Hepatosplenic T cell lymphoma: Difference between revisions

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===Physical Examination===
===Physical Examination===
Patients with [disease name] usually appear [general appearance]. Physical examination of patients with [disease name] is usually remarkable for [finding 1], [finding 2], and [finding 3].


OR
=== Temperature ===
* [[Fever]] is often present


Common physical examination findings of [disease name] include [finding 1], [finding 2], and [finding 3].
=== Skin ===
* [[Rash]]


OR
=== Thorax ===
* [[Pleural effusion]]
* Chest tenderness


The presence of [finding(s)] on physical examination is diagnostic of [disease name].
=== Abdomen ===
* [[Splenomegaly]]
* [[Hepatomegaly]]
* [[Ascites]]
* Abdomen tenderness


OR
=== Extremities ===
 
* Bone tenderness
The presence of [finding(s)] on physical examination is highly suggestive of [disease name].


===Laboratory Findings===
===Laboratory Findings===
An elevated/reduced concentration of serum/blood/urinary/CSF/other [lab test] is diagnostic of [disease name].
* Biopsy of the tumor:
 
** Histology - small-to intermediate sized T lymphocytes infiltrate the sinusoids of the liver and the splenic red pulp.
OR
** 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.
Laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].
* Bone marrow biopsy; The bone marrow is characterized by neoplastic cells in the sinusoids.
 
* Complete blood count; Pancytopenia
OR
* Liver function tests: Deranged LFT
 
** Elevated transaminases
[Test] is usually normal among patients with [disease name].
** Hyperbilirubinemia
 
** Hypoproteinemia
OR
** Elevated [[alkaline phosphatase]]  
 
** Elevated PT/INR
Some patients with [disease name] may have elevated/reduced concentration of [test], which is usually suggestive of [progression/complication].
 
OR
 
There are no diagnostic laboratory findings associated with [disease name].


===Electrocardiogram===
===Electrocardiogram===
There are no ECG findings associated with [disease name].
There are no ECG findings associated with hepatosplenic t cell lymphoma.
 
OR
 
An ECG may be helpful in the diagnosis of [disease name]. Findings on an ECG suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].


===X-ray===
===X-ray===
There are no x-ray findings associated with [disease name].
There are no x-ray findings associated with hepatosplenic t cell lymphoma but pleural effusion might be present.
 
OR
 
An x-ray may be helpful in the diagnosis of [disease name]. Findings on an x-ray suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
 
OR
 
There are no x-ray findings associated with [disease name]. However, an x-ray may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].


===Echocardiography or Ultrasound===
===Echocardiography or Ultrasound===
There are no echocardiography/ultrasound  findings associated with [disease name].
* There are no echocardiography findings associated with hepatosplenic t cell lymphoma.
 
* On ultrasound of abdomen:
OR
** Hepatomegaly
 
** Splenomegaly
Echocardiography/ultrasound may be helpful in the diagnosis of [disease name]. Findings on an echocardiography/ultrasound suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
 
OR
 
There are no echocardiography/ultrasound  findings associated with [disease name]. However, an echocardiography/ultrasound  may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].


===CT scan===
===CT scan===
There are no CT scan findings associated with [disease name].
Tumor mass can be seen in liver or spleen or both.
 
OR
 
[Location] CT scan may be helpful in the diagnosis of [disease name]. Findings on CT scan suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
 
OR
 
There are no CT scan findings associated with [disease name]. However, a CT scan may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].


===MRI===
===MRI===
There are no MRI findings associated with [disease name].
Tumor mass can be seen in liver or spleen or both.
 
OR
 
[Location] MRI may be helpful in the diagnosis of [disease name]. Findings on MRI suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
 
OR
 
There are no MRI findings associated with [disease name]. However, a MRI may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].


===Other Imaging Findings===
===Other Imaging Findings===
There are no other imaging findings associated with [disease name].
PET scan: On PET scan, tumor mass can be seen in liver or spleen or if it has metastatsized to any other organ.
 
OR
 
[Imaging modality] may be helpful in the diagnosis of [disease name]. Findings on an [imaging modality] suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
 
===Other Diagnostic Studies===
There are no other diagnostic studies associated with [disease name].
 
OR
 
[Diagnostic study] may be helpful in the diagnosis of [disease name]. Findings suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
 
OR
 
Other diagnostic studies for [disease name] include [diagnostic study 1], which demonstrates [finding 1], [finding 2], and [finding 3], and [diagnostic study 2], which demonstrates [finding 1], [finding 2], and [finding 3].


==Treatment==
==Treatment==

Revision as of 05:12, 23 January 2019


For patient information, click here

Hepatosplenic T cell lymphoma Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Epidemiology and Demographics

Risk Factors

Screening

Differentiating Hepatosplenic T cell lymphoma from other Diseases

Natural History, Complications and Prognosis

Diagnosis

Staging

History and Symptoms

Physical Examination

Laboratory Findings

Biopsy

CT

MRI

Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

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Most cited articles

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CME Programs

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MRI

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Risk calculators and risk factors for Hepatosplenic T cell lymphoma

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

Synonyms and keywords:

Overview

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 and leukemia[3].
  • Patients with inflammatory bowel diseases 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 deveopment 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]:

  • Inflammatory bowel disease
  • Organ transplant patients (reciever)
  • Immunosuppresent medications
  • Thiopurines
  • Infliximab
  • Cyclophosphamide
  • Vincristine
  • Doxorubicin

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:

  • Immunodeficiency diseases.
  • Patients on immunosuppresant medications.
  • Patients on chemotherapy.
  • Inflammatory bowel disease
  • Organ transplant patients.

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

  • Hepatomegaly
  • Hepatic failure
  • Portal vein thrombosis
  • Splenomegaly
  • Splenic infarction
  • Spleen rupture
  • Splenic vein thrombosis
  • Anemia
  • Thrombocytopenia
  • Neutropenia
  • Neurological dysfunction if metastasizes to brain.
  • Intestinal perforation
  • Intestinal obstruction

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
    • Elevated transaminases
    • Hyperbilirubinemia
    • Hypoproteinemia
    • Elevated alkaline phosphatase
    • Elevated PT/INR

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:
    • Hepatomegaly
    • Splenomegaly

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

There is no treatment for [disease name]; the mainstay of therapy is supportive care.

OR

Supportive therapy for [disease name] includes [therapy 1], [therapy 2], and [therapy 3].

OR

The majority of cases of [disease name] are self-limited and require only supportive care.

OR

[Disease name] is a medical emergency and requires prompt treatment.

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

Surgical intervention is not recommended for the management of [disease name].

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

There are no established measures for the primary prevention of [disease name].

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

There are no established measures for the secondary prevention of [disease name].

OR

Effective measures for the secondary prevention of [disease name] include [strategy 1], [strategy 2], and [strategy 3].

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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