hairy cell leukemia

出典: meddic

ヘアリー細胞白血病

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出典(authority):フリー百科事典『ウィキペディア(Wikipedia)』「2016/04/24 18:45:29」(JST)

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英文文献

  • Uncommon histiocytic disorders: Rosai-Dorfman, juvenile xanthogranuloma, and Erdheim-Chester disease.
  • Haroche J1, Abla O2.
  • Hematology / the Education Program of the American Society of Hematology. American Society of Hematology. Education Program.Hematology Am Soc Hematol Educ Program.2015 Dec 5;2015(1):571-8. doi: 10.1182/asheducation-2015.1.571.
  • Rosai-Dorfman disease (RDD), juvenile xanthogranuloma (JXG), and Erdheim-Chester disease (ECD) are non-Langerhans cell (non-LCH) disorders arising from either a dendritic or a macrophage cell. RDD is a benign disorder that presents with massive lymphadenopathy, but can have extranodal involvement. I
  • PMID 26637774
  • Hairy cell leukemia: Past, present and future.
  • Getta BM1, Park JH2, Tallman MS3.
  • Best practice & research. Clinical haematology.Best Pract Res Clin Haematol.2015 Dec;28(4):269-72. doi: 10.1016/j.beha.2015.10.015. Epub 2015 Oct 21.
  • This brief review highlights the sequence of therapeutic milestones and advances in our understanding of the biology of hairy cell leukemia (HCL) with a focus on recent molecular findings and how these may be applied to improve disease outcomes in the future. Targeted therapy is discussed in the con
  • PMID 26614906
  • What your hairy cell leukemia patients want you to know.
  • Stone EJ1, Margolin M2.
  • Best practice & research. Clinical haematology.Best Pract Res Clin Haematol.2015 Dec;28(4):264-8. doi: 10.1016/j.beha.2015.09.001. Epub 2015 Oct 8.
  • Hairy cell leukemia is different from most other hematologic malignancies because it is so rare. Patient concerns include finding a knowledgeable physician, confirming the diagnosis at the initial presentation, coping with treatment side effects and monitoring for potential relapse of this chronic d
  • PMID 26614905

和文文献

  • Tuberculous Meningoencephalitis in a Patient with Hairy Cell Leukemia in Complete Remission
  • GIRARDI K,PAVIGLIANITI A,CIRILLO M,BIANCHI A,GHERARDI G,ANNIBALI O,CERCHIARA E,MARCHESI F,TOMARCHIO V,MIGLIO C,TIRINDELLI MC,ONETTI MUDA A,AVVISATI G
  • Journal of clinical and experimental hematopathology 52(1), 31-34, 2012-05-01
  • NAID 10030615572
  • ヘアリー細胞白血病とBRAF変異

関連リンク

Hairy cell leukemia is an uncommon hematological malignancy characterized by an accumulation of abnormal B lymphocytes. It is usually classified as a sub-type of chronic lymphoid leukemia. Hairy cell leukemia makes up ...
Hairy Cell Leukemia. Hairy cell leukemia (HCL) is a chronic lymphoid leukemia, originally described in 1958 by Bouroncle and colleagues[1, 2] and named after the hairlike cytoplasmic projections seen on the surface of ...

関連画像

Hairy Cell LeukemiaHairy cell leukemia: variant - 3.Hairy cell leukemia: variant - 2.Hairy Cell LeukemiaHairy Cell LeukemiaHairy Cell Leukemia


★リンクテーブル★
国試過去問095G033」「107I014
リンク元慢性リンパ性白血病」「ヘアリー細胞白血病」「leukemic reticuloendotheliosis」「HCL
関連記事cell」「hair」「hairy」「leukemia

095G033」

  [★]

  • 56歳の男性。倦怠感と腹部膨満感とを訴えて来院した。6か月前から左上腹部の重圧態を自覚し、少量の摂食で満腹となるようになった。顔色は不良で、るいそうがある。右肋骨弓下に肝を4cm、左肋骨弓下に脾を5cm触知し、いずれも弾性硬で圧痛はない。血液所見:赤血球320万、Hb 9.5 g/dl、Ht31%、網赤血球13‰、白血球23,000(前骨髄球0.5%、好中性骨髄球3.0%、好中性後骨髄球3.5%、桿状核好中球13.0%、分葉核好中球55.0%、好酸球2.0%、好塩基球3.0%、単球4.0%、リンパ球16.0%、赤芽球5個/100白血球)、血小板57万。好中球アルカリホスファターゼスコア320(基準120~320)。末梢血塗抹May-Giemsa染色標本を以下に示す。
  • 最も考えられるのはどれか。


[正答]
※国試ナビ4※ 095G032]←[国試_095]→[095G034

107I014」

  [★]


[正答]


※国試ナビ4※ 107I013]←[国試_107]→[107I015

慢性リンパ性白血病」

  [★]

chronic lymphocytic leukemia, chronic lymphoid leukemia, CLL
白血病
  • first aid step1 2006 p.300,302,303,304,309,310,427,430

概念

  • the accumulation of nonproliferating mature-appearing lymphocytes in the blood, marrow, lymph nodes, and spleen.
  • monoclonal B lymphocytes.
  • CD5+ and CD23+CD5, CD23
  • derived from memory B cell.

細胞表面マーカー WCH.2438

condition smIg CD5 CD10 CD11c CD19 CD20 CD22 CD23 CD25 CD43 CD79b CD103 FMC7
chronic lymphocytic leukemia Dim ++ -/+ ++ Dim -/+ ++ +/- -/+
Waldenstrom macroglobulinemia ++ -/+ ++ ++ -/+ +/-
prolymphocytic leukemia +++ -/+ -/+ -/+ ++ +++ ++ ++ -/+ ++
hairy cell leukemia +++ ++ +++ +++ +++ +++ +++ +++
hairy cell leukemia variant +++ ++ +++ +++ +++ +++ +++
splenic lymphoma with villous lymphocytes ++ -/+ -/+ +/- ++ ++ ++ +/- -/+ ++ -/+ ++
marginal zone B-cell lymphoma ++ +/- ++ ++ +/- +/- -/+ ++
mantle cell lymphoma ++ ++ -/+ ++ ++ ++ ++ ++
follicular lymphoma ++ -/+ ++ ++ ++ ++ -/+ ++ ++

臨床像

HIM.693
  年齢 子供における頻度 男性(%) ステージI,II vs III,IV(%) B症状(%) 骨髄浸潤(%) 消化管浸潤(%) 5年生存率(%)
B細胞CLL/小リンパ球性リンパ腫 65 まれ 53 9 vs 91 33 72 3 51

疫学

YN.G-49
  • 全白血病の2-3%
  • 60歳以上に多い。
  • 男女比=2:1

病態

YN.G-49
  • 正常な免疫グロブリンの産生が抑制されるばかりでなく、異常な自己抗体を産生 → 続発性免疫不全症、自己免疫性溶血性貧血


USMLE

  • Q book p.246 42

参考

  • 1. [charged] Epidemiology and clinical manifestations of chronic lymphocytic leukemia - uptodate [1]
  • 2. [charged] Pathologic features, diagnosis, and differential diagnosis of chronic lymphocytic leukemia - uptodate [2]
  • 3. [charged] Pathophysiology and cytogenetics of chronic lymphocytic leukemia - uptodate [3]



[show details]


ヘアリー細胞白血病」

  [★]

hairy cell leukemia HCL
ヘアリーセル白血病、毛髪細胞白血病有毛細胞白血病毛様細胞白血病有毛細胞性白血病
2-クロロデオキシアデノシン


  • first aid step1 2006 p.295


leukemic reticuloendotheliosis」

  [★]

白血病性細網内皮症白血病性細網組織増殖症

hairy cell leukemia


HCL」

  [★] ヘアリー細胞白血病 hairy cell leukemia


cell」

  [★] 細胞

WordNet   license wordnet

「small room in which a monk or nun lives」
cubicle

WordNet   license wordnet

「a device that delivers an electric current as the result of a chemical reaction」
electric cell

WordNet   license wordnet

「a room where a prisoner is kept」
jail cell, prison cell

WordNet   license wordnet

「(biology) the basic structural and functional unit of all organisms; they may exist as independent units of life (as in monads) or may form colonies or tissues as in higher plants and animals」

WordNet   license wordnet

「any small compartment; "the cells of a honeycomb"」

WordNet   license wordnet

「a small unit serving as part of or as the nucleus of a larger political movement」
cadre

PrepTutorEJDIC   license prepejdic

「(刑務所の)『独房』;(修道院の)小さい独居室 / (ミツバチの)みつ房,巣穴 / 小さい部屋 / 『細胞』 / 電池 / 花粉室 / (共産党などの)細胞」


hair」

  [★]

WordNet   license wordnet

「filamentous hairlike growth on a plant; "peach fuzz"」
fuzz, tomentum

WordNet   license wordnet

「a filamentous projection or process on an organism」

WordNet   license wordnet

「any of the cylindrical filaments characteristically growing from the epidermis of a mammal; "there is a hair in my soup"」
pilus

WordNet   license wordnet

「a covering for the body (or parts of it) consisting of a dense growth of threadlike structures (as on the human head); helps to prevent heat loss; "he combed his hair"; "each hair consists of layers of dead keratinized cells"」

PrepTutorEJDIC   license prepejdic

「〈C〉(人間・動物の1本の)『毛』;(植物・昆虫などの)毛 / 〈U〉《集合的に》『髪の毛』,毛,毛髪;(動物の)体毛 / 《a~》1本の毛ほどの量(距離,程度),ごくわずか」


hairy」

  [★]

WordNet   license wordnet

「hazardous and frightening; "hairy moments in the mountains"」

PrepTutorEJDIC   license prepejdic

「毛深い,毛だらけの・粗い,ざらざらした・《俗》 ぞっとするほど危険な; 困難な,難しい」

WordNet   license wordnet

「having or covered with hair; "Jacob was a hairy man"; "a hairy caterpillar"」
haired, hirsute


leukemia」

  [★] 白血病

WordNet   license wordnet

「malignant neoplasm of blood-forming tissues; characterized by abnormal proliferation of leukocytes; one of the four major types of cancer」
leukaemia, leucaemia, cancer of the blood

PrepTutorEJDIC   license prepejdic

「白血病」




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