Eddou Hicham, Zinebi Ali, Khalloufi Abdelaziz, Sina Mohammed, Mahtat Mehdi, Doghmi Kamal, Mikdame Mohammed, Moudden Mohammed Karim, Baaj Mohammed El
Service de Médecine Interne, Hôpital Militaire Moulay Ismail, Meknès, Maroc.
Faculté de Médecine et de Pharmacie de Fès, Maroc.
Pan Afr Med J. 2017 Jan 23;26:32. doi: 10.11604/pamj.2017.26.32.9215. eCollection 2017.
Acquired amegakaryocytic thrombocytopenic purpura is a very rare condition characterized by severe thrombocytopenia linked to the reduction or disappearance of megakaryocytes in the bone marrow. It may be primary idiopathic or secondary to many pathological conditions including hematologic disorders. We report the case of a 24-year-old patient admitted for haemorrhagic syndrome caused by immunological thrombocytopenic purpura. The diagnosis was acquired amegakaryocytosis after the failure of corticotherapy and the performance of myelography. The patient was treated with ciclosporin with rapid progression to acute myeloblastic leukemia. The progression of acquired amegakaryocytosis to acute leukemia is reported but it is generally not so rapid and above all it is preceded by myelodysplastic syndrome or medullary aplasia. This study highlights the importance of a close follow-up of these pathologies with a benign-like appearance.
获得性无巨核细胞性血小板减少性紫癜是一种非常罕见的疾病，其特征是严重血小板减少，与骨髓中巨核细胞减少或消失有关。它可能是原发性特发性的，也可能继发于包括血液系统疾病在内的许多病理状况。我们报告一例24岁因免疫性血小板减少性紫癜导致出血综合征入院的患者。在皮质激素治疗失败并进行脊髓造影后诊断为获得性无巨核细胞血症。该患者接受环孢素治疗后迅速进展为急性髓细胞白血病。获得性无巨核细胞血症进展为急性白血病已有报道，但通常不会如此迅速，最重要的是，在此之前会有骨髓增生异常综合征或骨髓再生障碍。本研究强调了对这些看似良性的疾病进行密切随访的重要性。
