1/17. Long-term survival following non-Hodgkin's lymphoma arising in wiskott-aldrich syndrome.wiskott-aldrich syndrome (WAS) is a rare X-linked recessive trait, characterized by thrombocytopenia, eczema, immunodeficiency and a high risk of malignancy, usually leukaemia or lymphoma. Until recently, most patients died before the age of 10 years. A patient with WAS who developed extranodal non-Hodgkin's lymphoma at the age of 16 years is reported. Despite thrombocytopenia at presentation, chemotherapy was well tolerated. There was disease progression after first line chemotherapy and radiotherapy, but the patient responded to second line chemotherapy with cisplatin, vincristine and etoposide. He remains disease free 9 years after completing treatment.- - - - - - - - - - ranking = 1keywords = lymphoma (Clic here for more details about this article) |
2/17. Epstein-Barr virus-associated hodgkin's disease in a patient with wiskott-aldrich syndrome.wiskott-aldrich syndrome is a primary immunodeficiency syndrome in which the majority of malignant complications are non-Hodgkin's lymphoma. We report here a wiskott-aldrich syndrome patient who developed Epstein-Barr virus-positive Hodgkin's disease in the bilateral pulmonary hilar lymph nodes. The treatment was successful as the patient achieved a complete response and event-free survival for more than 4 y. CONCLUSION: This case is very rare, but highly suggestive of the immune-mediated mechanisms in the pathogenesis of Epstein-Barr virus-associated Hodgkin's disease in an immunodeficiency patient.- - - - - - - - - - ranking = 0.16666666666667keywords = lymphoma (Clic here for more details about this article) |
3/17. Mediastinal seminoma in a patient with wiskott-aldrich syndrome.Shortness of breath developed in an 18-year-old man with wiskott-aldrich syndrome, and he was found to have a large mediastinal mass. The gallium scan was positive, and biopsy indicated a seminoma. After treatment with four cycles of chemotherapy, the mass completely resolved. Despite severe thrombocytopenia, he required only two platelet transfusions during therapy. Although lymphomas make up the vast majority of mediastinal tumors in patients with wiskott-aldrich syndrome, a positive gallium scan should not preclude the diagnosis of seminoma or the need for confirmatory tissue diagnosis. This report shows the possibility of uneventful and successful treatment of malignancy in a patient with wiskott-aldrich syndrome and severe thrombocytopenia.- - - - - - - - - - ranking = 0.16666666666667keywords = lymphoma (Clic here for more details about this article) |
4/17. Isolated EBV lymphoproliferative disease in a child with wiskott-aldrich syndrome manifesting as cutaneous lymphomatoid granulomatosis and responsive to anti-CD20 immunotherapy.patients with primary immunodeficiencies such as the wiskott-aldrich syndrome (WAS) are prone to develop Epstein-Barr virus (EBV) related lymphoproliferative disorders (LPDs). EBV LPD is most frequently seen in patients receiving immunosuppressive treatment after organ transplantation (post-transplant lymphoproliferative disorder), but can also arise in the primary immunodeficiencies. Typically, EBV LPD presents as a diffuse systemic disease with lymphadenopathy and organ involvement. A rare angiocentric and angiodestructive form of EBV associated B cell LPD, lymphomatoid granulomatosis (LyG), has also been reported in association with WAS. LyG most commonly involves the lung, but can also be seen in brain, kidney, liver, and skin. This report describes the case of a 16 year old boy with WAS who presented with an isolated non-healing ulcerating skin lesion. biopsy revealed an EBV related LPD with the histological features of LyG. This cutaneous lesion responded dramatically to treatment with specific anti-CD20 immunotherapy and the patient remains clinically free of LPD at 18 months.- - - - - - - - - - ranking = 0.83333333333333keywords = lymphoma (Clic here for more details about this article) |
5/17. Malignant B cell non-Hodgkin's lymphoma of the larynx in children with Wiskott Aldrich syndrome.A 15 years old male with a primary diagnosis of Wiskott Aldrich syndrome presented a laryngeal B cell lymphoma associated with Epstein-Barr virus. A chemotherapy and a radiotherapy were started in association with an endoscopic debulking of the tumor. The child died of respiratory failure secondary to a lung infection. The incidence of NHL (non-Hodgkin's lymphoma) of laryngeal origin in infants is extremely low and to our knowledge it has never been found in Wiskott Aldrich syndrome.- - - - - - - - - - ranking = 1keywords = lymphoma (Clic here for more details about this article) |
6/17. Coincident Kaposi sarcoma and T-cell lymphoma in a patient with the wiskott-aldrich syndrome.A 24 year old male with a history of eczema, recurrent mild infections, and thrombocytopenia consistent with the wiskott-aldrich syndrome (WAS) presented with a mediastinal mass, generalized lymphadenopathy, splenomegaly, and severe thrombocytopenia. Studies of immune function including immunoglobulin levels and T-cell subsets were normal. Furthermore, his T lymphocytes proliferated normally in response to phytohemagglutinin, concanavalin a, and the combination of neuraminidase/galactose oxidase. However, their proliferative responses to anti-CD43 antibody and periodate were diminished, consistent with the clinical diagnosis of WAS. An initial inguinal lymph node biopsy surprisingly revealed Kaposi sarcoma. However, following splenectomy to increase the platelet count, biopsy of the mediastinal mass revealed T-cell large cell lymphoma. Studies of biopsied tissue for the presence of Epstein-Barr virus and cytomegalovirus were negative, as were studies of blood, including the polymerase chain reaction, for the presence of the human immunodeficiency virus (hiv). This is the first report of Kaposi sarcoma arising in a patient with a congenital immunodeficiency syndrome. Although Kaposi sarcoma can arise in the face of the severe immunosuppression that follows allograft transplantation and in patients infected with hiv, we postulate that longevity in the face of mild immunosuppression was the major factor in the development of Kaposi sarcoma in this patient.- - - - - - - - - - ranking = 0.83333333333333keywords = lymphoma (Clic here for more details about this article) |
7/17. wiskott-aldrich syndrome complicated by an atypical lymphoproliferative disorder: a case report.wiskott-aldrich syndrome (WAS) is an X-linked syndrome consisting of eczema, recurrent pyogenic infection, and thrombocytopenia with decreased platelet volume. Immunologic studies reveal normal immunoglobulin g (IgG), decreased IgM, elevated IgA and IgE levels, and decreased T-cell function. patients with WAS often have increased susceptibility to lymphoproliferative disorders (LPDs). We report a 3-year-old boy who had persistent thrombocytopenia with bleeding, recurrent infections, and chronic eczema with frequent skin infections since birth. A blood smear revealed small platelets (50% of normal size). Immunologic studies showed normal IgG (1880 mg/dL), decreased IgM (76 mg/dL) and increased IgA (228 mg/dL) and IgE (14,282 IU/mL) levels. The relative proportions of immune cells were CD2 52.2%, CD3 41.1%, CD4 23.4%, CD8 16.8%, CD19 8.0%, CD57 7.7% and active T cells 14.6%. T-cell dysfunction was detected on the multitest for cell-mediated immunity. The WAS diagnosis was confirmed by mutation analysis which demonstrated a 4-base pair deletion in WAS protein gene exon 1. His thrombocytopenia was uncontrolled despite intravenous immunoglobulin infusions, so splenectomy was performed. The platelet count then rose to about 60,000 to 80,000/microL. However, about 2 weeks after splenectomy, he developed generalized lymphadenopathy and lymphoma was misdiagnosed based on lymph node biopsy at another hospital where he was admitted for urgent care. However, our analysis of his lymph node pathology led to the diagnosis of atypical LPD (ALPD). The lymphadenopathy regressed spontaneously 1 month later without chemotherapy. Early and correct diagnosis of WAS complicated with ALPD is important to avoid unnecessary chemotherapy.- - - - - - - - - - ranking = 0.16666666666667keywords = lymphoma (Clic here for more details about this article) |
8/17. burkitt lymphoma-induced ileocolic intussusception in wiskott-aldrich syndrome.A 12-year-old patient with wiskott-aldrich syndrome (WAS) was referred because of recurrent abdominal pain and bloody stools. Ileocolic invagination was diagnosed and resection of the terminal ileum was performed. Pathologic examination identified submucosal tumor as the leading point of intussusception. immunohistochemistry confirmed the diagnosis of burkitt lymphoma. The use of chemotherapy with anti-CD20 monoclonal antibody led to complete clinical remission of lymphoma. Non-Hodgkin's lymphoma (NHL) accounts for more than 60% of the tumors in children with primary immunodeficiency, and it is the most common type of malignancy observed in WAS. burkitt lymphoma represents 40% to 50% of all NHL cases in childhood, but in WAS it has rarely been reported. mutation analysis of the WASP gene in this patient revealed missense mutation (105 C > T) in exon 1. WAS protein (WASP) of normal size was present at a reduced amount in peripheral blood lymphocytes. Complete lack of expression of WASP carries a greater risk for severe infections, bleeding, or malignancy development in WAS. However, rare patients with residual expression of mutated WASP, like this patient, still may develop lymphomas.- - - - - - - - - - ranking = 1.5keywords = lymphoma (Clic here for more details about this article) |
9/17. Fatal B-cell lymphoproliferative syndrome in allogeneic marrow graft recipients. A clinical, immunobiological and pathological study.We have studied four cases of fatal B-cell lymphoproliferative syndrome (LPS) developing among 333 patients (incidence 1.2%) treated with allogeneic bone marrow transplantation (BMT). All four patients had received a T-cell depleted graft. Onset of the first clinical symptoms (palpable lymph node enlargement in three and IgA-lambda paraproteinemia in two patients) occurred between 41 and 188 days post-BMT (median 76 days). The course of the LPS was rapidly progressive in all cases, leading to death in 2-5 weeks. The peripheral blood showed progressive pancytopenia with disproportionally high numbers of activated NK cells, apparently compensating for the T-cell deficiency. Post-mortem histological studies disclosed polymorphic B-cell proliferations, most pronounced in the lymph nodes, spleen, liver, lungs and kidneys. Lymphohemopoietic cells were of donor origin in three patients. In the fourth patient, graft failure suggested a host origin for the proliferating cells. immunophenotyping and gene rearrangement analysis revealed polyclonal proliferation in one patient, monoclonal proliferation in another patient, and an oligoclonal pattern in the other two patients. The clinical behavior of the LPS was independent of clonality. Immunohistologically, the proliferating cells showed characteristics of relatively mature B-cells in three cases, and pre-B-cell features in one case. Epstein Barr virus (EBV) serology indicated seroconversion (primary infection) in one child, and chronic active EBV infection in both adults. EBV dna as well as EBV nuclear antigen (EBNA) were detected in infiltrated tissues of all four patients. The labeling pattern on in situ hybridization suggested a replicative EBV infection comparable to that in lymphoblastoid cell lines. We conclude that EBV-associated LPS developing as a result of post-transplant immunodeficiency is a distinct clinicopathologic entity, differing from non-Hodgkin's lymphoma (including Burkitt's lymphoma) and infectious mononucleosis of the immunocompetent host.- - - - - - - - - - ranking = 0.33333333333333keywords = lymphoma (Clic here for more details about this article) |
10/17. Positive gallium-67 citrate scintigraphy in wiskott-aldrich syndrome with malignant lymphoma.This case describes the first detection of solid lymphoma lesions by gallium-67 scintigraphy in a 12-year-old patient with wiskott-aldrich syndrome. gallium 67 uptake was found in multiple sites in the patient who had developed an undifferentiated small, non-cleaved cell lymphoma. Response to chemotherapeutic treatment is correlated with findings on the gallium scintigraphy follow-up.- - - - - - - - - - ranking = 1keywords = lymphoma (Clic here for more details about this article) |
| | Next -> |