RT Book, Section
A1 Wahner-Roedler, Dietlind L.
A1 Kyle, Robert A.
A2 Kaushansky, Kenneth
A2 Lichtman, Marshall A.
A2 Prchal, Josef T.
A2 Levi, Marcel M.
A2 Press, Oliver W.
A2 Burns, Linda J.
A2 Caligiuri, Michael
SR Print(0)
ID 1121101639
T1 Heavy-Chain Disease
T2 Williams Hematology, 9e
YR 2015
FD 2015
PB McGraw-Hill Education
PP New York, NY
SN 9780071833004
LK hemonc.mhmedical.com/content.aspx?aid=1121101639
RD 2024/04/20
AB SUMMARYThe  heavy-chain  diseases  (HCDs)  are  B-cell  lymphoplasmacytic  proliferative  disorders  in  which  neoplastic  cells  produce  monoclonal  immunoglobulins  (Ig)  consisting  of  truncated  heavy  chains  (HCs)  without  attached  light  chains.  The  complex  abnormalities  of  HCD  proteins  and  the  usual  lack  of  normal  light  chains  are  a  result  of  several  distinct  gene  alterations,  including  somatic  mutations,  deletions,  and  insertions.  HCDs  involving  the  three  main  immunoglobulin  classes  have  been  described:  α-HCD  is  the  most  common  and  has  the  most  uniform  presentation;  γ-  and  μ-HCDs  have  variable  clinical  presentations  and  histopathologic  features.  The  diagnosis  is  established  from  immunofixation  of  serum,  urine,  or  secretory  fluids  in  the  case  of  α-HCD  or  by  immunohistologic  analysis  of  the  proliferating  lymphoplasmacytic  cells  in  nonsecretory  disease.  Treatment  of  α-HCD  consists  of  antibiotics.  If  there  is  no  response  to  antibiotics  or  if  aggressive  lymphoma  is  diagnosed,  chemotherapy  is  indicated.  Treatment  of  γ-  and  μ-HCDs  depends  on  the  underlying  clinicopathologic  features  rather  than  on  the  presence  of  the  abnormal  protein.  Table  110–1  summarizes  the  features  of  the  HCDs.