Hepatosplenic T cell lymphoma
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- Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [2]
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.
- It is also known as ‘gamma-delta hepatosplenic T-cell lymphoma.
- It usually occurs in young men with history of immunosuppression including solid organ transplantation and leukemia.
- 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.
- 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:
- 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.
- 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.
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
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].
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The presence of [finding(s)] on physical examination is diagnostic of [disease name].
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The presence of [finding(s)] on physical examination is highly suggestive of [disease name].
Laboratory Findings
An elevated/reduced concentration of serum/blood/urinary/CSF/other [lab test] is diagnostic of [disease name].
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Laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].
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[Test] is usually normal among patients with [disease name].
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Some patients with [disease name] may have elevated/reduced concentration of [test], which is usually suggestive of [progression/complication].
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There are no diagnostic laboratory findings associated with [disease name].
Electrocardiogram
There are no ECG findings associated with [disease name].
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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
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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].
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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
There are no echocardiography/ultrasound findings associated with [disease name].
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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].
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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
There are no CT scan findings associated with [disease name].
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[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].
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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
There are no MRI findings associated with [disease name].
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[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].
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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
There are no other imaging findings associated with [disease name].
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[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].
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[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].
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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
Medical Therapy
There is no treatment for [disease name]; the mainstay of therapy is supportive care.
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Supportive therapy for [disease name] includes [therapy 1], [therapy 2], and [therapy 3].
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The majority of cases of [disease name] are self-limited and require only supportive care.
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[Disease name] is a medical emergency and requires prompt treatment.
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The mainstay of treatment for [disease name] is [therapy].
OR The optimal therapy for [malignancy name] depends on the stage at diagnosis.
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[Therapy] is recommended among all patients who develop [disease name].
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Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].
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Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
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Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].
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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].
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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]
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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].
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The feasibility of surgery depends on the stage of [malignancy] at diagnosis.
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Surgery is the mainstay of treatment for [disease or malignancy].
Primary Prevention
There are no established measures for the primary prevention of [disease name].
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There are no available vaccines against [disease name].
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Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].
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[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].
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Effective measures for the secondary prevention of [disease name] include [strategy 1], [strategy 2], and [strategy 3].