Falé Cláudia, Araújo Ana, Lourenço Sílvia, Pais Maria
Serviço de Medicina. Hospital do Espírito Santo. Évora. Portugal.
Acta Med Port. 2013 Sep-Oct;26(5):613-6. Epub 2013 Oct 31.
Acute Panmyelosis with myelofibrosis is a rare and aggressive acute myeloid leukemia accounting for 2% of all acute leukemias. Clinically, it is characterized by an acute onset of cytopenias associated with extensive bone marrow fibrosis in the absence of splenomegaly and related morphological changes in the red blood cells such as dacryocytes. The presence of fibrosis further complicates the correct diagnosis of this rare disease, being important to differentiate this entity from other hematologic malignancies such as other acute myeloid leukemias and myelodysplastic syndromes associated with myelofibrosis. We report a case of a young adult of 16 years old, admitted with severe pancytopenia. The exposure of this case is justified by the rarity of this pathology associated with few cases reported in medical literature, allowing for a clinical sharing and a better understanding of the different clinical aspects of this entity of difficult diagnosis.
急性全髓增殖症伴骨髓纤维化是一种罕见且侵袭性的急性髓系白血病，占所有急性白血病的2%。临床上，其特征为血细胞减少急性起病，伴有广泛骨髓纤维化，无脾肿大及红细胞相关形态学改变如泪滴形红细胞。纤维化的存在使这种罕见疾病的正确诊断更加复杂，将该实体与其他血液系统恶性肿瘤如其他急性髓系白血病和与骨髓纤维化相关的骨髓增生异常综合征相鉴别很重要。我们报告一例16岁青年成人，因严重全血细胞减少入院。报道此病例的理由是这种病理情况罕见，医学文献中报道的病例很少，有助于临床经验分享并更好地理解这一诊断困难实体的不同临床方面。
