Heavy chain disease: Difference between revisions

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There are four forms:
* alpha chain disease<ref name="pmid1425927">{{cite journal |author=Fakhfakh F |title=Alpha heavy chain disease alpha mRNA contain nucleotide sequences of unknown origins |journal=Eur. J. Immunol. |volume=22 |issue=11 |pages=3037–40 |year=1992 |pmid=1425927 |doi=10.1002/eji.1830221141 |author-separator=, |author2=Dellagi K |author3=Ayadi H |display-authors=3 |last4=Bouguerra |first4=Annie |last5=Fourati |first5=Rashid |last6=Ayed |first6=Farhat Ben |last7=Brouet |first7=Jean Claude |last8=Tsapis |first8=Andréas}}</ref> (Seligmann's disease)
* gamma chain disease<ref name="Franklin Disease">{{cite journal|last=Weng CH|author2=Wang RC |author3=Hsieh TY |author4=Tsai CA |author5=Lin TH |title=Penicillium pneumonia in a patient with newly diagnosed Franklin disease|journal=Am J Med Sci.|date=July 2012|volume=344|issue=1|pages=69–71|doi=10.1097/MAJ.0b013e31824a8927|pmid=22543591|first1=Chien-Hsiang}}</ref> <ref name="pmid12861101">{{cite journal |author=Wahner-Roedler DL, Witzig TE, Loehrer LL, Kyle RA |title=Gamma-heavy chain disease: review of 23 cases |journal=Medicine (Baltimore) |volume=82 |issue=4 |pages=236–50 |year=2003 |pmid=12861101 |doi=10.1097/01.md.0000085058.63483.7f |url=http://meta.wkhealth.com/pt/pt-core/template-journal/lwwgateway/media/landingpage.htm?issn=0025-7974&volume=82&issue=4&spage=236|last2=Witzig |last3=Loehrer |last4=Kyle }}</ref> (Franklin's disease)
* mu chain disease<ref name="pmid1566748">{{cite journal |author=Wahner-Roedler DL, Kyle RA |title=Mu-heavy chain disease: presentation as a benign monoclonal gammopathy |journal=Am. J. Hematol. |volume=40 |issue=1 |pages=56–60 |year=1992 |pmid=1566748 |doi=10.1002/ajh.2830400112|last2=Kyle }}</ref>
* delta chain disease<ref name="pmid6777103">{{cite journal |author=Vilpo JA, Irjala K, Viljanen MK, Klemi P, Kouvonen I, Rönnemaa T |title=Delta-Heavy chain disease: A study of a case |journal=Clin Immunol Immunopathol. |volume=17 |issue=4 |pages=584–94 |year=1980 |pmid=6777103 |doi=10.1016/0090-1229(80)90154-3|last2=Irjala |last3=Viljanen |last4=Klemi |last5=Kouvonen |last6=Rönnemaa }}</ref>
 
===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.<ref name="item13">{{cite journal |author=Wahner-Roedler DL, Kyle RA |title=Heavy chain diseases |journal=Best Pract Res Clin Haematol |volume=18 |issue=4 |pages=729–46 |year=2005 |pmid=16026747 |doi=10.1016/j.beha.2005.01.029|last2=Kyle }}</ref>
 
===IgG/γHCD===
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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.<ref name="item3">{{cite journal |author=Fermand JP, Brouet JC, Danon F, Seligmann M |title=Gamma heavy chain "disease": heterogeneity of the clinicopathologic features. Report of 16 cases and review of the literature |journal=Medicine (Baltimore) |volume=68 |issue=6 |pages=321–35 |year=1989 |pmid=2509855 |doi=10.1097/00005792-198911000-00001|last2=Brouet |last3=Danon |last4=Seligmann }}</ref>
 
* 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.<ref name="item13" />