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
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
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）』「2014/04/30 13:11:31」(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 digestive form, however 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 in these patients.^[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 F, Klein U and Kuppers 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 et al. (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 et al. (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 et al. (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, Chien-Hsiang; 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. Cite uses deprecated parameters (help)
^ 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. PMID 2509855.

UpToDate Contents

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1. 重鎖病 the heavy chain diseases
2. 免疫グロブリン軽鎖（AL）アミロイドーシスおよび軽鎖重鎖沈着症の病因 pathogenesis of immunoglobulin light chain al amyloidosis and light and heavy chain deposition diseases
3. 免疫グロブリン軽鎖（AL）アミロイドーシスおよび軽鎖重鎖沈着症の予後および治療 prognosis and treatment of immunoglobulin light chain al amyloidosis and light and heavy chain deposition diseases
4. 多発性骨髄腫の臨床的特徴、検査所見、および診断 clinical features laboratory manifestations and diagnosis of multiple myeloma
5. 末梢動脈疾患（PAD）の疫学、危険因子、および自然経過 epidemiology risk factors and natural history of peripheral artery disease

English Journal

Cochlear implantation in a patient with Epstein syndrome.

Nishiyama N, Kawano A, Kawaguchi S, Shirai K, Suzuki M.SourceDepartment of Otorhinolaryngology, Tokyo Medical University, Tokyo, Japan. Electronic address: nobuoto@tokyo-med.ac.jp.
Auris, nasus, larynx.Auris Nasus Larynx.2013 Aug;40(4):409-12. doi: 10.1016/j.anl.2012.07.005. Epub 2012 Jul 31.
Epstein syndrome is a rare disease which is accompanied by nephritis, sensorineural hearing impairment and macrothrombocytopenia. It has been suggested that this syndrome is a hereditary disease associated with mutations in MYH9, which encodes non-muscle myosin heavy chain IIA. We report a case of a
PMID 22854055

Sexually dimorphic myofilament function and cardiac troponin I phosphospecies distribution in hypertrophic cardiomyopathy mice.

McKee LA, Chen H, Regan JA, Behunin SM, Walker JW, Walker JS, Konhilas JP.SourceDepartment of Physiology, Sarver Molecular Cardiovascular Research Program, University of Arizona, 1501 N. Campbell Ave., Tucson, AZ 85724, USA.
Archives of biochemistry and biophysics.Arch Biochem Biophys.2013 Jul 1;535(1):39-48. doi: 10.1016/j.abb.2012.12.023. Epub 2013 Jan 23.
The pathological progression of hypertrophic cardiomyopathy (HCM) is sexually dimorphic such that male HCM mice develop phenotypic indicators of cardiac disease well before female HCM mice. Here, we hypothesized that alterations in myofilament function underlies, in part, this sex dimorphism in HCM
PMID 23352598

Japanese Journal

A Unique Case of γ-heavy Chain Deposition Disease Characterized by Concomitant Deposition of γ2 and γ4 Subclasses

, , , ,
Internal Medicine 53(22), 2615-2618, 2014
… Heavy chain deposition disease (HCDD) is a rare entity. … γ-HCDD is the predominant subtype and is characterized by glomerular and tubular deposition of a single γ-heavy chain subclass. … We herein describe a unique case of γ-HCDD with concurrent deposition of two subclasses, presenting a new subtype to the disease spectrum. …
NAID 130004713251

基礎と臨床の連携による疾患バイオマーカーの開発

野村文夫
生物物理化学 58(1), 5-8, 2014
… <br/>血清プロテオーム解析により我々が報告したフィブリノーゲンα C-chainのnear C-terminal fragment（FIC5.9と略）は簡便なELISA系の確立により，多疾患・多検体の解析が可能となった。 …
NAID 130004704672