El Maachi Nora, El Mehdi Mahtat, Filali Imane Ait, Jennane Selim, El Maaroufi Hicham, Doghmi Kamal
Service d'Hématologie Clinique, Hôpital Militaire d´Instruction Mohamed V, Rabat, Maroc.
Pan Afr Med J. 2022 Jul 13;42:201. doi: 10.11604/pamj.2022.42.201.32754. eCollection 2022.
The combination of erythroblastopenia and primary myelofibrosis is very rare. We here report the unusual case of a 76-year-old Moroccan patient followed up since 2018 for idiopathic erythroblastopenia, initially treated with corticotherapy and then with ciclosporin. Two years later, the patient reported bone pain with splenomegaly. Assessment including myelogram, bone marrow biopsy and molecular biology showed myelofibrosis. Etiological assessment of myelofibrosis was negative confirming its primitive nature. The patient received ruxolitinib with transfusion support. Patient´s outcome was favorable and marked by improvement of general condition, splenomegaly and transfusion rate. The association between erythroblastopenia and myeloproliferative disorder is exceptional and only a few cases have been reported in the literature.
红细胞生成减少症与原发性骨髓纤维化并存的情况极为罕见。我们在此报告一例不寻常的病例，一名76岁的摩洛哥患者自2018年起因特发性红细胞生成减少症接受随访，最初接受皮质激素治疗，随后使用环孢素治疗。两年后，患者出现骨痛并伴有脾肿大。包括骨髓检查、骨髓活检和分子生物学在内的评估显示存在骨髓纤维化。骨髓纤维化的病因评估结果为阴性，证实其原发性。该患者接受了芦可替尼并辅以输血支持。患者的预后良好，总体状况、脾肿大和输血率均有改善。红细胞生成减少症与骨髓增殖性疾病之间的关联极为罕见，文献中仅报道了少数病例。
