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Adoptive therapy for EBV-induced cancers: driving success with post-transplant lymphoproliferative disorder to other EBV-derived tumors.

Epstein-Barr virus (EBV) infection is associated with a range of human malignancies of lymphocytic and epithelial cell origin. In addition to viral-mediated and genetic oncogenic events that lead to the establishment of EBV-associated malignancies, defects in the immune control of EBV likely play a significant role in promoting the survival of malignant cells. This breakdown in immune surveillance is most evident in immunocompromised transplant patients who are susceptible to the development of post-transplant lymphoproliferative disorders. Observations over the last two decades have shown tha…

HLA-DRB1 typing by micro-bead array assay identifies the origin of early lymphoproliferative disorder in a heart transplant recipient.

We report the case of a 68-year-old woman who underwent heart transplantation for hypertrophic cardiomyopathy. Two months after the transplant she developed mild fever and dyspnea with a marked drop in left ventricle ejection fraction of 31%. Coronary angiography was negative for cardiac allograft vasculopathy. Endomyocardial biopsy revealed ischemic damage with no evidence of acute cellular rejection, antibody-mediated rejection or viral myocarditis. A neoplastic process was suspected even though full-body computerized tomography was negative for malignancy. The patient died 4 months after tr

An adult case of systemic Epstein-Barr virus-positive T/natural killer-cell lymphoproliferative disorder with good outcome.

Epstein-Barr virus-positive T/natural killer (NK)-cell lymphoproliferative disorder (EBV+T/NK LPD) encompasses a heterogeneous group of disorders that have a common feature with excessive lymphoid proliferation of mainly T cells and/or NK cells. This disease is rare, predominantly affects children and young adults, and associated with high mortality. Herein, we report a case of EBV+T/NK LPD that occurred in an old woman with good outcome. The patient presented with fever, splenomegaly, and pancytopenia. Computed tomography (CT) scan of the abdomen showed splenomegaly. The clinical impression w

Dominance of nonmalignant T-cell clones and distortion of the TCR repertoire in the peripheral blood of patients with cutaneous CD30+ lymphoproliferative disorders.

The CD30-positive cutaneous lymphoproliferative disorders (CLPD) include lymphomatoid papulosis (LyP) and primary cutaneous anaplastic large T-cell lymphoma (cALCL). Despite the malign-appearing histology, an excellent prognosis and spontaneous regression of single lesions characterize LyP. Even after years of clinical remission newly erupting lesions often harbor a T-cell clone identical to the initial one. This fact raises the question whether the clonal T-cell population persists in the peripheral blood. Therefore we investigated genomic DNA of 126 samples of lesional skin and peripheral bl…

EBV(+) B-cell lymphoproliferative disorder associated with subsequent development of Burkitt lymphoma in a patient with idiopathic CD4(+) T-lymphocytopenia.

We report here a case of idiopathic CD4(+) T-lymphocytopenia (ICL) associated with Epstein-Barr virus (EBV)(+) lymphoproliferative disorder (LPD) terminating in Burkitt lymphoma (BL). A 33-year-old Japanese male was admitted to the hospital showing severe CD4(+) lymphocytopenia and neutropenia that was diagnosed as ICL in 1993. Twenty months after the onset of disease, right cervical lymphadenopathy was detected. Biopsy of the specimen showed reactive lymph node hyperplasia and interfollicular B-cell hyperplasia. Ninety-one months later, polypoid tumors were resected from the bilateral nasal c

Epstein-Barr virus nuclear antigen-2 detection and typing in immunocompromised children correlated with lymphoproliferative disorder biopsy findings.

Epstein-Barr virus (EBV), the causative agent of infectious mononucleosis, plays a significant role as a cofactor in the process of tumorigenesis, and has consistently been associated with a variety of malignancies especially in immunocompromised patients. Forty-four children and adolescents (21 liver transplant patients, 7 heart transplant, 5 AIDS, 3 autoimmune hepatitis, 2 nephritic syndromes, 2 medullar aplasia, 2 primary immunodeficiency disorder patients, 1 thrombocytopenic purpura and 1 systemic lupus erythematosus) presenting with chronic active EBV infection (VCA-IgM persistently posit

T-cell lymphoproliferative disorder of hand-mirror cell morphology presenting in an eosinophilic loculated peritoneal effusion, with omental "caking".

BACKGROUND: Cells with "hand mirror" morphology have not, to the best of our knowledge, been described in a primary effusion sample. This paper describes a case of T-cell lymphoma with eosinophilia in a patient with suspected peritoneal carcinomatosis. Rarely, a T-cell lymphoproliferative process may mimic primary peritoneal carcinomatosis, clinically suggested by a presentation in CT imaging of omental caking with bilateral massive loculated effusions in a patient without lymphadenopathy or splenomegaly. METHODS: A 60 year old caucasian male presented with vague abdominal discomfort and incr

Pure red cell aplasia in B-cell lymphoproliferative disorder treated with rituximab: report of two cases and review of the literature.

Pure red cell aplasia (PRCA) is an unusual cause of anemia in patients with chronic lymphoproliferative disorders. Here, we present two cases of PRCA, one associated with chronic lymphocytic leukemia (CLL) and the other with splenic marginal zone lymphoma, in which the PRCA responded dramatically to treatment with rituximab. We then review the literature on PRCA in lymphoma and response to rituximab. PRCA associated with CLL or lymphoma may be another indication for rituximab therapy.

Hodgkin's disease as unusual presentation of post-transplant lymphoproliferative disorder after autologous hematopoietic cell transplantation for malignant glioma.

BackgroundPost-transplant lymphoproliferative disorder (PTLD) is a complication of solid organ and allogeneic hematopoietic stem cell transplantation (HSCT); following autologous HSCT only rare cases of PTLD have been reported. Here, a case of Hodgkin's disease (HD), as unusual presentation of PTLD after autologous HSCT for malignant glioma is described.Case presentation60-years old man affected by cerebral anaplastic astrocytoma underwent subtotal neurosurgical excision and subsequent high-dose chemotherapy followed by autologous HSCT. During the post HSCT course, cranial irradiation and cort

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… Background Post-transplant lymphoproliferative disorders (PTLDs) are severe complications that occur in solid organ and allogeneic hematopoietic cell transplantation (HSCT), and their incidence ranges from 1% to 20% [ 1 ]. … Successful traetment of post-transplantation lymphoproliferative disorder in autologous blood stem cell transplant recipients. Bone Marrow Transplant. 2002;30:321–6. doi: 10.1038/sj.bmt.1703603. …

Gain of chromosome 3/3q in B-cell chronic lymphoproliferative disorder is associated with plasmacytoid differentiation with or without IgM overproduction.

Trisomy 3 has been reported to be associated with marginal zone B-cell lymphoma. However, its occurrence and significance in other B-cell chronic lymphoproliferative disorders has not been fully defined. We report five cases of B-cell chronic lymphoproliferative disorders showing gain of chromosome 3 or 3q. The patients were elderly males who presented with splenomegaly with or without hepatomegaly and lymphadenopathy. The diagnoses included chronic lymphocytic leukemia (3 cases), prolymphocytic leukemia (1 case), and Waldenstrom macroglobulinemia (1 case). Distinctive feature in this group of…

Humanized anti-CD20 monoclonal antibody (Rituximab) in post transplant B-lymphoproliferative disorder: a retrospective analysis on 32 patients.

BACKGROUND: B-lymphoproliferative post-transplant disorder (BLPD) is a severe complication of organ and bone marrow transplantation. The reduction of immuno-suppressive therapy or surgery for localized disease may cure some BLPDs. Other therapeutic approaches such as chemotherapy and antiviral drugs are toxic and of limited efficacy. Adoptive immunotherapy with donor T-cell infusions has yielded promising results but is, at the present time, easily applicable only in bone marrow-transplanted patients. Anti-B-cell Murine monoclonal antibodies (MoAbs) have proven effective but are no longer avai

Age-dependent intestinal lymphoproliferative disorder due to stem cell factor receptor deficiency: parameters in small and large intestine.

Signaling through c-Kit/stem cell factor (SCF) is crucial for normal development of erythroid and myeloid hematopoietic precursors and of melanocytes and germ cells. While peripheral lymphoid populations of W/Wv and SI/SId mice appear normal, we demonstrated that the intraepithelial lymphocyte (IEL) populations of small (SI) and large (LI) intestine were significantly affected. IEL populations of young W/Wv animals were indistinguishable from those of their control littermates, but an age-dependent decrease in SI and LI TCRgamma delta IEL occurred in c-Kit mutant mice. In SI, but not in LI, th

Ibrutinib as the Unifying Culprit: A Case Report of Multisystem Complications in an Elderly Patient With Low-Grade B-Cell Lymphoproliferative Disorder.

Ibrutinib, an irreversible inhibitor of Bruton's tyrosine kinase (BTK), is a cornerstone of therapy for low-grade B-cell lymphoproliferative disorders (B-cell LPD), including Waldenstrom's macroglobulinaemia (WM). While highly effective, its adverse effect profile is broad and potentially life-threatening, particularly in elderly patients with multiple comorbidities. We report an 82-year-old woman with a seven-year history of CD5-negative low-grade B-cell LPD and IgM paraproteinaemia, managed sequentially with rituximab and ibrutinib. During ibrutinib therapy, she developed a cascade of compli…

Donor-Recipient Epstein-Barr Virus Serostatus and Time-Varying Risk of Posttransplant Lymphoproliferative Disorder in Kidney Transplant Recipients.

BACKGROUND: Prior studies and guidelines emphasize PTLD-risk for Epstein-Barr virus (EBV) mismatched (D+/R-) recipients. However, risk among D-/R- kidney recipients remains poorly defined. We evaluated PTLD-risk by donor-recipient EBV serostatus, including D-/R-, and time-varying impact of PTLD on mortality in kidney transplant recipients. METHODS: This retrospective cohort included deceased donor kidney transplants from the US Scientific Registry of Transplant Recipients (2003-2023). Recipient and donor-recipient EBV serostatus were categorical exposures. Time-to-PTLD was analyzed using Kapl

Spontaneous Regression of a Retinal Mass With Epstein-Barr Virus-Positive and Cytomegalovirus-Positive Panuveitis in Methotrexate-Associated Lymphoproliferative Disorder.

Methotrexate (MTX) has been widely used for patients with autoimmune diseases as a corticosteroid-sparing agent. We here report an 86-year-old Japanese woman treated with MTX for rheumatoid arthritis who presented with panuveitis along with a yellowish retinal mass. Both Epstein-Barr virus (EBV) and cytomegalovirus (CMV) were detected in aqueous humor and vitreous fluid by quantitative polymerase chain reaction. The retinal mass gradually regressed over eight months after MTX withdrawal. The present case indicates that intraocular MTX-lymphoproliferative disorder (MTX-LPD) should be considered

CD30-Positive Lymphoproliferative Disorder With DUSP22-IRF4 Rearrangement and Gamma-Delta T-Cell Phenotype: A Novel Indolent Presentation.

We present a case of an 84-year-old female with an isolated and asymptomatic 1.0 cm red papule on the left medial knee. A shave biopsy revealed a diffuse atypical lymphoid infiltrate in the dermis with epidermotropism, diffuse expression of CD30 and positive T-cell receptor (TCR) gamma-delta immunophenotype. Fluorescence in situ hybridization (FISH) identified a chromosomal rearrangement involving dual-specificity phosphatase-22 (DUSP22) and interferon regulatory factor-4 (IRF4). Next generation sequencing (NGS) indicated a low tumor mutational burden with no gene variants. Upon follow-up, the

Intraoral CD30+ T-Cell Lymphoproliferative Disorder with Lymphomatoid Papulosis Type C Features Mimics Lymphoma Histopathologically and Immunohistochemically.

BACKGROUND: Previous studies have shown that at least a of intraoral eosinophilic ulcer is best classified as a CD30 + T-cell lymphoproliferative disorder (LPD), with histopathology reminiscent of lymphomatoid papulosis (LyP) of the skin. Microscopically, a mixed population of inflammatory cells, often including eosinophils and varying numbers of atypical lymphoid cells, frequently expressing CD30, is typical for LyP, whose clinicopathological spectrum includes type A, B, C, D, E, and LyP with DUSP22/IRF4 rearrangement. To date, about 27 intraoral LyP cases have been reported. Of them, 7 cases

Post-Transplant Lymphoproliferative Disorder Presenting as a Gastrointestinal Fistulous Tract in a Heart Transplant Recipient: Case Report and Literature Review.

We present a challenging case of Epstein-Barr virus-related isolated small bowel post-transplant lymphoproliferative disorder (PTLD) in a pediatric heart transplant recipient presenting as recurrent gastrointestinal (GI) bleeding and subsequently a GI fistulous tract with associated intra-abdominal abscess. Diagnosis was not confirmed until exploratory laparoscopy was performed, with excision of the fistulous tract revealing evidence of PTLD on pathology. Early diagnosis of GI-PTLD remains a challenge, especially if isolated in the small intestine. Diagnosis may rely on positron emission tomog

Genomic landscape of T-large granular lymphocyte leukemia and chronic lymphoproliferative disorder of NK cells: a single institution experience.

LGLL is a rare and chronic lymphoproliferative disorder including T-LGLL and CLPD-NK. Here, we investigated the genomic profiles of LGLL with a focus on STAT3 and STAT5B mutations in a cohort of 49 patients (41 T-LGLL, 8 CLPD-NK). Our study indicated that STAT3 was identified in 38.8% (19/49) of all patients, while STAT5B occurred in only 8.2% (4/49) of patients. We found that STAT3 mutations were associated with lower ANC in T-LGLL patients. The average number of pathogenic/likely pathogenic mutations in STAT3/STAT5B-mutated patients was significantly higher than that in WT patients (1.78 ± 1