Dahiya Surbhi, Kotwal Jyoti, Langer Sabina, Saraf Amrita, Prakhar Pallavi, Gupta Deepika, Gupta Nitin, Arya Vandana
Department of Hematology, Sir Ganga Ram Hospital, New Delhi, Delhi, India.
Indian J Pathol Microbiol. 2025 Jan 1;68(1):171-175. doi: 10.4103/ijpm.ijpm_391_23. Epub 2024 Feb 19.
Acute panmyelosis with myelofibrosis (APMF) corresponds to <1% cases of acute myeloid leukemia, which could be an underestimation due to missed diagnosis. Due to its rapidly fatal course, it warrants a timely and correct diagnosis. We present a case of a 44-year male who came with a short history of fever, generalised weakness, revealed pancytopenia with occasional circulating blast in the peripheral blood smear. Bone marrow aspirate was dry tap,biopsy revealed panmyelosis with myelofibrosis with increased (22%) blasts. Flowcytometric immunophenotyping, cytogenetics and molecular tests were undertaken. Together with clinical details, immunophenotypic profile, cytogenetics and molecular studies, the diagnosis of Acute panmyelosis with myelofibrosis was made and managed accordingly. 32 The WHO 2017 describes APMF as an acute panmyeloid proliferation with increased blasts (≥20% in the bone marrow or peripheral blood) and accompanying marrow fibrosis. APMF is rare with poor prognosis thus, must be differentiated especially from Acute megakaryoblastic leukemia to arrive at the correct diagnosis which will help reduce/prevent the early mortality by providing timely chemotherapy followed by upfront hemopoietic stem cell transplantation.
急性全髓增殖症伴骨髓纤维化（APMF）占急性髓系白血病病例的比例不到1%，由于漏诊，这一比例可能被低估。因其病程迅速致命，所以需要及时、正确的诊断。我们报告一例44岁男性病例，该患者因发热、全身无力病史较短前来就诊，外周血涂片显示全血细胞减少，偶见循环原始细胞。骨髓穿刺干抽，活检显示全髓增殖伴骨髓纤维化，原始细胞增多（22%）。进行了流式细胞免疫表型分析、细胞遗传学和分子检测。结合临床细节、免疫表型特征、细胞遗传学和分子研究，做出了急性全髓增殖症伴骨髓纤维化的诊断并进行了相应处理。世界卫生组织2017年版将APMF描述为一种急性全髓增殖，原始细胞增多（骨髓或外周血中≥20%）并伴有骨髓纤维化。APMF罕见，预后较差，因此必须特别与急性巨核细胞白血病相鉴别，以做出正确诊断，这将有助于通过及时化疗及随后的早期造血干细胞移植来降低/预防早期死亡率。
