The CARE Checklist has been completed by the authors for this case report and attached as online supplementary material (for all online suppl. Here, we present a series of 6 patients diagnosed with HSTCL over a 10-year period, describing the collective presentation, diagnosis, management, and outcomes. The disease course is progressively rapid, and a standard therapy has yet to be established. In most cases, the diagnosis is made by liver, spleen, and/or bone marrow biopsies. Given the rarity of this disease and the absence of significant nodal involvement, delays in reaching diagnosis and initiation of treatment are substantial and common problems for hematologists. Diagnosing HSTCL can be challenging, especially when the associated signs and symptoms are nonspecific and easily mimic various other conditions, mostly infectious etiologies, and other malignant disorders. T-cell lymphomas are uncommon compared to their B-cell counterparts with HSTCL making up 1–2% of all peripheral T-cell lymphomas. It usually comprises medium-sized lymphoid cells that exhibit marked sinusoidal infiltration of the spleen, liver, and bone marrow. HSTCL results from the proliferation of γ/δ T-cell receptor-type cytotoxic T cells. This malignant lymphoma was recognized as a hepatosplenic disease displaying sinusal/sinusoidal pattern, and the gamma delta phenotype of malignant cells. It was first described in two adults presenting with hepatosplenomegaly and minimal lymphadenopathy. Hepatosplenic T-cell lymphoma (HSTCL) is an aggressive subtype of lymphoma with a characteristic hepatosplenic presentation and without lymphadenopathy. Lymphomas are a group of heterogeneous malignancies that involve any tissue that houses lymphoid cells, especially the lymph nodes. Here, we highlight the clinicopathological features and management of hepatosplenic T-cell lymphoma over a 10-year span in a single hematology referral center and review the literature. There is a lack of international guidelines for its treatment, and in most cases, treatment is guided by case series. Managing this lymphoma is arduous because of its late presentation and aggressive nature, frequently resulting in rapid progression in its clinical course and refractoriness to conventional chemotherapy. The rarity of this disease, coupled with the lack of lymph node involvement that is usually seen in lymphomas, causes significant difficulty in diagnosis, which inevitably delays the initiation of treatment. Patients usually present with nonspecific constitutional symptoms and spleen and liver enlargement, with variable degrees of cytopenia. Hepatosplenic T-cell lymphoma can mimic various infectious diseases, immunological conditions, and other malignancies. Isochromosome 7q and trisomy 8 are the most prevalent chromosomal abnormalities associated with hepatosplenic T-cell lymphoma, and most patients have mutations in genes related to chromatin remodeling or the JAK/STAT system. Lymph nodes can also enlarge due to cancer in the lymphatic system, such as Hodgkin disease.Hepatosplenic T-cell lymphoma is a rare form of T-cell lymphoma that predominantly emerges from neoplastic proliferation of cytotoxic T cells of γ/δ T-cell receptor-expressing lymphocytes. Sometimes, the lymph nodes themselves can become inflamed and enlarged, a condition called lymphadenitis. However, the lymphadenopathy may be generalized, with lymph node enlargement in more than one area, which is typical of a viral illness. Swollen lymph nodes around the jaw may be due to an infection in the teeth or mouth. For example, an infant with a scalp infection may have enlarged lymph nodes at the back of the neck. Since enlarged lymph nodes are often near the source of infection, their location can help determine the cause. Bacterial infections, such as strep throat caused by the streptococcus bacterium, can also cause lymphadenopathy. Nearly all children will develop lymphadenopathy at some time, as the condition commonly occurs in response to an infection from a virus, such as an upper respiratory infection. As infection-fighting cells and fluid accumulate, the lymph nodes enlarge to many times their normal size. The lymphatic system is part of the immune system and functions to fight disease and infections.
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