Department of Immunohematology and Transfusion Medicine, AFMC, Pune - 411040 India.
Mediterr J Hematol Infect Dis. 2013 Jun 4;5(1):e2013042. doi: 10.4084/MJHID.2013.042. Print 2013.
One case of acute panmyelosis with myelofibrosis (APMF) is here reported. A 45 year old male presented with abrupt onset of rapidly progressing low backache, weakness and pancytopenia. On examination there was no organomegaly. Peripheral blood examination revealed normocytic normochromic red blood cells with 10% circulating blasts. Flowcytometric examination of peripheral blood revealed blasts which were positive for CD34, HLA- DR and myeloid associated antigens (i.e. CD13 and CD33). Blasts were negative for anti MPO. Bone marrow aspirate resulted in a dry tap. Bone marrow biopsy revealed panmyeloid proliferation with scattered blasts which were CD34 positive on imunohistochemistry and negative for anti MPO. Reticulin stain showed grade III myelofibrosis (WHO). Differential diagnosis considered included AML-M7, MDS-RAEB II and AML with myelodysplasia. He was started on chemotherapy [idarubicin and cytarabine; 3+7 induction regimen followed by three cycles of HIDAC (High dose cytosine arabinoside)] after which patient was in complete morphological remission with markedly reduced bone marrow fibrosis. He is now being worked up for allogeneic stem cell transplantation. Patient is asymptomatic at eight months of diagnosis. In conclusion these patients should be managed aggressively with AML therapy and this case report reaffirms the fact that APMF is subtype of AML.
现报道 1 例急性全髓细胞增生伴骨髓纤维化（APMF）。一名 45 岁男性，以突发性、进行性腰痛、乏力和全血细胞减少为首发表现。体格检查无肝脾肿大。外周血检查示正细胞正色素红细胞，有 10%的循环原始细胞。外周血流式细胞术检查示原始细胞 CD34、HLA-DR 和髓系相关抗原（即 CD13 和 CD33）阳性。原始细胞抗髓过氧化物酶阴性。骨髓穿刺未抽得骨髓液。骨髓活检示全髓细胞增生，散在原始细胞，免疫组化 CD34 阳性，抗髓过氧化物酶阴性。网状纤维染色示 III 级骨髓纤维化（WHO）。需要鉴别的诊断包括 AML-M7、MDS-RAEB II 和伴骨髓发育异常的 AML。他接受了化疗[阿柔比星和阿糖胞苷；3+7 诱导方案，随后进行了 3 个周期的 HIDAC（高剂量阿糖胞苷）]，随后患者完全形态缓解，骨髓纤维化明显减少。现在正在为他进行异基因造血干细胞移植的准备。诊断 8 个月后患者无症状。总之，这些患者应采用 AML 治疗方案积极治疗，本病例报告再次证实 APMF 是 AML 的一个亚型。
