Romiplostim in Life-Threatening Bleeding Situations − Case Reports

Case Report 1

A 65-year-old man, who was first diagnosed with steroid-responsive ITP three years ago, suddenly developed large bruises. Upon admission, severe thrombocytopenia (3.0 × 10⁹/L) was detected, and the condition was assessed as an exacerbation of chronic ITP. The patient was immediately treated with high-dose intravenous immunoglobulins (IVIG), prednisolone (40 mg daily orally), and romiplostim (1 μg/kg). Over the next four days, he developed respiratory failure, with CT scans indicating alveolar hemorrhage. The man's medical history included treatment for atypical mycobacteriosis. On the 11th day of hospitalization, the romiplostim dose was increased to 10 μg/kg, and he received bolus doses of methylprednisolone (1 g daily for three days) and a second cycle of IVIG. The platelet count normalized on the 21st day of hospitalization, and the platelet count remained stable during the following year under chronic therapy with 12.5 mg of prednisolone daily.

Case Report 2

A 77-year-old man was admitted to the hospital for gingival bleeding, hemoptysis, and dyspnea. Three months before admission, a chest X-ray revealed consolidation of the right lung field, and two days before admission, he underwent a transbronchial lung biopsy, which described cryptogenic organizing pneumonia. Upon admission, severe thrombocytopenia (6 × 10⁹/L) was detected, and the patient was diagnosed with acute ITP with alveolar hemorrhage. Immediate treatment with IVIG and corticosteroids was initiated; however, the patient's condition worsened on the second day of hospitalization with increased dyspnea. At this point, treatment with a higher initial dose of romiplostim (10 μg/kg) was started. Over the following week, respiratory symptoms normalized, and platelet count rose to 9.0 × 10⁹/L. A month after admission, the man was discharged without symptoms to home care.

Case Report 3

A 71-year-old man was admitted due to dyspnea and hemoptysis. His medical history included Stevens-Johnson syndrome caused by sulfasalazine and pulmonary emphysema. Shortly after admission, respiratory failure necessitated non-invasive support. At this time, a low platelet count (2.0 × 10⁹/L) was detected, although a normal blood count was documented two months earlier. Pulmonary CT showed ground-glass opacities, without signs of acute infection. The condition was diagnosed as acute ITP with alveolar hemorrhage. The patient was treated with bolus doses of methylprednisolone for five consecutive days and IVIG. However, respiratory deterioration progressed rapidly over the next two days. At this point, administration of romiplostim at a dose of 10 μg/kg was initiated. Over the next four days, the platelet count increased to 25 × 10⁹/L, and signs of hemoptysis and dyspnea disappeared.

Discussion and Conclusion

The causes of severe diffuse alveolar hemorrhage can vary, with ITP being one potential cause. The prognosis of DAH associated with thrombocytopenia is particularly poor. Romiplostim can be added in therapy, though standard initial doses may not be sufficient in these situations. Higher initial doses of romiplostim (6−9 μg/kg) may be necessary in patients with severe acute or persistent ITP who are resistant to conventional treatment.

The presented case reports demonstrated a rapid response. Despite the increase in platelet count to 600 × 10⁹/L, no thrombotic complications were noted in the following period. Early and higher dosing of romiplostim may be effective in potentially fatal bleeding situations.

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Source: Nagaharu K., Masuya M., Kawakami K., Katayama N. Successful management of immune thrombocytopenia presenting with lethal alveolar hemorrhage. Case Rep Hematol 2019; 2019: 5170282, doi: 10.1155/2019/5170282.