Hepatosplenic T-cell lymphoma
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| Hepatosplenic T-cell lymphoma Classification and external resources | |
| ICD-O: | 9716/3 |
|---|---|
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Synonyms
Hepatosplenic γδ T-cell lymphoma[1]
ICD-O Code
9716/3[1]
Definition
Hepatosplenic T-cell lymphoma is a systemic neoplasm comprised of medium-sized cytotoxic T-cells that show a significant sinusoidal infiltration in the liver, spleen, and bone marrow.[1]
Epidemiology
This lymphoma is rare, comprising less than 5% of all cases, and is most common in young adults and adolescents. A distinct male gender preference has been described.[1]
Clinical Features
Etiology
The cell of origin for this disease is an immature cytotoxic T-cell clonally expressing the γδ T-cell receptor. This disease is seen more often in immunosuppressed solid organ transplant recipients, an association that has led to the hypothesis that long-term immune stimulation in the setting of immunosuppression is the causative agent.[1][1]
Clinical Presentation
The typical clinical finding in a patient with hepatosplenic T-cell lymphoma is hepatosplenomegaly.[1]
Laboratory Findings
The constellation of thrombocytopenia, anemia, and leukocytosis is common in patients with hepatosplenic T-cell lymphoma.[1] The discovery of neoplastic cells in peripheral blood is often a finding late in the clinical course.[1]
Sites of Involvement
The spleen and liver are always involved, with bone marrow involvement frequently present. Nodal involvement is exceedingly rare.[1][1]
Morphology
The neoplastic cells in this disorder show a monotonous appearance, with a small amount of cytoplasm and inconspicuous nucleoli.[1]
Spleen and liver
This disease shows a distinct sinusoidal pattern of infiltration which spares the splenic white pulp and hepatic portal triads.[1]
Bone marrow
While the bone marrow is commonly involved, the detection of the neoplastic infiltrate may be difficult due to diffuse, interstitial pattern. Immunohistochemistry can aid in the detection of this lymphoma.[1]
Peripheral blood
Cells of a similar morphology observed in solid organs are observed in peripheral blood.[1]
Molecular Findings
Immunophenotype
The immunophenotype for hepatosplenic T-cell lymphoma is a post-thymic, immature T-cell.[1][1]
| Status | Antigens |
| Positive | CD3, TCRδ1, TIA-1 |
| Negative | CD4, CD5, CD8 |
Genetic Findings
Clonal rearrangement of the γ gene of the T-cell receptor is the hallmark of this disease. A few cases have shown rearrangement of the T-cell receptor β gene.[1] Isochromosome 7q has been observed in all cases described so far, sometimes in conjunction with other chromosomal abnormalities such as trisomy 8.[1]
References
WikiDoc Research Resources for Hepatosplenic T-cell lymphoma | |
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| Articles on Hepatosplenic T-cell lymphoma | Most recent articles on Hepatosplenic T-cell lymphoma • Most cited articles on Hepatosplenic T-cell lymphoma • Review articles on Hepatosplenic T-cell lymphoma • Articles on Hepatosplenic T-cell lymphoma in N Eng J Med, Lancet, BMJ |
| Media (Slides, Video, Images, MP3) on Hepatosplenic T-cell lymphoma | Powerpoint slides on Hepatosplenic T-cell lymphoma • Images of Hepatosplenic T-cell lymphoma • Photos of Hepatosplenic T-cell lymphoma • Podcasts & MP3s on Hepatosplenic T-cell lymphoma • Videos on Hepatosplenic T-cell lymphoma |
| Evidence Based Medicine Regarding Hepatosplenic T-cell lymphoma | Cochrane Collaboration on Hepatosplenic T-cell lymphoma • Bandolier on Hepatosplenic T-cell lymphoma • TRIP on Hepatosplenic T-cell lymphoma |
| Cost Effectiveness of Hepatosplenic T-cell lymphoma | Cost Effectiveness of Hepatosplenic T-cell lymphoma |
| Clinical Trials Involving Hepatosplenic T-cell lymphoma | Ongoing Trials on Hepatosplenic T-cell lymphoma at Clinical Trials.gov • Trial results on Hepatosplenic T-cell lymphoma • Clinical Trials on Hepatosplenic T-cell lymphoma at Google |
| Guidelines / Policies / Government Resources (FDA/CDC) Regarding Hepatosplenic T-cell lymphoma | US National Guidelines Clearinghouse on Hepatosplenic T-cell lymphoma • NICE Guidance on Hepatosplenic T-cell lymphoma • NHS PRODIGY Guidance • FDA on Hepatosplenic T-cell lymphoma • CDC on Hepatosplenic T-cell lymphoma |
| Textbook Information on Hepatosplenic T-cell lymphoma | Books and Textbook Information on Hepatosplenic T-cell lymphoma |
| Pharmacology Resources on Hepatosplenic T-cell lymphoma | Dosing of Hepatosplenic T-cell lymphoma • Drug interactions with Hepatosplenic T-cell lymphoma • Side effects of Hepatosplenic T-cell lymphoma • Allergic reactions to Hepatosplenic T-cell lymphoma • Overdose information on Hepatosplenic T-cell lymphoma • Carcinogenicity information on Hepatosplenic T-cell lymphoma • Hepatosplenic T-cell lymphoma in pregnancy • Pharmacokinetics of Hepatosplenic T-cell lymphoma • |
| Genetics, Pharmacogenomics, and Proteinomics of Hepatosplenic T-cell lymphoma | Genetics of Hepatosplenic T-cell lymphoma • Pharmacogenomics of Hepatosplenic T-cell lymphoma • Proteomics of Hepatosplenic T-cell lymphoma |
| Newstories on Hepatosplenic T-cell lymphoma | Hepatosplenic T-cell lymphoma in the news • Be alerted to news on Hepatosplenic T-cell lymphoma • News trends on Hepatosplenic T-cell lymphoma |
| Commentary on Hepatosplenic T-cell lymphoma | Blogs on Hepatosplenic T-cell lymphoma |
| Patient Resources on Hepatosplenic T-cell lymphoma | Patient resources on Hepatosplenic T-cell lymphoma • Discussion groups on Hepatosplenic T-cell lymphoma • Patient Handouts on Hepatosplenic T-cell lymphoma • Directions to Hospitals Treating Hepatosplenic T-cell lymphoma • Risk calculators and risk factors for Hepatosplenic T-cell lymphoma |
| Healthcare Provider Resources on Hepatosplenic T-cell lymphoma | Symptoms of Hepatosplenic T-cell lymphoma • Causes & Risk Factors for Hepatosplenic T-cell lymphoma • Diagnostic studies for Hepatosplenic T-cell lymphoma • Treatment of Hepatosplenic T-cell lymphoma |
| Continuing Medical Education (CME) Programs on Hepatosplenic T-cell lymphoma | CME Programs on Hepatosplenic T-cell lymphoma |
| International Resources on Hepatosplenic T-cell lymphoma | Hepatosplenic T-cell lymphoma en Espanol • Hepatosplenic T-cell lymphoma en Francais |
| Business Resources on Hepatosplenic T-cell lymphoma | Hepatosplenic T-cell lymphoma in the Marketplace • Patents on Hepatosplenic T-cell lymphoma |
| Informatics Resources on Hepatosplenic T-cell lymphoma | List of terms related to Hepatosplenic T-cell lymphoma |
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Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
