WordNet

unconscious from a blow or shock or intoxication; "the boxer was out cold"; "pass out cold"
a mild viral infection involving the nose and respiratory passages (but not the lungs); "will they never find a cure for the common cold?" (同)common_cold
the sensation produced by low temperatures; "he shivered from the cold"; "the cold helped clear his head" (同)coldness
without compunction or human feeling; "in cold blood"; "cold-blooded killing"; "insensate destruction" (同)cold-blooded, inhuman, insensate
sexually unresponsive; "was cold to his advances"; "a frigid woman" (同)frigid
lacking originality or spontaneity; no longer new; "moth-eaten theories about race"; "stale news" (同)stale, dusty, moth-eaten
(color) giving no sensation of warmth; "a cold bluish grey"
extended meanings; especially of psychological coldness; without human warmth or emotion; "a cold unfriendly nod"; "a cold and unaffectionate person"; "a cold impersonal manner"; "cold logic"; "the concert left me cold"
feeling or showing no enthusiasm; "a cold audience"; "a cold response to the new play"
having a low or inadequate temperature or feeling a sensation of coldness or having been made cold by e.g. ice or refrigeration; "a cold climate"; "a cold room"; "dinner has gotten cold"; "cold fingers"; "if you are cold, turn up the heat"; "a cold beer"
having lost freshness through passage of time; "a cold trail"; "dogs attempting to catch a cold scent"
lacking the warmth of life; "cold in his grave"
marked by errorless familiarity; "had her lines cold before rehearsals started"
of a seeker; far from the object sought
so intense as to be almost uncontrollable; "cold fury gripped him"
a sudden uncontrollable attack; "a paroxysm of giggling"; "a fit of coughing"; "convulsions of laughter" (同)fit, convulsion
presence of hemoglobin in the urine (同)haemoglobinuria
a pass between mountain peaks (同)gap
accompanied by or of the nature of paroxysms

PrepTutorEJDIC

『寒い』,冷たい;冷えた,冷やした / (性格・態度などが)『冷たい』,冷淡な,熱意のない;無情な,冷酷な / (物事が)人をがっかりさせる,つまらない / (色調が)冷たい,寒色の / (獲物のにおい臭が)かすかな,弱い / 《話》(打撃などで)意識を失った,失神した / 完全に,全く / 〈U〉《しばしばthe~》『寒さ』,寒け,冷たさ / 〈C〉,時に〈U〉『かぜ』,感冒
(病気の周期的な)発作《+『of』+『名』》 / (感情・行動などの)激発,発作的行動《+『of』+『名』》
(山脈の)鞍部(あんぶ),山あい

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/11/24 22:10:35」(JST)

wiki en

Paroxysmal cold hemoglobinuria (PCH), also known as Donath-Landsteiner syndrome, is a disease of humans that is characterized by the sudden presence of hemoglobin in the urine (called hemoglobinuria), typically after exposure to cold temperatures.

Presentation

People with PCH, a polyclonal IgG anti-P autoantibody binds to red blood cell surface antigens in the cold. This can occur in a susceptible individual as blood passes through cold extremities in cold weather. When the blood returns to the warmer central circulation, the red blood cells are lysed with complement, causing intravascular hemolysis. Hemoglobinuria and anemia can then occur. The anemia may be mild or severe.

Causes

PCH is a rare autoimmune haemolytic anaemia that can occur following an infection, when a microorganism triggers the formation of antibodies that cross-react with the P antigen on the red blood cell membrane (see article on the P antigen system). Viral infections that can cause PCH include measles, mumps, influenza, adenovirus, chickenpox, cytomegalovirus, and Epstein-Barr virus. Bacterial infections that can cause PCH include syphilis, Haemophilus influenzae and Mycoplasma pneumoniae. PCH can also be a side effect of some vaccinations. Chronic idiopathic (of unknown cause) PCH also occurs, but it is rare.

Treatment and prognosis

Acute PCH tends to be transient and self-limited,^[1] particularly in children. Chronic PCH associated with syphilis resolves after the syphilis is treated with appropriate antibiotics. Chronic idiopathic PCH is usually mild.

Risk factors

People with PCH are sometimes advised to avoid exposure to cold temperatures.^[2] If anemia is severe, blood transfusion may be needed. Careful compatibility testing by the blood bank is necessary because autoantibodies may interfere with blood typing. Prednisone may be used in individuals with PCH and severe anemia.

References

^ Ogose T, Wakata Y, Kaneko M, Shinahara K, Takechi T, Kotani H (October 2007). "A case of recurrent paroxysmal cold hemoglobinuria with the different temperature thresholds of Donath-Landsteiner antibodies". J. Pediatr. Hematol. Oncol. 29 (10): 716–9. doi:10.1097/MPH.0b013e31814d6845. PMID 17921855.
^ Sokol RJ, Booker DJ, Stamps R (1999). "Erythropoiesis: Paroxysmal Cold Haemoglobinuria: A Clinico-Pathological Study of Patients with a Positive Donath-Landsteiner Test". Hematology. 4 (2): 137–164. PMID 11399560.

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.

1. 発作性寒冷血色素尿症 paroxysmal cold hemoglobinuria
2. 小児における溶血性貧血の概要 overview of hemolytic anemias in children
3. Autoimmune hemolytic anemia in children: Treatment and outcome
4. Autoimmune hemolytic anemia in children: Classification, clinical features, and diagnosis
5. Cold agglutinin disease

English Journal

Treatment of atypical hemolytic uremic syndrome and thrombotic microangiopathies: a focus on eculizumab.

Schmidtko J, Peine S, El-Housseini Y, Pascual M, Meier P.SourceDivision de Néphrologie, Reseau Santé Valais, Hôpital du Valais, Sion, Switzerland; Centre de Transplantation d'Organes, Centre Hospitalier Universitaire Vaudois (CHUV), Lausanne, Switzerland. Electronic address: jan.schmidtko@hopitalvs.ch.
American journal of kidney diseases : the official journal of the National Kidney Foundation.Am J Kidney Dis.2013 Feb;61(2):289-99. doi: 10.1053/j.ajkd.2012.07.028. Epub 2012 Nov 7.
Uncontrolled complement activation is central to the occurrence of atypical hemolytic uremic syndrome (aHUS) and can result in thrombotic microangiopathies (TMAs).These terms encompass a group of heterogenic inherited or acquired diseases that recent research suggests may be triggered by the complem
PMID 23141475

Differential expression profile of Th1/Th17/Th2-related chemokines and their receptors in patients with acquired bone marrow failure syndromes.

Ge M, Zheng Y, Li X, Lu S, Li H, Chen F, Chen D, Shao Y, Shi J, Feng S.SourceState Key Laboratory of Experimental Hematology, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Science & Peking Union Medical College, 288 Nanjing Road, Tianjin 300020, PR China.
Human immunology.Hum Immunol.2013 Feb;74(2):176-80. doi: 10.1016/j.humimm.2012.11.001. Epub 2012 Nov 29.
Th1/Th17/Th2-related chemokines (CXCL10/CCL20/CCL2) and their receptors (CXCR3/CCR6/CCR2) have rarely been studied in acquired bone marrow failure syndromes (BMFs). We evaluated the concentrations of CXCL10, CCL20 and CCL2 in plasma and BM fluid from aplastic anemia (AA), paroxysmal nocturnal hemogl
PMID 23200757

Japanese Journal

Unusual association of paroxysmal cold hemoglobinuria as the first sign of disease in myelodysplastic patient

STEFANIZZI Caterina,BRECCIA Massimo,SANTOPIETRO Michelina,COLUZZI Serelina,CANNELLA Laura,GIRELLI Gabriella,ALIMENA Giuliana
International journal of hematology 89(5), 720-721, 2009-06-15
NAID 10025162247

自己免疫性溶血性貧血 (特集貧血--最新の基礎と臨床) -- (臨床編一次性貧血)

唐沢正光
日本臨床 66(3), 520-523, 2008-03
NAID 40015862601

Related Pictures

★リンクテーブル★

リンク元	「溶血性貧血」「発作性寒冷血色素尿症」「paroxysmal hemoglobinuria」「PCH」
関連記事	「cold」「paroxysm」「hemoglobinuria」「COLD」「paroxysmal」

「溶血性貧血」

Paroxysmal cold hemoglobinuria
Classification and external resources
ICD-10	D59.6
ICD-9-CM	283.2
DiseasesDB	9679
eMedicine	ped/512
MeSH	D006457
	[edit on Wikidata]

　　[★]

英: hemolytic anemia
同: 溶血性黄疸 hemolytic jaundice
関: 貧血

溶血の種類	機序	脾腫	ヘモグロビン尿ヘモジデリン尿	疾患
血管外溶血	Mφで貪食される	＋	－	球状赤血球症 HS 自己免疫性溶血性貧血 AIHA (温式) ↑冷式より多い
血管内溶血	血管内で溶血	－	＋	自己免疫性溶血性貧血 AIHA (冷式) 発作性夜間ヘモグロビン尿症 PNH G6PD欠損症赤血球破砕症候群不適合輸血

概念

赤血球寿命が種々の原因により短縮することに伴う貧血の総称。

病因

赤血球が何らかの原因により破壊され、骨髄の代償能力を超えて赤血球が減少することにより貧血を呈する。　→　溶血が存在するが貧血ではない状態がある。
溶血の原因

先天性疾患

遺伝性球状赤血球症(70%)
遺伝性楕円赤血球症
ヘモグロビン異常症、酵素欠乏症

後天性疾患

自己抗体による溶血

自己免疫性溶血性貧血(温式抗体、冷式抗体)、発作性夜間ヘモグロビン尿症、赤血球破砕症候群

HIM.653

赤血球内の素因

1. ヘモグロビンの異常
2. 膜-細胞骨格複合体の異常

遺伝性球状赤血球症(溶血性貧血のなかで一般的なもの。赤血球の細胞膜蛋白・細胞骨格の異常により球状となり脾臓で捕捉され溶血する)、hereditary elliptocytosis HE
stomatocytosis

3. 代謝経路

解糖系の異常：pyruvate kinase deficiency
還元系の異常：glucose 6-phosphate dehydrogenase deficiency
核酸異化異常：pyrimidine 5'-nucleotidase deficiency

赤血球外の素因

先天性

家族性溶血尿毒症 familial hemolytic uremic syndrome

後天性

赤血球の機械的損傷
毒物・薬物
感染
自己免疫性溶血性貧血 AIHA

発作性夜間血色素尿症 PNH

症状

貧血、無胆汁色素尿性または溶血性黄疸、脾腫

参考

[charged]Extrinsic nonimmune hemolytic anemia due to mechanical damage: Fragmentation hemolysis and hypersplenism - uptodate

http://www.uptodate.com/contents/extrinsic-nonimmune-hemolytic-anemia-due-to-mechanical-damage-fragmentation-hemolysis-and-hypersplenism?source=search_result&selectedTitle=2%7E150

「発作性寒冷血色素尿症」

　　[★]

英: paroxysmal cold hemoglobinuria PCH
同: 発作性寒冷ヘモグロビン尿症、

[show details]

発作性寒冷血色素尿症 : 約 19,300 件
発作性寒冷ヘモグロビン尿症 : 約 82 件

「paroxysmal hemoglobinuria」

　　[★]

発作性ヘモグロビン尿症

関: paroxysmal cold hemoglobinuria、paroxysmal nocturnal hemoglobinuria

「PCH」

　　[★] 発作性寒冷血色素尿症 paroxysmal cold hemoglobinuria

「cold」

　　[★]

adj.

寒い、冷たい、寒気がする。冷やした、冷えた(opp.hot)。加熱せずに処理する
冷たくなった、死んでいる
冷静な。冷淡な。よそよそしい。(やっと抑えている)激しい(怒り)。不感症の。気を滅入らせる、寒々とした
興ざましの、つまらない。気乗りのしない。(味が)弱い
客観的な(事実)

「paroxysm」

　　[★]

n.

(医)(寒気などの周期的な)発作。(医)痙攣。激発。発作的活動、激動

関: (adj.)paroxysmal
関: attack、bout、crises、crisis、insult、seizure、stroke

「hemoglobinuria」

　　[★] ヘモグロビン尿症、血色素尿症、ヘモグロビン尿、血色素尿

「COLD」

　　[★]

同: chronic obstructive lung disease, 慢性閉塞性肺疾患

「paroxysmal」

　　[★]

adj.

発作性の

関: ictal, paroxystic, paroxysm

[pmid17921855-1] Ogose T, Wakata Y, Kaneko M, Shinahara K, Takechi T, Kotani H (October 2007). "A case of recurrent paroxysmal cold hemoglobinuria with the different temperature thresholds of Donath-Landsteiner antibodies". J. Pediatr. Hematol. Oncol. 29 (10): 716–9. doi:10.1097/MPH.0b013e31814d6845. PMID 17921855.

[pmid11399560-2] Sokol RJ, Booker DJ, Stamps R (1999). "Erythropoiesis: Paroxysmal Cold Haemoglobinuria: A Clinico-Pathological Study of Patients with a Positive Donath-Landsteiner Test". Hematology. 4 (2): 137–164. PMID 11399560.

匿名

検索

案内

案内

paroxysmal cold hemoglobinuria