{"title":"一例80岁男性急性泛髓性白血病伴骨髓纤维化:一种罕见的急性髓性白血病亚型","authors":"P. Mandal, P. Prasad","doi":"10.4103/2384-5589.197968","DOIUrl":null,"url":null,"abstract":"Acute panmyelosis with myelofibrosis (APMF) is a rare, fatal hematological neoplasm that is characterized by acute onset of cytopenias and fibrosis in the bone marrow in the absence of splenomegaly or fibrosis-related morphological changes in the red blood cells. We report the case of an 80-year-old male who presented with progressive pallor and weakness of 1-month duration. Peripheral smear revealed severe anemia, leukopenia with occasional circulating blasts and a normal platelet count. The marrow was heavily fibrotic, and no aspirate material could be obtained; the biopsy showed extensive infiltration with small to medium size megakaryocytes, dysplastic changes in the erythroid compartment, and focal clusters of blasts. A diagnosis of APMF was made and the patient was started on low dose lenalidomide and supportive transfusions. However, the patient died due to intracranial bleeding. Cases of APMF have a dismal outcome and should be managed aggressively.","PeriodicalId":93249,"journal":{"name":"African journal of medical and health sciences","volume":"13 1","pages":"103 - 106"},"PeriodicalIF":0.0000,"publicationDate":"2016-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"An 80-year-old man with acute panmyelosis with myelofibrosis: A rare subtype of acute myeloid leukemia\",\"authors\":\"P. Mandal, P. Prasad\",\"doi\":\"10.4103/2384-5589.197968\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Acute panmyelosis with myelofibrosis (APMF) is a rare, fatal hematological neoplasm that is characterized by acute onset of cytopenias and fibrosis in the bone marrow in the absence of splenomegaly or fibrosis-related morphological changes in the red blood cells. We report the case of an 80-year-old male who presented with progressive pallor and weakness of 1-month duration. Peripheral smear revealed severe anemia, leukopenia with occasional circulating blasts and a normal platelet count. The marrow was heavily fibrotic, and no aspirate material could be obtained; the biopsy showed extensive infiltration with small to medium size megakaryocytes, dysplastic changes in the erythroid compartment, and focal clusters of blasts. A diagnosis of APMF was made and the patient was started on low dose lenalidomide and supportive transfusions. However, the patient died due to intracranial bleeding. Cases of APMF have a dismal outcome and should be managed aggressively.\",\"PeriodicalId\":93249,\"journal\":{\"name\":\"African journal of medical and health sciences\",\"volume\":\"13 1\",\"pages\":\"103 - 106\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2016-07-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"African journal of medical and health sciences\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.4103/2384-5589.197968\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"African journal of medical and health sciences","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.4103/2384-5589.197968","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
An 80-year-old man with acute panmyelosis with myelofibrosis: A rare subtype of acute myeloid leukemia
Acute panmyelosis with myelofibrosis (APMF) is a rare, fatal hematological neoplasm that is characterized by acute onset of cytopenias and fibrosis in the bone marrow in the absence of splenomegaly or fibrosis-related morphological changes in the red blood cells. We report the case of an 80-year-old male who presented with progressive pallor and weakness of 1-month duration. Peripheral smear revealed severe anemia, leukopenia with occasional circulating blasts and a normal platelet count. The marrow was heavily fibrotic, and no aspirate material could be obtained; the biopsy showed extensive infiltration with small to medium size megakaryocytes, dysplastic changes in the erythroid compartment, and focal clusters of blasts. A diagnosis of APMF was made and the patient was started on low dose lenalidomide and supportive transfusions. However, the patient died due to intracranial bleeding. Cases of APMF have a dismal outcome and should be managed aggressively.