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Urgent Administration of Romiplostim in Life-Threatening Bleeding Associated with ITP

10. 8. 2022

Recommendations for the care of acute, especially major bleeding in individuals with severe immune thrombocytopenia (ITP) are not entirely clear. Various methods (steroid boluses, intravenous immunoglobulins, platelet transfusions, etc.) can be used to quickly increase platelet count, but the response may not always be straightforward. Recently, reports have been published regarding the use of thrombopoietin receptor agonists (TPO-RAs) specifically aimed at rapidly increasing platelet count. The use of romiplostim in patients with ITP and intracranial hemorrhage is illustrated by the following case studies.

Case 1

A 29-year-old man was admitted to the hospital for sepsis with eosinophilic pneumonia. A blood test revealed thrombocytopenia (31 × 109/l). Bone marrow involvement and an autoimmune systemic disease were ruled out. Physicians considered the diagnosis of secondary ITP accompanying a severe overall condition; the patient was given intravenous immunoglobulins (IVIG) along with antibiotics and antiparasitics.

On the 6th day after admission, spontaneous intracerebral and abdominal bleeding developed. The CT scan showed bleeding in the right frontal lobe with surrounding edema and a large hematoma in the area of the left adrenal gland. ITP treatment was expanded to include corticosteroid boluses and platelet transfusions to achieve a platelet count of > 100 × 109/l.

Over the next 6 days, the condition worsened; status epilepticus appeared, the Glasgow Coma Score reached 8, and persistently elevated intracranial pressure was measured. A new brain CT showed an enlarging frontal hematoma and worsening surrounding edema. For this reason, a mini-craniectomy with hematoma evacuation was performed, but massive platelet transfusions failed to prevent perioperative bleeding.

The day after surgery, romiplostim administration was started. Stable response with a platelet count above 100 × 109/l was achieved by the 8th day after the first dose of romiplostim, and no further bleeding complications were noted. The patient was discharged 2 months after admission with persistent brachiofacial paresis.

Case 2

A 14-year-old adolescent girl with a history of chronic ITP (duration of illness 3 years) was admitted to the hospital for gum bleeding, drowsiness, and intense headaches. Blood tests showed severe thrombocytopenia (13 × 109/l) and anemia with hemoglobin (Hb) of 80 g/l. A brain CT scan showed a large right-sided parietooccipital hematoma with significant surrounding edema. At that time, a bolus of corticosteroids, IVIG, and platelet transfusions was given.

On the 3rd day after admission, the neurological condition further deteriorated, with seizures, coma, and anisocoria. The patient was induced into a barbiturate coma, corticosteroid therapy was intensified, and massive platelet transfusions were administered around the craniotomy for hematoma evacuation. Despite all administered treatments, the platelet count remained < 15 × 109/l, and the operation was complicated by significant subcutaneous bleeding.

The day after surgery, romiplostim administration was started, and a sustained platelet count response was achieved by the 6th day after its administration. 18 days after admission, the girl was discharged from the intensive care unit and fully recovered over the following months.

Discussion and Conclusion

In cases of major or life-threatening bleeding, achieving a stable and safe platelet count can be very challenging in ITP, even with a combination of various approaches. Second-line treatments (especially immunosuppressants) are often not feasible for critically ill patients. The presented cases show that the administration of romiplostim in an emergency situation can lead to a prompt and sustained response even in patients unresponsive to other treatments. The time to treatment response was short, and no serious adverse events were observed. The intensity of the platelet count increase may depend on the dose of romiplostim, as suggested by some other published experiences.

(eza)

Source: Gellens R., Habchi S., Freppel S. et al. Romiplostim for the emergency management of severe immune thrombocytopenia with intracerebral hemorrhage. Front Neurol 2018; 8: 737, doi: 10.3389/fneur.2017.00737.



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Authors: prof. MUDr. Tomáš Kozák, Ph.D., MBA

Authors: prof. MUDr. Tomáš Kozák, Ph.D., MBA

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