Homayra Tahseen Hossain, A. Rahman, M. Mazumder, Mahbub Mayukh Rishad, Q. Islam, Mohammad Zahiruddin
{"title":"一例年轻女孩原发性骨髓纤维化病例报告","authors":"Homayra Tahseen Hossain, A. Rahman, M. Mazumder, Mahbub Mayukh Rishad, Q. Islam, Mohammad Zahiruddin","doi":"10.3329/jbcps.v41i3.66913","DOIUrl":null,"url":null,"abstract":"Myelofibrosis (MF) is a rare disorder that is classified as one of the myeloproliferative disorders. It is a BCR-ABL1- negative myeloproliferative neoplasm characterized by abnormal proliferation of hemopoietic stem cells within the bone marrow, which leads to overproduction of fibrous tissue. Our patient a young girl of 17 year old presented with lump in left upper abdomen, shortness of breath & generalized swelling. The diagnosis was made based on severe anaemia, pancytopenia in peripheral blood film. Bone marrow trephine biopsy from the tibia revealed myelofibrosis. Splenectomy was done in an attempt to reduce the total volume of malignant cells and improve the features of hypersplenism. Myelofibrosis with hypersplenism in a 17- year- old girl is reported rarely. However, when it does, it usually runs rapid and fatal course.\nJ Bangladesh Coll Phys Surg 2023; 41: 239-243","PeriodicalId":89579,"journal":{"name":"Journal of Bangladesh College of Physicians & Surgeons","volume":" ","pages":""},"PeriodicalIF":0.0000,"publicationDate":"2023-07-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Primary Myelofibrosis in a Young Girl- A Case Report\",\"authors\":\"Homayra Tahseen Hossain, A. Rahman, M. Mazumder, Mahbub Mayukh Rishad, Q. Islam, Mohammad Zahiruddin\",\"doi\":\"10.3329/jbcps.v41i3.66913\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Myelofibrosis (MF) is a rare disorder that is classified as one of the myeloproliferative disorders. It is a BCR-ABL1- negative myeloproliferative neoplasm characterized by abnormal proliferation of hemopoietic stem cells within the bone marrow, which leads to overproduction of fibrous tissue. Our patient a young girl of 17 year old presented with lump in left upper abdomen, shortness of breath & generalized swelling. The diagnosis was made based on severe anaemia, pancytopenia in peripheral blood film. Bone marrow trephine biopsy from the tibia revealed myelofibrosis. Splenectomy was done in an attempt to reduce the total volume of malignant cells and improve the features of hypersplenism. Myelofibrosis with hypersplenism in a 17- year- old girl is reported rarely. However, when it does, it usually runs rapid and fatal course.\\nJ Bangladesh Coll Phys Surg 2023; 41: 239-243\",\"PeriodicalId\":89579,\"journal\":{\"name\":\"Journal of Bangladesh College of Physicians & Surgeons\",\"volume\":\" \",\"pages\":\"\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2023-07-27\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Journal of Bangladesh College of Physicians & Surgeons\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.3329/jbcps.v41i3.66913\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of Bangladesh College of Physicians & Surgeons","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.3329/jbcps.v41i3.66913","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
Primary Myelofibrosis in a Young Girl- A Case Report
Myelofibrosis (MF) is a rare disorder that is classified as one of the myeloproliferative disorders. It is a BCR-ABL1- negative myeloproliferative neoplasm characterized by abnormal proliferation of hemopoietic stem cells within the bone marrow, which leads to overproduction of fibrous tissue. Our patient a young girl of 17 year old presented with lump in left upper abdomen, shortness of breath & generalized swelling. The diagnosis was made based on severe anaemia, pancytopenia in peripheral blood film. Bone marrow trephine biopsy from the tibia revealed myelofibrosis. Splenectomy was done in an attempt to reduce the total volume of malignant cells and improve the features of hypersplenism. Myelofibrosis with hypersplenism in a 17- year- old girl is reported rarely. However, when it does, it usually runs rapid and fatal course.
J Bangladesh Coll Phys Surg 2023; 41: 239-243
求助内容：
应助结果提醒方式：
京公网安备 11010802042870号
