A Case of Post-Essential Thrombocythemia Myelofibrosis with Severe Osteosclerosis.
10.3343/kjlm.2010.30.2.122
- Author:
Kyo Kwan LEE
1
;
Han Ik CHO
;
Hyun Sook CHI
;
Do Yeun KIM
;
Seok Lae CHAE
;
Hee Jin HUH
Author Information
1. Department of Laboratory Medicine, Dongguk University Ilsan Hospital, Goyang, Korea. hjhuh@duih.org
- Publication Type:Case Report ; English Abstract
- Keywords:
Essential thrombocythemia;
Myelofibrosis;
Osteosclerosis
- MeSH:
Bone Marrow/pathology;
Female;
Humans;
Megakaryocytes/pathology;
Middle Aged;
Osteosclerosis/complications/*diagnosis;
Primary Myelofibrosis/complications/*diagnosis;
Splenomegaly/etiology;
Thrombocythemia, Essential/complications/*diagnosis;
Tomography, X-Ray Computed
- From:The Korean Journal of Laboratory Medicine
2010;30(2):122-125
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Essential thrombocythemia (ET) is a chronic myeloproliferative neoplasm that involves primarily the megakaryocytic lineage. After many years, a few patients with ET may develop bone marrow (BM) fibrosis and rarely develop osteosclerosis. A 60-yr-old female was admitted due to severe left upper quadrant abdominal discomfort. She had been diagnosed as ET 19 yrs ago. On liver computed tomography severe splenomegaly was shown. Laboratory tests revealed WBC 24.3x10(9)/L, hemoglobin 13.4 g/dL, platelets 432x10(9)/L, lactate dehydrogenase 4,065 IU/L (reference range; 240-480). Blood smear demonstrated leukoerythroblastosis, teardrop cells, and giant and hypogranular platelets. BM study revealed inadequate aspirate due to dry tap. BM biopsy showed clusters of dysplastic megakaryocytes, grade 3 fibrosis, and severe osteosclerosis. Major/minor BCR-ABL1 rearrangement and JAK2 V617F mutation were not detected. Cytogenetic studies revealed normal karyotype. According to the 2008 WHO diagnostic criteria, the patient was diagnosed as having post-essential thrombocythemia myelofibrosis with severe osteosclerosis.
