The Histopathology of γ Heavy-Chain Disease

Wester, Susan M.; Banks, Peter M.; Li, Chin‐Yang

doi:10.1093/ajcp/78.4.427

Cited by 31 publications

(14 citation statements)

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“…In contrast to ␣-HCD, ␥-HCD has no specific histologic pattern [13]. The most frequent pattern is a pleomorphic malignant lymphoplasmacytic proliferation observed predominantly in the bone marrow and lymph nodes.…”

Section: ␥-Heavy Chain Diseasementioning

confidence: 91%

Heavy chain disease

Witzig

Wahner‐Roedler

2002

Curr. Treat. Options in Oncol.

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The heavy chain diseases (HCDs) are rare B-cell malignancies that are distinguished by the production of a monoclonal immunoglobulin heavy chain (HC) without an associated light chain by the malignant B-cells. There are three types of HCD defined by the class of immunoglobulin (Ig) HC produced: IgA (alpha-HCD), IgG (gamma-HCD), and IgM (mu-HCD). Alpha-HCD is the most common and occurs most commonly as intestinal malabsorption in a young adult from a country bordering the Mediterranean Sea. Treatment consists of antibiotics and improved nutrition and hygiene. Surgery is occasionally required for patients with bulky masses at risk for bowel perforation. If there is no response to antibiotics or if aggressive non-Hodgkin's lymphoma (NHL) is diagnosed, the patient should be treated with chemotherapy. Gamma- and mu-HCD are rare and essentially are found in patients with a B-cell NHL that produces an abnormal Ig heavy chain. These patients occasionally may be diagnosed with a monoclonal gammopathy of undetermined significance (MGUS). Patients with MGUS with NHL should be administered chemotherapy. Screening the serum and urine of patients with lymphoplasmacytoid NHL would likely identify more patients with gamma- or mu-HCD.

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Section: ␥-Heavy Chain Diseasementioning

confidence: 91%

Heavy chain disease

Witzig

Wahner‐Roedler

2002

Curr. Treat. Options in Oncol.

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“…The most frequent histopathological finding is a pleomorphic malignant lymphoplasmacytic proliferation in bone-marrow and lymph nodes. Aspirate and biopsy specimens may show an increase in plasma cells, lymphocytes, and/or plasmacytoid lymphocytes (similar to the bone-marrow findings in Waldenström macroglobulinemia) ( 3 , 6 ). Nevertheless, in some cases γ-HCD are concurrent with other lymphoid neoplasms, including Hodgkin and non-Hodgkin lymphomas, which usually precede the serologic diagnosis of γ-HCD ( 2 , 7 , 8 ).…”

Section: Introductionmentioning

confidence: 66%

“…The most frequent histopathological finding is a pleomorphic malignant lymphoplasmacytic proliferation in bone marrow and lymph nodes ( 3 , 6 ). These features of HCD infiltration were not found in our patient.…”

Section: Discussionmentioning

confidence: 99%

“…One third of these cases are associated with autoimmune diseases such as rheumatoid arthritis, autoimmune haemolytic anaemia, or idiopathic thrombocytopenic purpura ( 5 , 6 ). Some authors divide γ-HCD into three broad categories based on the underlying pathologic process and its distribution: 1) those with disseminated proliferative disease (55 - 60%); 2) those with localized proliferative disease (25%); and 3) those with no apparent proliferative disease (10 - 15%) ( 3 , 6 ).…”

Section: Introductionmentioning

confidence: 99%

“…Some authors divide γ-HCD into three broad categories based on the underlying pathologic process and its distribution: 1) those with disseminated proliferative disease (55 - 60%); 2) those with localized proliferative disease (25%); and 3) those with no apparent proliferative disease (10 - 15%) ( 3 , 6 ). …”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Gamma heavy-chain disease accompanied with follicular lymphoma: a case report

San-José

Aguadero²,

Perea

et al. 2018

Biochemia Medica

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Heavy chain diseases (HCD) are B-cell lymphoprolipherative disorders characterized by the production of monoclonal heavy chains without associated light chains. Some cases of gamma-HCD (γ-HCD) are concurrent with other lymphoid neoplasm. The monoclonal component is not always detectable by serum electrophoresis, and often an immunofixation procedure is necessary to detect this component. Prognosis is variable, and no established guidelines for follow-up are available. We describe a case of a challenging diagnosis of γ-HCD due to the absence of clinical signs frequently reported in the disease (anaemia and palatal oedema among others). Haematological malignancy was the first suspicion but bone marrow examination was negative. In addition, the presence of an autoimmune bicytopenia and a Klinefelter syndrome complicated the clinical context of the patient. A thoracoabdominal computed tomography reported many small adenopathies whose pathological and immunohystochemical study revealed a follicular lymphoma. Shortly after, serum inmunofixation secondary to an abnormal electrophoretic pattern revealed a gamma paraprotein without light chains. Eventually, γ-HCD in association with follicular lymphoma was the final diagnosis. This is the first case reporting this association.

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