Hodgkin's disease associated with IgA and IgG subclass deficiency

Zénone, Thierry; Souquet, Pierre Jean; Cunningham‐Rundles, Charlotte; Bernard, J.-P.

doi:10.1046/j.1365-2796.1996.514837000.x

Cited by 20 publications

(9 citation statements)

References 24 publications

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“…Although CVID patients may be expected to have an increased risk of infectious events following pharmacological immunosuppression, the management of autoimmunity in these patients does not differ from non-CVID cases [14,81,82]. Lastly, malignancy is not uncommon in patients with CVID, secondary to an increased risk of gastric cancer and non-Hodgkin’s lymphoma [19,83,84,85], among others [19,36,45,84,85,86,87]. Of note, neoplasia may modify the disease classification since humoral immunodeficiencies with benign or malignant thymoma may manifest as CVID but should be classified as a separate entity [14,34,37,54,88,89,90,91].…”

Section: Cvid Clinical Featuresmentioning

confidence: 99%

Common Variable Immunodeficiency: Etiological and Treatment Issues

Deane

Selmi

Naguwa

et al. 2009

Int Arch Allergy Immunol

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One of the great advances in clinical medicine was the recognition of the pleomorphism of the immune response and the multiple afferent and efferent limbs of antigen processing and responsiveness. A significant contribution to this understanding was derived from studies of human immunodeficiency states, including both inherited and acquired syndromes. Amongst these syndromes, one of the most common, and least understood, is common variable immune deficiency (CVID). CVID is a syndrome that leads to a reduction in serum immunoglobulins and complications including recurrent infections. Management includes immunoglobulin replacement therapy; however, patients with CVID are at risk for complications of exogenous immunoglobulin administration as well as CVID-associated diseases such as autoimmune processes and malignancies. To assess the current state of knowledge in the field, we performed a literature review of a total of 753 publications covering the period of 1968 until 2008. From this list, 189 publications were selected for discussion. In this review, we demonstrate that while the molecular basis of CVID in many cases remains incompletely understood, significant strides have been made and it is now clear that there is involvement of several pathways of immune activation, with contributions from both T and B cells. Furthermore, despite the current gaps in our knowledge of the molecular pathogenesis of the syndrome, there have been dramatic advances in management that have led to improved survival and significantly reduced morbidity in affected patients.

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Section: Cvid Clinical Featuresmentioning

confidence: 99%

Common Variable Immunodeficiency: Etiological and Treatment Issues

Deane

Selmi

Naguwa

et al. 2009

Int Arch Allergy Immunol

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“…The selective IgA deficiency in our patient probably represents the most relevant predisposing factor for lymphoma evolvement. Patients with selective IgA deficiency—the most common primary immunodeficiency disease—are prone to infections or autoimmune disorders and bear a higher propensity to develop neoplasias . Lack of secretory IgA has been hypothesized to compromise the defense against infection with H. pylori which is pathogenetically related to gastric cancer and MALT lymphoma .…”

Section: Discussionmentioning

confidence: 99%

Primary cutaneous marginal zone lymphoma with sequential development of nodal marginal zone lymphoma in a patient with selective immunoglobulin A deficiency

Wobser

Kerstan²,

Kneitz³

et al. 2013

J Cutan Pathol

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Multiple lymphoma subtypes occurring within one patient is rare in the context of B-cell lymphoma, and only few such cases have been reported in association with primary cutaneous marginal zone lymphoma (PCMZL). We herein describe the case of a 43-year-old patient who was diagnosed with PCMZL and subsequently developed a clonally unrelated nodal marginal zone lymphoma (MZL). At the time of diagnosis of PCMZL, multiple skin lesions were present. The atypical lymphoid infiltrate showed monotypic expression of immunoglobulin light chain lambda and heavy chain (IgM) on immunohistochemistry and an identical B-cell clone. No sign of systemic lymphoma was present in staging examinations. Complete remission was achieved utilizing rituximab. After a 3-year clinical course of repetitive cutaneous relapses and remissions, the patient additionally developed nodal lymphoma involvement by MZL which, however, harbored an immunophenotype and a genetic clone distinct from the cutaneous lymphoma counterpart. Therefore, the rare occurrence of two different types of MZL with sequential evolution was diagnosed. In this uncommon case, we hypothesize that selective immunoglobulin A deficiency may play a promoting role for the metachronous development of the two MZL that occurred in our patient.

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“…Malignancy is not uncommon in patients with CVID [8][9][10][11][12][13][14][15]. Patients with CVID have a high risk of neoplastic diseaseboth hematological and solid tumor (breast, prostate, ovary, skin, and colon).…”

Section: Discussionmentioning

confidence: 99%

“…In particular, the incidence of lymphoma is increased in patients with CVID [10,11]. The most common malignancies were non-Hodgkin's lymphoma [5,10,[12][13][14][15] and gastric cancers [11][12][13]. Neoplasias developed in the fifth or sixth decade of life in a study by Cunningham-Rundles et al [13].…”

Section: Discussionmentioning

confidence: 99%