title: Severe enteropathy caused by α-heavy chain disease lacking detectable M-proteins.
abstract: A 57-year-old Japanese man was admitted to our hospital with diarrhea, weight loss and malabsorption. Due to a high serum IgA level, we suspected α-heavy chain disease (α-HCD). However, no monoclonal IgA was detected on protein electrophoresis or immunofixation. Immunohistochemical staining of intestinal biopsy specimens showed proliferation of CD138(+)IgA(+) cells, compatible with a diagnosis of α-HCD. Most α-HCD patients exhibit M-proteins in the serum on electrophoresis or immunoelectrophoresis; however, some patients lack detectable M-proteins, similar to our patient. Therefore, when α-HCD is suspected based on the clinical features, immunohistochemistry is required to make a diagnosis.
authors.affiliation: Department of Hematology/Oncology, Wakayama Medical University, Japan.
authors.forename: Miwa
authors.initials: M
authors.lastname: Kurimoto
authors.forename: Takashi
authors.initials: T
authors.lastname: Sonoki
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Nakamura
authors.forename: Yasuhide
authors.initials: Y
authors.lastname: Yamamoto
authors.forename: Yoshimasa
authors.initials: Y
authors.lastname: Maeda
authors.forename: Kodai
authors.initials: K
authors.lastname: Kuriyama
authors.forename: Toshiki
authors.initials: T
authors.lastname: Mushino
authors.forename: Hiroki
authors.initials: H
authors.lastname: Hosoi
authors.forename: Shogo
authors.initials: S
authors.lastname: Murata
authors.forename: Masaya
authors.initials: M
authors.lastname: Shimanuki
authors.forename: Kazuo
authors.initials: K
authors.lastname: Hatanaka
authors.forename: Nobuyoshi
authors.initials: N
authors.lastname: Hanaoka
authors.forename: Jun
authors.initials: J
authors.lastname: Kato
authors.forename: Hideki
authors.initials: H
authors.lastname: Nakakuma
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage*; D003520:Cyclophosphamide / Q000008:administration & dosage; D003967:Diarrhea / Q000209:etiology*; D004317:Doxorubicin / Q000008:administration & dosage; D004586:Electrophoresis; D006801:Humans; D007070:Immunoglobulin A / Q000097:blood*; D007150:Immunohistochemistry; D007161:Immunoproliferative Small Intestinal Disease / Q000175:diagnosis* / Q000188:drug therapy; D008286:Malabsorption Syndromes / Q000209:etiology*; D008297:Male; D008875:Middle Aged; D009194:Myeloma Proteins / Q000378:metabolism*; D049268:Positron-Emission Tomography; D011241:Prednisone / Q000008:administration & dosage; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage; D015431:Weight Loss
chemical_list: D007070:Immunoglobulin A; D009194:Myeloma Proteins; C085016:multiple myeloma M-proteins; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D011241:Prednisone