Hepatosplenic T cell lymphoma.

Area of Science:

• Hematology
• Oncology
• Pathology

Background:

• Hepatosplenic T-cell lymphoma (HSTCL) is an aggressive non-Hodgkin lymphoma primarily affecting adolescents and young adults.
• HSTCL is characterized by infiltration of the spleen, liver, and bone marrow, often presenting with cytopenias and constitutional symptoms.

Observation:

• A 26-year-old female presented with eight months of intermittent fever, abdominal pain, decreased appetite, jaundice, ascites, and lower extremity swelling.
• Radiological findings included hepatomegaly, portal vein dilation, massive splenomegaly, and ascites, with no lymphadenopathy.
• Liver biopsy revealed sinusoidal infiltration by lymphoid cells positive for LCA, CD3, and CD43, but negative for CD20, CD34, CD4, CD8, and c-kit.

Findings:

• The diagnostic workup, including blood and bone marrow studies, was inconclusive.
• Immunophenotyping and histological findings from the liver biopsy were crucial for diagnosing Hepatosplenic T-cell lymphoma.
• The patient's clinical presentation and biopsy results were consistent with HSTCL.

Implications:

• This case highlights the diagnostic challenges of Hepatosplenic T-cell lymphoma, particularly when initial investigations are inconclusive.
• Early and accurate diagnosis through liver biopsy and immunophenotyping is critical for appropriate management of HSTCL.
• The aggressive nature of HSTCL underscores the need for further research into effective therapeutic strategies and improved patient outcomes.