Hepatosplenic T cell lymphoma: Difference between revisions

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==Overview==
==Overview==
Hepatosplenic t cell lymphoma is a rare type of non Hodgkins lymphoma that occurs in states of immunosupression such as post organ transplant and treatment of inflammatory bowel disease. Hepatosplenic T cell lymphoma was discovered by Farcet ''et al in 1990.'' It is also known as ‘gamma-delta hepatosplenic T-cell lymphoma. The lymphoma is characterized by malignant T-cell proliferation in the sinusoids of the liver, sinuses and red pulp of the spleen, and sinuses of the bone marrow. The incidence of hepatosplenic t cell lymphoma is 0.3 per 100000 individuals per year. If left untreated, patients can develop liver failure, pancytopenia or spleen rupture. Biopsy of the tumor is the gold standard diagnostic test for diagnosing hepatosplenic t cell lymphoma. Chemotherapy including CHOP is the mainstay of the treatment alongwith bone marrow transplant and radiation therapy.


==Historical Perspective==
==Historical Perspective==
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==Pathophysiology==
==Pathophysiology==
* Hepatosplenic T-cell lymphoma is a peripheral T-cell lymphoma, a type of non Hodgkin's lymphoma<ref name="pmid28516671">{{cite journal| author=Armitage JO| title=The aggressive peripheral T-cell lymphomas: 2017. | journal=Am J Hematol | year= 2017 | volume= 92 | issue= 7 | pages= 706-715 | pmid=28516671 | doi=10.1002/ajh.24791 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28516671  }} </ref>.
* Hepatosplenic T-cell lymphoma is a [[T-cell lymphoma classification|peripheral T-cell lymphoma]], a type of [[Non-Hodgkin lymphoma|non Hodgkin's lymphoma]]<ref name="pmid28516671">{{cite journal| author=Armitage JO| title=The aggressive peripheral T-cell lymphomas: 2017. | journal=Am J Hematol | year= 2017 | volume= 92 | issue= 7 | pages= 706-715 | pmid=28516671 | doi=10.1002/ajh.24791 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28516671  }} </ref>.
* It is also known as ‘gamma-delta hepatosplenic T-cell lymphoma<ref name="pmid30656034">{{cite journal| author=Brandt PH, Rahmat LT, Ali SS| title=A rare case of hepatosplenic gamma-delta T-cell lymphoma and secondary hemophagocytic lymphohistiocytosis. | journal=Clin Case Rep | year= 2019 | volume= 7 | issue= 1 | pages= 164-169 | pmid=30656034 | doi=10.1002/ccr3.1924 | pmc=6333078 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30656034  }} </ref>.
* It is also known as ‘gamma-delta hepatosplenic T-cell lymphoma<ref name="pmid30656034">{{cite journal| author=Brandt PH, Rahmat LT, Ali SS| title=A rare case of hepatosplenic gamma-delta T-cell lymphoma and secondary hemophagocytic lymphohistiocytosis. | journal=Clin Case Rep | year= 2019 | volume= 7 | issue= 1 | pages= 164-169 | pmid=30656034 | doi=10.1002/ccr3.1924 | pmc=6333078 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30656034  }} </ref>.
* It usually occurs in young men with history of immunosuppression including solid organ transplantation and leukemia<ref name="pmid29933321">{{cite journal| author=Choi Y, Jeon SY, Yoo WH| title=Hepatosplenic T-Cell Lymphoma Arising in a Patient Treated With Tumor Necrosis Factor-α Inhibitors for Ankylosing Spondylitis. | journal=J Clin Rheumatol | year= 2018 | volume=  | issue=  | pages=  | pmid=29933321 | doi=10.1097/RHU.0000000000000805 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29933321  }} </ref>.
* It usually occurs in young men with history of [[immunosuppression]] including solid [[Organ transplant|organ transplantation]]<ref name="pmid29933321">{{cite journal| author=Choi Y, Jeon SY, Yoo WH| title=Hepatosplenic T-Cell Lymphoma Arising in a Patient Treated With Tumor Necrosis Factor-α Inhibitors for Ankylosing Spondylitis. | journal=J Clin Rheumatol | year= 2018 | volume=  | issue=  | pages=  | pmid=29933321 | doi=10.1097/RHU.0000000000000805 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29933321  }} </ref>.
* Patients with inflammatory bowel diseases receiving immunosuppressants and anti-tumor necrosis factor-α agent are also at risk for developing hepatosplenic T-cell lymphoma.  
* Patients with [[inflammatory bowel disease]] receiving [[immunosuppressants]] and [[Tumor necrosis factor-alpha|anti-tumor necrosis factor-α]] agent are also at risk for developing hepatosplenic T-cell lymphoma.  
* The T-cell  receptor consists of either a gamma delta or aplha beta entity on their cell surface which are a part of the innate immune system<ref name="pmid30596219">{{cite journal| author=Gowda L, Foss F| title=Hepatosplenic T-Cell Lymphomas. | journal=Cancer Treat Res | year= 2019 | volume= 176 | issue=  | pages= 185-193 | pmid=30596219 | doi=10.1007/978-3-319-99716-2_9 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30596219  }} </ref><ref name="pmid1698028">{{cite journal| author=Gaulard P, Bourquelot P, Kanavaros P, Haioun C, Le Couedic JP, Divine M et al.| title=Expression of the alpha/beta and gamma/delta T-cell receptors in 57 cases of peripheral T-cell lymphomas. Identification of a subset of gamma/delta T-cell lymphomas. | journal=Am J Pathol | year= 1990 | volume= 137 | issue= 3 | pages= 617-28 | pmid=1698028 | doi= | pmc=1877506 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1698028  }} </ref>.
* The T-cell  receptor consists of either a gamma delta or aplha beta entity on their cell surface which are a part of the [[Innate intelligence|innate]] immune system<ref name="pmid30596219">{{cite journal| author=Gowda L, Foss F| title=Hepatosplenic T-Cell Lymphomas. | journal=Cancer Treat Res | year= 2019 | volume= 176 | issue=  | pages= 185-193 | pmid=30596219 | doi=10.1007/978-3-319-99716-2_9 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30596219  }} </ref><ref name="pmid1698028">{{cite journal| author=Gaulard P, Bourquelot P, Kanavaros P, Haioun C, Le Couedic JP, Divine M et al.| title=Expression of the alpha/beta and gamma/delta T-cell receptors in 57 cases of peripheral T-cell lymphomas. Identification of a subset of gamma/delta T-cell lymphomas. | journal=Am J Pathol | year= 1990 | volume= 137 | issue= 3 | pages= 617-28 | pmid=1698028 | doi= | pmc=1877506 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1698028  }} </ref>.
* Gamma delta T cells represent the first line of defense against bacterial peptides, such as heat-shock proteins.
* Gamma delta T cells represent the first line of defense against [[Bacteria|bacterial]] [[Peptide|peptides]], such as [[Heat shock protein|heat-shock proteins]].
* Gamma delta T cells are CD4 and CD8 negative, but CD56 positive which is NK cell marker.
* Gamma delta T cells are [[CD4]] and [[CD8]] negative, but CD56 positive which is [[NK cell marker]].
* Gamma delta cells respond to a stimuli and are responsible for lymphokine production and proliferation.
* Gamma delta cells respond to a stimuli and are responsible for [[lymphokine]] production and proliferation.
* Gamma delta cells are predominantly located in the spleen, liver sinusoids and intestinal epithelium.
* Gamma delta cells are predominantly located in the [[spleen]], [[liver]] sinusoids and [[Intestine|intestinal]] epithelium.
* 75 % of the cases are of gamma delta phenotype and the rest are apha beta phenotype.
* 75 % of the cases are of gamma delta phenotype and the rest are apha beta phenotype.
* Chronic antigen stimulation in states of immunosuppression is responsible for the deveopment of the lymphoma.
* Chronic antigen stimulation in states of immunosuppression is responsible for the development of the [[lymphoma]].
* Isochromosome of the long arm of chromosome 7 is a genetic abnormality described in hepatosplenic t cell lymphoma. It could also be in association with trisomy 8 and a loss of a sex chromosome.
* [[Isochromosome]] of the long arm of [[chromosome 7]] is a genetic abnormality described in hepatosplenic t cell lymphoma. It could also be in association with [[trisomy]] 8 and a loss of a [[sex chromosome]].
* Mutations in ''SETD2, INO80, TET3'' and ''STAT5B'' occur exclusively in hepatosplenic t cell lymphoma as compared to other T and B cell lymphoma types.
* Mutations in ''SETD2, INO80, TET3'' and ''STAT5B'' occur exclusively in hepatosplenic t cell lymphoma as compared to other T and B cell lymphoma types.
* The lymphoma is characterized by malignant T-cell proliferation in the sinusoids of the liver, sinuses and red pulp of the spleen, and sinuses of the bone marrow.
* The lymphoma is characterized by malignant T-cell proliferation in the sinusoids of the liver, sinuses and red pulp of the spleen, and sinuses of the bone marrow.
* Splenic involvement is characterized by diffuse involvement of the red pulp with small-to-medium-sized atypical lymphocytes.  
* Splenic involvement is characterized by diffuse involvement of the [[red pulp]] with small-to-medium-sized [[atypical lymphocytes]].  
* The atypical lymphocytes are present within the cords and sinuses of the red pulp.
* The atypical lymphocytes are present within the cords and sinuses of the red pulp.
* There occurs a complete loss of the white pulp.
* There occurs a complete loss of the [[white pulp]].
* The liver also shows sinusoidal infiltration by neoplastic lymphoid cells.  
* The liver also shows sinusoidal infiltration by neoplastic lymphoid cells.  
* The bone marrow is characterized by neoplastic cells in the sinusoids.
* The bone marrow is characterized by neoplastic cells in the sinusoids.
* Bone marrow infiltration results in pancytopenia.
* Bone marrow infiltration results in [[pancytopenia]].
* The lymphoma cells are typically CD2+, CD3+, CD4−, CD5−, CD7+, CD8− CD42+, CD52+, CD76+, CD82+ with either gamma-delta or alpha-beta T-cell phenotypic receptor expression.
* The lymphoma cells are typically CD2+, CD3+, CD4−, CD5−, CD7+, CD8− CD42+, CD52+, CD76+, CD82+ with either gamma-delta or alpha-beta T-cell phenotypic receptor expression.
* It manifests as hepatosplenomegaly without peripheral lymphadenopathy.
* It manifests as [[hepatosplenomegaly]] without peripheral [[lymphadenopathy]].
* Pancytopenia and abnormal liver functions are the laboratory findings.
* [[Pancytopenia]] and abnormal liver functions are the laboratory findings.
* Histology and immunohistochemistry of the tumor biopsy shows portal and sinusoidal infiltration by atypical small-to-medium sized lymphocytes with hyperchromatic nuclei and low mitotic activity.
* Histology and immunohistochemistry of the tumor biopsy shows portal and sinusoidal infiltration by atypical small-to-medium sized lymphocytes with hyperchromatic nuclei and low mitotic activity.


==Causes==
==Causes==
Common causes of hepatosplenic t cell lymphoma are<ref name="pmid29337025">{{cite journal| author=Yabe M, Miranda RN, Medeiros LJ| title=Hepatosplenic T-cell Lymphoma: a review of clinicopathologic features, pathogenesis, and prognostic factors. | journal=Hum Pathol | year= 2018 | volume= 74 | issue=  | pages= 5-16 | pmid=29337025 | doi=10.1016/j.humpath.2018.01.005 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29337025  }} </ref>:
Common causes of hepatosplenic t cell lymphoma are<ref name="pmid29337025">{{cite journal| author=Yabe M, Miranda RN, Medeiros LJ| title=Hepatosplenic T-cell Lymphoma: a review of clinicopathologic features, pathogenesis, and prognostic factors. | journal=Hum Pathol | year= 2018 | volume= 74 | issue=  | pages= 5-16 | pmid=29337025 | doi=10.1016/j.humpath.2018.01.005 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29337025  }} </ref>:
* Inflammatory bowel disease
* [[Inflammatory bowel disease]]
* Organ transplant patients (reciever)
* [[Organ transplant]] patients (reciever)
* Immunosuppresent medications
* [[Immunosuppresive drug|Immunosuppressive]] medications
* Thiopurines
* [[Thiopurine|Thiopurines]]
* Infliximab
* [[Infliximab]]
* Cyclophosphamide
* [[Cyclophosphamide]]
* Vincristine
* [[Vincristine sulfate|Vincristine]]
* Doxorubicin
* [[Doxorubicin hydrochloride|Doxorubicin]]


==Differentiating hepatosplenic t cell lymphoma from Other Diseases==
==Differentiating hepatosplenic t cell lymphoma from Other Diseases==
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==Risk Factors==
==Risk Factors==
Common risk factors include:
Common risk factors include:
* Immunodeficiency diseases.
* [[Autoimmune diseases]]
* Patients on immunosuppresant medications.
* Patients on immunosuppresant medications.
* Patients on chemotherapy.
* Patients on [[Chemotherapy|chemotherapy.]]
* Inflammatory bowel disease
* Inflammatory bowel disease
* Organ transplant patients.
* Organ transplant patients.
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=== Natural history ===
=== Natural history ===
* Patients have a history of immunosupression such as inflammatory bowel disease under treatment or organ transplant<ref name="pmid19237479">{{cite journal| author=Falchook GS, Vega F, Dang NH, Samaniego F, Rodriguez MA, Champlin RE et al.| title=Hepatosplenic gamma-delta T-cell lymphoma: clinicopathological features and treatment. | journal=Ann Oncol | year= 2009 | volume= 20 | issue= 6 | pages= 1080-5 | pmid=19237479 | doi=10.1093/annonc/mdn751 | pmc=4092251 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19237479  }} </ref>.
* Patients have a history of [[immunosupression]] such as inflammatory bowel disease under treatment or organ transplant<ref name="pmid19237479">{{cite journal| author=Falchook GS, Vega F, Dang NH, Samaniego F, Rodriguez MA, Champlin RE et al.| title=Hepatosplenic gamma-delta T-cell lymphoma: clinicopathological features and treatment. | journal=Ann Oncol | year= 2009 | volume= 20 | issue= 6 | pages= 1080-5 | pmid=19237479 | doi=10.1093/annonc/mdn751 | pmc=4092251 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19237479  }} </ref>.
* The mean age group is 35 years and most of the patients are males.
* The mean age group is 35 years and most of the patients are males.
* Initial symptoms are fever, weight loss, night sweats and then progress to more severe symptoms depending on the organ involvement but there is no lymphadenopathy.
* Initial symptoms are fever, weight loss, night sweats and then progress to more severe symptoms depending on the organ involvement but there is no [[Lymphadenopathy|lymphadenopathy.]]
* Patients also present with symptoms of liver, spleen and bone marrow dysfunction.
* Patients also present with symptoms of liver, spleen and bone marrow dysfunction.
* If left untreated, patients can develop liver failure, pancytopenia or spleen rupture.
* If left untreated, patients can develop [[Hepatic failure|liver failure]], [[pancytopenia]] or [[spleen rupture]].


=== Complications ===
=== Complications ===
* Hepatomegaly
* [[Hepatomegaly]]
* Hepatic failure
* [[Hepatic failure]]
* Portal vein thrombosis
* [[Portal vein thrombosis]]
* Splenomegaly
* [[Splenomegaly]]
* Splenic infarction
* [[Splenic infarction]]
* Spleen rupture
* Spleen rupture
* Splenic vein thrombosis
* [[Splenic vein thrombosis]]
* Anemia
* [[Anemia]]
* Thrombocytopenia
* [[Thrombocytopenia]]
* Neutropenia
* [[Neutropenia]]
* Neurological dysfunction if metastasizes to brain.
* Neurological dysfunction if metastasizes to brain.
* Intestinal perforation
* [[Gastrointestinal perforation|Intestinal perforation]]
* Intestinal obstruction
* [[Intestinal obstruction]]
*  
*  


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* Night sweats
* Night sweats
* Pain abdomen
* Pain abdomen
* Jaundice
* [[Jaundice]]
* Fatigue
* Fatigue
* Recurrent infections
* Recurrent infections
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===Laboratory Findings===
===Laboratory Findings===
* Biopsy of the tumor:
* Biopsy of the tumor:
** Histology - small-to intermediate sized T lymphocytes infiltrate the sinusoids of the liver and the splenic red pulp.
** [[Histology]] - small-to intermediate sized T lymphocytes infiltrate the sinusoids of the liver and the splenic red pulp.
** Flow cytometry and immunophenotyping - The lymphoma cells are typically CD2+, CD3+, CD4−, CD5−, CD7+, CD8− CD42+, CD52+, CD76+, CD82+ with either gamma-delta or alpha-beta T-cell phenotypic receptor expression.
** [[Flow cytometry]] and [[immunophenotyping]] - The lymphoma cells are typically CD2+, CD3+, CD4−, CD5−, CD7+, CD8− CD42+, CD52+, CD76+, CD82+ with either gamma-delta or alpha-beta T-cell phenotypic receptor expression.
** Karyotyping - Isochromosome of the long arm of chromosome 7 is a genetic abnormality described in hepatosplenic t cell lymphoma. It could also be in association with trisomy 8 and a loss of a sex chromosome.
** [[Karyotype|Karyotyping]] - Isochromosome of the long arm of chromosome 7 is a genetic abnormality described in hepatosplenic t cell lymphoma. It could also be in association with trisomy 8 and a loss of a sex chromosome.
* Bone marrow biopsy; The bone marrow is characterized by neoplastic cells in the sinusoids.
* Bone marrow biopsy; The bone marrow is characterized by [[Cancer|neoplastic]] cells in the sinusoids.
* Complete blood count; Pancytopenia
* Complete blood count; [[Pancytopenia]]
* Liver function tests: Deranged LFT
* Liver function tests: Deranged [[Liver function tests|LFT]]
** Elevated transaminases
** Elevated [[Transaminase|transaminases]]
** Hyperbilirubinemia
** [[Jaundice|Hyperbilirubinemia]]
** Hypoproteinemia
** [[Hypoproteinemia]]
** Elevated [[alkaline phosphatase]]  
** Elevated [[alkaline phosphatase]]  
** Elevated PT/INR
** Elevated PT/[[Prothrombin time|INR]]


===Electrocardiogram===
===Electrocardiogram===
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===X-ray===
===X-ray===
There are no x-ray findings associated with hepatosplenic t cell lymphoma but pleural effusion might be present.
There are no x-ray findings associated with hepatosplenic t cell lymphoma but [[pleural effusion]] might be present.


===Echocardiography or Ultrasound===
===Echocardiography or Ultrasound===
* There are no echocardiography findings associated with hepatosplenic t cell lymphoma.
* There are no echocardiography findings associated with hepatosplenic t cell lymphoma.
* On ultrasound of abdomen:
* On ultrasound of abdomen:
** Hepatomegaly
** [[Hepatomegaly]]
** Splenomegaly
** [[Splenomegaly]]


===CT scan===
===CT scan===
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==Treatment==
==Treatment==
===Medical Therapy===
===Medical Therapy===
There is no treatment for [disease name]; the mainstay of therapy is supportive care.
* [[Induction (biology)|Induction]] therapy
 
** [[Cyclophosphamide]]
OR
** [[Vincristine sulfate|Vincristine]]
 
** [[Doxorubicin hydrochloride|Doxorubicin]]
Supportive therapy for [disease name] includes [therapy 1], [therapy 2], and [therapy 3].
** [[Prednisolone]]
 
* [[Consolidation (medicine)|Consolidation]] therapy
OR
** [[Radiation therapy]]
 
** Allogenic [[Hematopoietic stem cell transplantation|bone marrow transplant]]
The majority of cases of [disease name] are self-limited and require only supportive care.
** Autologous [[Hematopoietic stem cell transplantation|bone marrow transplant]]
 
* Additional medications given during chemotherapy
OR
** [[Acyclovir]]
 
** [[Fluconazole]]
[Disease name] is a medical emergency and requires prompt treatment.
** [[Sulfamethoxazole-Trimethoprim|TMP/sulphamethaxozole]]
 
OR
 
The mainstay of treatment for [disease name] is [therapy].
 
OR
 
The optimal therapy for [malignancy name] depends on the stage at diagnosis.
 
OR
 
[Therapy] is recommended among all patients who develop [disease name].
 
OR
 
Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].
 
OR
 
Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
 
OR
 
Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].
 
OR
 
Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].


===Surgery===
===Surgery===
Surgical intervention is not recommended for the management of [disease name].
Surgical intervention is not recommended for the management of hepaosplenic t cell lymphoma.
 
OR
 
Surgery is not the first-line treatment option for patients with [disease name]. Surgery is usually reserved for patients with either [indication 1], [indication 2], and [indication 3]
 
OR
 
The mainstay of treatment for [disease name] is medical therapy. Surgery is usually reserved for patients with either [indication 1], [indication 2], and/or [indication 3].
 
OR
 
The feasibility of surgery depends on the stage of [malignancy] at diagnosis.
 
OR
 
Surgery is the mainstay of treatment for [disease or malignancy].


===Primary Prevention===
===Primary Prevention===
There are no established measures for the primary prevention of [disease name].
There are no established measures for the primary prevention of hepatosplenic t cell lymphoma.
 
OR
 
There are no available vaccines against [disease name].
 
OR
 
Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].
 
OR
 
[Vaccine name] vaccine is recommended for [patient population] to prevent [disease name]. Other primary prevention strategies include [strategy 1], [strategy 2], and [strategy 3].


===Secondary Prevention===
===Secondary Prevention===
There are no established measures for the secondary prevention of [disease name].
There are no established measures for the secondary prevention of hepatosplenic t cell lymphoma.
 
OR
 
Effective measures for the secondary prevention of [disease name] include [strategy 1], [strategy 2], and [strategy 3].


==References==
==References==

Revision as of 21:16, 28 January 2019


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Synonyms and Keywords: Hepatosplenic γδ T-cell lymphoma, Hepatosplenic gamma delta T-cell lymphoma, HSTCL

Synonyms and keywords:

Overview

Hepatosplenic t cell lymphoma is a rare type of non Hodgkins lymphoma that occurs in states of immunosupression such as post organ transplant and treatment of inflammatory bowel disease. Hepatosplenic T cell lymphoma was discovered by Farcet et al in 1990. It is also known as ‘gamma-delta hepatosplenic T-cell lymphoma. The lymphoma is characterized by malignant T-cell proliferation in the sinusoids of the liver, sinuses and red pulp of the spleen, and sinuses of the bone marrow. The incidence of hepatosplenic t cell lymphoma is 0.3 per 100000 individuals per year. If left untreated, patients can develop liver failure, pancytopenia or spleen rupture. Biopsy of the tumor is the gold standard diagnostic test for diagnosing hepatosplenic t cell lymphoma. Chemotherapy including CHOP is the mainstay of the treatment alongwith bone marrow transplant and radiation therapy.

Historical Perspective

Hepatosplenic T cell lymphoma was discovered by Farcet et al in 1990.

Classification

There is no established system for the classification of hepatosplenic t cell lymphoma.

Pathophysiology

  • Hepatosplenic T-cell lymphoma is a peripheral T-cell lymphoma, a type of non Hodgkin's lymphoma[1].
  • It is also known as ‘gamma-delta hepatosplenic T-cell lymphoma[2].
  • It usually occurs in young men with history of immunosuppression including solid organ transplantation[3].
  • Patients with inflammatory bowel disease receiving immunosuppressants and anti-tumor necrosis factor-α agent are also at risk for developing hepatosplenic T-cell lymphoma.
  • The T-cell receptor consists of either a gamma delta or aplha beta entity on their cell surface which are a part of the innate immune system[4][5].
  • Gamma delta T cells represent the first line of defense against bacterial peptides, such as heat-shock proteins.
  • Gamma delta T cells are CD4 and CD8 negative, but CD56 positive which is NK cell marker.
  • Gamma delta cells respond to a stimuli and are responsible for lymphokine production and proliferation.
  • Gamma delta cells are predominantly located in the spleen, liver sinusoids and intestinal epithelium.
  • 75 % of the cases are of gamma delta phenotype and the rest are apha beta phenotype.
  • Chronic antigen stimulation in states of immunosuppression is responsible for the development of the lymphoma.
  • Isochromosome of the long arm of chromosome 7 is a genetic abnormality described in hepatosplenic t cell lymphoma. It could also be in association with trisomy 8 and a loss of a sex chromosome.
  • Mutations in SETD2, INO80, TET3 and STAT5B occur exclusively in hepatosplenic t cell lymphoma as compared to other T and B cell lymphoma types.
  • The lymphoma is characterized by malignant T-cell proliferation in the sinusoids of the liver, sinuses and red pulp of the spleen, and sinuses of the bone marrow.
  • Splenic involvement is characterized by diffuse involvement of the red pulp with small-to-medium-sized atypical lymphocytes.
  • The atypical lymphocytes are present within the cords and sinuses of the red pulp.
  • There occurs a complete loss of the white pulp.
  • The liver also shows sinusoidal infiltration by neoplastic lymphoid cells.
  • The bone marrow is characterized by neoplastic cells in the sinusoids.
  • Bone marrow infiltration results in pancytopenia.
  • The lymphoma cells are typically CD2+, CD3+, CD4−, CD5−, CD7+, CD8− CD42+, CD52+, CD76+, CD82+ with either gamma-delta or alpha-beta T-cell phenotypic receptor expression.
  • It manifests as hepatosplenomegaly without peripheral lymphadenopathy.
  • Pancytopenia and abnormal liver functions are the laboratory findings.
  • Histology and immunohistochemistry of the tumor biopsy shows portal and sinusoidal infiltration by atypical small-to-medium sized lymphocytes with hyperchromatic nuclei and low mitotic activity.

Causes

Common causes of hepatosplenic t cell lymphoma are[6]:

Differentiating hepatosplenic t cell lymphoma from Other Diseases

Epidemiology and Demographics

  • The incidence of hepatosplenic t cell lymphoma is 0.3 per 100000 individuals per year.
  • It occurs in younger group of patients, most cases falling in 20-40 years of age group.
  • Men are more affected than the females.

Risk Factors

Common risk factors include:

Screening

There is insufficient evidence to recommend routine screening for hepatosplenic t cell lymphoma.

Natural History, Complications, and Prognosis

Natural history

  • Patients have a history of immunosupression such as inflammatory bowel disease under treatment or organ transplant[7].
  • The mean age group is 35 years and most of the patients are males.
  • Initial symptoms are fever, weight loss, night sweats and then progress to more severe symptoms depending on the organ involvement but there is no lymphadenopathy.
  • Patients also present with symptoms of liver, spleen and bone marrow dysfunction.
  • If left untreated, patients can develop liver failure, pancytopenia or spleen rupture.

Complications

Prognosis

  • The prognosis is very poor, with patients dying within 2-3 years of diagnosis even after recieving treatment[8].

Diagnosis

Diagnostic Study of Choice

  • Biopsy of the tumor is the gold standard diagnostic test for diagnosing hepatosplenic t cell lymphoma.
  • CT scan and PET scan are used to assess the spread of the lymphoma.

Symptoms

  • Fever
  • Weight loss
  • Night sweats
  • Pain abdomen
  • Jaundice
  • Fatigue
  • Recurrent infections
  • Bleeding

Physical Examination

Temperature

Skin

Thorax

Abdomen

Extremities

  • Bone tenderness

Laboratory Findings

  • Biopsy of the tumor:
    • Histology - small-to intermediate sized T lymphocytes infiltrate the sinusoids of the liver and the splenic red pulp.
    • Flow cytometry and immunophenotyping - The lymphoma cells are typically CD2+, CD3+, CD4−, CD5−, CD7+, CD8− CD42+, CD52+, CD76+, CD82+ with either gamma-delta or alpha-beta T-cell phenotypic receptor expression.
    • Karyotyping - Isochromosome of the long arm of chromosome 7 is a genetic abnormality described in hepatosplenic t cell lymphoma. It could also be in association with trisomy 8 and a loss of a sex chromosome.
  • Bone marrow biopsy; The bone marrow is characterized by neoplastic cells in the sinusoids.
  • Complete blood count; Pancytopenia
  • Liver function tests: Deranged LFT

Electrocardiogram

There are no ECG findings associated with hepatosplenic t cell lymphoma.

X-ray

There are no x-ray findings associated with hepatosplenic t cell lymphoma but pleural effusion might be present.

Echocardiography or Ultrasound

  • There are no echocardiography findings associated with hepatosplenic t cell lymphoma.
  • On ultrasound of abdomen:

CT scan

Tumor mass can be seen in liver or spleen or both.

MRI

Tumor mass can be seen in liver or spleen or both.

Other Imaging Findings

PET scan: On PET scan, tumor mass can be seen in liver or spleen or if it has metastatsized to any other organ.

Treatment

Medical Therapy

Surgery

Surgical intervention is not recommended for the management of hepaosplenic t cell lymphoma.

Primary Prevention

There are no established measures for the primary prevention of hepatosplenic t cell lymphoma.

Secondary Prevention

There are no established measures for the secondary prevention of hepatosplenic t cell lymphoma.

References

  1. ↑ Armitage JO (2017). "The aggressive peripheral T-cell lymphomas: 2017". Am J Hematol. 92 (7): 706–715. doi:10.1002/ajh.24791. PMID 28516671.
  2. ↑ Brandt PH, Rahmat LT, Ali SS (2019). "A rare case of hepatosplenic gamma-delta T-cell lymphoma and secondary hemophagocytic lymphohistiocytosis". Clin Case Rep. 7 (1): 164–169. doi:10.1002/ccr3.1924. PMC 6333078. PMID 30656034.
  3. ↑ Choi Y, Jeon SY, Yoo WH (2018). "Hepatosplenic T-Cell Lymphoma Arising in a Patient Treated With Tumor Necrosis Factor-α Inhibitors for Ankylosing Spondylitis". J Clin Rheumatol. doi:10.1097/RHU.0000000000000805. PMID 29933321.
  4. ↑ Gowda L, Foss F (2019). "Hepatosplenic T-Cell Lymphomas". Cancer Treat Res. 176: 185–193. doi:10.1007/978-3-319-99716-2_9. PMID 30596219.
  5. ↑ Gaulard P, Bourquelot P, Kanavaros P, Haioun C, Le Couedic JP, Divine M; et al. (1990). "Expression of the alpha/beta and gamma/delta T-cell receptors in 57 cases of peripheral T-cell lymphomas. Identification of a subset of gamma/delta T-cell lymphomas". Am J Pathol. 137 (3): 617–28. PMC 1877506. PMID 1698028.
  6. ↑ Yabe M, Miranda RN, Medeiros LJ (2018). "Hepatosplenic T-cell Lymphoma: a review of clinicopathologic features, pathogenesis, and prognostic factors". Hum Pathol. 74: 5–16. doi:10.1016/j.humpath.2018.01.005. PMID 29337025.
  7. ↑ Falchook GS, Vega F, Dang NH, Samaniego F, Rodriguez MA, Champlin RE; et al. (2009). "Hepatosplenic gamma-delta T-cell lymphoma: clinicopathological features and treatment". Ann Oncol. 20 (6): 1080–5. doi:10.1093/annonc/mdn751. PMC 4092251. PMID 19237479.
  8. ↑ Yabe M, Medeiros LJ, Tang G, Wang SA, Ahmed S, Nieto Y; et al. (2016). "Prognostic Factors of Hepatosplenic T-cell Lymphoma: Clinicopathologic Study of 28 Cases". Am J Surg Pathol. 40 (5): 676–88. doi:10.1097/PAS.0000000000000614. PMID 26872013.


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