Primary cutaneous CD30 positive large cell lymphoma is a cutaneous T-cell lymphoma with a favorable prognosis, which is characterized by solitary or localized skin lesions with a tendency of spontaneous regression and frequent relapses. On the basis of the morphologic and immunophenotypic similarities between the large atypical cells in lymphomatoid papulosis and the neoplastic cells in primary cutaneous CD30 positive large cell lymphoma as well as their favorable prognosis, these two diseases are now regarded to be in a spectrum of primary cutaneous CD30 positive lymphoproliferative disorder. We present a borderline case of primary cutaneous CD30 positive lymphoproliferative disorder which recurred on the anatomic site different to the primary lesions despite multiple-agent chemotherapy.
Drug Therapy ; Lymphoma ; Lymphoma, T-Cell, Cutaneous ; Lymphomatoid Papulosis ; Lymphoproliferative Disorders* ; Prognosis ; Recurrence ; Skin

Cytophagic histiocytic panniculitis gap (CHP) was described in 1980 as a chronic histiocytic disease of the subcutaneous tissue and associated with systemic manifestations such as fever, serositis, and hepatosplenomegaly. The current concept of CHP is that it represents a spectrum of lymphoproliferative disorders that induce secondary histiocytic cytophagocytosis. The pathogenesis of hemophagocytosis in CHP is unknown but may be related to histiocyte reaction to an abnormal cytokine milieu brought about by disordered T-cell function. In most cases the disease follows a fulminant course, but in some patients the disease seems limited to the skin and subcutaneous tissue and follows a more benign, chronic course. We report a case of cytophagic histiocytic panniculitis which had a long chronic course over 16 years, but recently became aggravated with the involvement of the extrapleural fat tissue and was treated with combination chemotherapy.
Cytophagocytosis ; Drug Therapy, Combination ; Fever ; Histiocytes ; Humans ; Lymphoproliferative Disorders ; Panniculitis* ; Serositis ; Skin ; Subcutaneous Tissue ; T-Lymphocytes

Posttransplant lymphoproliferative disorder (PTLD) are among the most serious and potentially fatal complications of chronic immunosuppression in organ transplant recipient and also the most common malignancies, accounting for 21 percent of all malignancies in organ transplants versus 5 percent of malignancies in the general population. PTLD is associated with immunosuppression and Epstein Barr virus (EBV). Treatment modality of PTLD includes antiviral agent, interferon, intensive chemotherapy and monoclonal antibody. Choice of treatment modality depends on clinical presentation of PTLD. We report here a case of PTLD involving liver and renal allograft treated with rituximab.
Allografts ; Drug Therapy ; Herpesvirus 4, Human ; Immunosuppression ; Interferons ; Kidney Transplantation ; Liver ; Lymphoproliferative Disorders ; Transplantation* ; Transplants ; Rituximab

Primary cutaneous CD30+ lymphoproliferative disorders include primary cutaneous CD30+ (anaplastic) large T cell lymphoma, lymphomatoid papulosis, and borderline cases. These represent a clinical and histologic continuum rather than a separate entity. We report a 39-year-old woman with the clinical features of lymphomatoid papulosis. Histology favored primary CD30+ anaplastic large cell lymphoma. Although she was treated with aggressive combination chemotherapy, the lesions recurred shortly after discontinuation of chemotherapy. She is now regularly followed up without any treatment.
Adult ; Drug Therapy ; Drug Therapy, Combination ; Female ; Humans ; Lymphoma, Large-Cell, Anaplastic ; Lymphoma, T-Cell ; Lymphomatoid Papulosis* ; Lymphoproliferative Disorders

A case of primary oral mucosal diffuse large B-cell lymphoma(DLBCL)due to long-term use of methotrexate(MTX)for the treatment of rheumatoid arthritis(RA)was admitted to the Department of Hematology,Fujian Medical University Union Hospital.We analyzed and discussed the clinical features,diagnosis and treatment,and prognosis of specific malignant lymphoma induced by MTX in this RA patient.Our purpose is to improve the awareness and knowledge of other iatrogenic immunodeficiency-associated lymphoproliferative disorders of clinicians and pathologists.This study provides a new reference for the clinical diagnosis and treatment of MTX-associated DLBCL.
Arthritis, Rheumatoid/drug therapy* ; Humans ; Lymphoma, Large B-Cell, Diffuse/drug therapy* ; Lymphoproliferative Disorders ; Methotrexate/adverse effects*

Primary cutaneous CD30-positive large cell lymphoma and lymphomatoid papulosis; both entities are characterized by CD30-positive large atypical cells predominantly of T cell origin. We report a borderline case between CD30-positive large cell lymphoma and lymphomatoid papulosis in a 57-year-old woman presenting as a spontaneous disappearing and recurrent solitary erosive nodule on the right upper eyelid. Histopathological findings of two biopsies from each lesion showed mixed CD30-positive large atypical cells, many eosinophils and neutrophils histologically. The nodule disappeared after chemotherapy and subsequent radiotherapy. She is well without recurrence for a follow-up of 9 months.
Biopsy ; Drug Therapy ; Eosinophils ; Eyelids ; Female ; Follow-Up Studies ; Humans ; Lymphoma ; Lymphomatoid Papulosis ; Lymphoproliferative Disorders* ; Middle Aged ; Neutrophils ; Radiotherapy ; Recurrence

OBJECTIVETo study the role of flow cytometry (FCM) in detection of polymorphic post-transplant lymphoproliferative disorders (PTLD).

METHODS AND RESULTSTwo patients presented with fever and multiple lymphadenopathy on day 46 and day 50 respectively after successful allogeneic hematopoietic stem cell transplantation (allo-HSCT). The symptoms couldn't be controlled by antibiotics. The polymorphic PTLD was diagnosed based on the elevation of bone marrow EB virus DNA and detection of subsets of light chain restricted B cells and/or plasma cells in peripheral blood (PB) samples. The lymphocyte immunophenotypes from PB and/or bone marrow (BM) samples were serially tested by FCM after lowering the dose of immunosupressive agents and treating with antivirus drugs, anti-CD20 antibodies, and cytotoxic T cell infusion. B cells were undetable in two patient, but monoclonal plasma cells appeared or maintained. One patient died after two weeks. Another patient was still on treatment. B cells and plasms cells couldn't be detected in her PB, but there were monoclonal plasma cells in her BM. FCM have a prominent advantage in detect polymorphic PTLD, since it can effectively recognize different cell groups in blood and identify monoclonal subsets. Besides, the immunophenotype of plasma cells in polymorphic PTLD might be different from that in typical plasma cell myeloma.

CONCLUSIONPolymorphic PTLD can be detected and followed up by FCM. BM is more suitable than PB for monitoing the disease. Besides lymph node biopsy, B cell abnormaliity could be detected in PB in allo-HSCT patients.

A 67 year old woman with a 10 year history of rheumatoid arthritis (RA) treated with methotrexate and prednisone, presented with a 2 year history of worsening multiple cutaneous plaques of variable appearance. Two distinct skin lesions were biopsied to reveal a composite cutaneous lymphoma, possibly caused by long term methotrexate therapy. An [18F] fluoro-2-deoxy-D-glucose (¹⁸F-FDG) positron emission tomography/computed tomography (PET/CT) was performed to stage the malignancy, and was later repeated to evaluate response to chemotherapy, which guided subsequent management. We present the PET/CT imaging findings of this very rare iatrogenic (methotrexate induced) immunodeficiency-associated lymphoproliferative disorder.
Arthritis, Rheumatoid ; Composite Lymphoma ; Drug Therapy ; Electrons ; Female ; Humans ; Lymphoma ; Lymphoproliferative Disorders ; Methotrexate ; Positron-Emission Tomography and Computed Tomography ; Prednisone ; Skin

Epstein-Barr virus (EBV)-associated post-transplantation lymphoproliferative disease (EBV-PTLD) is a potentially life-threatening complication after hematopoietic stem cell transplantation or solid organ transplantation. In the last decade, the survival of patients with EBV-PTLD has been significantly improved by immunotherapeutic interventions among high-risk patients. The immunotherapeutic interventions for EBV-PTLD include reduction in immunosuppression, CD20 monoclonal antibodies (rituximab) as monotherapy or in combination with chemotherapy, and adoptive immunotherapy with EBV-specific T cells. This paper reviews the latest update on the high-risk factors, clinical manifestations and immunotherapy of EBV-PTLD.
Epstein-Barr Virus Infections ; complications ; therapy ; Hematopoietic Stem Cell Transplantation ; adverse effects ; Humans ; Immunotherapy ; methods ; Lymphoproliferative Disorders ; etiology ; therapy ; Postoperative Complications ; etiology ; therapy ; Risk Factors

A 38-year-old man was admitted with a high fever, sore throat, and right upper quadrant pain. Nine months before his admission, he had undergone right hemicolectomy under the impression of intestinal lymphoma. But there had been no evidence of lymphoma on microscopic examination. Under the postoperative diagnosis of inflammatory bowel disease, corticosteroid therapy was tried without response. On the follow-up colonoscopic examination, an ovoid ulcer, with convergence of the surrounding mucosal folds at the descending colon and an irregularly shaped ulcer at the ileocolic anastomotic site, were found. The colonoscopic diagnosis was Behcet's colitis. After pathologic slides of biopsy and surgical specimens obtained from the palatine tonsil and colon were reviewed, the diagnosis of polymorphic reticulosis was made. The patient received anticancer chemotherapy, including cyclophophamide and glucocorticosteroid. To date, colonic involvement of polymorphic reticulosis has not been reported. Because of the similarity of the colonoscopic findings to those of Behcet's colitis, polymorphic reticulosis should be included in the differential diagnosis of inflammatory bowel disease. We assume that this is the first case of polymorphic reticulosis involving the colon with characteristic colonoscopic findings.
Adult ; Colonic Neoplasms/*diagnosis/drug therapy/pathology ; Cyclophosphamide/therapeutic use ; Diagnosis, Differential ; Drug Therapy, Combination ; Glucocorticoids/therapeutic use ; Humans ; Lymphoproliferative Disorders/*diagnosis ; Male