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#author("2024-01-31T22:08:01+09:00","","") #author("2024-01-31T22:10:05+09:00","","") [[Acute megakaryoblastic leukaemia]] [[中部交見会症例]] *CHUBU1215 [#e4e8e315] 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.PMID:17324277、PMCID:PMC1808440 Free PMC Article 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. 本症例は、41歳男性の頭頸部に発生した紡錘細胞成分を主成分とする組織球性肉腫（HS）の初めての症例報告である。腫瘍は、多形小胞状の核、顕著な核小体、および豊富な好酸球性細胞質を有する大きな円形から楕円形の細胞のシートから構成されていた。多核形態、多数の有糸分裂、および腫瘍壊死も認められた。紡錘形～卵形の小胞状核、小～中サイズの明瞭な核小体、好酸球性細胞質を有する紡錘形細胞のシート、束、渦が頻繁に観察された。腫瘍細胞の免疫組織化学染色では、CD163、CD68、リゾチーム、CD45、NSEが陽性であった。CD4とS-100の局所発現も認められた。電子顕微鏡検査では、腫瘍細胞の細胞質に豊富なリソソームが認められた。染色体検査では、57-80の超2倍体 [7]/46, XY [13] の核型が認められ、様々な染色体の3-4コピーを含んでいた。免疫組織化学的所見および超微細構造学的所見から、HSと診断された。(DeepL訳) *骨髄線維化/線維症を呈する造血器疾患の鑑別 [#taf72050] 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.¬e{:Tefferi A, et al., Agnogenic myeloid metaplasia. Semin Oncol; 22: 327-333, 1995};¬e{:Reilly JT Idiopathic myelofibrosis:pathogenesis, natural history and management Blood Rev 11; 233-242, 1997}; ***Secondary myelofibrosisの原因疾患 [#mc920409] ''Neoplastic disease'' -Chronic myeloproliferative disease --PV(spent phase) --[[PV(spent phase)>myelofibrosis#gfa8ef20]] --CML(advanced phase)¬e{:Honma K, et al., Chronic myelocytic leukemia with marked myelofibrosis and osteosclerosis. Acta Pathol Jpn. 1983 May;33(3):599-607. PMID:6578670}; --ET(advanced phase) --Mast cell disease -Acute myelogenous leukaemia --[[acute megakaryoblastic leukaemia>Acute megakaryoblastic leukaemia]] (M7) --Therapy-related --Other subtypes ie, M3¬e{: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}; ¬e{: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}; -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
