scholarly journals Epitheliotropic T-cell lymphoma in 2 half-sibling bontebok

2020 ◽  
pp. 104063872098411
Author(s):  
Sierra M. Imanse ◽  
Colleen F. Monahan ◽  
Kimberly A. Thompson ◽  
Judilee C. Marrow ◽  
Sarah M. Corner

We diagnosed epitheliotropic T-cell lymphoma of the forestomachs in 2 aged, half-sibling, zoo-managed bontebok ( Damaliscus pygargus pygargus). One bontebok also had mesenteric lymph node and cutaneous involvement. Both animals had a history of chronic abdominal distension and diminished body condition that resulted in euthanasia. At autopsy, both animals had marked ruminal distension with diffusely blunted ruminal papillae and reticular crests. In case 1, there was an increased amount and particle length of the ruminoreticular fibrous material with scant fluid, and a 2-cm diameter focus of cutaneous crusting adjacent to a mammary teat. In case 2, the rumen and reticulum were fluid-distended with decreased fibrous material. Histologically in case 1, the rumen, reticulum, omasum, and skin had intraepithelial nests and sheets of neoplastic small lymphocytes; in case 2, the rumen and reticulum had a similar neoplastic cell population. Immunohistochemically, neoplastic lymphocytes were immunoreactive for CD3 and negative for CD20, confirming the diagnosis of epitheliotropic T-cell lymphoma.

2007 ◽  
Vol 135 (1-2) ◽  
pp. 80-84
Author(s):  
Milena Bakrac ◽  
Branka Bonaci-Nikolic ◽  
Natasa Colovic ◽  
Sanja Simic-Ogrizovic ◽  
Miodrag Krstic ◽  
...  

Enteropathy associated T-cell lymphoma (EATCL) is a high grade, pleomorphic peripheral T-cell lymphoma with usually cytotoxic phenotype. This is a case report of three patients with EATCL. The first patient was 50 year-old woman with four year history of gluten sensitive enteropathy (GSE). Diagnosis of lymphoma was confirmed after the resection of the jejunum (small intestine obstruction). Pathohistological (PAS, Reticulin, Giemsa) and immunohistochemical (anti-LCA, anti-CD20, anti- CD45RO, anti-CD3) methods revealed the diagnosis of EATCL: CD45RO+, CD3+. After the third cycle of chemotherapy, the disease progressed with massive lung infiltration. Patient died due to complications of bone marrow aplasia. The second patient was 23 year-old woman with long earlier history of GSE. She presented with the acute renal failure. According to established diagnosis of tubulointerstitial nephritis, she was treated with pulse doses of steroid therapy. After temporary improvement, she had dissemination of the disease. On MRI, small intestinal wall was thickened, and abdominal lymph nodes were enlarged with extraluminal compression of common bile duct. Laparotomy with mesenterial lymph node biopsy and consecutive pathohistological and immunohistochemical analyses revealed the diagnosis of EATCL. The patient received chemotherapy, but she died with signs of pulmonary embolization. The third patient was 53 year-old woman without previous history of GSE. Diagnosis of EATCL was revealed after the resection of jejunum because of small intestinal obstruction. She received two cycles of chemotherapy, but she died with signs of disease progression. IgA antiendomysial antibodies were detected in the serum of all patients. The overall survival of patients was 7 months. The possibility of lymphoma rising in patients with clinical progression of GSE despite gluten free diet must be kept in mind.


2011 ◽  
Vol 144 (4) ◽  
pp. 312-316 ◽  
Author(s):  
M. Suzuki ◽  
Y. Kanae ◽  
Y. Kagawa ◽  
N. Ano ◽  
K. Nomura ◽  
...  

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 2730-2730
Author(s):  
Jennifer E. Amengual ◽  
Bruce G. Raphael

Abstract 2730 Poster Board II-706 Angioimmunoblastic T-cell lymphoma (AITL) is a rare subtype representing 2% of Non-Hodgkin's Lymphoma characterized by lymphadenopathy, hepatosplenomegaly, anemia, hypergammaglobulinemia and immune dysfunction. Prognosis is poor with a median survival of less than 36 months. There is no standard treatment for AITL. Most patients initially respond to treatment, but relapse within short time intervals. Alemtuzumab is a humanized monoclonal antibody that binds to CD52 antigen, a cell surface glycoprotein with high expression on T-cells. We report three patients with refractory AITL, with confirmed T-cell receptor gene rearrangements, who achieved sustained, durable responses with alemtuzumab. The table below lists the treatment regimens, duration of remissions and complications for all 3 cases. Infectious and autoimmune complications were effectively treated in all.PatientPrevious Treatment (Response duration, months)Alemtuzumab (Response duration)InfectionsAutoimmune manifestationsACHOP (10) Cytoxan-P (1) Gemzar-P (1)24 monthsCMVBCHOPE (10) ICE (0) Gemcitabine-P followed by Cyclosporine maintenance (1)>24 monthsAspergillusAgranulocytosis Autoimmune hemolytic anemiaCCHOP (1)>14 monthsCMV Legionella Patient A was a 73 year-old female who presented with lymphadenopathy and biopsy proven AITL. Her longest remission was 10 months following CHOP. She was started on alemtuzumab 30 mg 3 times per week for 4 weeks in June 2007 after relapsing. Her only complication from treatment was CMV infection. She remained in remission until June 2009 when she relapsed in her liver and colon. She was treated with alemtuzumab and prednisone for 2 weeks, but developed neutropenic fever, CMV and died July 2009. Patient B is a 73 year-old male with a history of ITP who presented in July 2005 with fevers, lymphadenopathy and anemia, and biopsy proven AITL. His longest remission was 10 months with CHOPE. In June 2007, the patient was treated with alemtuzumab for 7 weeks after relapsing. Treatment complications included Aspergillus pneumonia, agranulocytosis and autoimmune hemolytic anemia. He achieved a complete response as evidenced by PET/CT scan. He remains in remission 2 years later. Patient C is a 62 year-old woman with a history of MGUS who presented in 2007 with rapidly growing lymphadenopathy and a biopsy that revealed AITL. She never achieved a sustained remission with chemotherapy. June 2008, the patient was treated with alemtuzumab for 6 weeks, complicated by CMV and Legionella pneumonia. She remains in remission now over 14 months. Here we have shown remarkable success with short courses of alemtuzumab. Three patients remained disease free for an average of 21 months; two remissions are on-going. This report demonstrates sustained responses for patients with AITL, suggesting that alemtuzumab is a valid and rational treatment choice in heavily pretreated patients. We propose using anti-CD52 therapy as consolidation after primary response to conventional chemotherapy in patients with AITL. Disclosures: Off Label Use: Alemtuzumab is not licensed for use in Angioimmunoblastic T-cell Lymphoma.


2011 ◽  
Vol 2011 ◽  
pp. 1-3 ◽  
Author(s):  
Rehan Hussain ◽  
Amir Bajoghli

Primary cutaneous CD30-positive large cell lymphoma (CD30+ PCLCL) is a rare subtype of cutaneous T-cell lymphoma (CTCL) that can present in a variety of ways. We report a patient with a three-month history of an enlarging, exophytic mass with two smaller satellite lesions on the left forearm. Biopsy of the skin stained positive for CD30, and, after thorough systemic evaluation, a diagnosis of CD30+ PCLCL was made. When PCLCL is suspected, it is important to perform immunohistological studies for CD30 types and conduct a thorough workup to rule out systemic LCL. These measures will reduce the use of unnecessarily aggressive chemotherapy regimens for CD30+ PCLCL, an indolent disease with a favorable prognosis.


2010 ◽  
Vol 2010 ◽  
pp. 1-5 ◽  
Author(s):  
Anna Vasku ◽  
Julie Bienertova Vasku ◽  
Miroslav Nečas ◽  
Vladimir Vasku

The aim of the study was to investigate the DNA polymorphic genotype in MMP-2 promoter gene as a potential candidate region for the development of the cutaneous T-cell lymphoma (CTCL) and/or its progression. A total of 89 Czech patients with CTCL (including 23 patients with large plaque parapsoriasis) were compared to 198 controls of similar age and sex distribution, without personal or family history of chronic skin diseases and without personal history of malignancy. The three selected polymorphisms in the promoter of MMP-2 gene (−1575G/A,−1306C/T, and−790T/G) were determined using the PCR-based methodology with RFLP. In our cohort, the associatedGGCCTTMMP-2 promoter genotype was highly significantly more frequent in CTCL-Ia stage patients compared to patients with parapsoriasis, the tests having high sensitivity and specificity (78%, 83%, resp.). To conclude, use of associated MMP-2 promoter genotype as a DNA marker might make it possible to distinguish between the patients with parapsoriasis and those with CTCL stage Ia, which could substantially improve possibilities of clinical diagnostics, therapy design, and prognosis of this serious condition in the early stages.


2019 ◽  
Vol 3 (4) ◽  
pp. 588-595 ◽  
Author(s):  
Chantana Polprasert ◽  
Yasuhide Takeuchi ◽  
Nobuyuki Kakiuchi ◽  
Kenichi Yoshida ◽  
Thamathorn Assanasen ◽  
...  

Abstract Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare subtype of peripheral T-cell lymphoma affecting younger patients and associated with hemophagocytic lymphohistiocytosis. To clarify the molecular pathogenesis of SPTCL, we analyzed paired tumor and germline DNAs from 13 patients by whole-exome sequencing. All cases were Asians and were phenotypically sporadic with no family history of SPTCL. Consistent with a recent report, germline mutations in HAVCR2, encoding T-cell immunoglobulin mucin 3 (TIM3), were identified in 11 of 13 (85%) cases. All mutated cases were primary SPTCL, whereas the 2 cases without mutation were secondary SPTCL associated with underlying diseases, including viral infection and autoimmune disease. Ten patients harbored homozygous p.Y82C mutations, and 1 showed compound heterozygous mutations (p.Y82C and p.T101I). Both missense mutations altered highly conserved residues located in the extracellular immunoglobulin variable–like domain. According to the Genome Aggregation Database of >138 500 general individuals, both mutations were documented with minor allele frequencies < 0.007, indicating remarkable enrichment of these HAVCR2 alleles in SPTCL. SPTCL cells also harbored somatic mutations (6.2 per patient) that are frequently identified in genes associated with epigenetic regulation and signal transduction. In conclusion, individuals harboring biallelic HAVCR2 (TIM3) germline mutations were highly susceptible to sporadic SPTCL, which was also associated with clonal somatic mutations.


2017 ◽  
Vol 05 (01) ◽  
pp. e68-e70 ◽  
Author(s):  
Kaja Gizewska-Kacprzak ◽  
Katarzyna Karpinska-Kaczmarczyk ◽  
Tomasz Ociepa

AbstractDuring infancy, skin inflammation is usually treated in basic pediatric care. In this study, we present a case of an 8-month-old girl with a 2-month history of an inflammation of the thigh treated locally by ointments and oral antibiotics in basic and dermatological care. The patient had a history of fever, sweating, and failure to thrive. The lactate dehydrogenase was elevated up to 869 U/L with low C-reactive protein (1.04 mg/L). Magnetic resonance imaging of the thigh reassured the diagnosis of local inflammation. Intravenous antibiotic caused mild local improvement, but the episodes of high fever sustained. The patient was transferred to our pediatric surgery department for treatment and surgical biopsy of the lesion. Histopathological examination confirmed a subcutaneous panniculitis-like T-cell lymphoma, which is a rare cytotoxic T-cell lymphoma representing less than 1% of non-Hodgkin lymphomas, uncommon in children. The patient was introduced to a chemotherapy protocol EURO-LB 2002 with good response. In a skin lesion that is associated with systemic symptoms and responding untypically to antibiotic treatment malignancy should be considered and biopsy not be postponed.


2007 ◽  
Vol 48 (9) ◽  
pp. 1780-1784 ◽  
Author(s):  
Faek R. Jamali ◽  
Zaher K. Otrock ◽  
Assaad M. Soweid ◽  
Ghassan N. Al-Awar ◽  
Rami A. Mahfouz ◽  
...  

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