Perforated rectal lymphoma in a renal transplant recipient: report of a case

PURPOSE: We report the case of a renal transplant recipient with rectal lymphoma manifested by sudden onset of abdominal pain from a perforated rectum who was treated successfully with prompt surgical resection and reduction of immunosuppressants. METHODS: An emergent anterior resection with Hartmann's procedure was done. Immunosuppressants were drastically reduced by discontinuation of cyclosporine. RESULTS: Pathologic examination showed diffusely infiltrated large-cell malignant lymphoma with an immunoblastic feature. The patient has been followed-up for four years, with no tumor recurrence or graft rejection. CONCLUSION: Rectal lymphoma, although rare, should be kept in the list of differential diagnoses for transplant recipients who exhibit lower gastrointestinal bleeding, intestinal obstruction, or abdominal pain.     

Myelodysplastic syndrome as a late complication following autologous bone marrow transplantation for non-Hodgkin's lymphoma

PURPOSE: To determine the incidence, natural history, and risk factors associated with myelodysplastic syndrome (MDS) occurring as a late complication following autologous bone marrow transplantation for patients with non-Hodgkin's lymphoma. METHODS: We retrospectively reviewed the charts of all 262 patients who underwent autologous bone marrow transplantation for non-Hodgkin's lymphoma at the Dana-Farber Cancer Institute from 1982 through 1991. Although patients received a variety of treatments before they were eligible for transplant, identical myeloablative therapy (cyclophosphamide 60 mg/kg/d for 2 days plus total-body irradiation twice daily for 3 days) was administered in each case. By collecting data on pretransplant and early posttransplant variables, we attempted to identify risk factors for the development of MDS. RESULTS: The crude overall incidence of posttransplant MDS or acute myeloid leukemia (AML) was 7.6%. The actuarial risk at 6 years was 18% +/- 9%. The median time of onset was 31 months (range, 10 to 101) after transplant or 69 months (range, 27 to 141) after initial treatment for lymphoma. Pretreatment variables predictive for the development of MDS (univariate analysis) included prolonged interval between initial treatment and the transplant procedure (P = .003), increased duration of exposure to chemotherapy (P = .019) or to alkylating agents (P = .045), and use of radiation therapy (P = .032) or pelvic radiation (P = .003) before transplant. CONCLUSION: MDS is a potential complication of autologous bone marrow transplantation for non-Hodgkin's lymphoma; bone marrow stem-cell damage sustained before the transplant may be the most important risk factor.     

Hemophagocytic syndrome: pitfalls in its diagnosis

The hemophagocytic syndrome (HS) is characterized by a clinical picture of fever, hepatosplenomegaly, lymphadenopathy and peripheral pancytopenia. The morphologic hallmark of this syndrome is the phagocytosis of hematopoietic elements by morphologically normal macrophages. HS is considered rare and may be a primary disease or associated to viral, infection, neoplasias or autoimmune diseases. Treatment is controversial and its evolution is often fatal. Anatomo-pathological evaluation shows the phenomenon of hemophagocytosis in several organs, especially the hematopoietic tissues. We describe a case of HS, discuss its possible causes, its clinical and pathologic features, its pathophysiology and therapeutic possibilities.     

Bone marrow transplantation for non-Hodgkin's lymphoma: a review

High dose chemotherapy and stem-cell rescue (bone marrow transplantation) is used increasingly in the treatment of malignant disorder. Numerous trials have demonstrated the effectiveness of bone marrow transplantation in the treatment of non-Hodgkin's lymphoma. However, there are many unanswered questions as to the role of high-dose therapy in certain subtypes of lymphoma, the timing of transplant, and even the type of transplant to perform. An attempt will be made to clarify many of these unanswered questions. The utilization of high-dose therapy for non-Hodgkin's lymphoma is recommended for most patients who have relapsed after initial therapy. Transplantation in first remission is not recommended routinely. Allogeneic bone marrow transplantation should by reserved for individuals with poorly responding disease or in individuals with bone marrow involvement. The precise roles of purging and transplantation of individuals with low grade lymphoma are being investigated.     

Longitudinal observation and outcome of nonfamilial childhood haemophagocytic syndrome receiving etoposide-containing regimens

The long-term outcome of 22 children treated with etoposide-containing regimens for haemophagocytic syndrome (HS) were longitudinally studied; none of them had a family history of the disease. All patients received etoposide-containing (150 mg/m2/d) regimens, combined, in 16 cases, with intravenous immunoglobulin (IVIG) and prednisolone. Complete remission (CR) was achieved in 12 patients, partial remission in seven, and early mortality occurred in three. Of the 12 CR patients, only four remain alive and disease-free, with a median follow-up of 47.4 months; one CR patient died due to infection and the remaining seven had relapsed diseases. Three patients with a partial response or with relapsed disease progressed to T-cell lymphoma, characterized, in the two cases tested, by clonal chromosomal abnormalities. Epstein-Barr virus (EBV) infection was implicated in disease pathogenesis in 15/22 patients. The overall survival was 45.5%, 40.9% and 40.9% at 1, 3 and 5 years, respectively, and disease-free survival for CR patients at these same times was 45.5%, 36.4% and 36.4%. The etoposide-containing regimen would appear to be an effective initial therapeutic option for childhood HS. However, in view of the frequency of partial remissions and relapsed disease, a more intensive chemotherapy or bone marrow transplantation should be applied. The progression to EBV-containing T-cell lymphoma in three patients is consistent with the previous observation that EBV-associated HS is a potentially malignant disease.     

Lymphoma with bladder involvement and renal transplantation

Based on the case of a renal transplant recipient who developed a monoclonal B cell lymphoma with plasma cell differentiation, arising in the bladder, the authors discuss the responsibility of intensive immunosuppressant protocols in the development of post-transplantation lymphoproliferative syndromes. The Epstein-Barr virus favours the development of these lymphomas. The bladder is a rare site. Diagnosis was based on endoscopic resection with immunohistochemical analysis. The prognosis depends on the stage at the time of diagnosis. At an early stage, resection of the tumour combined with reduction of immunosuppressant therapy may be sufficient, but more aggressive treatment is required for more advanced stages. Anti-B lymphocyte monoclonal antibodies (CD21, CD24) give good results, especially in polyclonal forms and Epstein-Barr virus primo-infections. Anti-CD38 monoclonal antibodies are currently being investigated and appear to be very promising. Conventional radiotherapy and chemotherapy are disappointing.     

Diagnosis and management of asthma: occupational health nurses'' awareness and use of national consensus practice guidelines

In 7 patients who received liver transplants, 12 plasma proteins were subjected to phenotype analysis in donor and recipient before and after transplantation. The plasma proteins analyzed were haptoglobin, transferrin, glycoprotein GC, alpha-1-antitrypsin, complement factor 3 (C3), orosomucoid 1, properdin factor B, complement factor 6, alpha-2-HS-glycoprotein (A2HS), plasminogen, factor B of coagulation factor XIII, and interalpha-trypsin-inhibitor (ITI). Classification was done with isoelectric focusing or agarose gel electrophoresis (C3). A change from recipient to donor type was observed for all systems with the exception of C3. This is the first time such data have been obtained for the A2HS and ITI systems. The time is indicated at which the recipient type disappeared and the donor type appeared. In addition to the expected phenotype changes from recipient to donor type, unexpected results were found in some systems. For instance, in 2 patients a mixed type was synthesized, or new phenotypes appeared. Possible explanations include blood transfusions, immunosuppressive therapy, extrahepatic sites of synthesis and disturbed transplant function. The usefulness of phenotype determination as a diagnostic criterion for transplant function is discussed.     

Cranial cystic epidermoid: report of two cases and review of the literature

Among non-Hodgkin's lymphomas occurring in childhood two major histologic subgroups can be identified: (1) Burkitt's lymphoma and (2) T-cell lymphoblastic lymphoma, an uncommon high-grade malignant non-Hodgkin's lymphoma. Although Burkitt's lymphoma with maxillofacial involvement is a well-documented disease, T-cell lymphoblastic lymphoma in the perioral region is rare. An unusual case of T-cell lymphoblastic lymphoma with initial oral manifestation in an 18-month-old child is presented.     

非霍奇金淋巴瘤中枢神经系统累及的诊断和预防

非霍奇金淋巴瘤(NHL)患者中枢神经系统(CNS)累及预后不良,其中位生存期2～6个月.与NHLCNS累及相关参数是年轻、进展期、累及结外部位数、乳酸脱氢酶(LDH)增高和国际预后指标(IPI)积分.最有希望的治疗为自体造血于细胞移植,可延长中数生存期10～26个月.处于CNS侵袭高危状态的某些NHL亚型患者需要早期进行CNS预防,如伯基特淋巴瘤(BL)和淋巴母细胞淋巴瘤(LBL).弥漫性大B细胞淋巴瘤(DLBCL)初期治疗时是否需应用CNS预防久有争议,因为它属于CNS累及(≈5%)的低危群体.危险模式的确定有助于预示NHL的CNS复发.     

Late outcome and quality of life after complicated heart operations

The coexistence of two cutaneous non-Hodgkin's lymphomas of different lineage is rare. We report a patient with an indolent erythrodermic cutaneous T-cell lymphoma followed by an aggressive B-cell lymphoma. To our best knowledge, this is the first report describing Epstein-Barr virus-associated B-cell lymphoma in a patient with cutaneous T-cell lymphoma. We suggest that the long-standing cutaneous T-cell lymphoma, as well as the long-term chemotherapy, suppressed host immunity and caused reactivation of latent Epstein-Barr virus.     

Primary cerebral lymphoma following+ kidney transplant: a case report and review of the literature

We report a 30 year old male, presenting eight years after receiving a kidney transplant with intracranial hypertension and two hyperdense masses detected in a brain CAT scan, whose histopathological study revealed a giant cell immunoblastic lymphoma. The patient was successfully treated with chemo and radiotherapy and after 18 months of follow up there is no evidence of tumoral relapse. Immunocompromised patients, specially transplant recipients, had a several fold higher incidence of malignant tumors, specially primary lymphomas of the central nervous system. These are generally of B type, are associated to Epstein Barr virus and have a high mortality. Cancer must be considered in the differential diagnosis of masses of uncertain origin in transplant recipients.     

Hippocampal sclerosis contributes to dementia in the elderly

OBJECTIVE: To assess the relevance of hippocampal sclerosis (HS) to dementia in the elderly. BACKGROUND: HS is a prominent pathologic finding in some demented elderly, but the anatomic substrate and cognitive profiles of this dementia have not been well established. DESIGN/METHODS: An autopsy series, including dot-immunobinding assay to estimate neocortical synaptic density, of eight patients (three men, five women) with HS on whom extensive antemortem neuropsychological testing was available. RESULTS: Mean age at onset was 72.0 (+/-9.8) (range, 59 to 89) with a mean duration of symptoms of 6.5 (+/-2.9) years. Patients were only mildly impaired with a mean MMSE of 20.9 (+/-4.9) and a mean DRS of 103.1 (+/-12.5) at presentation. Cardiovascular disease was present in 88%, with a mean Hachinski score of 3.4 (+/-2.2). No patient had a history of seizures. Sixty-three percent had depression or depressive symptoms. Neuropsychologically, most patients presented with prominent memory and language deficits and became progressively demented. Neuropathologically, isolated HS was a rare finding; many patients had either very mild or neocortical "plaque only or plaque predominant" Alzheimer's disease (AD) in addition to HS changes. Midfrontal neocortical synaptophysin counts were significantly reduced in all HS patients compared with controls (p = 0.0006). CONCLUSIONS: In the elderly, HS can be a neuropathologic substrate of dementia. Clinically, it can be associated with a course that is difficult to distinguish from AD although cardiac disease and depression are frequent concomitants. Deterioration of cognitive function in these subjects may relate to other pathologic features such as neocortical synapse loss.     

Gastric malt lymphoma with crystalline immunoglobulin inclusions and secondary immunoblastic lymphoma in a cervical lymph node

A case of gastric B cell lymphoma of the mucosa-associated lymphoid tissue (MALT) with crystalline immunoglobulin inclusions in monotypic plasma cells is reported, which initially presented as a secondary immunoblastic lymphoma in a cervical lymph node. The low-grade MALT lymphoma was diagnosed 14 months after detection of the nodal high-grade lymphoma, but rare crystal-containing plasma cells of identical immunoglobulin isotype found in the immunoblastic lymphoma retrospectively confirmed a common clonal origin. The diagnostic significance of crystalline immunoglobulin inclusions and the unusual initial presentation of MALT lymphoma as a secondary high-grade lymphoma at a distant nodal site are discussed.     

稀土铁基自熔基合金抗硫化氢腐蚀性能的实验研究

近年来关于稀土在热喷涂（焊）材料中的应用研究正日益受到人们的重视。铁基自熔合金末作为一种广泛使用的热喷涂（焊）材料具有价格低廉，喷涂（焊）层的耐磨性较好等优点，但其耐蚀性较差。为此，本研究引入稀土对其改性。通过熔炼－雾化工艺制备了稀土铁基自熔合金粉末，并考察了稀土对铁基自熔合金喷焊层在硫化氢介质中气蚀和在含硫化氢的中性氯化钠溶液中浸蚀的影响。结果表明，铁基自熔合金（含稀土和不含稀土）喷焊层试样表现出较强的抗硫化氢能力。铁基合金喷焊层试样在含硫化氢的4Wt%NaCl溶液中的腐蚀速率随稀土的添加量增加量增加而逐渐降低，且稀土添加量有最佳值0.2Wt%。     

Primary lymphoma of the breast. Three cases of non-Hodgkin lymphoma

Primary and sole breast lymphoma is a very rare disease. With the review of our series of 616 cases operated on for breast cancer, only 3 cases (0.48%) of primary breast non-Hodgkin lymphoma (LNH) have been observed. The authors outline the problems concerning diagnosis and therapy of this rare disease: pathological and immunohistochemical aspects are discussed. They stress the importance of staging in order to plan a correct multidisciplinary approach.     

Indications and relative indications for stem cell transplantation in non-Hodgkin's lymphoma

High dose chemotherapy with or without total body irradiation and autologous stem cell rescue has proven to be effective treatment to cure patients with relapsed intermediate grade and high grade non-Hodgkin's lymphoma. Important factors for selection of candidates most likely to do well with these approaches include patients whose disease is responsive to conventional therapy and those who have minimal disease volume at the time of transplant. The treatment-related mortality of autologous stem cell transplantation has diminished from 20% to less than 5% with improved supportive care and selection of patients with less advanced disease. Although the treatment-related mortality of allogeneic stem cell transplantation may be as high as 20-40%, a graft versus lymphoma effect may decrease relapse with the result that overall survival is not substantially different between autologous and allogeneic transplantation. The definitive indications for stem cell transplantation include patients who have relapsed with intermediate or high grade NHL. Relative indications include intermediate/high grade non-Hodgkin's lymphoma patients, "high risk" first complete remission (CR), resistant relapse; low grade non-Hodgkin's lymphoma in sensitive or resistant relapse, advanced disease (sensitive or resistant relapse, transformation), first CR (younger patients). Relative contraindications include specific patient profiles such as bulky high grade lymphoma which progresses on appropriate conventional therapy, poor performance status, active serious infection, serious cardiac, renal, pulmonary or liver dysfunction, active, central nervous system (CNS) disease unresponsive to cranial irradiation/intrathecal therapy. For patients with previous marrow involvement or active marrow involvement at the time of harvest or transplant, "purged" autografts, peripheral blood stem cell transplantation and allografts have been used successfully.     

Discrepancy between tau immunoreactivity and argyrophilia by the Bodian method in neocortical neurons of corticobasal degeneration

BACKGROUND: The purpose of this investigation was to identify and characterize abdominal lymphomas as they occur in a large solid-organ-transplant population. METHODS: A large transplant population was isolated, and all patients developing an abdominal lymphoma were identified. These patients were further characterized after review of their medical records and radiologic examinations. RESULTS: Twenty-eight (1%) of 2925 patients developed lymphoma following transplantation. Of these 28 patients, 14 developed abdominal manifestations of disease. Examples of the wide variety of abdominal manifestations of posttransplant lymphoma are presented. Most of these patients had positive titers for Epstein-Barr virus and were treated with cyclosporin as a part of their immunotherapy. The majority of patients died secondary to this aggressive disease process. CONCLUSION: The development of lymphoma following solid organ transplantation is more common than in the general population. One-half of the patients in our study population developed abdominal manifestations of this disease.     

Hidradenitis suppurativa

BACKGROUND: Hidradenitis suppurativa (HS) is a recurrent, suppurative disease manifested by abscesses, fistulas, and scarring. METHODS: We reviewed the literature to identify reliable information regarding epidemiology, pathogenesis, clinical manifestations, evaluation and differential diagnosis, treatment, complications, and prognosis. RESULTS: Hidradenitis suppurativa usually affects young women, with a prevalence of 0.3% to 4% in industrialized countries. Once considered to be "apocrine acne," HS is actually a defect of terminal follicular epithelium. Obesity, chemical irritants, or hyperandrogenism are not consistently associated; bacterial involvement is secondary. Hidradenitis suppurativa should be suspected in young adults with recurrent, deep furuncular lesions in flexural sites, especially when such lesions respond poorly to antibiotic therapy. Clindamycin and isotretinoin may be useful, though wide excision with healing by granulation is considered most efficacious. Anemia, arthropathy, and squamous cell carcinoma are potential complications. CONCLUSIONS: Since spontaneous resolution is rare and progressive disability the rule, early definitive surgical treatment of HS is advisable.     

An overview of rheumatological research in the European Union

We describe a case of a 73-year-old male with a rare T-cell lymphoma that presented deceptively as progressive hepatic failure with fever, weight loss, pancytopenia, mental confusion, splenomegaly, and no lymphadenopathy. An alcoholic history supported the diagnosis of cirrhosis, but a liver biopsy was not performed. A bone marrow biopsy was considered unremarkable. Death occurred after a course of four months. Postmortem examination showed hepatic, splenic, lymph node, and marrow infiltration by characteristically sparse, isolated, bizarre, medium-to-large sized neoplastic cells with extensive hepatic centrilobular necrosis, steatosis, and predominant splenic involvement. Immunohistochemical markers indicated a T-cell lymphoma consistent with either an alpha/beta peripheral T-cell lymphoma or a gamma/delta lymphoma. Definitive immunotyping was not available. However, the pathologic features are most consistent with a gamma/delta T-cell lymphoma. This case is an example of a rare, rapidly progressive lymphoma, which is a recognized clinical entity, easily missed, and treatable. Its diagnostic consideration must be explicitly communicated to pathologists, because the isolated or sparse tumor cells in a lymph node, liver, or bone marrow biopsy may easily be mistaken for variants of megakaryocytes or histiocytes.     

Severe acute liver failure as the initial manifestation of haematological malignancy

Acute liver failure is rarely secondary to lymphoma or leukaemia and it is extremely uncommon as the initial presentation of malignancy. We report a case of a young adult patient with severe acute liver failure referred for liver transplant, in which a Burkitt acute lymphoblastic leukaemia was diagnosed by bone marrow examination. A complete recovery and long remission were obtained with chemotherapy.