Diagnosis

Hi Everyone, I have a question about diagnosis. My hematologist said he was diagnosing ITP. After further research I see it is a diagnosis of exclusion. He ran labs to check PTT, Prothrombin time and hiv. All were normal. WBC is a little low. It seems I could ask for autoimmune testing, I read there is a marker all with ITP will have. I feel like more tests should have been done? My integrative dr, did find low nk 57 wbc count and low immunoglobulin subclass 3. The hematologist could not answer why and also said that shortness of breath and heart palpitations were unrelated to the platelet count. I am going to see a cardiologist about that tomorrow. Main question: what tests are essential to diagnose ITP? Is there a diagnosis code for it because I see on my record it says thrombosis, unspecified. He told me ITP so I am confused and will follow up with his office, maybe they entered the diagnosis wrong. Thanks!

Jen:

Protocol states:

Diagnostic tools for adults and children with suspected ITP were grouped into 3 sections of recommendation. A presumptive diagnosis of ITP is made when the history, physical examination, complete blood count, and examination of the peripheral blood smear do not suggest other etiologies for the thrombocytopenia. There is no “gold standard” test that can reliably establish the diagnosis. Response to ITP-specific therapy, for example, intravenous immunoglobulin (IVIg) and intravenous anti-D, is supportive of the diagnosis, but a response does not exclude secondary ITP.


Physical examination

Physical examination should be normal aside from bleeding manifestations. Mild splenomegaly may be found in younger patients, but moderate or massive splenomegaly suggests an alternative cause. Constitutional symptoms, such as fever or weight loss, hepatomegaly, or lymphadenopathy might indicate underlying disorder such as HIV, systemic lupus erythematosus (SLE), or a lymphoproliferative disease.

Peripheral blood count

ITP is characterized by isolated thrombocytopenia with an otherwise normal complete blood count. Anemia from blood loss may be present, but it should be proportional to the amount, and the duration, of bleeding and may result in iron deficiency (evidence level IV). If anemia is found, the reticulocyte count may help define whether it the result of poor production or increased destruction of red blood cells (RBCs).

Evaluation of peripheral blood smear

Evaluation of the peripheral blood smear by a qualified hematologist or pathologist is paramount to the diagnosis of ITP. This may demonstrate abnormalities that are not consistent with ITP, such as schistocytes in patients with thrombotic thrombocytopenic purpura–hemolytic uremic syndrome, or leukocyte inclusion bodies in MYH9-related disease. Excessive numbers of giant or small platelets may indicate an inherited thrombocytopenia. Pseudo-thrombocytopenia due to ethylenediaminetetra acetic acid (EDTA)–dependent platelet agglutination should also be excluded.

Bone marrow examination may be informative in patients older than 60 years of age, in those with systemic symptoms or abnormal signs, or in some cases in which splenectomy is considered. Both a bone marrow aspirate and a biopsy should be performed. In addition to the morphologic assessment, flow cytometry and cytogenetic testing should be considered (evidence level IIb-IV). Flow cytometry may be particularly helpful in identifying patients with ITP secondary to chronic lymphocytic leukemia (CLL).

Helicobacter pylori testing

The detection of H pylori infection, preferably with the urea breath test or the stool antigen test, should be considered in the work-up of adults with typical ITP where it may have clinical impact20 (evidence level IIa). Serologic detection may be used but is less sensitive and less specific than the other tests; furthermore, the test may produce false positive results after IVIg therapy. Except in high-prevalence areas, the literature does not support routine testing in children with ITP.

HIV and HCV testing

The thrombocytopenia associated with HIV and hepatitis C virus (HCV) infections may be clinically indistinguishable from primary ITP and can occur several years before patients develop other symptoms. Routine serologic evaluation for HIV and/or HCV infection in adult patients with suspected ITP, regardless of local background prevalence and personal risk factors documented in the patient history, is recommended. Control of these infections may result in complete hematologic remission.

www.bloodjournal.org/content/115/2/168.full?sso-checked=true

I'm not sure what 'marker' you are referring to. There really isn't one. Some people with ITP do have detectable antibodies but as of now, that is not considered to be a reliable way to diagnose ITP. There really isn't a list of tests to diagnose ITP and by exclusion, they generally mean in the absence of any physical symptoms unrelated to ITP or problems with the blood smear other than low platelets.

Hi Jen,

what is your current count?
In my case the diagnosis was pretty simple. I had a count of 0 and they gave me steroiods. They said if steroids work you have ITP. If not we need to look further since the low count will than have a different reason.

Best regards,
Joerg

They can usually tell ITP from the blood smear or blood film, whichever they call it. If it's ITP then everything is normal except few platelets which are all large as they are all new. Few small platelets is something else. Some other anomaly with other cells is something else. Then as Joerg says if the steroids or IVIG don't work they will probably do a bone marrow biopsy to check for other things.

My count was 80 last week, up from 54. My integrative dr put me on antibiotics for a lyme relapse. They are not going to treat ITP unless it gets below 50 and treatment would be prednisone. I was hoping they would go straight to ivig because of lyme disease and don't want to take steroids. I already have low immunoglobulins so I feel I might qualify for ivig even without ITP. The hematologist doesn't recognize chronic lyme so I have a second opinion hematology appointment next week just to see if I can line up a possibility of other treatment if I need it for the ITP. I'm also having shortness of breath, arrythmias and they are checking my heart now. I think the antibiotics helped my count but its possible it just goes up and down? We've only been watching it since May and it was downward each check until I started the antibiotics. Thanks for your input.

My doc always says that everything above 50 is luxury.

I'm closely monitored between 30 and 50. Treatment usually only starts below 30.
I can't really recommend anything, but I personally prefer not to take any medication.

Cheers,
jw

Jen:

Yes, counts go up and down a lot with ITP. Counts can change that much in a day. It's good that yours are going up. 50k is still a good count and most would not treat at that number unless there were symptoms, but you have to do what you are comfortable with. IVIG is known to be a very temporary treatment; it normally only lasts a few days so you might want to take that into consideration.

If your counts are truly down due to Lymes, then it could be possible that the antibiotics that treat Lymes could cause your counts to rise.

Lyme carditis could explain palpitations and shortness of breath. Is that what they are looking at? Is that why they diagnosed Lyme relapse?

I would ask the question of your doctor whether the presence of Lyme disease would affect the decision to treat ITP with prednisone, which is immunosuppressing.

Good article about Lymes if you haven't already seen it.

www.envita.com/lyme-disease/necessary-treatment-for-chronic-lyme-disease

From Sandi's article:

Steroidal therapy that is often administered to patients who suffer from autoimmune diseases can be particularly harmful to Lyme patients. Steroidal therapy can suppress patients' immune systems, allowing the Borrelia and other co-infections to grow, rather than attacking the infections.

No, the hematologist doesn't think its lyme causing the ITP. The dr who originally treated me for lyme is an integrative MD and he is looking at clinical symptoms, cd 57 nk cell test very low and past positive lyme (late stage untreated neuro lyme). She also thinks I have babesia. The cardiologist asked about the lyme but since its already been treated I doubt he will consider lyme carditis. I live in SC which is not considered endemic for lyme and I had to see the out of network doctor to even get a western blot test to begin with. I have an abnormal ecg rhythm and will do a stress test Wednesday. I have on a monitor right now and they can see that my heart rate is too high and I have arrhythmia so they called in a beta blocker, atenolol. I took half, I'm to build up over a few days but I then read that a side effect of atenolol can be lowered platelets! I'll call him tomorrow to make sure but we discussed the ITP/platelet issue so hopefully its ok. If not, other beta blockers?

I am seeing a new hematologist on Wednesday to discuss how steroids might impact me with lyme and low immunolglobuilins already. I want a plan in place in case they drop very low.

This is what my lyme dr said too. She wants me to talk to the hematologist about other possibilities for treatment, especially ivig since my immunoglobulins are low anyway.

Jen, I would be much more concerned about the possibility of lyme carditis than your ITP. The coincidence of having a relapse of lyme and new symptoms consistent with lyme carditis is compelling, and lyme carditis is a serious complication: sometimes the arrhythmias are severe enough to require temporary use of a pacemaker.

ITP does not require treatment unless counts go much lower, especially if you are not having symptoms other than minor bruising. Let's hope that your counts and bleeding symptoms do not demand treatment, but if they do, I would push hard against using steroids or other immunosuppressives. IVIG is very expensive, and you may get pushback from insurance. If your platelets do drop, your hematologist can make the argument that steroids are contraindicated due to your lyme disease, but a firm diagnosis of lyme carditis would be an especially convincing argument.

Hopefully the antibiotics will work quickly, the cardio symptoms will disappear, and the lyme treatment will be complete before you need any treatment for low platelets.

For what it's worth, human monocytic ehrlichiosis and rocky mountain spotted fever are both tick-borne. Both are endemic to the Southeast, and both are associated with carditis. Both are known to cause thrombocytopenia, but lyme is not known to be a cause (though controversy exists that it may be an autoimmune trigger).

Please keep us updated.

Jen:

I don't know a whole lot about Lymes, but I've read enough articles to know that even if treated, the affects of Lymes can go on for a long time, even with negative test results.

well.blogs.nytimes.com/2013/07/08/when-lyme-disease-lasts-and-lasts/?_r=0

With all that you have going on, I'd consider IVIG too and refuse steroids. As Rob said, you may not even need treated for ITP. The fact that your counts went up a bit with the antibiotics may be an indication that your platelet count may be related to Lymes. There was another girl here with Lymes and ITP who posted some good articles about it. I'll try to find them.

Here is a whole thread that you might find interesting:

pdsa.org/forum-sp-534/6-general-itp-discussion/28117-is-it-lyme-disease-or-just-bruising.html#42057

Hi, I am doing a lot better since starting the beta blocker Friday night. The heart pain, shortness of breath and palpitations are now much less frequent. Also my headache went away.

Thank you guys for the great information.

Hi, I wanted to give an update. I met for a second opinion with a new hematologist and he agreed he would not use steroids if my counts fall too much and I need treatment. He thinks I might do well with n plate if treatment is needed. He said IVIG is not dramatic platelet increase but he would consider it.

He ran hepatitis tests, normal, EBV which was high and said he might do bone marrow biopsy if it continues to drop. I got abdominal ultrasound. I go back Thursday.

My stress echo was good, so cardiologist said abnormal ekg was wrong and he is waiting to see results of the monitor about the arrhythmias. He can't see a reason for my fatigue related to my heart.

It is good to feel that I am working this out and lining up a treatment that would be best for me should it come to that. I feel empowered to be taking action and hopeful that I will be feeling better very soon.

Good for you,Jen! It does help to take control. Keep us posted!

I had a bone marrow biopsy and it shows some hypersegmented neutrophils and slightly increased and atypical megakaryocytes. I meet with the dr in a couple of weeks but will call tomorrow to see if I can go ahead and get my b12 and folate levels checked as it seems that deficiency will need to be clinically ruled out. Everything else looked good and if it were MDS it would be very low, early level. Thoughts? Still in lyme/babesia treatment. Echocardiogram was normal and e monitor still outstanding. Counts were up to 78 at last visit, much better.