Angioimmunoblastic T-Cell Lymphoma With Cutaneous Involvement: A Case Report With Subtle Histologic Changes and Clonal T-Cell Proliferation

2004 ◽  
Vol 128 (10) ◽  
pp. e122-e124
Author(s):  
Chien-Tai Huang ◽  
Shih-Sung Chuang

Abstract Angioimmunoblastic T-cell lymphoma is a nodal peripheral T-cell lymphoma that rarely involves the skin. We describe a 62-year-old Taiwanese man who developed a second relapse of angioimmunoblastic T-cell lymphoma with generalized erythroderma and numerous plaquelike and nodular lesions. Biopsy of the erythematous skin lesion demonstrated mild infiltrate of atypical small lymphocytes, some with clear cytoplasm. The lymphoid infiltrate was located mainly around skin appendages and in the upper dermis without epidermotropism. Immunohistochemically, these atypical lymphocytes expressed CD3. Polymerase chain reaction analysis for T-cell receptor γ-chain gene rearrangement using paraffin section showed the same-sized monoclonal bands in the skin and 2 previous nodal biopsies. We conclude that the histologic features of angioimmunoblastic T-cell lymphoma involving skin may be very subtle, showing only mild lymphoid infiltrate. Awareness of the history of angioimmunoblastic T-cell lymphoma with ancillary studies, including clonality testing for T-cell receptor gene rearrangement, is crucial for reaching an accurate diagnosis.

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 5111-5111
Author(s):  
Rong Liang ◽  
Xie qun Chen ◽  
Zhe Wang ◽  
Bai qin Xian ◽  
qin-Guo guo Yan ◽  
...  

Abstract Objectives To improve the understanding of extranodal natural killer(NK)/ T cell lymphoma (ENKTCL) with poor prognosis and provide experiential references for individual therapy via a retrospective analysis of the clinical and pathological features. Methods 120 NKTCL cases from April 2007 to Oct 2012 in single center of Northwestern China were retrospectively analyzed on their pathologic diagnosis and clinical manifestations. Pathological examinations were mainly depended on morphology, immunohistochemisty for immunophenotype and In situ hybridizationc for epstein - barr virus (EBV) small encoded RNA£¨EBER£©. Polymerase chain reaction (PCR) for the amount of EBV DNA in whole-blood and T-cell receptor (TCR) gene rearrangement were performed. Chemotherapy and or radiotherapy were the main treatments. Complete remission(CR), 2 year(2y) overall survival (OS) and progressive free survival (PFS) according to clinical characteristics were analyzed. Results The median age of 120 NKTCL cases was 43.19+13.7 years old. 98 primary nasal ENKTCL cases accounted for 81.4%, whose 2y OS and PFS were 88.6% and 69.1% respectively. 22 non-nasal ENKTCL accounted for 18.3%£€whose 2y OS and PFS were 58.8% and 45.5% respectively, which was statistically significant comparing with primary nasal ENKTCL cases. It was found that patients with primary intestinal or Ki67 greater than 80% were dead in first year. Patients with primary liver and intestinal had higher Ki67 than patients with primary nasal. Rate of 2y OS of 43 patients with Ki67 from 60% to 80% was 60% and PFS was 36% as compared to 86.3% of rate of OS and 57.5% for PFS of 30 patients with Ki67 from 30% to 50% and 100% of rate of OS and 78% for PFS of 7 patients with Ki67 less than 30%. Basing on analysis showed that from the cell of origin, it was found that primary site was nasal and 2y OS 100%¡¢PFS 75%£€in 8 cases with CD56+,CD3+ and T-cell receptor (TCR) gene rearrangement, which was statistically significant comparing with CD56- nasal ENKTCL cases. EBER was positive in every case. Among the 13 EBV –DNA samples detected, there were 5 samples with more than 6.1X107 copies/ml with OS 60%. 2y OS and PFS of Patients with normal ferritin ¦Â-microglobulin were longer than that of higher ferritin and ¦Â-microglobuli( P). 2y OS and PFS of 10 cases with I stage who had only radiotherapy was 100% respectively. 47 cases with II-IV stage received only chemotherapy. Their 2y OS and PFS was 100% and 90% respectively for patients with II stage better than that of III and IV stage. 63 cases with treatment of chemotherapy and radiotherapy. Among them, 2y OS and PFS of local nasal and invasion nasal were different. International prognostic index, IPI was good for prognosis for OSºÍPFS. Conclusion£º It was implied that Ki67, CD56, EBER, EBV-DNA and primary site was related with the prognosis of NKTCL. It needs further and more clinical observation and verification to judge if they could be additional markers for prognostication and clinical stratification to be incorporated in clinical individual management algorithms. The chemotherapies containing asparaginase or polyehylene glycol asparaginase are more effective. Autologous stem cell transplantation could make patients long live and pretransplant CR status was a major prognostic factors. Disclosures: No relevant conflicts of interest to declare.


Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 4343-4343
Author(s):  
Ashok Malani ◽  
Robert Weigand ◽  
Vicram Gupta ◽  
Lawrence Hertzberg ◽  
Gautam Rangineni

Abstract Immunophenotyping by flow cytometry has revolutinized the diagnosis of blood cell disorders such as leukemias and lymphomas and is now commonly used in diagnosis and prognosis of such patients. We describe a case of human ehrlichiosis mimicking T-cell lymphoma/leukemia based on flow cytometry of bone marrow cells and confirmed by T-cell receptor gene rearrangement (TCR) by polymerase chain reaction (PCR). Treatment with doxycycline reversed these findings. A 20-year-old, Amish female presented with fatigue, fever, chills, sweating, low back pain, and lower abdominal pain for 2 days. She admitted to multiple bites from ticks 2 weeks prior to presentation and also reported having numerous animals such as cats, dogs, cows, goats, horses at her farm where she lived. Clinical exam was significant for fever of 101.4 F, heart rate of 118/min, BP of 80/60 mm Hg and a distended urinary bladder which was treated by catheter drainage. Relevant laboratory tests are shown in table 1. Table 1 Hemoglobin 9.7 12–16 gm/dl WBC 0.8 4–10.8 k/mm3 Platelets 16 150–400 k/mm3 Segments 62% 50–75% Lymphocytes 15% 20–40% Sodium 140 125–135 mmol/L AST 126 0–37 IU/L ALT 71 0–65 IU/L Alk. Phos. 49 50–136 IU/L LDH 691 91–190 IU/L Chest radiograph, Ultrasound and Computed tomography scan of the abdomen were within normal limits. With a provisional diagnosis of septic shock and suspicion for Ehrlichiosis, therapy with intravenous(IV) fluids, vasopressors and doxycycline was initiated. Blood was cultured and a sample was forwarded to CDC for analysis of tick borne infections. In order to evaluate and exclude blood disorders like leukemia and lymphoma in a patient with fever and pancytopenia, a bone marrow aspiration and biopsy was performed. It showed cytologically abnormal-appearing, large sized lymphocyte population with irregular nuclear membranes. Flow cytometry of the bone marrow cells revealed 8–10% of phenotypically abnormal T-cells with abnormally weak intensity of membrane surface CD3, CD5, and CD7 expression and negativeCD4 and CD8 expression. These cells also expressed HLA-DR and CD38 at uncommonly bright intensity and there were no CD34 benign immature B-cells. Cytogenetics however was normal. Interestingly, PCR analysis was positive for clonal TCR gamma gene rearrangement. These results were reported as consistent with involvement of marrow by a peripheral T-cell lymphoma/leukemia T-Cell receptor PCR analysis T-Cell receptor PCR analysis Since the patient was steadily improving with IV Doxycycline, we decided to wait and repeated the bone marrow aspiration a week later. This time the bone marrow exam was found to be normal morphologically, on flow cytometry and TCR gamma gene rearrangement by PCR. Patient was discharged on oral doxycycline after a stay of 13 days in the hospital. The blood test for ehrlichiosis from CDC was reported 3 weeks later as positive for Ehrlichia chaffeensis by PCR. Patient is doing well 6 months after the illness. This case illustrates that Ehrlichiosis can transiently cause T cell abnormalities resulting in false positive analysis on flow cytometry and TCR gamma gene rearrangement, thereby leading to false positive diagnosis of Ehrlichiosis. Reconfirmation with repeat studies need to be done before considering active treatment for lymphoma/leukemia.


Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 4396-4396
Author(s):  
Tanawan Kummalue ◽  
Teerawat Saesue ◽  
Tawatchai Pongpruttipan ◽  
Sanya Sukpanichnant ◽  
Nisarat Opartkiattikul

Abstract Thirteen Thai patients, seven with pathologic diagnosis of T-cell lymphoma, four with clinical and/or pathological suspicion of T-cell lymphoma, one with lymphadenitis and one with chronic hypertrophic tonsillitis, were investigated for T-cell receptor gene rearrangement (TCR-GR) by polymerase chain reaction (PCR). Clonal TCR-GR was demonstrated in each T-cell lymphoma whereas polyclonal TCR-GR was found in the rest of the patients, including the four cases that were suspected of T-cell lymphoma. Aim: To identify T-cell receptor gene rearrangement in malignant lymphoma in Thai patients. Material and method: Thirteen specimens of formalin-fixed, paraffin-embedded tissue were collected from Department of Pathology, Faculty of Medicine Siriraj Hospital, Mahidol University. After conventional morphological evaluation and immunohistochemical studies, DNA in each specimen was extracted from 10μm thick paraffin sections using QIAamp DNA mini kit (QIAGEN) for TCR-GR analysis by multiplex PCR. Two pairs of primers were selected for Vγ11 (VγI), Vγ101(VγIII/IV), Jγ12(Jγ1/2) and Jp12(JPγ1/2). DNA of the four cases suspected of malignant lymphoma were repeated by using IdentiClone™ TCRG Gene Clonality Assay kit (InVivoScribe Technologies). Results: According to the WHO classification (2001), the seven cases of T-cell lymphoma were angioimmunoblastic type (3 cases) and unspecified type (4 cases). All cases showed monoclonal TCR-GR. Only one of the four cases suspected of T-cell lymphoma was doubtful for nasal NK/T-cell lymphoma while the others were suspected clinically. All of these four cases and the two reactive conditions revealed polyclonal TCR-GR. After using IdentiClone™ kit twice, the four cases suspected of T-cell lymphoma still showed polyclonal TCR-GR. Conclusion: The molecular analysis of TCR-GR performed in thirteen cases demonstrated the monoclonal TCR-GR in all seven cases of T-cell lymphoma and polyclonal TCR-GR in the four cases suspected of T-cell lymphoma and the two cases of reactive condition. Therefore, this technique is very useful to confirm the diagnosis of T-cell lymphoma and to provide information for further management in patients suspected of malignant lymphoma.


2014 ◽  
Vol 55 (9) ◽  
pp. 2161-2164 ◽  
Author(s):  
Tomoko Miyata-Takata ◽  
Katsuyoshi Takata ◽  
Sachiko Yamanouchi ◽  
Yasuharu Sato ◽  
Mai Harada ◽  
...  

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