赤芽球癆 PRCA
WordNet
- free of extraneous elements of any kind; "pure air and water"; "pure gold"; "pure primary colors"; "the violins pure and lovely song"; "pure tones"; "pure oxygen"
- in a state of sexual virginity; "pure and vestal modesty"; "a spinster or virgin lady"; "men have decreed that their women must be pure and virginal" (同)vestal, virgin, virginal, virtuous
- (used of persons or behaviors) having no faults; sinless; "I felt pure and sweet as a new baby"- Sylvia Plath; "pure as the driven snow"
- concerned with theory and data rather than practice; opposed to applied; "pure science"
- free from discordant qualities
- small room in which a monk or nun lives (同)cubicle
- a device that delivers an electric current as the result of a chemical reaction (同)electric cell
- a room where a prisoner is kept (同)jail cell, prison cell
- (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
- any small compartment; "the cells of a honeycomb"
- a small unit serving as part of or as the nucleus of a larger political movement (同)cadre
- of a color at the end of the color spectrum (next to orange); resembling the color of blood or cherries or tomatoes or rubies (同)reddish, ruddy, blood-red, carmine, cerise, cherry, cherry-red, crimson, ruby, ruby-red, scarlet
- red color or pigment; the chromatic color resembling the hue of blood (同)redness
- the syllable naming the second (supertonic) note of any major scale in solmization (同)ray
- a tributary of the Mississippi River that flows eastward from Texas along the southern boundary of Oklahoma and through Louisiana (同)Red River
PrepTutorEJDIC
- (金など)『混じりけのない』,純粋の / (水など)『汚れていない』,きれいな / (道徳的に)汚れていない,清らかな / (血統が)純粋の,純血の,きっすいの / 《名詞の前にのみ用いて》《話》全くの / 《名詞の前にのみ用いて》(応用的に対して)理論的な,純粋の
- (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
- 〈U〉〈C〉『赤,』『赤色;』赤い絵の具(染料) / 〈U〉赤い服 / 〈C〉《しばしば『R-』》《話》《時に軽べつして》アカ,共産主義者;過激論(主義)者 / 〈U〉《通例the ~》(会計の)赤字,負債 / 『赤い』,赤色の / (顔・目などが)赤くなった;血に染った / 赤い服を着た;赤毛の / 《しばしば『R-』》《話》《軽べつして》共産主義の;過激な
- レ(全音階の第2音)
Wikipedia preview
出典(authority):フリー百科事典『ウィキペディア(Wikipedia)』「2013/10/15 12:52:36」(JST)
[Wiki en表示]
Pure red cell aplasia |
Classification and external resources |
ICD-10 |
D60 |
ICD-9 |
284.8 |
DiseasesDB |
29063 |
eMedicine |
med/1967 |
MeSH |
D012010 |
Pure red cell aplasia (PRCA) or erythroblastopenia refers to a type of anemia affecting the precursors to red blood cells but not to white blood cells. In PRCA, the bone marrow ceases to produce red blood cells. The condition has been first described by Paul Kaznelson in 1922.[1]
Contents
- 1 Causes
- 2 Treatment
- 3 See also
- 4 References
- 5 External links
Causes[edit]
Causes include:
- Viral infections such as HIV, herpes, parvovirus B19 (Fifth disease),[3] or hepatitis.[citation needed]
- Lymphoproliferative. Association of pure red cell aplasia with T large granular lymphocyte leukemia is well recognized, especially in China.[4]
- Idiopathic. Many cases of PRCA are considered idiopathic in that there is no discernible cause detected.[5]
- Drugs, e.g., erythropoietin[6] or mycophenolic acid.[7]
- Congenital. The term "hereditary pure red cell aplasia" has been used to refer to Diamond-Blackfan anemia.[8]
Treatment[edit]
PRCA is considered an autoimmune disease as it will respond to immunosuppressant treatment such as ciclosporin in many patients,[9] though this approach is not without risk.[10]
It has also been shown to respond to treatments with Rituxan and Tacrolimus.
See also[edit]
- Diamond-Blackfan anemia (genetic red cell aplasia)
- aplastic anemia (aplasia affecting other bone marrow cells as well)
References[edit]
- ^ Kaznelson, P (1922). "Zur Entstehung der Blutplättchen". Verh Dtsch Ges Inn Med. 34: 557–8.
- ^ Hirokawa M, Sawada K, Fujishima N, et al. (January 2008). "Long-term response and outcome following immunosuppressive therapy in thymoma-associated pure red cell aplasia: a nationwide cohort study in Japan by the PRCA collaborative study group". Haematologica 93 (1): 27–33. doi:10.3324/haematol.11655. PMID 18166782.
- ^ Geetha D, Zachary JB, Baldado HM, Kronz JD, Kraus ES (December 2000). "Pure red cell aplasia caused by Parvovirus B19 infection in solid organ transplant recipients: a case report and review of literature". Clin Transplant 14 (6): 586–91. doi:10.1034/j.1399-0012.2000.140612.x. PMID 11127313.
- ^ Kwong YL, Wong KF (1998). "Association of pure red cell aplasia with T large granular lymphocyte leukaemia". J. Clin. Pathol. 51 (9): 672–5. doi:10.1136/jcp.51.9.672. PMC 500904. PMID 9930071.
- ^ Miller, AC, Rashid, RM (2008). "Three episodes of acquired pure red cell aplasia restricted to pregnancy.". Journal of perinatal medicine 36 (3): 270–1. doi:10.1515/JPM.2008.041. PMID 18576941.
- ^ Bennett CL, Luminari S, Nissenson AR, et al. (2004). "Pure red-cell aplasia and epoetin therapy". N. Engl. J. Med. 351 (14): 1403–8. doi:10.1056/NEJMoa040528. PMID 15459301.
- ^ Petrochko C (2009). "FDA Strengthens Warning on Transplant Drug." Medpage Today. 14 August 2009. Accessed on 19 August 2009.
- ^ Online 'Mendelian Inheritance in Man' (OMIM) 105650
- ^ Sawada K, Hirokawa M, Fujishima N, et al. (August 2007). "Long-term outcome of patients with acquired primary idiopathic pure red cell aplasia receiving cyclosporine A. A nationwide cohort study in Japan for the PRCA Collaborative Study Group". Haematologica 92 (8): 1021–8. doi:10.3324/haematol.11192. PMID 17640861.
- ^ Sawada K, Fujishima N, Hirokawa M (August 2008). "Acquired pure red cell aplasia: updated review of treatment". Br. J. Haematol. 142 (4): 505–14. doi:10.1111/j.1365-2141.2008.07216.x. PMC 2592349. PMID 18510682.
External links[edit]
- Aplastic Anemia & MDS International Foundation
Pathology: hematology, hematologic diseases of RBCs and megakaryocytes / MEP (D50-69,74, 280-287)
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Red
blood cells |
↑ |
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↓ |
Anemia |
Nutritional |
- Micro-: Iron deficiency anemia
- Macro-: Megaloblastic anemia
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Hemolytic
(mostly Normo-) |
Hereditary |
- enzymopathy: G6PD
- glycolysis
- hemoglobinopathy: Thalassemia
- Sickle-cell disease/trait
- HPFH
- membrane: Hereditary spherocytosis
- Minkowski-Chauffard syndrome
- Hereditary elliptocytosis
- Southeast Asian ovalocytosis
- Hereditary stomatocytosis
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Acquired |
- Drug-induced autoimmune
- Drug-induced nonautoimmune
- Hemolytic disease of the newborn
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Aplastic
(mostly Normo-) |
- Hereditary: Fanconi anemia
- Diamond–Blackfan anemia
- Acquired: PRCA
- Sideroblastic anemia
- Myelophthisic
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Blood tests |
- MCV
- Normocytic
- Microcytic
- Macrocytic
- MCHC
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Other |
- Methemoglobinemia
- Sulfhemoglobinemia
- Reticulocytopenia
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Coagulation/
coagulopathy |
↑ |
Hyper-
coagulability |
- primary: Antithrombin III deficiency
- Protein C deficiency/Activated protein C resistance/Protein S deficiency/Factor V Leiden
- Prothrombin G20210A
- Sticky platelet syndrome
- acquired:Thrombocytosis
- DIC
- Congenital afibrinogenemia
- Purpura fulminans
- autoimmune
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↓ |
Hypo-
coagulability |
Thrombocytopenia |
- Thrombocytopenic purpura: ITP
- TM
- Heparin-induced thrombocytopenia
- May-Hegglin anomaly
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|
Platelet function |
- adhesion
- aggregation
- Glanzmann's thrombasthenia
- platelet storage pool deficiency
- Hermansky–Pudlak syndrome
- Gray platelet syndrome
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Clotting factor |
- Hemophilia
- von Willebrand disease
- Hypoprothrombinemia/II
- XIII
- Dysfibrinogenemia
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cell/phys (coag, heme, immu, gran), csfs
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rbmg/mogr/tumr/hist, sysi/epon, btst
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drug (B1/2/3+5+6), btst, trns
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UpToDate Contents
全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.
English Journal
- Improved analysis of TCRγδ variable region expression in humans.
- Christopoulos P1, Bukatz D2, Kock S3, Malkovsky M4, Finke J5, Fisch P6.
- Journal of immunological methods.J Immunol Methods.2016 Jul;434:66-72. doi: 10.1016/j.jim.2016.04.009. Epub 2016 Apr 21.
- Deeper understanding of γδ Τ cell increases in various clinical situations requires the assessment of TCRγ and δ variable (V) region gene expression and junctional diversity. Here we describe an improved TCRγ and δ spectratyping method used to study the γδ T-cell expansions in two patients
- PMID 27109705
- Bone marrow transplantation for CVID-like humoral immune deficiency associated with red cell aplasia.
- Sayour EJ1,2, Mousallem T3,4, Van Mater D5, Wang E6, Martin P7, Buckley RH4, Barfield RC5.
- Pediatric blood & cancer.Pediatr Blood Cancer.2016 Jun 8. doi: 10.1002/pbc.26092. [Epub ahead of print]
- Patients with common variable immunodeficiency (CVID) have a higher incidence of autoimmune disease, which may mark the disease onset; however, anemia secondary to pure red cell aplasia is an uncommon presenting feature. Here, we describe a case of CVID-like humoral immune deficiency in a child who
- PMID 27273469
- Monoclonal gammopathy-associated pure red cell aplasia.
- Korde N1,2, Zhang Y2, Loeliger K3, Poon A3, Simakova O4, Zingone A2, Costello R2, Childs R3, Noel P5, Silver S6, Kwok M7, Mo C7, Young N3, Landgren O1,2, Sloand E3, Maric I5.
- British journal of haematology.Br J Haematol.2016 Jun;173(6):876-83. doi: 10.1111/bjh.14012. Epub 2016 Mar 21.
- Pure red cell aplasia (PRCA) is a rare disorder characterized by inhibition of erythroid precursors in the bone marrow and normochromic, normocytic anaemia with reticulocytopenia. Among 51 PRCA patients, we identified 12 (24%) patients having monoclonal gammopathy, monoclonal gammopathy of undetermi
- PMID 26999424
Japanese Journal
- 症例報告 サンヤクとブクリョウ投与後に生じた赤芽球癆と低ガンマグロブリン血症
- 症例 赤芽球癆を合併した原発性マクログロブリン血症の1例
- 再生不良性貧血と赤芽球癆の病態と治療 (特集 貧血 : 基礎知識から治療の最前線まで)
Related Links
- Pure red cell aplasia (PRCA) is an uncommon disorder in which maturation arrest occurs in the formation of erythrocytes. Erythroblasts are virtually absent in bone marrow; however, WBC and platelet production is normal.
- Pure red cell aplasia (PRCA) is an uncommon disorder in which maturation arrest occurs in the formation of erythrocytes. Erythroblasts are virtually absent in bone marrow; however, WBC and platelet production is normal.
Related Pictures
★リンクテーブル★
[★]
- 英
- thymoma, thymomas
- 関
- 胸腺、縦隔腫瘍
概念
- 上縦隔の前部、前縦隔の前部に発生
- 胸腺固有の上皮細胞が腫瘍化したもの。precursor T cell(thymocytes)も存在することがあるが、悪性ではない。
分類
- begign or encapsulated thymoma
- malignant thymoma
- type I: cytologically benign but biologically aggressive and capable of local invasion and, rarely, distant spread
- type II: also called thymic carcinoma: cytologically malignant with all of the features of cancer and comparable behavior
- HIM. e89
型
|
組織型
|
割合,%
|
予後(10-year disease-free survival), %
|
A
|
medullary thymoma
|
8
|
100
|
AB
|
mixed thymoma
|
17
|
100
|
B1
|
predominantly cortical thymoma
|
27
|
83
|
B2
|
cortical thymoma
|
8
|
83
|
B3
|
well-differentiated thymic carcinoma
|
12
|
36
|
C
|
thymic carcinoma
|
28
|
28
|
病因
- 不明なことが多い。EBウイルスが関わっているかもしれない
疫学
- 胸腺腫は希であって、悪性のものはさらに希
- 全縦隔腫瘍の20-30%を占める。
- どの年齢にも起こりうるが、とりわけ中年に後発する。平均50歳代。
症状
- 30%:無症状。
- 30-40%:CTでとらえられ、咳嗽、胸痛、呼吸困難、上大静脈症候群などの周胸臓器圧迫症状
- 残り:全身病の合併
合併症
症例
- 40歳の女性。夕方になると、ものが二重に見える(複視)ことを主訴に来院した。眼瞼下垂を認める。血液検査では網赤血球の減少を認める。
参考
- 1. 15-year-old boy with noninvasive cystic thymoma
- http://www.ajronline.org/cgi/content-nw/full/186/4/1176/FIG5
- http://telomelysin.com/article/52766550.html
- 3. Imaging of Cystic Masses of the Mediastinum1
- http://radiographics.rsna.org/content/22/suppl_1/S79.full
[★]
- 癆 aplasia
- 英
- pure red cell aplasia, PRCA
- 同
- 純赤血球形成不全?
- 関
- 貧血、再生不良性貧血
概念
- 赤芽球形成が選択的に障害される再生不良性貧血
- 正球性正色素性貧血をきたす。
病態
- 何らかの原因により骨髄から赤血球系の前駆細胞が消失する。
- 無構造血、溶血性貧血も見られないし、間接ビリルビン、ハプトグロビン、LDHも上昇しない。
検査
- 血算:血小板数、白血球数は正常。網状赤血球が正常~減少。
- 骨髄穿刺:網状赤血球が正常~減少をみたら骨髄穿刺を施行し、過形成であれば脾機能亢進症、腎性貧血、悪性腫瘍、低形成であれば再生不良性貧血、
赤芽球癆?、正形成であれば赤芽球癆が疑われる。赤芽球癆の場合、赤血球系の細胞は存在しない。パルボウイルス感染症の場合にはgiant proerythroblastが見られる(参考1)。
- 血製鉄:上昇
- UIBC:↓ (トランスフェリンが鉄で飽和してしまう)
鉄の動態にまつわる検査
- %RCU:低下 ← 赤芽球が少ないので、ヘムにとりこまれ末梢血に出現するのが遅い
- PIDT1/2:延長 ← 赤芽球が少ないので骨髄で取り込まれるのが遅い
USMLE
参考
- 1. [charged] Acquired pure red cell aplasia - uptodate [1]
[★]
赤芽球癆 pure red cell aplasia
[★]
- 関
- erythrocyte、erythrocytic、erythroid、RBC、red blood cell、red corpuscle
[★]
- 関
- aplastic, dysgenesis, hypoplasia
[★]
- 関
- genuinely、purely、sincere
[★]
- 関
- erythro
[★]
細胞