We report here a case of angioimmunoblastic lyinphadenopathy with dysprote inemia(AILD) in a 65-year-old male associated with skin nodules on the face. Physical examina.tion showed generalized lymphadenopathy, hepatosplenomegaly, erythematous maculopapular eruptions on the trunk and extremities, and er the matous nodules on the face. Lahoratory findings showed hemolytic anernia, arid polyclonal gammopathy. Histopathological findings of lymph node revealed typical features of AILD. I-iistopathological findings of maculopapules revealed lymphohistiocytic infiltra tion and extravasated erythrocytes around the blocked vessels, whereas histological picture of nodules on the face mirrorred that of the lymph node. After taking systemic chemotherapy, his general condition and skin lesions were improved.
Aged ; Drug Therapy ; Erythrocytes ; Extremities ; Humans ; Immunoblastic Lymphadenopathy* ; Lymph Nodes ; Lymphatic Diseases ; Male ; Skin*

Aged ; Bendamustine Hydrochloride ; therapeutic use ; Humans ; Lymphadenopathy ; diagnosis ; Lymphoma ; diagnosis ; drug therapy ; Male ; Rituximab ; therapeutic use

Herein we report a 51-year-old male with angioimmunoblastic lymphadenopathy who had developed rnaculopapular eruptions after the administration of crystalline penicillin, acetylsalicylic acid and sulpyrine. Skin biopsy showed mild lyrnphohistiocytic infiltration and extravasated RBCs around the blood vessels in the upper dermis. He had run a progressively downhill course in spite of vigorous chemotherapy and ultimately died.
Aspirin ; Biopsy ; Blood Vessels ; Crystallins ; Dermis ; Dipyrone ; Drug Therapy ; Humans ; Immunoblastic Lymphadenopathy ; Lymphatic Diseases* ; Male ; Middle Aged ; Penicillins ; Skin

Angioimmunoblastic lymphadenopathy with dysproteinemia(AILD), first described at early seventies, is a clinico-pathologic syndrome which has common features of lymphadenopathy, hepatosplenomegaly, fever, rash and dysproteinemia. It is a lymphoproliferative disease, differs from malignant lymphoma by the high frequency of constitutional symptoms and by the presence of generalized lymph node enlargement, hepatosplenomegaly, cutaneous menifestations and polyclonal hypergammaglobulinemia at the time of clinical onset. Peripheral T cell lymphomas(PTCL) are morphologically and immunologically heterogeneous group of lymphoproliferative disorders that are composed of postthymic lymphocytes. There are group of cases showing features similar to AILD, and these cases are classified as AILD like PTCL. We report a rare case of AILD like PTCL in an 8 year old boy, who visited Seoul National University Hospital with fever, hepatosplenomegaly and cervical lymphadenopathy, and diagnosed as AILD like PTCL by lymph node biopsy. He did not respond to conventional chemotherapy and died 1 year after clinical onset during chemotherapy, showing feature of multiple brain infarction of unknown etiology.
Biopsy ; Brain Infarction ; Child ; Drug Therapy ; Exanthema ; Fever ; Humans ; Hypergammaglobulinemia ; Immunoblastic Lymphadenopathy* ; Lymph Nodes ; Lymphatic Diseases ; Lymphocytes ; Lymphoma ; Lymphoma, T-Cell, Peripheral* ; Lymphoproliferative Disorders ; Male ; Paraproteinemias ; Seoul

We present a case of angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) in a 57-year-old male who had suffered from fever and general weakness of one month's duration. He showed generalized lymphadenopathy, hepatomegaly and purpuric maculopapules on the whole body surface. Lahoratory findings show anemia, polyclonal gammaglobulinemia and thrombocytopenia. The lymph node biopsy specimens show morphologic triad of AILD except lack of interstitial deposits of eosinophilic materials and the skin biopsy specimen shows lymphohistiocytic vasculitis. Bone marrow biopsy specimen shows similar findings in lymph node, suggesting bone marrow involvement. He have been treated with COPP chemotherapy.
Anemia ; Biopsy ; Bone Marrow ; Drug Therapy ; Eosinophils ; Fever ; Hepatomegaly ; Humans ; Immunoblastic Lymphadenopathy* ; Lymph Nodes ; Lymphatic Diseases ; Male ; Middle Aged ; Skin ; Thrombocytopenia ; Vasculitis

We report a case of 74 year old man presented with generalized lymphadenopathy with hepatosplenomegaly. Lymph node biopsy revealed angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) and most areas of lymph node were replaced with large atypical lymphocytes which were revealed to be T cells by immunohistochemistry. All together, the patient was diagnosed as AILD-like T cell lymphoma with bone marrow involvement. The patient presented monoclonal gammopathy (IgG lambda) without any evidence of plasma cell dyscrasia or secondary amyloidosis. After three cycles of combination chemotherapy including cyclophosphamide, prednisone, vincristine and doxorubicin, the patient achieved partial remission with decreased level of serum and urine monoclonal protein.
Aged ; Amyloidosis ; Biopsy ; Bone Marrow ; Cyclophosphamide ; Doxorubicin ; Drug Therapy, Combination ; Humans ; Immunoblastic Lymphadenopathy ; Immunohistochemistry ; Lymph Nodes ; Lymphatic Diseases ; Lymphocytes ; Lymphoma, T-Cell* ; Paraproteinemias* ; Prednisone ; T-Lymphocytes ; Vincristine

Composite lymphoma (CL) involving B-cell lymphoma and T-cell lymphoma is extremely rare. Herein, we report three such cases using immunohistochemistry, flow cytometry, and the next-generation sequencing (NGS) to identify the pathological and molecular characteristics of CL. In the first case, the patient was admitted to hospital for generalized pruritic maculopapular rash over the whole body. An excisional biopsy of the skin lesions showed T-cell lymphoma. At the same time, the staging bone marrow (BM) biopsy revealed a diffuse large B-cell lymphoma (DLBCL). After R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) therapies, the patient produced a good response with substantial dissipation of the rashes and relief of skin. The other two patients were admitted to hospital due to lymphadenopathy and were diagnosed with DLBCL and follicular lymphoma (FL) after core needle biopsy of lymph nodes, BM biopsy, BM aspiration, and flow cytometry. Following R-CHOP and R-COP (rituximab, cyclophosphamide, vincristine, and prednisone) therapies, they achieved complete remission unconfirmed (CRu) and complete remission (CR). However, one or two years later, they suffered a relapse of lymphadenopathy. The shocking fact was that re-biopsy of lymphadenopathy revealed peripheral T-cell lymphoma (PTCL) and angioimmunoblastic T-cell lymphoma (AITL). NGS findings identified DNA methyltransferase 3a (DNMT3a), isocitrate dehydrogenase 2 (IDH2), Ras homolog gene family, member A (RHOA), splicing factor 3B subunit 1 (SF3B1), and tumor protein p53 (TP53) mutations. After immunochemotherapy, these patients achieved CRu and CR again. Nevertheless, they suffered a second relapse of T-cell lymphoma. Finally, they died due to progression of disease. We found that the occurrence of CL is associated with Epstein-Barr virus infection and DNMT3a, IDH2, and TP53 mutations, and the prognosis of the disease is closely related to the T-cell lymphoma components.
Humans ; Rituximab/therapeutic use* ; Vincristine/therapeutic use* ; Prednisone/therapeutic use* ; Epstein-Barr Virus Infections/drug therapy* ; Herpesvirus 4, Human ; Neoplasm Recurrence, Local ; Lymphoma, T-Cell/drug therapy* ; Cyclophosphamide/therapeutic use* ; Lymphoma, Large B-Cell, Diffuse/pathology* ; Doxorubicin/therapeutic use* ; Lymphadenopathy/drug therapy* ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use*

No abstract available.
Adult ; Biopsy ; Cholecystitis/diagnostic imaging/*etiology/therapy ; Churg-Strauss Syndrome/*complications/diagnosis/drug therapy ; Diagnosis, Differential ; Female ; Gallstones/diagnostic imaging/*etiology/therapy ; Glucocorticoids/therapeutic use ; Humans ; Lymphadenopathy/diagnostic imaging/*etiology/therapy ; Magnetic Resonance Imaging ; Mediastinum ; Methylprednisolone/therapeutic use ; Predictive Value of Tests ; Tomography, X-Ray Computed ; Treatment Outcome

OBJECTIVETo retrospectively analyze the clinical features and prognostic factors of patients with angioimmunoblastic T-cell lymphoma (AITL).

METHODSThe clinicopathological and follow-up data of 18 AITL patients undergoing integrated treatment from Feb. 1998 to April 2009 in our department were retrospectively analyzed. All of the patients received CHOP-like regimens as initial chemotherapy, including 4 once treated with radiotherapy and 1 with high dose therapy followed by autologous stem cell transplantation (HDT-ASCT) as upfront consolidation therapy. B-cell, T-cell and NK-cell subgroup proportions in the peripheral blood were tested by flow cytometry in 6 patients.

RESULTSThe median age of the 18 patients was 55 years, male and female ratio was 2.6:1. Seventy-two percent of the patients were in an advanced stage. 72% of them had B symptoms, 69% hypergammaglobulinemia, 60% elevated LDH and 47% anemia. Forty-four percent achieved CR after initial treatment with CHOP-like regimens. With the median follow-up of 26 months, the overall 2-year survival and disease free survival (DFS) rates were 62.2% and 44.4%, respectively. In the univariate analysis, only age > 30 years and primary refractory disease adversely affected overall survival (OS); age > 30 years, advanced stage, B symptoms and splenomegaly adversely affected DFS. Four patients suffered from severe pneumonia during treatment, 2 of them died of respiratory failure. Flow cytometry of peripheral blood lymphocytes showed that 5 of the 6 tested cases had decreasing proportion of CD3(+)CD4(+) T cells, B cells and NK cells but elevated CD3(+)CD8(+) T cells. Two heavily treated patients achieved partial and complete response by thalidomide therapy, with a progression free survival (PFS) of 2 and 6+ months, respectively.

CONCLUSIONAITL patients do not response well to CHOP-like regimens chemotherapy. Age < 30 years and sensitive to initial chemotherapy are associated with prolonged OS. Effectiveness of thalidomide in the treatment of AITL deserves further investigation. Peripheral blood lymphocytes test indicates that AITL patients suffered from both natural and acquired immune defects.

Adult ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Cyclophosphamide ; therapeutic use ; Disease-Free Survival ; Doxorubicin ; therapeutic use ; Female ; Follow-Up Studies ; Humans ; Immunoblastic Lymphadenopathy ; blood ; drug therapy ; pathology ; radiotherapy ; L-Lactate Dehydrogenase ; blood ; Lymphoma, T-Cell, Peripheral ; blood ; drug therapy ; pathology ; radiotherapy ; Male ; Middle Aged ; Pneumonia ; etiology ; Prednisone ; therapeutic use ; Retrospective Studies ; Stem Cell Transplantation ; Survival Rate ; Thalidomide ; therapeutic use ; Vincristine ; therapeutic use

Adult ; Anti-Inflammatory Agents, Non-Steroidal ; adverse effects ; therapeutic use ; CD3 Complex ; metabolism ; Colitis, Ulcerative ; drug therapy ; Diagnosis, Differential ; Drug Hypersensitivity ; etiology ; metabolism ; pathology ; Female ; Gastrointestinal Agents ; adverse effects ; therapeutic use ; Humans ; Immunoblastic Lymphadenopathy ; metabolism ; pathology ; Ki-1 Antigen ; metabolism ; Lymphadenitis ; chemically induced ; metabolism ; pathology ; Lymphoma, Large-Cell, Anaplastic ; metabolism ; pathology ; Lymphoma, T-Cell ; metabolism ; pathology ; Receptors, Complement 3d ; metabolism ; Sulfasalazine ; adverse effects ; therapeutic use