n.

巨脾症、巨脾

mild hepatomegaly may accompany the splenomegaly but is unusual in the absence of splenic enlargement(HIM.674)

WordNet

an abnormal enlargement of the spleen

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/07/15 17:40:52」(JST)

wiki en

[Wiki en表示]

Splenomegaly is an enlargement of the spleen. The spleen usually lies in the left upper quadrant (LUQ) of the human abdomen. It is one of the four cardinal signs of hypersplenism, some reduction in the number of circulating blood cells affecting granulocytes, erythrocytes or platelets in any combination; a compensatory proliferative response in the bone marrow; and the potential for correction of these abnormalities by splenectomy. Splenomegaly is usually associated with increased workload (such as in hemolytic anemias), which suggests that it is a response to hyperfunction. It is therefore not surprising that splenomegaly is associated with any disease process that involves abnormal red blood cells being destroyed in the spleen. Other common causes include congestion due to portal hypertension and infiltration by leukemias and lymphomas. Thus, the finding of an enlarged spleen, along with caput medusa, is an important sign of portal hypertension.^[1]

Normal spleen

Definition

Poulin et al.^[2] classify splenomegaly as:

Moderate splenomegaly, if the largest dimension is between 11–20 cm
Severe splenomegaly, if the largest dimension is greater than 20 cm
Splenomegaly refers strictly to spleen enlargement, and is distinct from hypersplenism, which connotes overactive function by a spleen of any size. Splenomegaly and hypersplenism should not be confused. Each may be found separately, or they may coexist.
Clinically if a spleen is palpable, it means it is enlarged as it has to undergo at least twofold enlargement to become palpable. However, the tip of the spleen may be palpable in a newborn baby up to 3 months of age.

Signs and symptoms

Symptoms may include abdominal pain, chest pain, chest pain similar to pleuritic pain when stomach, bladder or bowels are full, back pain, early satiety due to splenic encroachment, or the symptoms of anemia due to accompanying cytopenia.

Signs of splenomegaly may include a palpable left upper quadrant abdominal mass or splenic rub. It can be detected on physical examination by using Castell's sign or Traube's space, but an ultrasound can be used to confirm diagnosis. In patients where the likelihood of splenomegaly is high, the physical exam is not sufficiently sensitive to detect it; abdominal imaging is indicated in such patients.^[3]

Causes

The most common causes of splenomegaly in developed countries are infectious mononucleosis, splenic infiltration with cancer cells from a hematological malignancy and portal hypertension (most commonly secondary to liver disease, and Sarcoidosis). Splenomegaly may also come from bacterial infections, such as syphilis or an infection of the heart's inner lining (endocarditis).^[4]

The possible causes of moderate splenomegaly (spleen <1000 g) are many, and include:

Splenomegaly grouped on the basis of the pathogenic mechanism
Increased function	Abnormal blood flow	Infiltration
Removal of defective RBCs spherocytosis thalassemia hemoglobinopathies nutritional anemias early sickle cell anemia Immune hyperplasia Response to infection (viral, bacterial, fungal, parasitic) mononucleosis, AIDS,^[5] viral hepatitis subacute bacterial endocarditis, bacterial septicemia splenic abscess, typhoid fever brucellosis, leptospirosis, tuberculosis histoplasmosis malaria, leishmaniasis, trypanosomiasis ehrlichiosis^[6] Disordered immunoregulation rheumatoid arthritis systemic lupus erythematosus serum sickness familial hemophagocytic lymphohistiocytosis autoimmune hemolytic anemia autoimmune lymphoproliferative syndrome, an autosomal dominant disorder sarcoidosis drug reactions Extramedullary hematopoiesis myelofibrosis marrow infiltration by tumors, leukemias marrow damage by radiation, toxins	Organ Failure cirrhosis Vascular hepatic vein obstruction portal vein obstruction Budd–Chiari syndrome splenic vein obstruction Infections hepatic schistosomiasis hepatic echinococcosis	Metabolic diseases Gaucher disease Niemann–Pick disease alpha-mannosidosis Hurler syndrome and other mucopolysaccharidoses^[7] amyloidosis Tangier disease Benign and malignant “infiltrations” leukemias (acute, chronic, lymphoid, and myeloid) lymphomas (Hodgkins and non-Hodgkins) myeloproliferative disease metastatic tumors (commonly melanoma) histiocytosis X hemangioma, lymphangioma splenic cysts hamartomas eosinophilic granuloma littoral cell angioma^[8]^[9]^[10]

The causes of massive splenomegaly (spleen >1000 g) are fewer, and include:

visceral leishmaniasis (kala-azar)
chronic myelogenous leukemia
myelofibrosis
malaria
splenic marginal zone lymphoma

Treatment

If the splenomegaly underlies hypersplenism, a splenectomy is indicated and will correct the hypersplenism. However, the underlying cause of the hypersplenism will most likely remain; consequently, a thorough diagnostic workup is still indicated, as, leukemia, lymphoma and other serious disorders can cause hypersplenism and splenomegaly. After splenectomy, however, patients have an increased risk for infectious diseases.

Patients undergoing splenectomy should be vaccinated against Haemophilus influenzae, Streptococcus pneumoniae, and Meningococcus. They should also receive annual influenza vaccinations. Long-term prophylactic antibiotics may be given in certain cases.

In cases of infectious mononucleosis splenomegaly is a common symptom and health care providers may consider using abdominal ultrasonography to get insight into a person's condition.^[11] However, because spleen size varies greatly, ultrasonography is not a valid technique for assessing spleen enlargement and should not be used in typical circumstances or to make routine decisions about fitness for playing sports.^[11]

References

^ Ghazi, Ali (2010). "Hypercalcemia and huge splenomegaly presenting in an elderly patient with B-cell non-Hodgkin's lymphoma: a case report". Journal of Medical Case Reports 4 (334). doi:10.1186/1752-1947-4-330.
'^ Matacia-Murphy et al., Splenomegaly (Medscape, updated Apr. 2012)
^ Grover SA, Barkun AN, Sackett DL; Barkun; Sackett (1993). "The rational clinical examination. Does this patient have splenomegaly?". JAMA 270 (18): 2218–21. doi:10.1001/jama.270.18.2218. PMID 8411607. Ovid full text
^ Kaiser, Larry R.; Pavan Atluri; Giorgos C Karakousis; Paige M Porrett (2006). The surgical review: an integrated basic and clinical science study guide. Hagerstwon, MD: Lippincott Williams & Wilkins. ISBN 0-7817-5641-3.
^ Sproat, LO.; Pantanowitz, L.; Lu, CM.; Dezube, BJ. (Dec 2003). "Human immunodeficiency virus-associated hemophagocytosis with iron-deficiency anemia and massive splenomegaly". Clin Infect Dis 37 (11): e170–3. doi:10.1086/379613. PMID 14614691.
^ Friedman, AD.; Daniel, GK.; Qureshi, WA. (Jun 1997). "Systemic ehrlichiosis presenting as progressive hepatosplenomegaly". South Med J 90 (6): 656–60. doi:10.1097/00007611-199706000-00017. PMID 9191748.
^ Neufeld EF, Muenzer J. (1995). "The mucopolysaccharidoses". In Scriver CR, Beaudet AL, Sly WS, Valle D, eds. The metabolic and molecular bases of inherited disease.7th ed. Vol. 2. McGraw-Hill , New York. pp. 2465–94.
^ Suvajdzić, N.; Cemerikić-Martinović, V.; Saranović, D.; Petrović, M.; Popović, M.; Artiko, V.; Cupić, M.; Elezović, I. (Oct 2006). "Littoral-cell angioma as a rare cause of splenomegaly". Clin Lab Haematol 28 (5): 317–20. doi:10.1111/j.1365-2257.2006.00801.x. PMID 16999722.
^ Dascalescu, CM.; Wendum, D.; Gorin, NC. (Sep 2001). "Littoral-cell angioma as a cause of splenomegaly". N Engl J Med 345 (10): 772–3. doi:10.1056/NEJM200109063451016. PMID 11547761.
^ Ziske, C.; Meybehm, M.; Sauerbruch, T.; Schmidt-Wolf, IG. (Jan 2001). "Littoral cell angioma as a rare cause of splenomegaly". Ann Hematol 80 (1): 45–8. doi:10.1007/s002770000223. PMID 11233776.
^ ^a ^b American Medical Society for Sports Medicine (24 April 2014), "Five Things Physicians and Patients Should Question", Choosing Wisely: an initiative of the ABIM Foundation (American Medical Society for Sports Medicine), retrieved 29 July 2014 , which cites
- Putukian, M; O'Connor, FG; Stricker, P; McGrew, C; Hosey, RG; Gordon, SM; Kinderknecht, J; Kriss, V; Landry, G (Jul 2008). "Mononucleosis and athletic participation: an evidence-based subject review". Clinical journal of sport medicine : official journal of the Canadian Academy of Sport Medicine 18 (4): 309–15. doi:10.1097/jsm.0b013e31817e34f8. PMID 18614881.
- Spielmann, AL; DeLong, DM; Kliewer, MA (Jan 2005). "Sonographic evaluation of spleen size in tall healthy athletes.". AJR. American journal of roentgenology 184 (1): 45–9. doi:10.2214/ajr.184.1.01840045. PMID 15615949.

External links

PatientPlus Splenomegaly and hypersplenism
Medical dictionary
11-141b. at Merck Manual of Diagnosis and Therapy Professional Edition (Hypersplenism)
/Splenomegaly and Hypersplenism

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. 脾腫およびその他の脾疾患を有する成人患者に対するアプローチ approach to the adult patient with splenomegaly and other splenic disorders
2. 脾腫を有する小児に対するアプローチ approach to the child with an enlarged spleen
3. アルコール性肝炎：臨床症状および診断 alcoholic hepatitis clinical manifestations and diagnosis
4. 成人における慢性門脈血栓症：臨床症状、診断、および管理 chronic portal vein thrombosis in adults clinical manifestations diagnosis and management
5. 進行期（III/IV）濾胞性リンパ腫の初期治療 initial treatment of advanced stage iii iv follicular lymphoma

English Journal

Symptomatic Profiles of Patients With Polycythemia Vera: Implications of Inadequately Controlled Disease.

Geyer H1, Scherber R1, Kosiorek H1, Dueck AC1, Kiladjian JJ1, Xiao Z1, Slot S1, Zweegman S1, Sackmann F1, Fuentes AK1, Hernández-Maraver D1, Döhner K1, Harrison CN1, Radia D1, Muxi P1, Besses C1, Cervantes F1, Johansson PL1, Andreasson B1, Rambaldi A1, Barbui T1, Bonatz K1, Reiter A1, Boyer F1, Etienne G1, Ianotto JC1, Ranta D1, Roy L1, Cahn JY1, Maldonado N1, Barosi G1, Ferrari ML1, Gale RP1, Birgegard G1, Xu Z1, Zhang Y1, Sun X1, Xu J1, Zhang P1, Te Boekhorst PA1, Commandeur S1, Schouten H1, Pahl HL1, Griesshammer M1, Stegelmann F1, Lehmann T1, Senyak Z1, Vannucchi AM1, Passamonti F1, Samuelsson J1, Mesa RA1.
Journal of clinical oncology : official journal of the American Society of Clinical Oncology.J Clin Oncol.2016 Jan 10;34(2):151-9. doi: 10.1200/JCO.2015.62.9337. Epub 2015 Nov 23.
PURPOSE: Polycythemia vera (PV) is a myeloproliferative neoplasm (MPN) associated with disabling symptoms and a heightened risk of life-threatening complications. Recent studies have demonstrated the effectiveness of JAK inhibitor therapy in patients with PV patients who have a history of prior hydr
PMID 26598745

Sirolimus is effective in relapsed/refractory autoimmune cytopenias: results of a prospective multi-institutional trial.

Bride KL1, Vincent T1, Smith-Whitley K2, Lambert MP2, Bleesing JJ3, Seif AE1, Manno CS4, Casper J5, Grupp SA1, Teachey DT1.
Blood.Blood.2016 Jan 7;127(1):17-28. doi: 10.1182/blood-2015-07-657981. Epub 2015 Oct 26.
Patients with autoimmune multilineage cytopenias are often refractory to standard therapies requiring chronic immunosuppression with medications with limited efficacy and high toxicity. We present data on 30 patients treated on a multicenter prospective clinical trial using sirolimus as monotherapy.
PMID 26504182

Breed Distribution and Clinical Characteristics of B Cell Chronic Lymphocytic Leukemia in Dogs.

Bromberek JL1, Rout ED1, Agnew MR1, Yoshimoto J1, Morley PS2, Avery AC1.
Journal of veterinary internal medicine / American College of Veterinary Internal Medicine.J Vet Intern Med.2016 Jan 6. doi: 10.1111/jvim.13814. [Epub ahead of print]
BACKGROUND: B-cell chronic lymphocytic leukemia (B-CLL) is the most common hematopoietic malignancy in humans in the developed world and the primary risk factor is genetic. Dogs also develop B-CLL, but there is no systematic description of the disease in dogs. Understanding the epidemiology of B-CLL
PMID 26740174

Japanese Journal

症例報告甲状腺クリーゼに全身リンパ節腫脹,脾腫および汎血球減少,さらには複数の自己抗体の上昇を認め,造血系疾患や自己免疫疾患の鑑別を要したBasedow病の一例(簡潔表題 : 造血系,自己免疫疾患の鑑別を要したBasedow病の一例)

日本甲状腺学会雑誌 = Journal of the Japan Thyroid Association 6(2), 125-128, 2015-10
NAID 40020630306

Modifications in B-Lymphocyte Number and Phenotype in the Course of Pregnancy in a Woman with Persistent Polyclonal B-Cell Lymphocytosis: A Flow Cytometric Study

Journal of Clinical and Experimental Hematopathology 55(2), 77-82, 2015
NAID 130005104668

Successful Treatment of Bing-Neel Syndrome Accompanying Waldenström's Macroglobulinemia with R-MPV: A Case Report

Journal of Clinical and Experimental Hematopathology 55(2), 113-119, 2015
NAID 130005104663

Related Pictures

★リンクテーブル★

リンク元	「脾腫」「脾腫大」
拡張検索	「fibrocongestive splenomegaly」

「脾腫」

Splenomegaly
	CT scan showing splenomegaly in a patient with chronic lymphoid leukemia
Classification and external resources
Specialty	General surgery
ICD-10	Q89.0, R16.1
ICD-9-CM	759.0, 789.2
DiseasesDB	12375
MedlinePlus	003276
eMedicine	ped/2139 med/2156
MeSH	D013163

　　[★]

英: splenomegaly
関: 脾臓

定義

脾臓が腫大した状態(250以上(正常は90-120g))
脾の大きさは長径と重量がよく相関し、超音波診断では長径×短径(SI スプリーンインデックス)が脾腫の指標となる。
正常の2倍程度となると脾腫として左季肋部で診察可能となる
慢性骨髄増殖症候群、慢性リンパ性白血病、ヘアリー細胞白血病、遺伝性溶血性貧血、フェルティ症候群、マラリアでは巨大脾腫を認める。

頻度

急性ウイルス感染の約50%、銃声細菌感染症の約20%、慢性肝疾患の50-70%、溶血性貧血で約70%、血液造血器疾患で約40%に出現する。(IMD.544)

症状

ほぼない。
胃か圧排するほど大きくなれば、胃の膨満感が出現しうる。

診察

仰臥位で右季肋部を触診
小児では触れても良いが、健常成人では触れない。触れたら異常である。
臍まで脾臓を脾臓を触知できる場合を特に巨脾という。
打診または触診で診察する

打診

IMD 544

Nixon法

右側臥位とする
後腋窩線上を胸部から腹部に向かい打診する。
肺鼓音から濁音に変わった部位から左肋骨弓に直角に向かい打診をする。
正常では濁音界が6-8cmであるが、8cmを超えた場合異常と判断する。

Castell法

仰臥位とする
- 前腋窩線上を胸部から腹部に向かい打診する。
第8または第9肋間は正常では鼓音であるが、濁音であれば異常と判断する。

Traube法

左第6肋骨上縁と左中腋窩線および左肋骨弓に囲まれた部位を(トラウベ半月腔)という。
空腹時、通常の呼吸ではこの領域は鼓音であるが、濁音であれば異常と判断する。

検査

腹部エコー、腹部CT
腹部エコーで長径が10cmを超えると脾腫と判断しうる

鑑別疾患

病因による鑑別

V 血管：全身性うっ血(心不全)、門脈系うっ血(肝硬変、門脈血栓症)
I 感染症：細菌性心内膜炎、伝染性単核球症、結核、梅毒、エキノコックス、日本住血吸収症、マラリア
N 血液悪性腫瘍(悪性リンパ腫、白血病、骨髄線維症、慢性骨髄増殖症候群)
D
I
C 血液系疾患(溶血性貧血(自己免疫性溶血性貧血、遺伝性球状赤血球症、サラセミア)、悪性貧血、特発性血小板減少性紫斑病)、免疫異常(関節リウマチ、フェルティ症候群、全身性エリテマトーデス)、蓄積病(ムコ糖質蓄積など)、嚢胞性疾患
A
T
E

病態による鑑別疾患

脾臓は巨大なリンパ器官と考えられるために、原因は組織を考えれば鑑別を挙げることが容易となる。

	産生増加	新生物	破壊亢進	閉塞	浸潤
赤血球	再生不良性貧血骨髄癆性貧血	赤血球増加症	溶血性貧血エリテマトーデス悪性貧血
白血球	骨髄異形成感染症	白血病	無顆粒球症
血小板			特発性血小板減少性紫斑病
リンパ組織	伝染性単核球症	ホジキンリンパ腫嚢胞性リンパ管腫
支持組織		転移性癌	エリテマトーデスコラーゲン病		ヘモクロマトーシス細網内皮症ハーラー病アミロイドーシスサルコイドーシス
動脈				塞栓症動脈瘤
静脈		血管腫		うっ血性心不全肝硬変血栓症バンチ病膵尾部膵癌

治療

基礎疾患の治療
脾摘：脾機能亢進、遺伝性溶血性貧血、特発性血小板減少性紫斑病、脾破裂

「脾腫大」

　　[★]

英: splenomegaly
関: 脾腫

「fibrocongestive splenomegaly」

　　[★] 線維性うっ血性脾腫

[1] Ghazi, Ali (2010). "Hypercalcemia and huge splenomegaly presenting in an elderly patient with B-cell non-Hodgkin's lymphoma: a case report". Journal of Medical Case Reports 4 (334). doi:10.1186/1752-1947-4-330.

[2] '^ Matacia-Murphy et al., Splenomegaly (Medscape, updated Apr. 2012)

[pmid8411607-3] Grover SA, Barkun AN, Sackett DL; Barkun; Sackett (1993). "The rational clinical examination. Does this patient have splenomegaly?". JAMA 270 (18): 2218–21. doi:10.1001/jama.270.18.2218. PMID 8411607. Ovid full text

[4] Kaiser, Larry R.; Pavan Atluri; Giorgos C Karakousis; Paige M Porrett (2006). The surgical review: an integrated basic and clinical science study guide. Hagerstwon, MD: Lippincott Williams & Wilkins. ISBN 0-7817-5641-3.

[pmid14614691-5] Sproat, LO.; Pantanowitz, L.; Lu, CM.; Dezube, BJ. (Dec 2003). "Human immunodeficiency virus-associated hemophagocytosis with iron-deficiency anemia and massive splenomegaly". Clin Infect Dis 37 (11): e170–3. doi:10.1086/379613. PMID 14614691.

[pmid9191748-6] Friedman, AD.; Daniel, GK.; Qureshi, WA. (Jun 1997). "Systemic ehrlichiosis presenting as progressive hepatosplenomegaly". South Med J 90 (6): 656–60. doi:10.1097/00007611-199706000-00017. PMID 9191748.

[Neufeld1995-7] Neufeld EF, Muenzer J. (1995). "The mucopolysaccharidoses". In Scriver CR, Beaudet AL, Sly WS, Valle D, eds. The metabolic and molecular bases of inherited disease.7th ed. Vol. 2. McGraw-Hill , New York. pp. 2465–94.

[pmid16999722-8] Suvajdzić, N.; Cemerikić-Martinović, V.; Saranović, D.; Petrović, M.; Popović, M.; Artiko, V.; Cupić, M.; Elezović, I. (Oct 2006). "Littoral-cell angioma as a rare cause of splenomegaly". Clin Lab Haematol 28 (5): 317–20. doi:10.1111/j.1365-2257.2006.00801.x. PMID 16999722.

[pmid11547761-9] Dascalescu, CM.; Wendum, D.; Gorin, NC. (Sep 2001). "Littoral-cell angioma as a cause of splenomegaly". N Engl J Med 345 (10): 772–3. doi:10.1056/NEJM200109063451016. PMID 11547761.

[pmid11233776-10] Ziske, C.; Meybehm, M.; Sauerbruch, T.; Schmidt-Wolf, IG. (Jan 2001). "Littoral cell angioma as a rare cause of splenomegaly". Ann Hematol 80 (1): 45–8. doi:10.1007/s002770000223. PMID 11233776.

[AMSSMfive-11] American Medical Society for Sports Medicine (24 April 2014), "Five Things Physicians and Patients Should Question", Choosing Wisely: an initiative of the ABIM Foundation (American Medical Society for Sports Medicine), retrieved 29 July 2014 , which cites

Putukian, M; O'Connor, FG; Stricker, P; McGrew, C; Hosey, RG; Gordon, SM; Kinderknecht, J; Kriss, V; Landry, G (Jul 2008). "Mononucleosis and athletic participation: an evidence-based subject review". Clinical journal of sport medicine : official journal of the Canadian Academy of Sport Medicine 18 (4): 309–15. doi:10.1097/jsm.0b013e31817e34f8. PMID 18614881.

Spielmann, AL; DeLong, DM; Kliewer, MA (Jan 2005). "Sonographic evaluation of spleen size in tall healthy athletes.". AJR. American journal of roentgenology 184 (1): 45–9. doi:10.2214/ajr.184.1.01840045. PMID 15615949.

[12] Putukian, M; O'Connor, FG; Stricker, P; McGrew, C; Hosey, RG; Gordon, SM; Kinderknecht, J; Kriss, V; Landry, G (Jul 2008). "Mononucleosis and athletic participation: an evidence-based subject review". Clinical journal of sport medicine : official journal of the Canadian Academy of Sport Medicine 18 (4): 309–15. doi:10.1097/jsm.0b013e31817e34f8. PMID 18614881.

[13] Spielmann, AL; DeLong, DM; Kliewer, MA (Jan 2005). "Sonographic evaluation of spleen size in tall healthy athletes.". AJR. American journal of roentgenology 184 (1): 45–9. doi:10.2214/ajr.184.1.01840045. PMID 15615949.

匿名

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案内

案内

splenomegaly