Panmielosis aguda con mielofibrosis / Acute panmyelosis with myelofibrosis

La panmielosis aguda con mielofibrosis (PMAF) es un raro desorden hematológico, definido como un subtipo de leucemia aguda. Se reporta un paciente masculino de 31 años de edad con historia de decaimiento marcado, fiebre vespertina y gingivorragia. El examen físico muestra palidez cutáneo mucosas, esplenomegalia ligera y en exámenes complementarios pancitopenia con 5 por ciento de blastos. En el medulograma no se obtuvo material y la impronta sugiere leucemia mieloide aguda no promielocítica. El estudio de inmunofenotipo por citometría de flujo confirma incremento de mieloblastos positivo para (CD34, CD13, CD17, CD117, CD38) y disminuidos en (CD11c y HLA-DR), con una mielofibrosis marcada en biopsia de medula ósea sin la presencia de blastos. Recibe tratamiento de inducción con esquema 3 + 7 (Citosar + Rubidomicina) después del cual el paciente se encuentra en remisión hematológica con persistencia de la fibrosis medular. Posteriormente inicia tratamiento con lenalidomida, Bifosfonatos (Ácido Zoledronico) y se encuentra en remisión hematológica 11 meses después del diagnóstico, hasta marzo 2020. Se realizan estudios de histocompatibilidad (HLA) para trasplante alogénico(AU)

Acute panmyelosis with myelofibrosis (PMAF) is a rare hematologic disorder, defined as a subtype of acute leukemia. A 31-year-old male patient with a history of marked decay, evening fever, and gingivorrhagia is reported. The physical examination showed mucous skin paleness, slight splenomegaly and the complementary examinations showed pancytopenia with 5 percent blasts. In the medullogram no material was obtained and the imprint suggests non-promyelocytic acute myeloid leukemia. Immunophenotype study by flow cytometry confirmed an increase in myeloblasts positive for (CD34, CD13, CD17, CD117, CD38) and decreased in (CD11c and HLA-DR), with marked myelofibrosis in bone marrow biopsy without the presence of blasts. He received induction treatment with a 3 + 7 scheme (Citosar + Rubidomycin) after which the patient was in hematological remission with persistence of spinal fibrosis. Later, he started treatment with lenalidomide, bisphosphonates (Zoledronic Acid) and was in hematological remission 11 months after diagnosis, until March 2020. Histocompatibility studies (HLA) were performed for allogeneic transplantation(AU)

Reduced Intensity Conditioning Regimen Followed by Allogeneic Peripheral Blood Stem Cell Transplantation for Acute Panmyelosis with Myelofibrosis

A 44-year-old male presented with a month history of exertional dyspnea and dizziness. A peripheral blood smear revealed a pancytopenia with 3% of blasts. We were not able to obtain a bone marrow aspirate, but a biopsy specimen showed hypercellularity, proliferation of trilineage cell lines (panmyelosis) with extensive myelofibrosis, and clusters of immature cells at the paratrabecular area. After remission induction therapy with idarubicin 12mg/m2 (D1-3) and cytosine arabinoside 100mg/m2 (D1-7), the bone marrow blast count was decreased, but the marrow fibrosis and pancytopenia persisted. Peripheral blood stem cell transplantation from his HLA-matched brother was performed after administering fludarabine 30mg/m2 for 5 days and busulfan 3.2mg/kg for 2 days. Early engraftment occurred and the bone marrow reticulin fibrosis disappeared. Full-donor chimerism was demonstrated at day 22 by performing short tandem repeats analysis and this was maintained for 1 year. The patient has survived 20 months after transplantation without any complication.

A Case of Acute Panmyelosis with Myelofibrosis / 대한진단검사의학회지

We experienced a case of acute panmyelosis with myelofibrosis, one of the acute myeloblastic leukemia (AML) subtypes according to WHO classification. A 44 year old man presented with a laborious dyspnea, dizziness, and generalized weakness. On admission, the peripheral blood revealed pancytopenia (hemoglobin 4.0 g/dL, WBC 2, 300/microliter, platelet 92, 000/microliter) with leukoerythroblastosis. The bone marrow (BM) aspirate yielded inadequate material but histological sections showed hyper-cellularity, proliferation of trilineage cell lines (panmyelosis) with extensive myelofibrosis, and clusters of immature cells at paratrabecular area. On ultrasonographic examination, no evidence of hepato-splenomegaly or any other abnormalities were noted. The follow-up BM study after the chemotherapy with Ara C for 7 days and idarubicin for 3 days showed no significant changes in pancytopenia and myelofibrosis but revealed a significant decrease in BM cellularity and blasts. We review and report the literature on acute panmyelosis with myelofibrosis.

A Case of Acute Panmyelosis with Myelofibrosis according to WHO Classification / 대한혈액학회지

A 61-year-old man developed a rapidly progressive disease with anemia, thrombocytopenia and large number of blasts (10%) in the peripheral blood. The bone marrow revealed hypercellular marrow with extensive fibrosis and proliferation of immature cells of trilineage. Immunophenotyping and chromosome study with the bone marrow aspirate showed myeloblasts with aberrant expression of CD19 and complex karyotype including structural abnormalities within chromosome 5 and 7. After induction chemotherapy with Ara-c and daunorubicin, the number of blasts in peripheral blood was increased and the bone marrow showed hypercellular marrow with immature cells of trilineage and fibrosis. Cases like this have been called acute myelofibrosis, acute myelosclerosis or acute myelodysplasia with myelofibrosis. Recently this rare disease with rapidly fatal course was categorized into 'acute panmyelosis with myelofibrosis in acute myelogenous leukemia (AML) not otherwise categorized' by WHO classification.