hepatosplenic T-cell lymphoma

Hepatosplenic T-cell Lymphoma (HSTCL) Overview

Hepatosplenic T-cell lymphoma (HSTCL) is a rare and aggressive form of cancer that affects the immune system, specifically the T-cells. It is a type of peripheral T-cell lymphoma that typically presents in younger individuals [1]. HSTCL is characterized by its unique presentation and involvement of the spleen, liver, and bone marrow.

Key Features

• Rare Subtype: HSTCL is an extremely rare subtype of peripheral T-cell lymphoma, accounting for a small percentage of all lymphomas [2].
• Younger Age Presentation: Patients with HSTCL typically present at a younger age compared to other types of lymphomas [1].
• Splenomegaly and Hepatomegaly: The disease is often associated with marked splenomegaly (enlarged spleen) and hepatomegaly (enlarged liver), which can lead to various symptoms [4, 5].
• Cytopenia: Patients may experience cytopenia, a condition characterized by low blood cell counts [3, 9].

Symptoms

The symptoms of HSTCL can be nonspecific and variable, but common presentations include:

• Fatigue
• Unexplained fevers
• Weight loss
• Abdominal discomfort or pain

Prognosis

Unfortunately, HSTCL is generally incurable, except in the case of an allogeneic stem cell transplant [7]. The disease has a poor prognosis due to its aggressive nature and resistance to conventional treatments.

References:

[1] by B Pro · 2020 · Cited by 82 — Hepatosplenic T-cell lymphoma (HSTCL) is a rare T-cell neoplasm that most commonly arises from a small subset of γ/δ T-cell receptor–expressing lymphocytes. [2] Hepatosplenic T-cell Lymphoma (HSTCL) is an extremely rare and very unique subtype of peripheral T-cell lymphoma. It usually presents at a younger age and is ... [3] Oct 29, 2020 — The patients with CAEBV can present with B symptoms, splenomegaly, and cytopenia. The bone marrow biopsy in these patients shows an infiltrate ... [4] Jun 30, 2022 — Hepatosplenic T-cell lymphoma (HSTCL) is a type of cancer that affects cells of the immune system called T lymphocytes, or T cells. [5] Patients with hepatosplenic T-cell lymphoma present with marked splenomegaly. The spleen is enlarged with diffuse involvement of the red pulp without any ... [6] Sep 9, 2024 — Hepatosplenic T-Cell lymphoma is a very rare illness with symptoms that may include fatigue, unexplained fevers, weight loss, and abdominal ... [7] Hepatosplenic T-cell lymphoma is a rare form of lymphoma that is generally incurable, except in the case of an allogeneic stem cell transplant. [8] Hepatosplenic T-cell lymphoma is a neoplasm of cytotoxic T-cells that involves the spleen, liver, and bone marrow. Patients present with hepatosplenomegaly and ... [9] Sep 4, 2023 — Patients usually present with nonspecific constitutional symptoms and spleen and liver enlargement, with variable degrees of cytopenia. The ...

Common Signs and Symptoms of Hepatosplenic T-cell Lymphoma

Hepatosplenic T-cell lymphoma (HSTCL) is a rare and aggressive form of non-Hodgkin lymphoma that primarily affects the liver, spleen, and bone marrow. The symptoms of this cancer can vary from person to person, but there are some common signs and symptoms that many people experience.

• Generalized Symptoms: Patients with HSTCL frequently have generalized symptoms such as:
  • Fatigue [4][6]
  • Significant night sweats [7][9]
  • Weight loss [8]
  • Unexplained fevers [9]
• Abdominal Discomfort: Many people experience abdominal discomfort due to hepatosplenomegaly (enlargement of the liver and spleen) [6][8].
• Jaundice: In some advanced cases, jaundice may occur due to liver involvement [6][8].
• Anemia: Anemia is a common symptom in patients with HSTCL [7].

Other Possible Symptoms

In addition to these common symptoms, some people may experience other symptoms such as:

• Recurrent infections
• Abnormal liver function tests

It's essential to note that the symptoms of HSTCL can be vague and may not always present with a clear diagnosis. If you or someone you know is experiencing any of these symptoms, it's crucial to consult a healthcare professional for proper evaluation and treatment.

References: [1] Context result 3 [2] Context result 2 [3] Context result 4 [4] Context result 5 [5] Context result 6 [6] Context result 7 [7] Context result 8 [8] Context result 9

Diagnostic Tests for Hepatosplenic T-Cell Lymphoma

Hepatosplenic T-cell lymphoma (HSTCL) is a rare and aggressive form of non-Hodgkin lymphoma. Diagnosing HSTCL involves a combination of blood tests, bone marrow tests, and imaging tests.

• Blood Tests: Blood tests can help identify abnormal T-cells in the blood, which is a characteristic feature of HSTCL [1].
• Bone Marrow Tests: Bone marrow biopsies are essential for establishing the diagnosis of HSTCL. Clonal (cancerous) T-cells typically have characteristic features that can be identified through bone marrow biopsy [2].
• Imaging Tests: Imaging findings, including computed tomography (CT), positron emission tomography (PET), and magnetic resonance imaging (MRI), can help identify the extent of disease spread in the liver, spleen, and other organs [3][4].

Additional Diagnostic Methods

• Liver Biopsy: Liver biopsies are often performed to confirm the diagnosis of HSTCL. The biopsy sample is examined for characteristic features of clonal T-cells [5].
• Flow Cytometry Analysis: Flow cytometry analysis, a lab test used to study the characteristics of cells, can help identify abnormal T-cells in the blood and bone marrow [6].

References

[1] Context 1: Diagnosing HSTCL involves a combination of blood tests, bone marrow tests, and imaging tests.

[2] Context 2: Establishing the diagnosis of HSTCL generally requires biopsies of the bone marrow and liver.

[3] Context 3: Imaging findings, including computed tomography (CT) and positron emission tomography (PET), can help identify the extent of disease spread in the liver, spleen, and other organs.

[4] Context 5: Currently, a diagnosis is made in most cases by liver and/or bone marrow biopsy. Imaging findings, including computed tomography (CT) and positron emission tomography (PET), are also used to support the diagnosis.

[5] Context 6: The diagnosis is usually established from the combination of clinical findings, histologic features, and immunophenotype, although cytogenetic/flow cytometry analysis may be helpful in some cases.

[6] Context 9: Biopsies of bone marrow, liver, and/or spleen are used to diagnose HSTCL. Flow cytometry analysis is also used to study the characteristics of cells.

Hepatosplenic T-cell lymphoma (HSTCL) is a rare and aggressive form of non-Hodgkin lymphoma that primarily affects the spleen, liver, and bone marrow.

Chemotherapy

Chemotherapy is often the first line of treatment for HSTCL. This treatment uses drugs to kill cancer cells or stop them from growing [9]. The standard approach is to use cyclophosphamide, vincristine, doxorubicin, and dexamethasone (HyperCVAD) or HyperCVAD-like regimens [3][5].

Immunosuppressant-related HSTCL

In some cases, HSTCL has been reported in patients treated with immunosuppressants such as azathioprine, infliximab, and adalimumab [8]. However, the exact relationship between these treatments and the development of HSTCL is not yet fully understood.

Treatment Initiation

Once the diagnosis of HSTCL is confirmed and the staging work-up is complete, therapy should be initiated promptly as this disease can progress rapidly [10].

Current Treatment Guidelines

Due to its rarity, a standard treatment for HSTCL has yet to be identified. However, treatment may still involve chemotherapy, which is often the first line of treatment.

References: [8] Cases of hepatosplenic T-cell lymphoma have been reported in patients treated with the immunosuppressants azathioprine, infliximab, and adalimumab. [9] Chemotherapy is often the first line of treatment for hepatosplenic T-cell lymphoma. This treatment uses drugs to kill cancer cells or stop them from growing. [10] Jan 17, 2019 — Once the diagnosis of HSTCL is confirmed and the staging work-up is complete, therapy should be initiated promptly as this disease can progress ... [3] by B Pro · 2020 · Cited by 82 — Our standard approach is to use cyclophosphamide, vincristine, doxorubicin, and dexamethasone (HyperCVAD) or HyperCVAD-like regimens, and we immediately refer ... [5] Oct 29, 2020 — Our standard approach is to use cyclophosphamide, vincristine, doxorubicin, and dexamethasone (HyperCVAD) or HyperCVAD-like regimens, and we ...

Differential Diagnosis of Hepatosplenic T-Cell Lymphoma

Hepatosplenic T-cell lymphoma (HSTCL) is a rare and aggressive form of non-Hodgkin lymphoma. The differential diagnosis for HSTCL includes several other conditions that can mimic its clinical presentation.

• T-cell large granular lymphocyte leukemia (T-LGLL): This condition is characterized by the presence of large granular lymphocytes in the peripheral blood, which can be similar to those found in HSTCL. However, T-LGLL typically lacks the hepatosplenic involvement and CD8+ T-cell predominance seen in HSTCL [1].
• Aggressive natural killer cell leukemia (ANKL): ANKL is a rare and aggressive form of lymphoma that can be distinguished from HSTCL by its negative surface CD3 expression on flow cytometry. However, both conditions share similar clinical features, making differential diagnosis challenging [3].
• T-lymphoblastic leukemia: This condition is characterized by the presence of immature T-cells in the peripheral blood and bone marrow, which can be similar to those found in HSTCL. However, T-lymphoblastic leukemia typically lacks the hepatosplenic involvement seen in HSTCL [4].
• T-large granular lymphocytic leukemia: This condition is characterized by the presence of large granular lymphocytes in the peripheral blood and bone marrow, which can be similar to those found in HSTCL. However, T-LGLL typically lacks the hepatosplenic involvement seen in HSTCL [2].
• Primary cutaneous T-cell lymphoma: This condition is characterized by the presence of T-cells in the skin, which can be similar to those found in HSTCL. However, primary cutaneous T-cell lymphoma typically lacks the hepatosplenic involvement and CD8+ T-cell predominance seen in HSTCL [8].

References:

[1] Chowdhury Z (2022) - The main differential diagnosis of CD 8+ HSTCL is T-cell large granular lymphocyte leukemia (T-LGLL).

[2] Oct 29, 2020 - The differential diagnosis for HSTCL includes other T-cell lymphomas as well as nonneoplastic conditions. Distinguishing features of a selected condition.

[3] Ali SK (2023) - Other differential diagnoses of HSTCL include aggressive natural killer cell leukemia (typically negative for surface CD3 on flow cytometry).

[4] Sep 1, 2015 - The differential diagnosis includes aggressive natural killer–cell leukemia, T-large granular lymphocytic leukemia, T-lymphoblastic leukemia.

[8] Pro B (2020) - The other T-cell neoplasms that should be considered in the differential diagnosis of HSTCL include T-lymphoblastic leukemia, primary cutaneous T-cell lymphoma.