Posttransplantation lymphoproliferative disease with features of lymphomatoid granulomatosis in a lung transplant patient

Kwon, Eun Ji; Katz, Kenneth A.; Seykora, John T.; Rook, Alain H.; Wasik, Mariusz A.; Junkins‐Hopkins, Jacqueline M.

doi:10.1016/j.jaad.2005.11.1066

Cited by 25 publications

(6 citation statements)

References 58 publications

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“…Following diagnosis of LG, underlying immunocompromise can usually be demonstrated in affected patients. Cutaneous lesions of LG have been seen in association with Wiskott–Aldrich syndrome, myeloproliferative disorders and as a form of post‐transplant lymphoproliferative disorder (PTLD) following organ transplantation . Lymphomatoid granulomatosis shows a variable clinical course ranging from spontaneous remission to progression to EBV + diffuse large B‐cell lymphoma .…”

Section: Discussionmentioning

confidence: 99%

Lymphomatoid granulomatosis presenting with cutaneous involvement: a case report and review of the literature

Rysgaard

Stone

2014

J Cutan Pathol

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Lymphomatoid granulomatosis (LG) is a rare Epstein-Barr virus (EBV)-associated B-cell lymphoproliferative disorder presenting in middle adulthood that nearly always affects the lungs and shows cutaneous involvement in up to 50% of cases. Skin lesions are present at the time of diagnosis in roughly one-third of patients and may precede the development of lung lesions in as many as 10-15%. Recognition by both the dermatologist and dermatopathologist is therefore crucial for early and accurate diagnosis. While skin involvement is grossly and microscopically diverse, the disease most commonly presents as erythematous subcutaneous and dermal nodules showing the classic histopathologic triad of transmural lymphocytic angiitis, atypical B-lymphocytes in a polymorphous T-cell background, and necrotic foci within lymphoid aggregates. We present a case of lymphomatoid granulomatosis initially presenting with cutaneous lesions, with an accompanying review of the literature.

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Section: Discussionmentioning

confidence: 99%

Lymphomatoid granulomatosis presenting with cutaneous involvement: a case report and review of the literature

Rysgaard

Stone

2014

J Cutan Pathol

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“…Lymph nodes, spleen, liver and bone marrow are typically spared 176 . LyG can occur in association with constitutional immune-deficiency syndromes such as Wiskott-Aldrich, myeloproli-ferative neoplasms and in post-transplant settings 177–180 . In the setting of immune deficiency the differential diagnosis with other EBV+ B-LPD is challenging 169 .…”

Section: Lymphomatoid Granulomatosis (Lyg)mentioning

confidence: 99%

Cutaneous EBV-related lymphoproliferative disorders

Gru

Jaffe

2017

Seminars in Diagnostic Pathology

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This article will focus on the cutaneous lymphoproliferative disorders associated with EBV, with an emphasis on the upcoming changes in the revised 4th Edition of the WHO classification of tumors of the hematopoietic system, many of which deal with cutaneous disorders derived from NK-cells or T-cells. Extranodal NK/T-cell lymphoma usually presents in the upper aerodigestive tract, but can involve the skin secondarily. EBV-associated T- and NK-cell lymphoproliferative disorders (LPD) in the pediatric age group include the systemic diseases, chronic active EBV infection (CAEBV) and systemic EBV+ T-cell lymphoma of childhood. Hydroa vacciniforme (HV)-like LPD is a primarily cutaneous form of CAEBV and encompasses the lesions previously referred to as HV and HV-like lymphoma (HVLL). All the T/NK-cell-EBV-associated diseases occur with higher frequency in Asians, and indigenous populations from Central and South America and Mexico. Among the B-cell EBV-associated LPD two major changes have been introduced in the WHO. The previously designated EBV-positive diffuse large B-cell lymphoma (EBV-DLBCL) of the elderly, has been changed to EBV-DLBCL with 'not otherwise specified' as a modifier (NOS). A new addition to the WHO system is the more recently identified EBV+ mucocutaneous ulcer, which involves skin and mucosal-associated sites.

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“…Underlying this EBV reactivation, a functional immune dysregulation has been seen in almost all patients suffering from LYG[ 20 41 ] and, consistent with this observation, the occurrence of LYG has been associated with several autoimmune diseases, congenital immunodeficiencies, and conditions following either leukemia therapy or organ transplantation. [ 23 27 31 ] In addition, discontinuing immunosuppressants has been observed to induce a remission in patients with LYG, and this option may be considered for each patient, if reasonable. [ 35 ] In more severe cases with evidence of a progressive disease, patients have been treated with corticosteroids, rituximab, interferon-α-2b, and chemotherapeutic agents such as prednisone, etoposide, vincristine, cyclophosphamide, and doxorubcin.…”

Section: Discussionmentioning

confidence: 99%

Unique case of trigeminal neuralgia due to Epstein-Barr-virus-associated B-cell lymphomatoid granulomatosis of the Meckel's cave and cavernous sinus: Important clinical and therapeutic implications

Sponton¹,

Ayyad²,

Archavlis³

et al. 2018

Surg Neurol Int

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Background:Trigeminal neuralgia (TN) represents one of the most disabling pain syndromes. Several diseases have been described as etiological triggers of TN, vascular compression of the trigeminal nerve being the most frequent cause. Here, we describe for the first time a rare case of TN caused by an infiltration of an isolated Epstein–Barr virus (EBV) B-cell lymphomatoid granulomatosis (LYG) mass into the Meckel's cave and cavernous sinus.Case Description:A 51-year-old woman undergoing immunosuppressant treatment for Crohn's disease presented due to right-sided TN. Magnetic resonance imaging (MRI) scans revealed an isolated lesion affecting the right Meckel's cave and lateral wall of the cavernous sinus. We accomplished tumor resection through a subtemporal extradural approach and the patient recovered successfully from surgery. Histological examination revealed an LYG, and a blood test confirmed low but positive EBV counts. The immunosuppressant therapy was discontinued and we assumed a watchful waiting management. During a 41-months’ follow-up there was neither evidence of LYG recurrence nor an increase of EBV counts.Conclusions:LYG, an angiodestructive disease associated with EBV reactivation in the context of immune dysfunction and often associated with an aggressive behavior or even malignant transformation, should be considered as a rare differential diagnosis of TN associated with skull base lesions. The management of this rare disease is still controversial and varies from limiting the treatment to correcting immune dysfunction up to chemotherapy. In this case of an isolated mass, surgical excision and discontinuation of immunosuppressants were effective to prevent the relapse of the disease in a long-term follow-up.

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Posttransplantation lymphoproliferative disease with features of lymphomatoid granulomatosis in a lung transplant patient

Cited by 25 publications

References 58 publications

Lymphomatoid granulomatosis presenting with cutaneous involvement: a case report and review of the literature

Lymphomatoid granulomatosis presenting with cutaneous involvement: a case report and review of the literature

Cutaneous EBV-related lymphoproliferative disorders

Unique case of trigeminal neuralgia due to Epstein-Barr-virus-associated B-cell lymphomatoid granulomatosis of the Meckel's cave and cavernous sinus: Important clinical and therapeutic implications

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