Korean J Lab Med.  2010 Apr;30(2):122-125. 10.3343/kjlm.2010.30.2.122.

A Case of Post-Essential Thrombocythemia Myelofibrosis with Severe Osteosclerosis

• Lee KK
• Cho HI
• Chi HS
• Kim DY
• Chae SL
• Huh HJ

• KMID: 1096796
• DOI: http://doi.org/10.3343/kjlm.2010.30.2.122

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.

Keyword

Essential thrombocythemia; Myelofibrosis; Osteosclerosis

MeSH Terms

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

Figure

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  Fig. 1. Initial bone marrow biopsy showing enlarged megakaryocytes. They reveal abundant amount of cytoplasm and deeply lobulated and hyperlobulated (stag-horn-like) nuclei (H&E stain, ×400).

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  Fig. 2. Follow-up bone marrow biopsy after 19 yr. (A) Osteosclerosis characterized by broad, irregular bony trabeculae (H&E stain, ×100). (B) Coarse bundles of collagen fibers (Masson's trichrome stain, ×200).

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