Gamma Heavy-chain Disease

Bieliauskas, Shannon; Tubbs, Raymond R.; Bacon, Chris M.; Eshoa, Camellia; Foucar, Kathy; Gibson, Sarah E.; Kroft, Steven H.; Sohani, Aliyah R.; Swerdlow, Steven H.; Cook, James R.

doi:10.1097/pas.0b013e318240590a

Cited by 37 publications

(27 citation statements)

References 30 publications

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“…Heavy chain disease (HCD) is a rare B-cell lymphoproliferative disorder characterized by the production of monoclonal, abnormally truncated, immunoglobulin heavy-chain proteins (alpha, gamma, and/or mu) without associated light chains ( 1 , 2 ). Heavy chain disease can be thought of as a variant of non-Hodgkin’s lymphoma.…”

Section: Introductionmentioning

confidence: 99%

“…Clinical manifestations depend on the chain isotype involved and range from asymptomatic to aggressive lymphoma. Prognosis is variable, and no standardized effective treatment programs are available ( 2 , 3 ). …”

Section: Introductionmentioning

confidence: 99%

“…Classic presentation includes generalized lymphadenopathies and splenomegaly ( 2 ). The most distinctive symptom is palatal oedema resulting from enlargement of nodes in Waldeyer’s ring, sometimes leading to respiratory compromise ( 3 , 5 ).…”

Section: Introductionmentioning

confidence: 99%

“…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: 99%

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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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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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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 deposition diseaseconfined to the respiratory tract isextremely rare consisting of parenchymal masses and enlarged lymph nodes [208]. …”

Section: Other Diseases Due To Nonamyloid Iggsmentioning

confidence: 99%

The Lung in Dysregulated States of Humoral Immunity

Uzunhan¹,

Jeny²,

Kambouchner³

et al. 2017

Respiration

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In common variable immunodeficiency, lung manifestations are related to different mechanisms: recurrent pneumonias due to encapsulated bacteria responsible for diffuse bronchiectasis, diffuse infiltrative pneumonia with various patterns, and lymphomas, mostly B cell extranodal non-Hodgkin type. The diagnosis relies on significant serum Ig deficiency and the exclusion of any primary or secondary cause. Histopathology may be needed. Immunoglobulin (IgG) replacement is crucial to prevent infections and bronchiectasis. IgG4-related respiratory disease, often associated with extrapulmonary localizations, presents with solitary nodules or masses, diffuse interstitial lung diseases, bronchiolitis, lymphadenopathy, and pleural or pericardial involvement. Diagnosis relies on international criteria including serum IgG4 dosage and significantly increased IgG4/IgG plasma cells ratio in pathologically suggestive biopsy. Respiratory amyloidosis presents with tracheobronchial, nodular, and cystic or diffuse interstitial lung infiltration. Usually of AL (amyloid light chain) subtype, it may be localized or systemic, primary or secondary to a lymphoproliferative process. Very rare other diseases due to nonamyloid IgG deposits are described. Among the various lung manifestations of dysregulated states of humoral immunity, this article covers only those associated with the common variable immunodeficiency, IgG4-related disease, amyloidosis, and pulmonary light-chain deposition disease. Autoimmune connective-vascular tissue diseases or lymphoproliferative disorders are addressed in other chapters of this issue.

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