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Lymphomatoid granulomatosis (LYG) is a rare Epstein-Barr virus (EBV)-driven B-cell lymphoproliferative disease affecting mainly extranodal sites such as the lung, central nervous system (CNS), skin, kidney, and liver. We report a case of low-grade LYG involving the CNS that was successfully treated with interferon alpha (IFNα). A 69-year-old woman developed necrotic erythema of the skin and was initially diagnosed with pyoderma gangrenosum based on skin biopsy. She showed a limited response to prednisolone. Approximately 6 months after the initial onset, low-grade LYG was diagnosed after detection of CNS lesions on brain biopsy. The whole blood EBV-DNA load determined by real-time polymerase chain reaction was slightly elevated. Two months into IFNα therapy, skin and CNS lesions had responded favorably and the EBV-DNA load decreased. IFNα plays an important role in treatment of LYG through its antiproliferative, immunomodulatory, and anti-EBV effects. To our knowledge, this is the first case report of successful treatment with IFNα in Japan. Further investigation is necessary to determine optimal use of IFNα for LYG., (© 2021. Japanese Society of Hematology.)

Lymphomatoid granulomatosis (LYG) is a rare lymphoproliferative disease with angiocentric and angiodestructive infiltrates, and by definition, Epstein-Barr virus (EBV)-associated B-cell malignancy. It most frequently involves the lung, and in some cases, the lesions are confined to the central nervous system (isolated CNS-LYG). However, it remains a controversial disease in terms of pathophysiology, especially in those confined to the CNS. We report the case of a 37-year-old man with CNS lesion pathologically characterized by angiocentric, T-cell-rich lymphoid cell infiltrates that resembled CNS-LYG. The lesion was clinically aggressive with subacute onset and irregular ring-like enhancement on MRI. The resected specimen showed no cytological atypia, EBV-infected cells, or monoclonality for IgH and TCR gene rearrangements. Considering the possibility of latent malignancy, the patient was successfully treated with corticosteroid and chemoradiotherapy with high-dose methotrexate. The present case and the literature suggest that EBV-negative CNS lesions with angiocentric lymphoid infiltrates are probably heterogeneous in their pathogenesis, including those that could fit into the so-called CNS-LYG and those with T-cell predominance. The accumulation of similar cases is warranted for the classification and appropriate treatment of these lesions., (© 2021. The Japan Society of Brain Tumor Pathology.)

A 75-year-old Japanese woman with a 20-year history of rheumatoid arthritis presented with symptomatic bilateral pleural effusion and lung and brain tumors. She had received methotrexate for five years and tacrolimus for one year. A brain biopsy specimen showed the pathological features of lymphoproliferative disease, but a bone marrow biopsy showed proliferation of plasma cells. She was finally diagnosed with coexistent lymphomatoid granulomatosis (LYG) of the brain and lung and multiple myeloma (MM) of the bone marrow and received chemotherapy for both. This report shows that immunodeficient patients are at risk of developing the unusual coexistence of LYG and MM.

Background: Angiocentric lymph proliferative disorder (ALPD) is a granulomatous lymphoproliferative condition associated with various primary and secondary immunodeficiency states. ALPD is so rare that its prevalence has not been established. Typically affecting middle-aged adults, this condition is often found in the context of Epstein Bar Virus infection and consists of angiocentric and angioinvasive pulmonary infiltrates. Herein, we present a biopsy-proven case of a patient manifesting with a viral meningoencephalomyelitis-like picture with brain, spinal cord, renal and splenic lesions. The diagnosis was confirmed to be ALPD in the context of newly diagnosed HIV infection., Case Presentation: A 35 year-old homosexual man presented with a 5-week history of headaches followed by a 3-week history of horizontal diplopia, limb weakness and right 6th cranial nerve palsy. Lumbar puncture revealed a lymphocytic pleocytosis, high protein and low glucose. Magnetic Resonance Imaging showed scattered lesions throughout the brain and spinal cord and Computed Tomography of the abdomen and pelvis revealed hypodensities involving the kidneys and spleen. HIV testing was positive, with a viral load of 11,096 copies/mL and CD4 count of 324 cells/μL. Serum Epstein Bar virus PCR was positive with 12,434 copies/ml. Right frontal brain biopsy revealed gray matter containing angiogentric cerebritis with organizing infarction but Epstein Bar Virus-in situ preparations were negative and no viral inclusions were identified. A diagnosis of ALPD (also known as lymphomatoid granulomatosis) was made. The patient was initiated on antiretroviral therapy and treated with intravenous rituximab every 3 weeks for 4 cycles and made progressive improvements. By the time of discharge his strength had improved and he was ambulating again although with a walker. Within 2 months, his HIV viral load was suppressed. Magnetic Resonance Imaging of the brain 6 months later revealed interval improvement. At his most recent follow-up, 34 months later, his neurological symptoms had almost completed resolved., Conclusion: Albeit rare, ALPD should be considered in the differential diagnosis of central nervous system lesions in persons with HIV once common etiologies have been eliminated. Furthermore, ALPD involving the central nervous system may occur in in the absence of documented EBV infection in the central nervous system.

The diagnosis of lymphoproliferative disorders associated with immunodeficiency can be challenging because many of these conditions have overlapping clinical and pathologic features and share similarities with their counterparts in the immunocompetent setting. There are subtle but important differences between these conditions that are important to recognize for prognostic and therapeutic purposes. This article provides a clinicopathologic update on how understanding of these B-cell lymphoproliferations in immunodeficiency has evolved over the past decade., (Published by Elsevier Inc.)

Autoimmune side effects are frequent in patients with cancer treated with immune checkpoint-targeting antibodies, but are rare with cancer vaccines. Here, we present a case report on a patient with metastatic melanoma who developed pulmonary sarcoid-like granulomatosis following repetitive vaccinations with peptides and CpG. Despite multiple metastases, including one lesion in the brain, the patient is alive and well more than 13 years after the diagnosis of metastatic disease. The strongly activated tumor-specific CD8(+) T cells showed robust long-term memory and effector functions. It is possible that long-term survival and adverse autoimmune events may become more common for vaccines inducing robust anticancer immune responses as were present in this patient., (©2014 American Association for Cancer Research.)

A 70-year-old woman with rheumatoid arthritis treated with methotrexate (MTX) complained of right arm weakness. On CT and MRI, tumors were found in the right frontal lobe, bilateral lungs, and left renal parenchyma. She was diagnosed as having lymphomatoid granulomatosis (LYG) grade 2 on thoracoscopic biopsy of the left lung. We discontinued MTX and treated a mass lesion in the right frontal lobe with stereotactic radiotherapy. As a result, the tumors showed a gradual reduction in size, and the patient achieved complete remission. LYG is a rare lymphoproliferative disorder, and has various clinical characteristics. We describe herein a patient with LYG grade 2 with cerebral, pulmonary, and renal lesions, who has maintained a complete remission for six months, to date, after treatment.

Lymphomatoid granulomatosis (LYG) is a rare B-cell lymphoproliferative disorder predominantly involving the lungs, but poorly-recognized among clinicians and pathologists. It is an Epstein-Barr virus (EBV)-driven disease mimicking several other diseases on clinical and radiological grounds, generally showing multiple, bilateral nodular, ill-defined infiltrates of the lungs tending to coalescence and/or cavitation. LYG often affects middle-aged males with an underlying immunodeficiency and commonly involves skin and central nervous system during disease progression. Diagnosis requires a generous biopsy and careful histologic examination with immunohistochemical staining and molecular demonstration of EBV genome in large atypical B-cells. LYG is graded as I to III based on the number of large EBV-positive B-cells; grades II/III are now considered as a peculiar variant of T-cell rich diffuse large B-cell lymphoma. In this brief review, clinical, radiologic and pathologic features of LYG will be analyzed with focus on differential diagnosis, the most appropriate treatment and prognosis.

Epstein-Barr virus (EBV) is a DNA virus from the herpes virus group affecting only humans. More than 90% adults are infected by the virus, but very few ever have symptoms. Within the abdomen, both solid and hollow viscera can be involved in symptomatic disease. Awareness of the spectrum of imaging findings, high index of suspicion, and appropriate clinical and laboratory information can help in the early and accurate diagnosis in these cases., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Bartonella henselae is the causative agent of cat-scratch disease and other disorders, including hepatosplenic granulomatosis. This infection has only rarely been reported after solid organ transplantation, where it can mimic the more common post-transplant lymphoproliferative disease. Here we present a case of asymptomatic B. henselae hepatic and lymph nodal granulomatosis in a pediatric patient who had received orthotopic liver transplant 2 months before; we hypothesize that the causative agent was transmitted from the donor. This infection developed early in the post-transplant period; the disease involved only the graft liver and the regional lymph nodes, and the patient did not have a cat or any history of contact, scratches, or bites by a cat. In our patient this infection resolved successfully with a combination of 2 associated antibiotics and reduction of immunosuppressive therapy.

We report on a patient of rheumatoid arthritis (RA) who sequentially developed an axillary mass and a fatal interstitial pneumonia during a 2-year course of methotrexate (MTX) therapy. Autopsy revealed a systemic lymph node involvement and the diagnosis of Epstein-Barr virus (EBV)-related lymphoproliferative disease (LPD) with the features of lymphomatoid granulomatosis was made. The lung tissue specimens revealed a typical diffuse alveolar damage (DAD), and small nodules consisting of atypical B lymphocytes showing positive staining for EBV were sparsely recognized only in basal lungs. This is the first report of a RA patient receiving MTX therapy sequentially developing MTX-associated lymphomatoid granulomatosis and DAD.

Recent studies point to a role of Epstein-Barr virus in the pathogenesis of lymphomatoid granulomatosis. Little is however known about the association of the pathogenesis of the disease with Epstein-Barr virus reactivation. A 46-year-old man presented with fever and general malaise. He had been admitted with fever of unknown origin 10 years previously. Chest radiographs and CT scans showed multiple nodular shadows in both lung fields. PET/CT showed strong accumulation in lung nodules, femoral muscle, lumbar subcutaneous nodule. He died of rapidly progressive neurological lesions. The definitive diagnosis of lymphomatoid granulomatosis was made from histological findings of the surgical biopsy specimen and at autopsy. Elevation of early antigen-IgG titer in serum was shown in this case. This result suggests that reactivation of Epstein-Barr virus is involved in the development of the disease.

A 58-year-old lung transplant patient developed worsening shortness of breath and indurated erythematous plaques on the lower left leg. A skin biopsy specimen revealed a dense angiocentric and angioinvasive infiltrate in the mid to reticular dermis and panniculus containing large, atypical lymphocytes with convoluted nuclei and prominent nucleoli. Immunohistochemical stains showed that the atypical cells were of B-cell origin, and that Epstein-Barr virus was present. Molecular studies demonstrated B-cell clonality. The patient was successfully treated with rituximab. The clinical and pathologic features in this case represent posttransplantation lymphoproliferative disease with features of lymphomatoid granulomatosis. The case also highlights the importance of clinical and pathologic examination of cutaneous lesions in the evaluation of lymphoproliferative disorders for patients undergoing transplantation.

Aim: To analyse causes of reactive lymphadenitis regarding histological changes in the lymph node, of diagnostic errors; to determine frequency of establishment of nosological diagnosis., Material and Methods: A retrospective analysis of diagnostic examination of 375 patients with reactive lymphadenitis observed from 1992 to 2000 was made., Results: For differential diagnosis 6 groups of reactive lymphadenopathies with different histological picture were identified: follicular hyperplasia, paracortical hyperplasia, granulomatous lymphadenitis, granulomatous pyonecrotic lymphadenitis, sinus histiocytosis as a leading sign and node necrosis as a leading sign. The analysis of making nosological diagnosis for each group and of diagnostic errors was conducted., Conclusion: Characteristics of lymph node changes in reactive lymphadenitis is of great importance. Better cooperation between histologists and clinicians must improve efficacy of reactive lymphadenitis diagnosis.

A patient was recently evaluated who had longstanding sarcoidosis with lymphadenopathy and multiple, small lung nodules, and who developed a new, 9 cm solitary pulmonary mass in the right lower lobe. After thoracotomy, this lesion was ultimately found to be lymphomatoid granulomatosis, a rare lymphoproliferative disorder. Radiographic evaluations of patients with this disorder characteristically show multiple, bilateral reticulonodular opacities that follow the bronchovascular bundles; however, presentation with a solitary, large pulmonary mass is rare. The present case illustrates the need for complete evaluation of new clinical and radiographic findings in the setting of chronic lung disease.

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.

Angiocentric lymphomatoid granulomatosis is a rare lymphoproliferative disease, mainly associated with pulmonary manifestation. Its origin is unknown, but Epstein-Barr virus may be one of the etiological factors. A 51-year-old male had an abdominal laparotomy in 1994 and a large granulomatous mass was removed from behind the cecum. No specific therapy was administered. In February 1998 multiple pulmonary lesions were found by X-ray and thoracoscopic biopsy was made. The histopathological diagnosis was angiocentric lymphomatoid granulomatosis. The patient received 6 cycles of CHOP chemotherapy, with which a complete remission was achieved. A consistent severe hypogammaglobulinaemia was detected, so the diagnosis of common variable immunodeficiency (CVID) was established. The diagnosed CVID was the probable causative factor of the angiocentric lymphomatoid granulomatosis. After the CHOP treatment, the patient is on intravenous immunoglobulin substitution and is well up to today.

Lymphomatoid granulomatosis is a necrotizing angiocentric and angiodestructive infiltrative process involving primarily the lung, skin, central nervous system, and kidney. The incidence is highest in middle-aged men and is rare in children. We report a case of lymphomatoid granulomatosis involving both skin and lung in a 4-year-old boy. The disease progressed to peripheral T-cell lymphoma, which was unusual in light of recent evidence suggesting a B-cell origin in the majority of cases.

Background: Lymphomatoid granulomatosis is an Epstein-Barr virus-associated B-cell lymphoproliferative disease. It is angiocentric and angiodestructive and involves the lungs, central nervous system and skin. Exclusive cutaneous involvement is rare and may be associated with a better outcome. Contrarily to the extra-cutaneous forms of lymphomatoid granulomatosis, it is difficult or impossible to detect Epstein-Barr virus DNA sequences in primary and isolated cutaneous lymphomatoid granulomatosis., Case Report: A 54-year-old woman developed erythemato-violaceous lesions on both legs 3 years after a heart-lung transplantation. The diagnosis of erythema multiforme and of drug-induced vasculitis were first made. Because of fever and of the rapid extension of the lesions, the patient was hospitalized. The histologic examination of the first lesions showed a perivascular infiltrate, without epidermotropism, composed of histiocytes, lymphocytes and plasma cells. Immunohistochemistry revealed the presence of a predominantly T-cell infiltrate with some large B cells. Subsequent biopsies were diagnosed as high grade B-cell lymphoma. Polymerase chain reaction analysis as well as in situ hybridation study showed the presence of Epstein-Barr virus load in the lesions. There was however no serologic evidence of viral reactivation. Extensive systemic evaluation revealed no visceral or bone marrow involvement. Despite antiviral treatment and CHOP polychemotherapy, the patient died 3 months after her admission., Discussion: This observation of lymphomatoid granulomatosis is particular because of its exclusive cutaneous involvement associated with a fulminant evolution to high grade B lymphoma. The presence of a context of iatrogenic immunosuppression underlies the role of altered immune cellular functions in the initiation and/or progression of lymphomatoid granulomatosis and strengthens the role of a viral agent in its pathogenesis. We suggest that the presence of Epstein-Barr virus, which is generally not associated with the isolated cutaneous forms of lymphomatoid granulomatosis, may have played a role in this fulminant evolution to high grade B lymphoma.

Lymphomatoid granulomatosis (LYG) is a rare angio-destructive lymphoproliferative disorder (LPD) of uncertain etiology, with prominent pulmonary involvement. Recent studies indicate that LYG is an Epstein-Barr virus (EBV)-associated B cell LPD with large numbers of background reactive T lymphocytes (T cell-rich B cell lymphoma). Although the disease frequently, but not exclusively, occurs in various immunodeficiency states, it has not been reported in association with the transient immunosuppression following autologous bone marrow/peripheral stem cell transplantation (ABM/PSCT). We describe a patient who developed lymphomatoid granulomatosis of the lung approximately 2 weeks after high-dose chemotherapy and autologous peripheral stem cell transplantation for multiple myeloma. Although molecular studies showed no evidence of EBV genome in the biopsy material, the serologic profile with high IgM titers was suggestive of primary EBV infection. Complete radiologic remission occurred following reconstitution of the patient's immune response after a 2-week course of ganciclovir treatment. Despite the apparently low frequency of LPD (both LYG and EBV-associated post-transplant lymphoma) in the ABMT setting, we believe that it should be considered in the differential diagnosis of patients whose clinical course following ABMT is complicated by fevers, in the absence of an identifiable infectious process.

A 5-year-old dog showed remarkable edematous swelling of the left hock with lameness and local cellulitis, and paecillomyces fungus was isolated from ulcerative lesion of the hock joint and mediastinum. At autopsy severe effusive pleuritis was shown and numerous necrotizing and granulomatous lesions with fungal elements were seen in the liver, pancreas, kidney and mediastinal lymph nodes.

A 48-year-old woman was admitted to our hospital because of cough and low-grade fever. Chest X-ray films showed multiple ill-defined nodular shadows and laboratory findings showed that she was an ATL-virus carrier. Because the specimens obtained by transbronchial biopsy were not diagnostic, open thoracotomy was performed. Open lung specimens showed proliferation of lymphoid cells composed of polymorphorous lymphocytes, which do not represent cytologic atypia, plasma cells and histiocytes without necrosis. Several vessels were occluded by the infiltration of these cells. A diagnosis of angiocentric immunoproliferative lesions (Grade I), which was originally coined by Jaffe, was made. Complete remission was achieved by treatment with prednisolone and cyclophosphamide. This disease, which corresponds to lymphomatoid granulomatosis, is graded into three categories depending on the severity of cytologic and histologic malignancy. Although it has recently been suggested that its pathogenesis may be related to EB-virus infection, ATL-virus may have been a contributory factor in this case.

A 49 years-old man presented with dry cough, low grade fever, and abnormal shadow on a chest X-ray. He had suffered from follicular lymphoma of the liver 5 years previously. He received irradiation therapy in combination with chemotherapy for approximately three years and had been in complete remission. Physical and radiological examination revealed pleural effusion and softly dense masses in the right lung. The laboratory data were within normal limits. He was diagnosed as having lymphomatoid granulomatosis (LYG) by open lung biopsy. The lung lesion was mainly infiltrated with T cells. The patient received prednisolone and the lung lesions disappeared. However, when a lung mass was noted two months later, he started to receive combination chemotherapy consisting of cyclophosphamide, adriamycin, vincristine, and prednisolone every three months. He has not shown relapse of LYG so far. To investigate the association between the preceding follicular lymphoma and subsequent LYG at this time, DNA analysis using the PCR technique was carried out. The LYG lesion did not show a rearranged band for the JH probe, while the paraffin-embedded specimen of the preceding follicular lymphoma had shown rearranged band for the JH band. Southern blot analysis of the LYG lesion, showed no rearrangement for TCR beta, gamma or JH probe. These findings indicate that the LYG was different from the preceding follicular lymphoma in terms of origin. LYG is considered to be induced in the immunosuppressive state due to lymphoma.

In a patient treated for acute myeloblastic leukemia (AML), we saw an angiocentric and angiodestructive lymphoma that resembled lymphomatoid granulomatosis (LG). The lesions tended to involve extranodal sites such as the lung, the parotid gland, and the skin. The immunologic studies showed that the proliferating lymphoid cells were mature T cells. Furthermore, genotypic studies disclosed a clonal rearrangement of the beta T-cell receptor gene. It is concluded that this case of LG is related to a neoplastic T-cell lymphoproliferative disorder. The relations between LG and the previous AML are discussed.

Numerous systemic diseases share immunopathogenic mechanisms. This article reviews the proposed etiologies and immunopathogenic mechanisms of a group of diseases which share pulmonary and renal abnormalities. Specifically, we discuss the following diseases: Good-pasture's syndrome, systemic lupus erythematosus, progressive systemic sclerosis, Wegener's granulomatosis, lymphomatoid granulomatosis, and Churg-Strauss syndrome.

A review of records of 44 patients who had lymphomatoid granulomatosis (LYG) with skin lesions showed that cutaneous manifestations are protean and generally transient. Subcutaneous or dermal nodules were present in more than 60% of cases. A maculopapular rash or macular erythema was less common, and ulceration was rare. Skin lesions were widely distributed. Frequently, in patients who had early cutaneous involvement with LYG, associated involvement of the nervous system and joints occurred. Findings from laboratory studies were generally inconclusive. Lymphomatoid granulomatosis progressed to lymphoma in 18% of the cases, and death from respiratory failure was common. Theories on the possible cause and the pathogenesis of LYG are presented.

Disseminated granulomatosis is a well-documented complication of intravenous narcotism. We present here an interesting case in which this complication was caused by a right-to-left shunt (following intravenous injection of medicinal tablets intended for oral use) through a patent foramen ovale resulting from pulmonary hypertension due to angiothrombosis.

The authors discussed the malign form of lymphomatoid granulomatosis on the basis of two cases and consider the possible role of an aerocontaminant which is usually responsible for the extrinsic allergic type of alveolitis. A detailed biochemical and cytological study of the alveolar fluid from one of these patients is presented in parallel with immunofluorescent histology and electron microscopy of the lung. The observed biochemical abnormalities in the alveolar fluid could be superimposed on those formed in certain types of hypertensive lung disease. On the other hand the cytology was different, the cytofluorimetric technique revealed a mononuclear cell population rich in RNA whose role in the genesis of the disease of malign nature are discussed. The hypothesis of a hypersecretion of IgA by this malign process is raised by immunofluorescent studies of the lung. The role of IgA in lymphomatoid granulomatosis and bird fanciers lung is discussed in the light of recent work on pigeon IgA.

This paper describes the first French case of lymphomatoid granulomatosis (LYG) in 46-year-old male. Autopsy revealed bilateral pulmonary involvement and multiple central nervous system (CNS) localizations. The originality of this study is due to 1st the scarcity of reported cases of LYG in Europe 2nd a detailed neuropathological study permitting diagnosis of multifocal central nervous involvement by LYG. In the literature only 12 previous cases of LYG have histologically proven CNS lesions. These lesions correspond to; 8 instances of LYG, one progressive multifocal leukencephalopathy,, one post-therapeutic disseminated necrotizing leukoencephalopathy, one immunoblastic sarcoma and one case of LYG associated with histiocytic malignant lymphoma. This paper also evokes clinical, histological, ultrastructural and prognostic characteristics of LYG gathered from the available literature. From a nosological point of view the relationship of LYG with Wegener granulomatosis, polymorphic reticulosis and certain lymphomas is still, to this day, not clearly established.

A case report of a patient with lymphomatoid granulomatosis presenting initially as a reactivated Epstein-Barr virus infection is presented. Epstein-Barr virus is proposed in the possible role of establishing of an immunologically compromised state that may have set the stage for dissemination of this disease process. Of interest is the fact that successful chemotherapeutic management of the disease was accomplished using prednisone and cyclophosphamide. Furthermore, this clinical success was reflected in a decreasing Epstein-Barr virus early antigen-antibody titers accompanied by increasing antivirus capsid antigen titers; hence, it appears that laboratory markers of the response of lymphomatoid granulomatosis to treatment are available in the form of soluble immune complexes, antibodies to Epstein-Barr virus-coded antigens early antigens, Epstein-Barr nuclear antigens and/or virus capsid antigens as well as the active E rosette assay for T-cells. Finally, these data, although supporting the role of Epstein-Barr virus in the pathogenesis of lymphomatoid granulomatosis, suggest the need for further study in additional patients to verify the results.

Lymphomatoid granulomatosis is a multi-system disorder of high mortality and characterised by angiocentric and angiodestructive lymphoreticular proliferative and granulomatous change. A case is described of this disease occurring in a renal transplant patient. The patient underwent a rapid remission of the disease after a course of combined cytotoxic therapy and remains free of disease three years after treatment. This case is the second described renal transplant patient to develop the disease and the first to be successfully treated. It is likely that the disease originated as a consequence of immunosuppression similar to the development of lymphomas in allograft recipients.