A female patient, who has been diagnosed as a case of ITP, presented in the medical OPD with bleeding gums and menorrhagia.
She is on prednisolone 5 mg two tablets twice daily and Eltrombopag (Revolade) 25 mg once daily for the past one year.
Her current platelet counts are 2000/ul.
How should we manage this patient?
Patient is already using steriods and eltrombopag and still have bleeding and low platelets count.This patient is candidate for second line treatments.
Splenectomy, rituximab, or a thrombopoietin receptor agonist (TPO-RA) are the three principal choices of second-line treatment; they differ in their mechanism of action. All three are effective in raising the platelet count in the majority of individuals, but they differ dramatically in their application, costs, burdens, and adverse effects profiles. Splenectomy is a one-time permanent surgical procedure that is most likely to result in a durable response; rituximab requires several intravenous administrations and may need to be re-administered; TPO-RAs typically require long-term administration, although responses are sometimes maintained even after stopping treatment. Splenectomy carries operative and other risks.choice of treatment require patient prefrence.
*Splenectomy may be a good choice for an individual who wishes to have a single potentially curative surgical procedure and who is willing to accept the increased risks of infection and venous thromboembolism. Laparoscopic splenectomy has a lower surgical mortality and complication rate, shorter hospitalization, and faster recovery.
Splenectomy is considered for any patient who does not respond to steroids,inability to tapper steriods,or recurrent itp refrectory to second line agents.spleenectomy has durable response rate over 50%.
If splenectomy is chosen, it is generally preferable to wait at least one year from the time of diagnosis in case a spontaneous remission occurs.
*Rituximab may be a good choice for an individual who wishes to avoid surgery and prefers not to take a medication long term.
*A TPO-RA may be a good choice for an individual who is especially concerned about immunosuppression following splenectomy or rituximab and who is less concerned about the need to take a long-term medication, including the associated costs and burdens.
*IVIG and anti D temporarily increases platelet counts(duration upto 3 weeks or rarly longer) although serial ivig and anti D treatment may allow adult patients to delay spleenectomy.
*Other options for persistent or worsening itp includes use of immunosuppresive drugs like Azathioprine,retuximab,cyclosporine ,danazol,MMF but these carry side effects of immunosuppression.
*Fostamatinib is a small molecule prodrug of a tyrosine kinase inhibitor that inhibits the spleen tyrosine kinase.It was approved by the US Food and Drug Administration in mid-2018 for the treatment of thrombocytopenia in adults with chronic ITP who have had an insufficient response to a previous treatment.Evidence is more limited than for splenectomy, rituximab, or TPO-RAs, but high-quality evidence for efficacy and safety is emerging, making it a reasonable choice among third-line treatment options.
How can we prepare this patient for splenectomy?
Pre-splenectomy considerations —
●Immunizations – Patients considering splenectomy should receive immunizations for encapsulated organisms at least two to four weeks prior to the procedure if possible and if these were not done already. These individuals are often prescribed antibiotics to take in the event of fever.Splenectomy increases the risk for serious, including life-threatening, infections, especially with encapsulated organisms such as Streptococcus pneumoniae, Haemophilus influenzae, and Neisseria meningitidis, and vaccination against these organisms may reduce the risk.
For individuals who do not receive pre-splenectomy vaccinations for any reason, there is evidence that vaccination induces adequate antibody responses when given approximately 14 days after the procedure.
●Optimizing the preoperative platelet count – If the platelet count needs to be increased for splenectomy, the patient can be treated with intravenous immunoglobulins (IVIG), glucocorticoids, or a thrombopoietin receptor agonist (TPO-RA). We prefer to perform splenectomy with a platelet count of ≥50,000/microL; however, many patients with ITP have undergone open or laparoscopic splenectomy safely in the setting of more severe thrombocytopenia, with platelets available for transfusion if urgently needed because of intraoperative bleeding.
*An accessory spleen can be detected using various imaging modalities (eg, ultrasound, computed tomography).