Habberstad Andreas Hanssønn, Tran Hoa Thi Tuyet, Randen Ulla, Spetalen Signe, Dybedal Ingunn, Tjønnfjord Geir E, Dahm Anders Erik Astrup
Department of Haematology, Akershus University Hospital, Lørenskog, Norway.
Department of Pathology, Akershus University Hospital, Lørenskog, Norway.
J Med Case Rep. 2018 Apr 24;12(1):105. doi: 10.1186/s13256-018-1663-6.
Polycythemia vera is a myeloproliferative disease that sometimes evolves to myelofibrosis, causing splenomegaly and neutropenia. In this case report, we describe a patient with polycythemia vera and unexplained neutropenia who later turned out to also have hairy cell leukemia.
A middle-aged Caucasian man with polycythemia vera presented to our hospital with chronic mouth ulcers. Later he developed leukopenia and pancytopenia. Bone marrow biopsies showed fibrosis. Further morphological analyses of bone marrow and blood smears revealed probable transformation into acute myeloid leukemia. However, there were also cells indicating hairy cell leukemia. Morphological and immunohistochemical analyses later confirmed the presence of hairy cell leukemia in biopsies that had been present for 3 years. Treatment with cladribine temporarily reversed the patient's neutropenia.
Hairy cell leukemia may mimic development to myelofibrosis in patients with polycythemia vera.
真性红细胞增多症是一种骨髓增殖性疾病，有时会演变为骨髓纤维化，导致脾肿大和中性粒细胞减少。在本病例报告中，我们描述了一名患有真性红细胞增多症和不明原因中性粒细胞减少的患者，后来发现其还患有毛细胞白血病。
一名患有真性红细胞增多症的中年白种男性因慢性口腔溃疡前来我院就诊。后来他出现白细胞减少和全血细胞减少。骨髓活检显示纤维化。对骨髓和血涂片进行的进一步形态学分析显示可能已转化为急性髓系白血病。然而，也有细胞提示毛细胞白血病。形态学和免疫组化分析后来证实活检中存在毛细胞白血病，且这种情况已存在3年。用克拉屈滨治疗暂时逆转了患者的中性粒细胞减少。
毛细胞白血病可能在真性红细胞增多症患者中模拟向骨髓纤维化的发展。
