Thrombocytopenia

Immune thrombocytopenic purpura or Idiopathic thrombocytopenic purpura is one of the commonest hematological problems in pediatrics . Most of the cases resolve without any treatment .Major complications occur in one percent of cases. Our challenge is to exclude other serious disorders which may look like ITP ,pick up dangerous complications early and manage . There are guidelines for management of new cases subacute and chronic cases. Once in a while we come across cases which pose challenges in the diagnosis and management . One such a case now under treatment in our ICU is presented here for expert comments .

Five year old boy was referred as a case of ITP one month back .Born out of non consanguinous marriage with no family history of excessive bleeding tendency . No significant bleeding during his vaccination teeth eruptions or minor falls . He developed ecchymotic patches over body and epistaxis. No joint or deep bleeds , no hemetemesis or malena , no evidence of CNS bleeds.

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As he did nt have further bleeds he was not given drugs initially .
While other tests done as part of work up
HIV Negative
Coombs test positive
ANA Positive significant titres
As he he had further epistaxis and one count came below 10000, we did a bone marrow examination and he was put on prednisolone 2mg per kg
Bone marrow was consistant with ITP but few atypical findings. There were evidence of hemophagocytosis .
He responded to steroids. No further bleeds and his platelet count steadily increased.
He was send home with frequent follow up , and the steroid were tapered
while on tapering he developed new eccymotic patches and mucosal bleeds. He is admitted again

ESR                                  8mms
Quantitative CRP              28

Coombs test Positive , Repeat test also .
Corrected Reticulocyte count      1.5 
ANA by flurescent method .. Positive homogenous pattern , Repeat test also 

As the bleeding this time is continuing and the platelet count is 7000 he was given platelet transfusion and is steroid dose again hiked to 2mg per kg
Since yesterday his bleeding stopped , Platelet count is now above 10000.
How to proceed further ?
In short he is a case of Aquired primary hemostatic disorder , out of which number of platelt is the problem.
Did we rule out congenital problems for sure........Not hundred percent. remote possibility of Von villibrand type II is there. Other inherited thrombocytopenias and platelet function disorders less likely . The peripheral smear report from an expert pathologist comments as normal morphology chances of hereditary functional disorders with thrombocytopenias less eg Bernad soulier , May hegglin anomaly.. Glanzman disorder usually presents earlier and severe. In case of Glanzmans totally normal past history wtithout majro bleeds unlikely. That point practially rules out all cases of hereditory disorders.
Out of the aquired disorders of thrmbocytopenia , other most important task is to rule out malignancies . Yes in our case unlikely that we have missed malignancies like Acute leukemia or lymphoma or neuroblastoma ,We did a marrow examination and already one month over after diagnosis.. No fever, No worsening of pallor , no organomegaly so far .

So .We are justified to make diagnosis of ITP.
But few questions un answered.

1. Why he is symptomatic after a course of steroids. and platelet count which showed inital response is now going down to significant extents, at the end of a month of treatment ?.We checked the compliance of treatment .
Is it just a normal course of ITP , what percentage of ITP can take such a course. ie rather than branding as subacute or chronic.. just a persistance of problem for few more week.Just a need for sterods for a few more week.
This is just as a parent having a false hope in any serious issue. What is the chance that my child is likely to respond if we continue few more days with the treatment sir ? I know your guidelines does nt support my view ?.. Genuine argument indeed .
2.As a physician can i adopt that policy , ie wait for few more weeks hoping he ll respond with steroids one more week or two without investigation.
I dont think. He need to have work up now .
Why ?
There are few Odd features here
1. His ANA is positive.
"Hey ANA can be false positive "
"No, Here we did a ANA with flurescence method . and the pattern of uniform flurescence is documented. that can not be ignored "
2. His Coombs test is positive
" Sir why worry, his hemoglobin is not dropping. i agree it is a bit low.His corrected reticulocyte count is just 1.5. You did nt do LDH. His peripheral smear did nt show hemolysis. so why worry ?
No dear. Coombs test positivity alone without hemolysis also to be viewed significant .As you said we have send for LDH we are waiting for it "
In this context what possibility and how to proceed.
What is the chance for Systemic lupus erythematosis. Indeed very rare in this age. But not impossible.
He does nt have many classical features. No fever. No arthritis or arthralgia. No skin or mucosal ulcers. Hair normal Systemic examination normal .
He has just thrombocytopenia persistant and postive coombs and significant ANA
Should we consider some underlying problems which make SLE possible in a young child . eg early complement deficiency ?
Yes i think
What we have done and our plan
We have sent for Ds DNA, LDH. ESR and quantitative CRP
(Few more odd finding is his ESR not high, His wbc count is a bit higher side , both are unusual in SLE )
Planning estimate early complement compoents
UPDATE  ON 9/1/2018
He was sent to CMC Vellore .Worked up from there and discharged last week