WordNet

unusually great in degree or quantity or number; "heavy taxes"; "a heavy fine"; "heavy casualties"; "heavy losses"; "heavy rain"; "heavy traffic"
a serious (or tragic) role in a play
an actor who plays villainous roles
slowly as if burdened by much weight; "time hung heavy on their hands" (同)heavily
requiring or showing effort; "heavy breathing"; "the subject made for labored reading" (同)labored, laboured
lacking lightness or liveliness; "heavy humor"; "a leaden conversation" (同)leaden
darkened by clouds; "a heavy sky" (同)lowering, sullen, threatening
slow and laborious because of weight; "the heavy tread of tired troops"; "moved with a lumbering sag-bellied trot"; "ponderous prehistoric beasts"; "a ponderous yawn" (同)lumbering, ponderous
(of sleep) deep and complete; "a heavy sleep"; "fell into a profound sleep"; "a sound sleeper"; "deep wakeless sleep" (同)profound, sound, wakeless
full and loud and deep; "heavy sounds"; "a herald chosen for his sonorous voice" (同)sonorous
full of; bearing great weight; "trees heavy with fruit"; "vines weighed down with grapes" (同)weighed down
(of an actor or role) being or playing the villain; "Iago is the heavy role in `Othello"
(physics, chemistry) being or containing an isotope with greater than average atomic mass or weight; "heavy hydrogen"; "heavy water"
dense or inadequately leavened and hence likely to cause distress in the alimentary canal; "a heavy pudding"
large and powerful; especially designed for heavy loads or rough work; "a heavy truck"; "heavy machinery"
made of fabric having considerable thickness; "a heavy coat"
marked by great psychological weight; weighted down especially with sadness or troubles or weariness; "a heavy heart"; "a heavy schedule"; "heavy news"; "a heavy silence"; "heavy eyelids"
of comparatively great physical weight or density; "a heavy load"; "lead is a heavy metal"; "heavy mahogany furniture"
of great intensity or power or force; "a heavy blow"; "the fighting was heavy"; "heavy seas"
of relatively large extent and density; "a heavy line"
of the military or industry; using (or being) the heaviest and most powerful armaments or weapons or equipment; "heavy artillery"; "heavy infantry"; "a heavy cruiser"; "heavy guns"; "heavy industry involves large-scale production of basic products (such as steel) used by other industries"
sharply inclined; "a heavy grade"
fasten or secure with chains; "Chain the chairs together"
a unit of length
a necklace made by a stringing objects together; "a string of beads"; "a strand of pearls"; (同)string, strand
a linked or connected series of objects; "a chain of daisies"
a series of (usually metal) rings or links fitted into one another to make a flexible ligament
anything that acts as a restraint
(chemistry) a series of linked atoms (generally in an organic molecule) (同)chemical chain
a series of things depending on each other as if linked together; "the chain of command"; "a complicated concatenation of circumstances" (同)concatenation
(business) a number of similar establishments (stores or restaurants or banks or hotels or theaters) under one ownership
connect or arrange into a chain by linking
an impairment of health or a condition of abnormal functioning
a unit of magnetic field strength equal to one-hundred-thousandth of an oersted
the 3rd letter of the Greek alphabet
caused by or altered by or manifesting disease or pathology; "diseased tonsils"; "a morbid growth"; "pathologic tissue"; "pathological bodily processes" (同)morbid, pathologic, pathological

PrepTutorEJDIC

『重い』,重量のある / (分量・程度・規模・強さなどが)『激しい』,大きい, / 『厳しい』,耐えがたい,つらい / 悲しい,陰気な / (動作が)のろい,ぎこちない / ものうい,だるい / (空が)曇った,うっとうしい,(海が)荒れた / (食物が)消化の悪い / 敵役,悪役
『鎖』;(装飾用の)鎖 / 《複数形で》『束縛』,拘束;囚人をつなぐ鎖 / (物事の)『連続』,つながり《+『of』+『名』》 / (商店・銀行・ホテルなどの)チェーン(一連の店が同一資本のもとで連携して経営される方式;その店) / チェーン(測量で用いられる単位;約21.7m) / …'を'『鎖でつなぐ』《+『up』(『together』)+『名,』+『名』+『up』(『together』)》
(体の)『病気』,疾患 / (精神・道徳などの)病気,病弊
女性の話術芸人 =diseur
ガンマ(ギリシア語アルファベットの第3字Γ,γ;英語のG,gに相当)
病気にかかった / 病的な,不健全な(morbid)

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2017/04/02 16:30:11」(JST)

wiki en

Heavy chain disease is a form of paraproteinemia with a proliferation of cells producing immunoglobulin heavy chains.^[1]

This disease is characterized by an excessive production of heavy chains that are short and truncated. These heavy chain disease proteins have various deletions, mainly in their amino-terminal part, which causes the heavy chains to lose the ability to form disulfide bonds with the light chains. The defect in the immunoglobulins presumably arises during somatic hypermutation.^[2] Deletion of the N-terminal part of the heavy chain disease protein leads to aggregation and signaling of the B cell receptor,^[3] presumably due to the loss of the anti-aggregating properties of the light chain.^[4]

Classification

There are four forms:

alpha chain disease^[5] (Seligmann's disease)
gamma chain disease^[6]^[7] (Franklin's disease)
mu chain disease^[8]
delta chain disease^[9]

IgA/αHCD

The most common type of heavy chain disease is the IgA type, known as αHCD. The most common type of αHCD is the gastrointestinal form (known as immunoproliferative small intestine disease or IPSID), but it has also been reported in the respiratory tract, and other areas of the body.^[10]

IgG/γHCD

Franklin's disease (gamma heavy chain disease) It is a very rare B-cell lymphoplasma cell proliferative disorder which may be associated with autoimmune diseases and infection is a common characteristic of the disease.^[6] It is characterized by lymphadenopathy, fever, anemia, malaise, hepatosplenomegaly, and weakness. The most distinctive symptom is palatal edema, caused by nodal involvement of Waldeyer's ring. Diagnosis is made by the demonstration of an anomalous serum M component that reacts with anti-IgG but not anti-light chain reagents. Bone marrow examination is usually nondiagnostic. Patients usually have a rapid downhill course and die of infection if left untreated or misdiagnosed.

Patients with Franklin disease usually have a history of progressive weakness, fatigue, intermittent fever, night sweats and weight loss and may present with lymphadenopathy (62%), splenomegaly (52%) or hepatomegaly (37%). The fever is considered secondary to impaired cellular and humoral immunity, and thus recurrent infections are the common clinical presentation in Franklin disease. Weng et al. described the first case of Penicillium sp. infection in a patient with Franklin disease and emphasized the importance of proper preparation for biopsy, complete hematologic investigation, culture preparation and early antifungal coverage to improve the outcome.^[6]^[10]

The γHCD can be divided into three categories based on the various clinical and pathological features. These categories are disseminated lymphoproliferative disease, localized proliferative disease and no apparent proliferative disease.

Disseminated lymphoproliferative disease is found in 57-66% of patients diagnosed with γHCD. Lymphadenopathy and constitutional symptoms are the usual features.^[11]
Localized proliferative disease is found in approximately 25% of γHCD patients. This is characterized by a localization of the mutated heavy chains in extramedullary tissue, or solely in the bone marrow.^[10]
No apparent proliferative disease is seen in 9-17% of patients with γHCD, and there is almost always an underlying autoimmune disorder.^[11]

IgM/μHCD

The IgM type of heavy chain disease, μHCD, is often misdiagnosed as chronic lymphoid leukemia (CLL) because the two diseases are often associated with each other and show similar symptoms.^[11]

References

^ "Heavy Chain Diseases: Plasma Cell Disorders: Merck Manual Home Edition". Retrieved 2008-02-29.
^ Goossens T, Klein U, Küppers R (1998). "Frequent occurrence of deletions and duplications during somatic hypermutation: Implications for oncogene translocations and heavy chain disease". PNAS. 95 (5): 2463–8. doi:10.1073/pnas.95.5.2463. PMC 19376. PMID 9482908.
^ Corcos D, Dunda O, Butor C, Cesbron JY, Lorès P, Bucchini D, Jami J (1995). "Pre-B-cell development in the absence of lambda 5 in transgenic mice expressing a heavy-chain disease protein". Curr. Biol. 5 (10): 1140–8. doi:10.1016/S0960-9822(95)00230-2. PMID 8548286.
^ Corcos D, Osborn MJ, Matheson LS, Santos F, Zou X, Smith JA, Morgan G, Hutchings A, Hamon M, Oxley D, Brüggemann M (2010). "Immunoglobulin aggregation leading to Russell body formation is prevented by the antibody light chain". Blood. 115 (2): 282–8. doi:10.1182/blood-2009-07-234864. PMID 19822901.
^ Fakhfakh F, Dellagi K, Ayadi H, Bouguerra A, Fourati R, Ben Ayed F, Brouet JC, Tsapis A (1992). "Alpha heavy chain disease alpha mRNA contain nucleotide sequences of unknown origins". Eur. J. Immunol. 22 (11): 3037–40. doi:10.1002/eji.1830221141. PMID 1425927.
^ ^a ^b ^c Weng CH, Wang RC, Hsieh TY, Tsai CA, Lin TH (July 2012). "Penicillium pneumonia in a patient with newly diagnosed Franklin disease". Am J Med Sci. 344 (1): 69–71. doi:10.1097/MAJ.0b013e31824a8927. PMID 22543591.
^ Wahner-Roedler DL, Witzig TE, Loehrer LL, Kyle RA (2003). "Gamma-heavy chain disease: review of 23 cases". Medicine (Baltimore). 82 (4): 236–50. doi:10.1097/01.md.0000085058.63483.7f. PMID 12861101.
^ Wahner-Roedler DL, Kyle RA (1992). "Mu-heavy chain disease: presentation as a benign monoclonal gammopathy". Am. J. Hematol. 40 (1): 56–60. doi:10.1002/ajh.2830400112. PMID 1566748.
^ Vilpo JA, Irjala K, Viljanen MK, Klemi P, Kouvonen I, Rönnemaa T (1980). "Delta-Heavy chain disease: A study of a case". Clin Immunol Immunopathol. 17 (4): 584–94. doi:10.1016/0090-1229(80)90154-3. PMID 6777103.
^ ^a ^b ^c Wahner-Roedler DL, Kyle RA (2005). "Heavy chain diseases". Best Pract Res Clin Haematol. 18 (4): 729–46. doi:10.1016/j.beha.2005.01.029. PMID 16026747.
^ ^a ^b ^c Fermand JP, Brouet JC, Danon F, Seligmann M (1989). "Gamma heavy chain "disease": heterogeneity of the clinicopathologic features. Report of 16 cases and review of the literature". Medicine (Baltimore). 68 (6): 321–35. doi:10.1097/00005792-198911000-00001. PMID 2509855.

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. 重鎖病 the heavy chain diseases
2. リンパ形質細胞性リンパ腫の臨床症状、病理学的特徴、および診断 clinical manifestations pathologic features and diagnosis of lymphoplasmacytic lymphoma
3. モノクローナル蛋白の認識 recognition of monoclonal proteins
4. 腎生検の適応および合併症 indications for and complications of renal biopsy

English Journal

Mucosa-associated lymphoid tissue lymphoma of the thyroid with abundant IgG4-positive plasma cells.

Miki K, Orita Y, Sato Y, Sugitani I, Noyama M, Fuji S, Domae S, Nose S, Hamaya K, Yoshino T, Nishizaki K.SourceDepartment of Otolaryngology, Head and Neck Surgery, Okayama Saiseikai General Hospital, Okayama, Japan.
Auris, nasus, larynx.Auris Nasus Larynx.2013 Dec;40(6):587-90. doi: 10.1016/j.anl.2012.12.004. Epub 2013 Feb 8.
A case of thyroidal mucosa-associated lymphoid tissue (MALT) lymphoma mimicking IgG4-related disease is described. A 54-year-old male presented with acute swelling of the anterior neck. Anaplastic thyroid carcinoma (ATC), malignant lymphoma (ML), or acute deterioration of Hashimoto's thyroiditis wer
PMID 23395549

Alternative splicing of the trout Pax5 gene and identification of novel B cell populations using Pax5 signatures.

Macmurray E, Barr M, Bruce A, Epp L, Zwollo P.SourceDepartment of Biology, The College of William and Mary, Williamsburg, VA 23185-8795, United States.
Developmental and comparative immunology.Dev Comp Immunol.2013 Oct;41(2):270-81. doi: 10.1016/j.dci.2013.06.008. Epub 2013 Jun 21.
Pax5 is an alternatively spliced transcription factor that regulates B cell development and activation. The function of specific Pax5 isoforms is unknown. Here we report the existence of seven alternatively spliced isoforms of Pax5 in the rainbow trout. We hypothesized that B cells differentially ex
PMID 23796789

Japanese Journal

A Case of .GAMMA. 1-Heavy Chain Deposition Disease Successfully Treated with Melphalan and Prednisolone Therapy

Oe Yuji,Nakaya Izaya,Yahata Mayumi,Sakuma Tsutomu,Sato Hiroshi,Soma Jun
Internal Medicine 49(14), 1411-1415, 2010
… Heavy chain deposition disease (HCDD) is characterized by glomerular and tubular deposition of non-amyloidotic monoclonal heavy chains without associated light chains. … Staining for the constant heavy chain domains showed the deletion of the first constant heavy chain domain. …
NAID 130000299549

Gamma-heavy chain deposition disease showing nodular glomeru-losclerosis

YASUDA T.
Clin Nephrol 44, 394-399, 1995
NAID 10007119860

Related Pictures

★リンクテーブル★

リンク元	「Franklin's disease」
関連記事	「disease」「heaving」「heavy」「chain」「gamma」

「Franklin's disease」

Heavy chain disease
Classification and external resources
Specialty	oncology
ICD-10	C88.1-C88.2
ICD-9-CM	273.2
DiseasesDB	442 32226
eMedicine	med/958 med/959
MeSH	D006362
	[edit on Wikidata]

　　[★]

関: gamma heavy chain disease

「disease」

　　[★]

n.

疾患：illnessより厳密な概念。「ある臓器に明確な障害が確認され、それによって症状が出ているとはっきり説明できる場合」 (PSY.9)

特定の原因、病態生理、症状、経過、予後、病理組織所見が全てそろった場合 (PSY.9)

something that is very wrong with people's attitudes, way of life or with society.

関: ail、ailment、disease entity、disorder、ill、illness、malady、sick、sickness

注意

disease ≠ illness ≠ disorder

「heaving」

　　[★]

n.

揚げること、持ち上げること、引き揚げ。(船体の)上下揺れ。隆起、(地盤の)ふくれあがり、ヒービング

physical findings of LVH may include a heaving lV impulse on chest palpation, indicative of increased muscle mass.(PHD.325)

関: heave

「heavy」

　　[★]

adj.

比較級heavier-最上級heaviest

重い

「chain」

　　[★]

n.

鎖、チェーン

adj.

鎖状の、鎖式の

関: open-chain、strand

「gamma」

　　[★] γ ガンマ　　　

[titleHeavy_Chain_Diseases:_Plasma_Cell_Disorders:_Merck_Manual_Home_Edition-1] "Heavy Chain Diseases: Plasma Cell Disorders: Merck Manual Home Edition". Retrieved 2008-02-29.

[pmid9482908-2] Goossens T, Klein U, Küppers R (1998). "Frequent occurrence of deletions and duplications during somatic hypermutation: Implications for oncogene translocations and heavy chain disease". PNAS. 95 (5): 2463–8. doi:10.1073/pnas.95.5.2463. PMC 19376. PMID 9482908.

[pmid8548286-3] Corcos D, Dunda O, Butor C, Cesbron JY, Lorès P, Bucchini D, Jami J (1995). "Pre-B-cell development in the absence of lambda 5 in transgenic mice expressing a heavy-chain disease protein". Curr. Biol. 5 (10): 1140–8. doi:10.1016/S0960-9822(95)00230-2. PMID 8548286.

[pmid19822901-4] Corcos D, Osborn MJ, Matheson LS, Santos F, Zou X, Smith JA, Morgan G, Hutchings A, Hamon M, Oxley D, Brüggemann M (2010). "Immunoglobulin aggregation leading to Russell body formation is prevented by the antibody light chain". Blood. 115 (2): 282–8. doi:10.1182/blood-2009-07-234864. PMID 19822901.

[pmid1425927-5] Fakhfakh F, Dellagi K, Ayadi H, Bouguerra A, Fourati R, Ben Ayed F, Brouet JC, Tsapis A (1992). "Alpha heavy chain disease alpha mRNA contain nucleotide sequences of unknown origins". Eur. J. Immunol. 22 (11): 3037–40. doi:10.1002/eji.1830221141. PMID 1425927.

[Franklin_Disease-6] Weng CH, Wang RC, Hsieh TY, Tsai CA, Lin TH (July 2012). "Penicillium pneumonia in a patient with newly diagnosed Franklin disease". Am J Med Sci. 344 (1): 69–71. doi:10.1097/MAJ.0b013e31824a8927. PMID 22543591.

[pmid12861101-7] Wahner-Roedler DL, Witzig TE, Loehrer LL, Kyle RA (2003). "Gamma-heavy chain disease: review of 23 cases". Medicine (Baltimore). 82 (4): 236–50. doi:10.1097/01.md.0000085058.63483.7f. PMID 12861101.

[pmid1566748-8] Wahner-Roedler DL, Kyle RA (1992). "Mu-heavy chain disease: presentation as a benign monoclonal gammopathy". Am. J. Hematol. 40 (1): 56–60. doi:10.1002/ajh.2830400112. PMID 1566748.

[pmid6777103-9] Vilpo JA, Irjala K, Viljanen MK, Klemi P, Kouvonen I, Rönnemaa T (1980). "Delta-Heavy chain disease: A study of a case". Clin Immunol Immunopathol. 17 (4): 584–94. doi:10.1016/0090-1229(80)90154-3. PMID 6777103.

[item13-10] Wahner-Roedler DL, Kyle RA (2005). "Heavy chain diseases". Best Pract Res Clin Haematol. 18 (4): 729–46. doi:10.1016/j.beha.2005.01.029. PMID 16026747.

[item3-11] Fermand JP, Brouet JC, Danon F, Seligmann M (1989). "Gamma heavy chain "disease": heterogeneity of the clinicopathologic features. Report of 16 cases and review of the literature". Medicine (Baltimore). 68 (6): 321–35. doi:10.1097/00005792-198911000-00001. PMID 2509855.

v t e Immunoproliferative immunoglobulin disorders (D89, 273)

PCDs/PP	Plasmacytoma Multiple myeloma (Plasma cell leukemia) MGUS IgM (Macroglobulinemia/Waldenström's macroglobulinemia) heavy chain (Heavy chain disease) light chain (Primary amyloidosis)

Other hypergammaglobulinemia	Cryoglobulinemia

匿名

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案内

案内

gamma heavy chain disease