Erbil Polytechnic University

MLT Department
3rd Semester
2023 - 2024

Hepatosplenic T-cell lymphoma

Student Name : Lana hoshang abdulmajid

Student Year : Second Stage

Subject : Hematology
Supervisor: Dr. Dldar Salih Ismae

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Table content ………………..

• introduction

• What is the outlook for people with hepatosplenic T-cell

lymphoma?

• How is hepatosplenic T-cell lymphoma diagnosed?

• Symptoms

• What is the treatment for hepatosplenic T-cell lymphoma?

•refrence

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Introduction

Hepatosplenic T-cell lymphoma (HSTCL) is a rare subtype of

peripheral T-cell lymphoma (PTCL), first described in 1981 and
included as provisional entity γδ hepatosplenic T-cell lymphoma in
the revised European American Lymphoma Classification in
1994.1 After the identification of rare cases with an αβ phenotype,
the term “hepatosplenic T-cell lymphoma,” first used in 1990,2 has
been adopted to describe this neoplasm in the World Health
Organization (WHO) classification.3 HSTCL accounts for <5% of all
PTCL cases with slightly over 200 cases reported in the literature.
According to a retrospective study of 1314 cases of T-cell
lymphoma diagnosed worldwide, the incidence of HSTCL is
1.4%.4 More recently, a prospective study, the T-cell Project,
reported a similar incidence of 2%, with more cases observed in
the United States compared with Europe (44% vs 25%), and
extremely rare cases in other countries.5

As its name indicates, HSTCL is characterized by a proliferation of

small-medium–sized mature T cells infiltrating the sinusoids of
liver and spleen. Clinical presentation, histologic features, and
molecular findings make this disease a unique entity among other
PTCLs.

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What is the outlook for people with hepatosplenic T-cell
lymphoma?

HSTCL tends to be aggressive, and the outlook for people with it

tends to be poor. Estimates on the 5-year overall survival rate
have been as low as 7%, with half of people living less than 10
months.

In a 2015 Dutch studyTrusted Source, only 2 out of 12 people

who receive a diagnosis of HSTCL lived for more than a year.

Despite the historically poor outlook for people with HSTCL,

researchers are continuing to examine the best way to treat it.
Due to the rarity of the disease, treatment protocols will likely
continue to improve, and the survival rate will increase.

Many estimates of survival rates for HSTCL draw on research

from decades ago. For example, in a 2021 study, researchers
calculated the following overall survival rates:

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How is hepatosplenic T-cell lymphoma diagnosed?

Diagnosing HSTCL can be challenging because its symptoms

can mimic those of other cancers or infectious diseases.

The process of diagnosing HSTCL is similar to other types of

non-Hodgkin’s lymphoma. Doctors or healthcare
professionals usuallyTrusted Source perform a liver or bone
marrow biopsy to make a diagnosis. They’ll analyze cells from
the biopsy in a laboratory using specialized tests such as flow
cytometry.

Imaging such as computed tomography (CT) scan or positron

emission tomography/CT may be used to identify abnormalities
in your liver and spleen.

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Symptoms

HSTL usually affects the liver or spleen. As a result, people with HSTL
often have a larger than normal liver or spleen or both.

This type of NHL often affects the bone marrow, which can cause:

 fatigue
 low red blood cell counts (called anemia)
 low platelet counts (called thrombocytopenia)

HSTL can cause B symptoms, which are unexplained fever, drenching

night sweats and unexplained weight loss.

People with HSTL may have skin sores. They usually don’t have larger
than normal lymph nodes.

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What is the treatment for hepatosplenic T-cell
lymphoma?

Due to the rarity of HSTCL, researchers have limited knowledge

about how to best treat it. Most of what’s known has come from
studies analyzing a small number of people treated at a single
hospital, which makes it difficult to judge the effectiveness of
various treatment options.

While there’s no standard treatment, in most studies, doctors

treat people with HSTCL with chemotherapy and a bone marrow
transplant if eligible.

Researchers have looked into various chemotherapy regimens

with varying levels of effectiveness. At least two small
studiesTrusted Source have investigated the CHOP regimen,
which consists of the drugs:

 cyclophosphamide

 doxorubicin (hydroxydaunorubicin)

 vincristine (Oncovin)

 prednisone

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In one of these studies, 9 out of 21 people achieved complete
remission, but half of the people lived less than 16 months. In
the second study, only two out of six people treated with CHOP
achieved remission. Remission only lasted an average of 8
months.

The role of bone marrow transplants in treatment remains under

investigation. Some studiesTrusted Source have found evidence
that they may improve outcomes.

Refreance

 Ammad Ud Din M, et al. (2020). Hepatosplenic T-cell lymphoma in

an immunocompetent young male: A challenging diagnosis.

 Carvão J, et al. (2019). Hepatosplenic T-cell lymphoma: A rare

complication of monotherapy with thiopurines in Chron’s disease.

 Hussain I, et al. (2022). Peripheral T-cell lymphoma.

 Key statistics for Hodgkin lymphoma. (2022).

 Key statistics for non-Hodgkin lymphoma. (2022).

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  Krishnan M, et al. (2019). Hepatosplenic γ-δ T-cell lymphoma: Who
is on your speed dial?

 Li Y, et al. (2021). Survival analysis of hepatosplenic T cell

lymphoma: A population-based study using SEER.

 Lolli G, et al. (2022). Hepatosplenic T-cell non-Hodgkin lymphoma

cured with tandem autologous and allogeneic stem cell
transplantation.

 Montgomery M, et al. (2015). Hepatosplenic T-cell lymphoma: A

population-based study assessing incidence and association with
immune-mediated disease.

 Pro B, et al. (2020). Hepatosplenic T-cell lymphoma: A rare but

challenging entity.

 Signs and symptoms. (n.d.).