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.
MeSH terms
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Antineoplastic Combined Chemotherapy Protocols / administration & dosage*
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Cyclophosphamide / administration & dosage
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Diarrhea / etiology*
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Doxorubicin / administration & dosage
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Electrophoresis
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Humans
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Immunoglobulin A / blood*
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Immunohistochemistry*
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Immunoproliferative Small Intestinal Disease / diagnosis*
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Immunoproliferative Small Intestinal Disease / drug therapy
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Malabsorption Syndromes / etiology*
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Male
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Middle Aged
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Myeloma Proteins / metabolism*
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Positron-Emission Tomography
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Prednisone / administration & dosage
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Treatment Outcome
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Vincristine / administration & dosage
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Weight Loss
Substances
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Immunoglobulin A
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Myeloma Proteins
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multiple myeloma M-proteins
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Vincristine
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Doxorubicin
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Cyclophosphamide
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Prednisone