scholarly journals Delayed diagnosis of Angioimmunoblast T-cell lymphoma presenting with type II Cryoglobulinemia and acute kidney injury: a case report and narrative review of the literature

2020 ◽  
Vol 21 (1) ◽  
Author(s):  
Xiang-Yang Li ◽  
Hai-Yan He ◽  
Shu-Ling Yue ◽  
Pearl Pai
Keyword(s):  
Acute Kidney Injury ◽  
T Cell ◽  
Cell Lymphoma ◽  
Kidney Injury ◽  
T Cell Lymphoma ◽  
Type Ii ◽  
Clinical Scenarios ◽  
Initial Symptoms ◽  
Mesangial Area ◽  
History Of

Abstract Background Angioimmunoblastic T cell lymphoma (AITL) is an infrequent hematological malignancy with variable and often atypical presentations. The presence of dysproteinemia, autoantibodies and systemic involvement in AITL has often led to a delay in diagnosis or even misdiagnosis in practice. We herewith present a case of AITL that primarily presented with acute kidney injury associated with type II Cryoglobulinemia, the underlying cause was only identified 8 months after the emergence of initial symptoms. Case presentation A 67-year old woman presented with 2-month history of intermittent joint pain and a 3-day history of bilateral lower limb edema and acute kidney injury. Initial laboratory investigations showed marked hypocomplementemia with positive autoantibodies of ANA, anti-cardiolipin-IgM and direct antiglobulin. The serum and urinary Immunofixation and serum cryoglobulin tests were negative, while the serum free κ to λ light chain ratio was 0.231. A renal biopsy showed a diffuse proliferative glomerulonephritis with intracapillary pseudothrombi formation. There were orderly arranged microtubular structures of 20–35 nm in diameter in the subendothelial and mesangial area on electron microscopy. Shortly afterwards, the patient developed tingling affecting her finger tips and weak hands and legs. A diagnosis of cryoglobulinemia complicated with cryoglobulinemic glomerulonephritis and polyneuropathy was made. She responded well to methylprednisolone, plasma exchange and rituximab. However, 3 months later, she presented with generalized pruritic rash, weight loss, and inguinal lymphadenopathy. A subsequent inguinal excisional lymph node biopsy at month 8 revealed AITL as the underlying disease. Conclusions AITL and its associated B cell dysregulation can give rise to autoimmunity and cryoglobulinemia which may conceal itself as the underlying disorder. In various clinical scenarios of auto-immune diseases, it is advisable that the clinicians should take into consideration the multi-faceted lymphoma.

Cytokine Removal under Hemodiafiltration with Endogenous Reinfusion in Acute Kidney Injury Secondary to Angioimmunoblastic T-Cell Lymphoma: A Case Report

2012 ◽  
Vol 34 (3-4) ◽  
pp. 349-353 ◽  
Author(s):  
Emiliano Staffolani ◽  
Rebeca Nicolais ◽  
Simone Manca di Villahermosa ◽  
Diego Galli ◽  
Massimiliano Postorino ◽  
...  
Keyword(s):  
Acute Kidney Injury ◽  
Case Report ◽  
T Cell ◽  
Cell Lymphoma ◽  
Kidney Injury ◽  
T Cell Lymphoma ◽  
Angioimmunoblastic T Cell Lymphoma

Serendipity in Refractory Celiac Disease: Full Recovery of Duodenal Villi and Clinical Symptoms after Fecal Microbiota Transfer

2016 ◽  
Vol 25 (3) ◽  
pp. 385-388 ◽  
Author(s):  
Yvette H. Van Beurden ◽  
Tom Van Gils ◽  
Nienke A. Van Gils ◽  
Zain Kassam ◽  
Chris J.J. Mulder ◽  
...  
Keyword(s):  
Celiac Disease ◽  
T Cell ◽  
Clostridium Difficile ◽  
Clostridium Difficile Infection ◽  
Cell Lymphoma ◽  
Fecal Microbiota ◽  
Disease Type ◽  
T Cell Lymphoma ◽  
Type Ii ◽  
Refractory Celiac Disease

Treatment of refractory celiac disease type II (RCD II) and preventing the development of an enteropathy associated T-cell lymphoma in these patients is still difficult. In this case report, we describe a patient with RCD II who received fecal microbiota transfer as treatment for a recurrent Clostridium difficile infection, and remarkably showed a full recovery of duodenal villi and disappearance of celiac symptoms. This case suggests that altering the gut microbiota may hold promise in improving the clinical and histological consequences of celiac disease and/or RCD II. Abbreviations: CDI: Clostridium difficile infection; EATL : enteropathy associated T-cell lymphoma; FMT: fecal microbiota transfer; IEL: intraepithelial lymphocytes; RCD II: refractory celiac disease type II; TPN: total parenteral nutrition.

scholarly journals Enteropathy associated 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 ◽  
...  
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
Previous History ◽  
Lymph Node Biopsy ◽  
Intestinal Wall ◽  
T Cell Lymphoma ◽  
The Third ◽  
History Of ◽  
Small Intestinal ◽  
Mesenterial Lymph Node

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.

A Case of Type II Enteropathy-associated T-cell Lymphoma Causing Perforation of the Small Intestine

2014 ◽  
Vol 75 (8) ◽  
pp. 2229-2233
Author(s):  
Chie SAKIMURA ◽  
Masahiro KOMOTO ◽  
Yukie GO ◽  
Eiji AKO ◽  
Shigeki FUJITA ◽  
...  
Keyword(s):  
Small Intestine ◽  
T Cell ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Type Ii

scholarly journals Type II enteropathy-associated T cell lymphoma in the duodenum

Medicine ◽  
2020 ◽  
Vol 99 (23) ◽  
pp. e20050 ◽  
Author(s):  
Zhicheng Liu ◽  
Liang He ◽  
Yan Jiao ◽  
Helei Wang ◽  
Jian Suo
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Type Ii

Sustained, Durable Responses with Alemtuzumab in Refractory Angioimmunoblastic T-Cell Lymphoma.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 2730-2730
Author(s):  
Jennifer E. Amengual ◽  
Bruce G. Raphael
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
Receptor Gene ◽  
Response Duration ◽  
T Cell Lymphoma ◽  
Surface Glycoprotein ◽  
Sustained Remission ◽  
Cell Surface Glycoprotein ◽  
Angioimmunoblastic T Cell Lymphoma ◽  
History Of

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.

scholarly journals Primary Cutaneous CD30-Positive Large T-Cell Lymphoma in an 80-Year-Old Man: A Case Report

2011 ◽  
Vol 2011 ◽  
pp. 1-3 ◽  
Author(s):  
Rehan Hussain ◽  
Amir Bajoghli
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
Large Cell ◽  
T Cell Lymphoma ◽  
Cutaneous T Cell Lymphoma ◽  
Left Forearm ◽  
Favorable Prognosis ◽  
History Of ◽  
Indolent Disease ◽  
Rule Out

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.

Sign in / Sign up

Export Citation Format

Share Document