereditary. I know that sounds confusing. My sister and I have both had ITP, but it is not considered familial ITP, it was more of a genetic predisposition toward autoimmune disorders and we just happened to get the same one. In families with autoimmune tendencies, one might have ITP and another sibling has Graves and another sibling has MS, etc. The autoimmune disorder can vary from person to person.

Genetic ITP is not really ITP; it is another platelet disorder that presents as ITP. Here is some info about that:

www.pdsa.org/resources/other-platelet-disorders.html?layout=item

just curious... what caused you to have your sons platelets checked? kids that age dont usually get routine bloodwork right? so was he having an issue that the dr. had bloodwork done?

He had bruises popping out everywhere. Also petechia all over his chest and back.
His platelets are 89,000 this week. I am so glad they are over 50.

Good Luck! My daughter was 3.5 when she was diagnosed. She is the unfortunate one that didn't get rid of it in 6-12 months. Be positive and try to live in the moment without thinking about the long term. 80% is pretty high to outgrow it. We did prednisone and when we took her off of it we saw her numbers go down. If they treat other ways, you have lots of options.

Good luck and keep us posted!!

hello....plz help did ur son ever recover from itp?? my son is 1 and a half been 6 months and is not getting better plz w/b at cutestbabygirl05@yahoo.com thank you... im just freaking out.....