scholarly journals The Development of Primary Effusion Lymphoma-Like Lymphoma in a Patient with Preexisting Essential Thrombocythemia

2021 ◽  
Vol 2021 ◽  
pp. 1-6
Author(s):  
Yoshiki Uemura ◽  
Marina Komoto ◽  
Mitsuo Okada
Keyword(s):  
Computed Tomography ◽  
Pericardial Effusion ◽  
Essential Thrombocythemia ◽  
Normal Limit ◽  
Primary Effusion Lymphoma ◽  
Cell Lineage ◽  
Jak2 V617f ◽  
Lymphoid Cells ◽  
Japanese Male ◽  
Old Japanese

A 71-year-old Japanese male was diagnosed with essential thrombocythemia (ET) with the JAK2 V617F mutation variation, in April 2011. He was mainly treated with hydroxyurea following which the number of platelets was maintained within the normal limit. At age 80, he was hospitalized due to cardiac tamponade. Computed tomography showed no evidence of tumor masses or lymphadenopathy. Pericardial drainage was performed, and cytopathologic examination of the fluid revealed atypical lymphoid cells consistent with an effusion lymphoma of B cell lineage. The pericardial effusion was completely drained, and complete remission was achieved. Ultimately, the patient was diagnosed with primary effusion lymphoma-like lymphoma (PEL-LL). To the best of our knowledge, this is the first report of PEL-LL following ET.

scholarly journals HHV8-Negative Primary Effusion Lymphoma of B-Cell Lineage: Two Cases and a Comprehensive Review of the Literature

2013 ◽  
Vol 2013 ◽  
pp. 1-12 ◽  
Author(s):  
Neeraj Saini ◽  
Ephraim P. Hochberg ◽  
Erica A. Linden ◽  
Smita Jha ◽  
Heinz K. Grohs ◽  
...  
Keyword(s):  
B Cell ◽  
English Language ◽  
Herpes Virus ◽  
Primary Effusion Lymphoma ◽  
Cell Lineage ◽  
Kaposi Sarcoma ◽  
Body Cavity ◽  
Lymphoid Cells ◽  
Human Herpes Virus 8 ◽  
Herpès Virus

Primary effusion lymphoma (PEL) is a rare extranodal lymphoma that typically presents in a body cavity in the absence of a detectable tumor mass and that occurs predominantly in immunosuppressed individuals. The neoplastic lymphoid cells are frequently infected with human herpes virus 8 (HHV8), also known as Kaposi sarcoma herpes virus (KSHV). We describe two HIV-negative patients who presented with primary effusion lymphoma of B-cell lineage involving the pleural cavity, but whose tumor cells lacked infection by HHV8. We review the English language literature of HHV8-negative PEL of B-cell lineage and compare these lymphomas to HHV8-associated PEL with regard to clinical and pathological characteristics, therapy, and outcome.

scholarly journals Primary Effusion Lymphoma

2013 ◽  
Vol 137 (8) ◽  
pp. 1152-1154 ◽  
Author(s):  
Sanjay Patel ◽  
Philip Xiao
Keyword(s):  
Primary Effusion Lymphoma ◽  
Cell Lineage ◽  
Human Herpesvirus ◽  
Large Cell ◽  
Lymphoid Cells ◽  
Human Herpesvirus 8 ◽  
Treatment Modalities ◽  
Herpesvirus 8 ◽  
Non Hodgkin Lymphoma ◽  
Immunodeficiency Virus

Primary effusion lymphoma is a large cell non-Hodgkin lymphoma localized predominantly in body cavities and occasionally in extracavitary regions. It presents with characteristic lymphomatous effusions in the absence of solid tumor masses, and pleural, peritoneal, and pericardial spaces are most often involved. It is typically associated with human herpesvirus 8 infection in immunocompromised individuals, in the setting of human immunodeficiency virus infection, organ transplantation, or in rare cases advanced age. Histologically, primary effusion lymphoma is characterized by atypical lymphoid cells of B-cell lineage with large nuclei and prominent nucleoli. Demonstration of human herpesvirus 8 latent antigens is required for diagnosis, and treatment modalities are limited at this time. In this review, we aim to summarize clinicopathologic features of this rare and unique entity.

Computed tomography manifestations of renal hemangiopericytoma

2007 ◽  
Vol 48 (8) ◽  
pp. 934-937 ◽  
Author(s):  
K. Hayasaka ◽  
T. Nihashi ◽  
Y. Nojiri ◽  
K. Okamuara
Keyword(s):  
Computed Tomography ◽  
Volume Rendering ◽  
Preoperative Diagnosis ◽  
Abdominal Mass ◽  
Maximum Intensity Projection ◽  
Maximum Intensity ◽  
Radiological Findings ◽  
Tumor Vascularity ◽  
Japanese Male ◽  
Old Japanese

Renal hemangiopericytoma (HPC) is a rare tumor. There are no unique radiological findings that can reliably aid in its preoperative diagnosis. A 60-year-old Japanese male was admitted for an abdominal mass. Ultrasound revealed a heterogeneous hyperechoic mass. Computed tomography (CT) showed a heterogeneous mass and tumor vascularity by a precontrast and dynamic study with maximum intensity projection (MIP) and volume-rendering technique (VRT). Radical nephrectomy was performed, and the histopathogic diagnosis revealed renal HPC.

scholarly journals Selective deletion of SHIP-1 in hematopoietic cells in mice leads to severe lung inflammation involving ILC2 cells

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Xujun Ye ◽  
Fengrui Zhang ◽  
Li Zhou ◽  
Yadong Wei ◽  
Li Zhang ◽  
...  
Keyword(s):  
Lung Inflammation ◽  
Airway Remodeling ◽  
Pulmonary Inflammation ◽  
Hematopoietic Cells ◽  
Cell Lineage ◽  
Allergic Inflammation ◽  
Cell Types ◽  
Lymphoid Cells ◽  
Whole Body

AbstractSrc homology 2 domain–containing inositol 5-phosphatase 1 (SHIP-1) regulates the intracellular levels of phosphotidylinositol-3, 4, 5-trisphosphate, a phosphoinositide 3–kinase (PI3K) product. Emerging evidence suggests that the PI3K pathway is involved in allergic inflammation in the lung. Germline or induced whole-body deletion of SHIP-1 in mice led to spontaneous type 2-dominated pulmonary inflammation, demonstrating that SHIP-1 is essential for lung homeostasis. However, the mechanisms by which SHIP-1 regulates lung inflammation and the responsible cell types are still unclear. Deletion of SHIP-1 selectively in B cells, T cells, dendritic cells (DC) or macrophages did not lead to spontaneous allergic inflammation in mice, suggesting that innate immune cells, particularly group 2 innate lymphoid cells (ILC2 cells) may play an important role in this process. We tested this idea using mice with deletion of SHIP-1 in the hematopoietic cell lineage and examined the changes in ILC2 cells. Conditional deletion of SHIP-1 in hematopoietic cells in Tek-Cre/SHIP-1 mice resulted in spontaneous pulmonary inflammation with features of type 2 immune responses and airway remodeling like those seen in mice with global deletion of SHIP-1. Furthermore, when compared to wild-type control mice, Tek-Cre/SHIP-1 mice displayed a significant increase in the number of IL-5/IL-13 producing ILC2 cells in the lung at baseline and after stimulation by allergen Papain. These findings provide some hints that PI3K signaling may play a role in ILC2 cell development at baseline and in response to allergen stimulation. SHIP-1 is required for maintaining lung homeostasis potentially by restraining ILC2 cells and type 2 inflammation.

scholarly journals Protein C Gene Mutation in an Older Adult Patient with Clostridium perfringens Septicemia-Related Visceral Vein Thrombosis

TH Open ◽  
2021 ◽  
Vol 05 (02) ◽  
pp. e171-e173
Author(s):  
Kiyoko Kanosue ◽  
Satomi Nagaya ◽  
Eriko Morishita ◽  
Masayoshi Yamanishi ◽  
Shinsaku Imashuku
Keyword(s):  
Gene Mutation ◽  
Clostridium Perfringens ◽  
Protein C ◽  
Later Life ◽  
Heterozygous Mutation ◽  
Protein C Deficiency ◽  
Vein Thrombosis ◽  
Thrombosis Risk ◽  
Japanese Male ◽  
Old Japanese

AbstractA 78-year-old Japanese male with Clostridium perfringens septicemia and cholecystitis was found to have thrombosis in the left branch of intrahepatic portal vein as well as superior mesenteric vein. Visceral vein thrombosis (VVT) in this case was associated with protein C deficiency, due to a heterozygous mutation, p. Arg185Met. Our experience emphasizes that VVT, or other thromboembolic events, may occur in later life, triggered by environmental thrombosis risk factors, together with underlying hereditary protein C gene mutation.

scholarly journals JAK2 V617F, MPL, and CALR Mutations in Korean Patients with Essential Thrombocythemia and Primary Myelofibrosis

2015 ◽  
Vol 30 (7) ◽  
pp. 882 ◽  
Author(s):  
Bo Hyun Kim ◽  
Young-Uk Cho ◽  
Mi-Hyun Bae ◽  
Seongsoo Jang ◽  
Eul-Ju Seo ◽  
...  
Keyword(s):  
Essential Thrombocythemia ◽  
Jak2 V617f ◽  
Primary Myelofibrosis ◽  
Korean Patients ◽  
Calr Mutations

Successful thrombolysis in essential thrombocythemia-related acute ischaemic stroke

2021 ◽  
Vol 14 (5) ◽  
pp. e242925
Author(s):  
Ishita Desai ◽  
Ashutosh Tiwari ◽  
Mritunjai Kumar Singh ◽  
Niraj Kumar
Keyword(s):  
Ischaemic Stroke ◽  
Essential Thrombocythemia ◽  
Acute Ischaemic Stroke ◽  
Jak2 V617f ◽  
Preliminary Evidence ◽  
Sudden Onset ◽  
Left Ventricular ◽  
Interesting Case ◽  
Increased Risk ◽  
Successful Thrombolysis

Essential thrombocythemia (ET)-related acute ischaemic stroke (AIS) may account for approximately 0.25%–0.5% of all ischaemic strokes. If left undiagnosed and untreated, patients with ET carry an increased risk of recurrent thrombosis involving major organs including the brain. We report an interesting case of a 67-year-old man, who was successfully thrombolysed for AIS resulting from ET. He presented with sudden onset of left-sided hemiparesis with a left-ventricular clot. His subsequent investigations including positive JAK2 V617F mutation confirmed the diagnosis of ET. He made a significant recovery with thrombolysis, anticoagulation, antiplatelet and hydroxyurea. A fear of post-thrombolytic haemorrhagic complications appears the major reason for the lack of reports of thrombolysis in ET-related AIS. Although the diagnosis of ET was confirmed on subsequent investigations, successful thrombolysis in our case provides preliminary evidence that ET-related AIS cases can undergo successful thrombolysis using tenecteplase. To date, ours is only the second case of ET-related AIS being thrombolysed.

scholarly journals Effect of JAK2 V617F on thrombotic risk in patients with essential thrombocythemia: measuring the uncertain

Haematologica ◽  
2008 ◽  
Vol 93 (9) ◽  
pp. 1412-1414 ◽  
Author(s):  
P. D. Ziakas
Keyword(s):  
Essential Thrombocythemia ◽  
Jak2 V617f ◽  
Thrombotic Risk
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