Case Of Angioimmunoblastic T-cell Lymphoma Research Articles

A Rare Case of Angioimmunoblastic T-Cell Lymphoma with Pleomorphic Cutaneous Manifestations

A Rare Case of Angioimmunoblastic T-Cell Lymphoma with Pleomorphic Cutaneous Manifestations

TRBC1 enables identification of an otherwise immunophenotypically silent case of angioimmunoblastic T-cell lymphoma.

TRBC1 enables identification of an otherwise immunophenotypically silent case of angioimmunoblastic T-cell lymphoma.

Cutaneous Involvement of Angioimmunoblastic T-cell Lymphoma

A 74-year-old man with chronic lymphocytic leukemia and angioimmunoblastic T-cell lymphoma (AITL) of the right lung was undergoing evaluation for initiation of chemotherapy. Bendamustine and rituximab were previously initiated but stopped after one month due to adverse effects. The patient subsequently completed 7 years of ibrutinib therapy, which was halted 3 weeks prior to presentation due to its ineffectiveness and significant hematologic lab abnormalities. Treatment with CHOP (cyclophosphamide, doxorubicin hydrochloride, vincristine sulfate, prednisone) was proposed, however the patient had a nodule concerning for cellulitis on the left fifth palmar digit requiring treatment prior to chemotherapy initiation. No improvement was seen after courses of cephalexin and doxycycline. Due to a lack of response to these two antibiotics, dermatology was consulted for further evaluation.
 On physical exam, there was a 1.3-cm red-violaceous crusted nodule on the palmar aspect of the left fifth digit (Figure 1, panel A). Dermoscopy showed central orange-tan pigmentation with loss of normal acral ridge pattern and surrounding irregular vessels (Figure 1, panel B). The differential diagnosis included Sweet syndrome, drug eruption, infection, and malignancy. A punch biopsy was performed and showed an atypical lymphocytic nodular infiltrate with angioinvasive and angiodestructive features, and immunohistochemical stains suggested AITL. The patient’s clinical stage was changed from stage II to stage IV AITL due to skin involvement, and the patient was started on CHOP treatment.
 AITL is a peripheral CD4+ follicular T-helper cell-based lymphoma that presents with B symptoms and generally has a poor prognosis after conventional chemotherapies [1]. Though rare, AITL has been reported to present with skin manifestations in up to 50% of cases, which typically present as macular or papular eruptions on the trunk, similar to toxic erythema of chemotherapy, drug eruptions, or viral exanthems [2]. However, there are multiple case reports of AITL mimicking a variety of systemic dermatologic or oncologic entities, including dermatomyositis, systemic lupus erythematosus, vasculitis, and urticaria [3].
 This case of AITL with cutaneous involvement illustrates an uncommon presentation of this peripheral lymphoma that often has skin manifestations. The presence of skin involvement implies an advanced clinical stage, which confers a poorer prognosis [4]. Due to its variable presentations in the skin, it is often misdiagnosed as a vasculitis or exanthem, particularly when its initial presentation is in the skin without prior diagnosis of internal AITL malignancy [3]. As in this case, most patients are treated for their condition with chemotherapy, starting with anthracycline-based chemotherapy, and often require higher-dose or modified therapy due to the short response duration [4]. Here, we highlight the diagnostic challenge of AITL and the clinical importance of its identification for appropriate staging and treatment of this condition.

Angioimmunoblastic T-cell lymphoma with extensive follicular dendritic cell and fibroblastic reticular cell network proliferation mimicking follicular dendritic cell sarcoma: A case report with pathologic, immunophenotypic, and molecular findings

Angioimmunoblastic T-cell lymphoma (AITL) is a common type of nodal peripheral T-cell lymphoma, which always presents with extensive follicular dendritic cell (FDC) meshwork. Here, we report a case of AITL combined with extensive spindle cell meshwork. Spindle cells occupied were positive for the FDC markers CD21, CD23, and CD35. Furthermore, some cells were positive for desmin and smooth muscle actin (SMA), suggesting the differentiation of fibroblastic reticular cell (FRC). Interestingly, the proliferation of spindle cells was so extensive that was easily misdiagnosed as FDC sarcoma (FDCS). Next-generation sequencing showed that the common mutations reported in AITL, including RHOA, TET2, and IDH2, were also detected in this case, while the genes that are recurrently mutated in FDCS were not detected. Regrettably, the patient died 19 months later. Overall, we highlight the unusual morphologic features in an AITL patient with extensive FDC and FRC network that may be misdiagnosed as FDCS, and careful morphological observation and immunochemical and molecular examinations are crucial for an accurate diagnosis.

A rare diagnostic case of angioimmunoblastic T-cell lymphoma

Angioimmunoblastic T-cell lymphoma is a malignancy of mature T-cells. A 45 years old Sudanese female with generalized lymphadenomegalyreferred from Tayba hospital to Al-tayseer reference medical cent in histopathology and cytology laboratory department (Sudan) for her lymph node histopathology analysis. A surgical lymph node biopsy for histopathology analysis stained by H&E was performed and sections showed effacement of normal architecture of lymph node with disappearance of follicles and germinal center composed of polymorphic infiltrate of small tumour cells and large tumour cells. The small tumour cells showed irregular nuclei and clear cytoplasm. In this case report, we state that the delay of diagnosis was mainly a consequence of an insufficient clinical history, which led to an incomplete histological analysis, delay of reporting and need for second opinions for interpretation. We also strongly recommend that to obtain an accurate and precise diagnosis for AITL, PAS stain should be performed firstly to recognize the capillary invasion which facilitate the early diagnosis.

A difficult case of angioimmunoblastic T-cell lymphoma with Epstein-Barr virus-negative large mononuclear atypical cells.

A difficult case of angioimmunoblastic T-cell lymphoma with Epstein-Barr virus-negative large mononuclear atypical cells.

Skin manifestation of angioimmunoblastic T-cell lymphoma mimicking Hansen’s disease on histology

<p>Angioimmunoblastic T-cell lymphoma (AITL) is an uncommon form of peripheral T-cell lymphoma now put under the category of nodal T-cell lymphoma with follicular T helper phenotype. 50% cases of AITL have varied cutaneous manifestations including macules, papules, petechiae, purpura, nodules, non-specific rash and urticaria. Herein we present a case of AITL initially presented as maculopapular rash which on histology was mimicking as Hansen’s disease with perineural and perivascular inflammation; however, an infective organism could not be demonstrated. Later on, a delayed diagnosis was made on lymph node biopsy and immunohistochemistry and patient responded well with chemotherapy.</p>

1505 Relapsed Angioimmunoblastic T-Cell Lymphoma Presenting as Severe Colitis With Bowel Perforation

INTRODUCTION: Angioimmunoblastic T-cell lymphoma (AITL) is a subtype of the uncommon peripheral T-cell lymphomas that usually presents as acute onset of systemic B symptoms and generalized lymphadenopathy. Here, we report a case of a woman with relapsed AITL involving the descending, sigmoid colon, and rectum, presenting as persistent diarrhea and bowel perforation. CASE DESCRIPTION/METHODS: A 78-year-old woman who was diagnosed with AITL in 2002 and had been stable on continuous therapy of romidepsin for the past 2.5 years for relapsed disease, presented with a 6-week history of diarrhea, fatigue, and decreased appetite. The diarrhea was watery, non-bloody, occurred 4-5 times a day, with fecal urgency and incontinence. The patient denied any abdominal pain, nausea, vomiting, or fever. There was no recent change in her medications, and no recent history of travel or contact with children or pets. During the 6-week period, she was hospitalized twice and was initially thought to have infectious colitis, for which she was given ciprofloxacin, metronidazole, and re-hydration therapy. Abdominal CT showed severe left-sided colitis with multiple enlarged perirectal and mesenteric lymph nodes. Upon her third admission, infectious etiologies were ruled-out and sigmoidoscopy showed markedly edematous and erythematous rectum. Biopsies from the rectum and sigmoid colon showed dense atypical T-cell infiltration consistent with involvement of her previous AITL. Her hospital course was further complicated by bowel perforation and polymicrobial positive blood cultures. She refused surgery and was referred to home hospice for palliative care. DISCUSSION: AITL is an aggressive peripheral T-cell lymphoma that is believed to arise from CD4 positive follicular helper T-cells. It accounts for 1-2% of non-Hodgkin lymphoma cases and has a higher incidence among Asian/Pacific Islanders and Hispanic Whites in the United States. Initial presentation commonly includes generalized lymphadenopathy, fever, unintentional weight loss, and night sweats. With progression, it can commonly involve the bone marrow, spleen, skin and soft tissues. However, gastrointestinal involvement is rare. In 1996, Goenka et al. reported AITL presenting as colonic polyps. We are reporting a case of AITL progressing to involve the descending, sigmoid colon, and rectum, whose presentation was initially mistaken for infectious colitis. However, due to high clinical suspicion, sigmoidoscopy was performed and biopsies confirmed the diagnosis of AITL of the colon.

A Case of Angioimmunoblastic T-cell Lymphoma Involving the Ipsilateral Parotid and Lateral Neck

A Case of Angioimmunoblastic T-cell Lymphoma Involving the Ipsilateral Parotid and Lateral Neck

Monoclonal plasma cell infiltrates in the setting of cutaneous follicular helper T cell lymphoproliferative disorders

There is a growing recognition that some primary cutaneous T cell lymphomas of the skin exhibit a follicular helper T cell phenotype best exemplified by primary cutaneous CD4+ small/medium sized pleomorphic T cell lymphoma. The follicular helper T cells is an evolutionary function in a common TH1 cell under the influence of other cell types most notably monocyte derived dendritic cells but also plasma cells. In addition, the skin defines a characteristic organ site of involvement for angioimmunoblastic T-cell lymphoma (AITL); the first recognized form of follicular helper T cell lymphoma.One of the hallmarks of the follicular helper T cell lymphomas a significant degree of post germinal center B cell hyperplasia. We encountered 7 cases of primary cutaneous follicular helper T cell and four cases of AITL, in which the biopsies contained a light chain restricted plasma cell infiltrate in the skin. There were no features that suggested an atypical or more aggressive clinical course in association with the identification of this light chain restricted plasmacytic infiltrates except one case of AITL in whom a diffuse large cell B cell lymphoma subsequently developed.There was no association with Epstein–Barr virus (EBV) infection light chain restricted plasma cell infiltrate in any of the eleven cases. The basis of these infiltrates is likely a reciprocal functional one reflecting the role of follicular helper T cells in the induction of B cell hyperplasia and the role of plasma cells as a countercheck balance controlling the extent of follicular helper T cell hyperplasia. B cell clonality, plasma cell atypia and blastic B cell transformation can occur without implying a malignant transformation.

A case of angioimmunoblastic T-cell lymphoma diagnosed by submandibular lymphadenopathy

A case of angioimmunoblastic T-cell lymphoma diagnosed by submandibular lymphadenopathy

A case of angioimmunoblastic T-cell lymphoma with diffuse gastric involvement

A case of angioimmunoblastic T-cell lymphoma with diffuse gastric involvement

REACTIVE PLASMACYTOSIS AT THE ONSET OF ANGIOIMMUNOBLASTIC T-CELL LYMPHOMA. CASE REPORT

Angioimmunoblastic T-cell lymphoma is a rare T-cell lymphoproliferative disease with generalized lymphadenopathy, hepatosplenomegaly, intoxication and polyclonal hypergammaglobulinemia. The persistence of plasma cells in peripheral blood can be a manifestation of both tumor and reactive processes. In our article, we described the case of angioimmunoblastic T-cell lymphoma with an increase in the number of peripheral blood plasma cells to 28 %, and in bone marrow to 9 %. The complex diagnostics, including plasma cells immunophenotyping, morphology of the lymph node biopsy and bone marrow samples, made it possible to verify the diagnosis of angioimmunoblastic T-cell lymphoma with polyclonal plasmacytosis.

A Case of Angioimmunoblastic T-cell Lymphoma Initially Suspected to be Erythema Exsudativum Multiforme

A Case of Angioimmunoblastic T-cell Lymphoma Initially Suspected to be Erythema Exsudativum Multiforme

A case of angioimmunoblastic T-cell lymphoma exhibiting false high fibrin/fibrinogen degradation product and D-dimer levels

A case of angioimmunoblastic T-cell lymphoma exhibiting false high fibrin/fibrinogen degradation product and D-dimer levels

A Synchronous Presentation of AITL with Adenocarcinoma of Lung

Angioimmunoblastic T-cell lymphoma (AITL) is a frequent subtype of peripheral T – cell lymphoma (PTCL) characterized by generalized lymphadenopathy, hepatosplenomegaly, and frequent B - symptoms. Extranodal manifestations are quite common in this subtype of Non Hodgkins Lymphoma which is characterized by frequent skin involvement. However, the extranodal disease should be evaluated thoroughly and a tissue diagnosis must be obtained to confirm lymphomatous involvement when the presentation is not classical. Here we report a case of AITL who presented with lung mass suspected to be due to lymphomatous involvement, but turned out to be a synchronous presentation of an adenocarcinoma of the lung. The possible relation between the two conditions is also elucidated.

Successful treatment of angioimmunoblastic T-cell lymphoma with clarithromycin.

TO THE EDITOR: Macrolide antibiotics (Macs) have been proposed as potential antineoplastic and immunomodulatory agents in addition to their primary role as antibacterial agents. They have anti-lymphoproliferative effects and are clinically applicable for treating lymphoid malignancies. Several previous reports have indicated that B-cell malignancies can be treated successfully with Macs [1,2,3]. With regard to T-cell lineage malignancies, we have reported previously a case of mixed phenotype acute leukemia, T/myeloid, not otherwise specified, treated successfully using clarithromycin (CAM), a Mac, in combination with prednisolone (PSL) [4]. Here, we report the first case of angioimmunoblastic T-cell lymphoma (AITL) treated successfully with CAM in combination with PSL.

A difficult case of angioimmunoblastic T-cell lymphoma to diagnose.

Angioimmunoblastic T-cell lymphoma (AITL) is a malignancy of mature T-cells. It is characterized as a polymorphic lymphonodal lymphoid infiltrate accompanied by prominent proliferation of endothelial venules and follicular dendritic cells. AITL was first described in 1974 by Frizzera et al. as an angioimmunoblastic lymphadenopathy with dysproteinemia.1 A short time later, the name was changed to immunoblastic lymphadenopathy, and then to lymphogranulomatosis X in 1979.2 AITL comprises 15–20% of all peripheral T-cell lymphomas and 1–2% of all non-Hodgkin lymphomas (NHL). Most frequently, it occurs in aged patients, with equal prevalence between males and females. Typically, AITL displays an aggressive behavior, which makes the diagnosis difficult and it must be differentiated from other malignant lymphoproliferative diseases, drug reactions and viral infections. Patients with AITL frequently exhibit B-symptoms (e.g., fever and weight loss) and a generalized enlargement of the lymph nodes. Other

A case of CD10-negative angioimmunoblastic T cell lymphoma with leukemic change and increased plasma cells mimicking plasma cell leukemia: A case report.

Angioimmunoblastic T cell lymphoma (AITL) is a peripheral T cell lymphoma, known to express CD3 and CD4, and, frequently, also CD10 and c-Maf-1. Hypergammaglobulinemia is not particularly rare in patients with AITL. However, AITL in conjunction with plasmacytosis in the peripheral blood is rare. The current report presents a case of CD10-negative AITL demonstrating leukemic change and plasmacytosis in the peripheral blood mimicking plasma cell leukemia. A 78-year-old male was admitted to hospital due to systemic lymph node enlargement, high serum IgG and IgA, and increased counts of plasmacytoid cells and lymphoid cells with atypical nuclei in the peripheral blood. Initially, plasma cell leukemia was suspected, due to the extreme increase in the number of plasma cells in the peripheral blood. However, the plasma cells did not show clonal expansion on examination by flow cytometry. Based on histological analyses, following a biopsy of an enlarged lymph node, the patient was diagnosed with AITL. This case suggests that when hypergammaglobulinemia and increases in B-lineage cells are observed, AITL should be considered in addition to disorders of B-lineage cells.

Angioimmunoblastic T-cell lymphoma: Case report and review of the literature

Objective To raise awareness of angioimmunoblastic T-cell lymphoma(AITL) by one case of AITL and review of literature reports.Methods The clinic and follow-up data of one patient with AITL were analyzed,and clinical features,diagnosis and treatment of AITL were discussed combining with literature.Results AITL is derived from centro-follicular T-helper cells.It is characterized by generalized lymphacenopathy,fever,skin rash,anemia and autoimmune features are frequently described.The diagnosis of AITL needs clinical syndrome and morphology.Despite treatment with polychemotherapy,the prognosis is poor.Conclusions Peripheral nodes pressing bronchia in AITL patients may induce cough.System physical examination helps to reduce misdiagnosis.