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

Wobser, Marion; Kerstan, Andreas; Kneitz, Hermann; Goebeler, Matthias; Kunzmann, Volker; Rosenwald, Andreas; Geissinger, Eva

doi:10.1111/cup.12230

Cited by 8 publications

(5 citation statements)

References 47 publications

(57 reference statements)

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“…Three studies reported three cases of selective IgA deficiency with B cell lymphoma; an adult patient with apparent selective IgA deficiency who presented with a primary cutaneous MZL, a 38-year-old male with unclassified NHL and a 2-year-old boy with Burkitt-like lymphoma (41, 54, 75). It is relevant to note that a combined Danish and Swedish study of 386 patients with IgA deficiency did not show an increased risk for cancer (standardized incidence ratio = 1.0) (54).…”

Section: B Cell Lymphomas In Piddsmentioning

confidence: 99%

A Systematic Review on Predisposition to Lymphoid (B and T cell) Neoplasias in Patients With Primary Immunodeficiencies and Immune Dysregulatory Disorders (Inborn Errors of Immunity)

et al. 2019

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Primary immunodeficiencies and immune dysregulatory disorders (PIDDs; now referred to as inborn errors in immunity) are rare disorders with a prevalence of 41. 4 or 50.5 per 100,000 persons ( 1 ). The incidence of malignancy in PIDD patents is the second-highest cause of death in children as well as adults, after infection, and is higher in certain PIDDs compared to others. We performed a systematic review of the literature to identify reports of B cell and T cell neoplasias in PIDDs and clustered them based on their classification in the IUIS schema. As would be expected, higher susceptibility to malignancies are typically reported in patients with Common Variable Immunodeficiency (CVID), combined immunodeficiencies affecting cellular immunity, in particular, DNA repair defects, or in the context of impaired immune regulatory control. There is not much evidence of increased risk for cancer in patients with innate immune defects, indicating that not all types of infection or genetic susceptibility predispose equally to cancer risk. Viral infections, in particular EBV, HHV and HPV, have been shown to increase susceptibility to developing cancer, but also patients with defects in immune regulation, such as Autoimmune Lymphoproliferative Syndrome (ALPS), activated p110delta syndrome (APDS type 1) and IL-10 receptor deficiency among others have a higher incidence of neoplastic disease, particularly lymphomas. In fact, lymphomas account for two-thirds of all malignancies reported in PIDD patients ( 2 ), with either a combined immunodeficiency or DNA repair defect predominating as the underlying immune defect in one registry, or antibody deficiencies in another ( 3 ). The vast majority of lymphomas reported in the context of PIDDs are B cell lymphomas, though T cell lymphomas have been reported in a few studies, and tend to largely be associated with chromosomal breakage disorders ( 4 ) or Cartilage Hair Hypoplasia ( 5 ). There appears to be a much higher prevalence of T cell lymphomas in patients with secondary immunodeficiencies ( 6 ), though this could reflect treatment bias. We reviewed the literature and summarized the reports of B and T cell lymphoma in PIDD patients to survey the current state of knowledge in this area.

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Section: B Cell Lymphomas In Piddsmentioning

confidence: 99%

A Systematic Review on Predisposition to Lymphoid (B and T cell) Neoplasias in Patients With Primary Immunodeficiencies and Immune Dysregulatory Disorders (Inborn Errors of Immunity)

et al. 2019

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“…Patients with X-linked immunodeficiency with hyper-IgM, caused by mutations in the CD40 ligand, have a high incidence of tumors of the pancreas and liver ( 121 ). Interestingly, a primary cutaneous marginal zone lymphoma (PCMZL) with the sequential development of nodal marginal zone lymphoma has been recently reported in a patient with selective immunoglobulin A deficiency ( 122 ). This is in addition to a previous report of a γ/δ type abdominal T-cell non-Hodgkin’s lymphoma in a patient with selective IgA deficiency ( 123 ).…”

Section: Iga Deficiencymentioning

confidence: 99%

Cancers Related to Immunodeficiencies: Update and Perspectives

et al. 2016

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The life span of patients with primary and secondary immunodeficiency is increasing due to recent improvements in therapeutic strategies. While the incidence of primary immunodeficiencies (PIDs) is 1:10,000 births, that of secondary immunodeficiencies are more common and are associated with posttransplantation immune dysfunction, with immunosuppressive medication for human immunodeficiency virus or with human T-cell lymphotropic virus infection. After infection, malignancy is the most prevalent cause of death in both children and adults with (PIDs). PIDs more often associated with cancer include common variable immunodeficiency (CVID), Wiskott–Aldrich syndrome, ataxia-telangiectasia, and severe combined immunodeficiency. This suggests that a protective immune response against both infectious non-self-(pathogens) and malignant self-challenges (cancer) exists. The increased incidence of cancer has been attributed to defective elimination of altered or “transformed” cells and/or defective immunity towards cancer cells. The concept of aberrant immune surveillance occurring in PIDs is supported by evidence in mice and from patients undergoing immunosuppression after transplantation. Here, we discuss the importance of PID defects in the development of malignancies and the current limitations associated with molecular pathogenesis of these diseases and emphasize the need for further knowledge of how specific mutations can modulate the immune system to alter immunosurveillance and thereby play a key role in the etiology of malignancies in PID patients.

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“…Histopathological examination was not performed in this case. Two further patients presented with a histologically proven non-related secondary nodal lymphoma (nodal marginal zone lymphoma, clonally divergent) with one of these cases being published previously (28). No more patients showed any distant organ involvement.…”

Section: Clinical Patient Characteristicsmentioning

confidence: 95%

Paraproteinaemia in Primary Cutaneous Marginal Zone Lymphoma

Frings

Röding²,

Schmeißer³

et al. 2018

Acta Derm Venerol

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Primary cutaneous marginal zone lymphomas (PCMZL) frequently exhibit lymphoplasmacytoid/plasmacytic differentiation, implying the capacity to produce monoclonal immunoglobulins. As these paraproteins are secreted, and thus are measurable in blood and urine, they may correlate with disease burden and serve as tumour markers reflecting therapeutic response. This study retrospectively analysed the records of 23 patients with PCMZL. During treatment and follow-up, laboratory tests, including full blood count, lactate dehydrogenase, serum protein electrophoresis and turbidimetric analyses, were conducted. Thirty-nine percent of cases showed a suspicious serum protein electrophoresis in terms of paraproteinaemia. In 44% of cases the heavy and light chain restriction in tissue samples correlated with serological findings. Altogether, 89% of the PCMZL patients with paraproteinaemia eventually experienced a relapse, in contrast to 62% of the group without paraproteinaemia. This study analysed the incidence and clinical implications of paraproteinaemia in patients with PCMZL. A clear correlation was found between paraproteinaemia, tumour relapse and therapeutic intervention.

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

Cited by 8 publications

References 47 publications

A Systematic Review on Predisposition to Lymphoid (B and T cell) Neoplasias in Patients With Primary Immunodeficiencies and Immune Dysregulatory Disorders (Inborn Errors of Immunity)

A Systematic Review on Predisposition to Lymphoid (B and T cell) Neoplasias in Patients With Primary Immunodeficiencies and Immune Dysregulatory Disorders (Inborn Errors of Immunity)

Cancers Related to Immunodeficiencies: Update and Perspectives

Paraproteinaemia in Primary Cutaneous Marginal Zone Lymphoma

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