Combining thrombopoietin receptor agonists with immunosuppressive drugs in adult patients with multirefractory immune thrombocytopenia, an update on the French experience.

Combining drugs could be an effective option for treating multirefractory ITP, that is, patients not responding to rituximab, thrombopoietin receptor agonists (TPO-RA) and splenectomy. We conducted a retrospective, multicenter, observational study including multirefractory ITP patients who received a combination of a TPO-RA and an immunosuppressive drug. We included 39 patients (67% women, median age 59 years [range 21-96]), with a median ITP duration of 57 months [3-393] and a median platelet count at initiation of 10 × 109 /L [1-35]. The combination regimen was given for a median duration of 12 months [1-103] and included eltrombopag (51%) or romiplostim (49%), associated with mycophenolate mofetil (54%), azathioprine (36%), cyclophosphamide (5%), cyclosporin (3%) or everolimus (3%). Overall, 30 patients (77%) achieved at least a response (platelet count ≥30 × 109 /L and at least doubling baseline during at least 3 months), including 24 complete responses (platelet count >100 × 109 /L during at least 3 months) with a median time to response of 30 days [7-270] and a median duration of response of 15 months [4-63]. Severe adverse event related to ITP treatment was observed in 31%. In conclusion, this study confirms that some patients with multirefractory ITP can achieve long lasting response with this combination.

Giant cell arteritis presenting as a breast lesion: report of a case and review of the literature.

Giant cell arteritis (GCA) mainly affects arteries arising from the external carotid arteries, although it may also be responsible for extracephalic clinical presentation related to impairment of vessels other than the carotids, such as the aorta as well arteries of the lower and upper limbs (10%-30% of cases). However, breast involvement is considered to be a rare complication of GCA. We recently observed a case of particular interest as the patient developed superficial mammary arteriolitis, bilaterally, revealing GCA with favorable outcome after institution of steroid therapy. Our case report reinforces the possibility of an unusual presentation of GCA. Indeed, we suggest that an evaluation for GCA affecting the breast should be made in elderly women exhibiting breast nodule(s)/mass(es) associated with other features of GCA (that are: headache, jaw claudication, constitutional signs, polymyalgia rheumatica). Furthermore, we also suggest that the prevalence of breast impairment may be underestimated in GCA as the breast is supplied mainly by 2 branches of the subclavian artery (ie, the internal mammary artery [as in the present case report] and the lateral thoracic artery), which is often involved in GCA.