Abstract Number: PB1009
Meeting: ISTH 2021 Congress
Background: A 4 Years -old boy was admitted in hospital due to gum bleeding in 2010. Laboratory studies performed including CBC, PT, PTT, INR and BMA. There was no hepatosplenomegaly and lymphadenopathy and fever in examination. PT, PTT, INR were normal.
Aims: The patient diagnosed as idiopathic thrombocytopenic purpura. He received IVIG and discharged in good condition with plt=40000/mm3. In addition, he had multiple admissions (20times) due to petechiae, epistaxis, gum bleeding and low pIT count until 2017.
Methods: He received in these admissions IVIG 8 times, steroid 3 periods (1-3 weeks), anti-D for 4 weeks and rituximab for
4 weeks. He responded to treatments as platelet count increase from 30000/mm3 to 80000/mm3. But after few weeks or months platelets drop again below 10000/mm3.
On 2018 the patient referred and admitted in pediatric surgery ward of our hospital for splenectomy with diagnosis chronic refractory ITP. Before surgery, our surgeon requested pediatric hematology consultation.
There were 4 important points in review of patient’s history:
– cousin marriage in his parents.
– Presence of low PIT in patients cousin
– History of repeated bruising from infancy
– Lack of favorable response to ITP treatment
These clues help me think about hereditary thrombocytopenia.
Results: Since BSS is the second most recognized inherited sever PLT disorder among hereditary thrombocytopenia and considering that in 9 patients registered in our center with BSS 6 cases had been treated and followed-up as ITP for a long time, I though bout BSS in this patient.
Therefore I requested peripheral blood smear and PLT aggregometry test for the patient that disclosed the diagnosis of BSS.
Conclusions: Inherited thrombocytopenia especially BSS has very close similarities to ITP, therefore BSS should be considered in differential diagnosis of persistent and refractory ITP to avoid inappropriate therapies and it’s cost and complications.
To cite this abstract in AMA style:Mirbehbahani N. Importance of Consideration to Differetial Diagnosis of ITP [abstract]. Res Pract Thromb Haemost. 2021; 5 (Suppl 1). https://abstracts.isth.org/abstract/importance-of-consideration-to-differetial-diagnosis-of-itp/. Accessed September 16, 2021.
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