A New Case of Gamma-Heavy Chain Disease

Gallart, M T; Canals, Josep Cornellà i; Cañadell, E; Cortez, Leonardo M.; Moragas, A

doi:10.1159/000207781

Cited by 10 publications

(3 citation statements)

References 14 publications

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“…The disease shows a wide clinical course varying from completely asymptomatic to rapidly progressive forms (4, 6). As shown in the present case, those cases having a histological picture of immunoblastic sarcoma have a rapidly progressive course (21). Although extensive bone destruction simulated advanced MM, the overall features of the patient corresponded to those of GHCD.…”

Section: Discussionsupporting

confidence: 63%

Osteolytic gamma heavy chain disease

Kanoh

Nakasato²

1987

European J of Haematology

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We report a case of gamma heavy chain disease (GHCD) with several unique features: extensive osteolytic lesions simulating advanced multiple myeloma (MM), skin involvement, under the age of 40 yr, and the appearance of a series of pathological cells with indented or lobulated nuclei. The most remarkable feature is a previously unreported extensive bone destruction. Except for the presence of this bone disease, the clinical and pathological features of the patient resembled those of GHCD rather than those of MM. We have reviewed 3 previously reported cases of GHCD associated with skeletal involvement and compared these with our case.

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Section: Discussionsupporting

confidence: 63%

Osteolytic gamma heavy chain disease

Kanoh

Nakasato²

1987

European J of Haematology

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“…The 24-hour urine samples, collected with natrium azide, were concentrated up to 100 times by means of dialysis with Visking membrane against polyethylene glycol (Carbowax). The abovereferred studies were carried out with three blood and urine samples taken on different days according to the aforesaid techniques [20]. The immunoelectrophoresis analyses were done on 1 % agarose gel (Kallestad) in barbital buffer (ionic strength 0.5, pH 8.4).…”

Section: Methodsmentioning

confidence: 99%

Generalized Castleman’s Disease with Urinary Elimination of Heavy Chain Fragments

Urbano-Márquez¹,

Estruch²,

Grau³

et al. 1984

Acta Haematol

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A generalized form of Castleman’s disease of transitional type is reported in a 66-year-old male who was found in addition to have gamma heavy chain fragments in his urine. One portion of them was constituted by Fc fragments and the other ones by fragments which reacted with anti-Fab/IgG and anti-Fc/IgG antisera but not with anti-κ and anti-λ antisera. The findings of the present case support an immunological basis for Castleman’s disease.

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“…Chromosomal abnormalities are uncommon in γHCD with no frequent abnormality identified [1] [4] [9]. There are isolated reports of trisomy 7 [31], trisomy 3q [1], trisomy 21 [32], complex karyotype [4] and aneuploidy in two cases [33] [34].…”

Section: Chromosomal Findingsmentioning

confidence: 99%

Rare Case of a Patient Presenting with Parathyroid Hormone-Related Peptide Mediated Hypercalcemia and IgG Heavy Chain Disease: Case Report and Review of Literature

Hansra¹,

Liege²,

Sujoy³

et al. 2015

OJBD

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IgG Heavy Chain Disease (γHCD) is a rare plasma cell disorder. Hypercalcemia related to plasma cell dyscrasias is related to non-PTHrP related mechanisms. Here we describe the first case of a patient with γHCD and PTHrP related hypercalcemia. Methods: Patient case derived from chart review from 2011 to 2015. Literature review performed searching PubMed 1968-current. Results: The patient was diagnosed with hypercalcemia with elevated PTHrP and exclusion of other etiologies of hypercalcemia. She was diagnosed with (γHCD) by M-spike 0.64 g/dL, IFE showing a broad band of IgG heavy chain, without associated light chains and severe depression of the non-monoclonal IgG. Serum immunoglobulins demonstrated elevated IgG (2110 mg/dL), normal IgA (46 mg/dL) and decreased IgM (<21 mg/dL). Bone marrow biopsy showed 5% PCs, non-clonal by kappa/lambda, but exclusive for IgG by IHC, without any staining for IgA or IgM. The patient was started on therapy with improved hypercalcemia and PTHrP levels. Conclusions: This is the first reported case of γHCD presenting with PTHrP related hypercalcemia. Given that skeletal involvement is uncommon in γHCD, hypercalcemia secondary to γHCD may at times be a PTHrP driven phenomenon and we recommend that this test be ordered in such cases.

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A New Case of Gamma-Heavy Chain Disease

Cited by 10 publications

References 14 publications

Osteolytic gamma heavy chain disease

Osteolytic gamma heavy chain disease

Generalized Castleman’s Disease with Urinary Elimination of Heavy Chain Fragments

Rare Case of a Patient Presenting with Parathyroid Hormone-Related Peptide Mediated Hypercalcemia and IgG Heavy Chain Disease: Case Report and Review of Literature

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