中部交見会症例

CHUBU1215

Speakers'Dx: histiocytic sarcomaであったが病理診断について意見が多く最終的結論はついてないと思われる。

histiocytic sarcomaの参考文献

Alexiev BA, et al., Primary histiocytic sarcoma arising in the head and neck with predominant spindle cell component. Diagn Pathol. 2007 Feb 26;2:7.

Abstract
This is the first case report of Histiocytic Sarcoma (HS) with predominant spindle cell component occurring in the head and neck region of a 41-year-old man. The tumor was composed of sheets of large round to oval cells with pleomorphic vesicular nuclei, prominent nucleoli and abundant eosinophilic cytoplasm. Multinucleated forms, numerous mitoses, and tumor necrosis were also noted. Sheets, fascicles, and whorls of spindle cells with spindled to ovoid vesicular nuclei, small to medium-sized distinct nucleoli, and eosinophilic cytoplasm were frequently observed. Immunohistochemical staining in the tumor cells was positive for CD163, CD68, lysozyme, CD45, and NSE. Focal expression of CD4 and S-100 was also noted. Electron microscopy demonstrated an abundance of lysosomes in the cytoplasm of tumor cells. Chromosome study revealed a 57-80 hyperdiploid [7]/46, XY [13] karyotype, including 3 to 4 copies of various chromosomes. The immunohistochemical and ultrastructural findings confirmed the diagnosis of HS.

PMID:17324277、PMCID:PMC1808440 Free PMC Article

骨髄線維化/線維症を呈する造血器疾患の鑑別

骨髄に線維化をきたす疾患には原発性骨髄線維症(慢性特発性骨髄線維症: chronic idiopathic myelofibrosis)と他疾患に随伴する二次性骨髄線維症がある。

Secondary myelofibrosisの原因疾患*1*2

Neoplastic disease

  • Chronic myeloproliferative disease
    • PV(spent phase)
    • CML(advanced phase)*3
    • ET(advanced phase)
    • Mast cell disease
  • Acute myelogenous leukaemia
  • Myelodysplastic syndromes(occasional)
  • Hairy cell leukaemia
  • Non-Hodgkin's lymphoma
  • Hodgkin's lymphoma
  • Metastatic carcinoma
  • Multiple myeloma, osteosclerotic subtype
  • Acute lymphoblastic leukaemia(rare)

Non neoplastic disease

  • various infections
  • renal osteodystrophy
  • VitaminD deficiency
  • collagen vascular disease
  • Hyper/hypothyroidism
  • radiation exposure
  • Benzen toxicity
  • storage disease
  • osteopetrosis
  • gray platelet syndrome

*1 Tefferi A, et al., Agnogenic myeloid metaplasia. Semin Oncol; 22: 327-333, 1995
*2 Reilly JT Idiopathic myelofibrosis:pathogenesis, natural history and management Blood Rev 11; 233-242, 1997
*3 Honma K, et al., Chronic myelocytic leukemia with marked myelofibrosis and osteosclerosis. Acta Pathol Jpn. 1983 May;33(3):599-607. PMID:6578670
*4 Dutta P, et al., Acute promyelocytic leukemia with secondary myelofibrosis -- case report and review of the literature. Am J Hematol. 2006 Jun;81(6):476-7. PMID: 16680747
*5 Lu Q, et al., Long-term remission in a case of acute promyelocytic leukemia patient with marked myelofibrosis treated with arsenic trioxide, all-trans retinoic acid and consolidation therapy with daunorubicin plus cytarabine. Leuk Res. 2012 Jun;36(6):e119-21. Epub 2012 Mar 2.PMID: 22386731

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Last-modified: 2014-08-29 (金) 12:41:20 (1331d)