PMID- 29326807
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20200930
IS  - 2035-3006 (Print)
IS  - 2035-3006 (Electronic)
IS  - 2035-3006 (Linking)
VI  - 10
IP  - 1
DP  - 2018
TI  - Heavy-Chain Diseases and Myeloma-Associated Fanconi Syndrome: an Update.
PG  - e2018011
LID - 10.4084/MJHID.2018.011 [doi]
LID - e2018011
AB  - The heavy chain diseases (HCDs) are rare B-cell malignancies characterized by the 
      production of a monoclonal immunoglobulin heavy chain without an associated light 
      chain. There are three types of HCD, defined by the class of immunoglobulin heavy 
      chain produced: IgA (alpha-HCD), IgG (gamma-HCD), and IgM (mu-HCD). Alpha-HCD is the most 
      common and usually occurs as intestinal malabsorption in a young adult from a 
      country of the Mediterranean area. Gamma- and mu-HCDs are rarer and associated 
      with a B-cell non-Hodgkin lymphoma that produces an abnormal Ig heavy chain. 
      These patients may occasionally be diagnosed with a monoclonal gammopathy of 
      undetermined significance (MGUS). Fanconi syndrome, on the other hand, can be 
      primary (inherited) or secondary (acquired). The only exception to this rule is 
      the idiopathic form. Adult acquired Fanconi syndrome can be a rare complication 
      of a monoclonal gammopathy. At diagnosis, most patients have an MGUS or 
      smoldering multiple myeloma, with renal failure and evidence of osteomalacia. 
      During follow-up, patients can develop an end-stage renal disease. Chemotherapy 
      provides little benefit on renal function.
FAU - Ria, Roberto
AU  - Ria R
AD  - Department of Biomedical Sciences and Human Oncology, Section of Internal 
      Medicine, University of Bari "Aldo Moro" Medical School, Bari, Italy.
FAU - Dammacco, Franco
AU  - Dammacco F
AD  - Department of Biomedical Sciences and Human Oncology, Section of Internal 
      Medicine, University of Bari "Aldo Moro" Medical School, Bari, Italy.
FAU - Vacca, Angelo
AU  - Vacca A
AD  - Department of Biomedical Sciences and Human Oncology, Section of Internal 
      Medicine, University of Bari "Aldo Moro" Medical School, Bari, Italy.
LA  - eng
PT  - Journal Article
PT  - Review
DEP - 20180101
PL  - Italy
TA  - Mediterr J Hematol Infect Dis
JT  - Mediterranean journal of hematology and infectious diseases
JID - 101530512
PMC - PMC5760076
OTO - NOTNLM
OT  - B-cell malignancies
OT  - Fanconi syndrome
OT  - Heavy chain disease
OT  - Monoclonal gammopathy
OT  - Multiple myeloma
COIS- Competing interests: The authors have declared that no competing interests exist.
EDAT- 2018/01/13 06:00
MHDA- 2018/01/13 06:01
PMCR- 2018/01/01
CRDT- 2018/01/13 06:00
PHST- 2017/10/05 00:00 [received]
PHST- 2017/12/23 00:00 [accepted]
PHST- 2018/01/13 06:00 [entrez]
PHST- 2018/01/13 06:00 [pubmed]
PHST- 2018/01/13 06:01 [medline]
PHST- 2018/01/01 00:00 [pmc-release]
AID - mjhid-10-1-e2018011 [pii]
AID - 10.4084/MJHID.2018.011 [doi]
PST - epublish
SO  - Mediterr J Hematol Infect Dis. 2018 Jan 1;10(1):e2018011. doi: 
      10.4084/MJHID.2018.011. eCollection 2018.