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: Miwa Kurimoto, Takashi Sonoki, Yasushi Nakamura, Yasuhide Yamamoto, Yoshimasa Maeda, Kodai Kuriyama, Toshiki Mushino, Hiroki Hosoi, Shogo Murata, Masaya Shimanuki, Kazuo Hatanaka, Nobuyoshi Hanaoka, Jun Kato, Hideki Nakakuma
• Journal: Internal medicine (Tokyo, Japan) (Intern Med) Vol. 53 Issue 6 Pg. 581-5 ( 2014) ISSN: 1349-7235 [Electronic] Japan
• PMID: 24633028 (Publication Type: Case Reports, Journal Article)

Chemical References

• Immunoglobulin A
• Myeloma Proteins
• multiple myeloma M-proteins
• Vincristine
• Doxorubicin
• Cyclophosphamide
• Prednisone

Topics

• Antineoplastic Combined Chemotherapy Protocols (administration & dosage)
• Cyclophosphamide (administration & dosage)
• Diarrhea (etiology)
• Doxorubicin (administration & dosage)
• Electrophoresis
• Humans
• Immunoglobulin A (blood)
• Immunohistochemistry
• Immunoproliferative Small Intestinal Disease (diagnosis, drug therapy)
• Malabsorption Syndromes (etiology)
• Male
• Middle Aged
• Myeloma Proteins (metabolism)
• Positron-Emission Tomography
• Prednisone (administration & dosage)
• Treatment Outcome
• Vincristine (administration & dosage)
• Weight Loss