Vývoj koagulačních markerů chronické diseminované intravaskulární koagulace (DIK) u pacienta s mnohočetnou angiomatózou v průběhu léčby antiangiogenními léky: interferonem alfa, thalidomidem a lenalidomidem
| Title in English | Chronic disseminated intravascular coagulation (DIC) markers in a patient with multiple angiomatosis during treatment with antiangiogenics: interferon alpha, thalidomide and lenalidomide |
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| Authors | |
| Year of publication | 2012 |
| Type | Article in Periodical |
| Magazine / Source | Vnitřní lékařství |
| MU Faculty or unit | |
| Citation | |
| Field | Neurology, neurosurgery, neurosciences |
| Keywords | hemangiomatosis; angiomatosis; thalidomide; lenalidomide; D-dimers; chronic disseminated intravascular coagulation; PET-CT imaging |
| Attached files | |
| Description | Multiple angiomatosis is a rare disease with angiomatous formations in multiple organs and tissues and associated with a risk of fatal bleeding. Case description: In this patient, the bones, pleural and peritoneal cavities and digestive tract were involved. The patient had long-term been administered zoledronate that provided relief from bone pain as early as after the second dose. The effect of antiangiogenics was evaluated on CT and MRI. Since angiomatous proliferation is associated with chronic disseminated intravascular coagulation (DIC) and anaemisation, blood count and fibrinogen as well as D-dimer and soluble fibrin monomer concentrations are also used to assess treatment response. Results: Before treatment, D-dimer levels were in excess of 20 micro g/mL, fibrinogen 1.4 g/L and soluble fibrin monomers were at measurable levels. During treatment with interferon alpha at a dose of 6 million units 3 times a week with the dose reduction after 10 month, the median fibrinogen concentration increased to 1.5 (1.2 – 2.0) g/L, the median D-dimer levels declined to 17.2 (13.4 - 20.0) micro g/mL and fibrin monomers were still detectable. Thalidomide therapy (100 mg/day) provided reduction in the median D-dimer levels to 6.07 (4.71 - 10.21) micro g/ml and increase in median fibrinogen concentration to 1.9 g/L; soluble fibrin monomers were unidentifiable. CT imaging suggested significant reduction of angiomatous mass. Progressing neuropathy required dose reduction of thalidomide to 50 mg/day, leading to D-dimer increase. Lenalidomide 10 mg/day provided an increase in median D-dimer concentration to 10.8 (10.8 – 17.35) and decline in the level of haemoglobin to a median of 124 (135 – 117) g/L. Soluble fibrin monomers became detectable again. Therefore, a low dose of lenalidomide 10 mg/day was combined with thalidomide 100 mg and, subsequently, 50 mg/day. Treatment with lenalidomide 10 mg and thalidomide 50 mg provided median D-dimer levels of 9.32 and the disease has remained stable for 9 months. Conclusion: Thalidomide 100 mg/day stabilized multiple angiomatosis better than interferon alfa. Thalidomide 50 mg/day was insufficient to maintain disease stability. Lenalidomide at a dose of 10 mg was tolerated really well but this dose was insufficient to maintain low D-dimer levels and normal haemoglobin concentrations. The combination of lenalidomide 10 mg and thalidomide 50 mg daily stabilized the disease for 9 months. |
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