Simultaneous hepatosplenic T-cell lymphoma and myelofibrosis

Area of Science:

• Hematology
• Oncology
• Pathology

Background:

• Hepatosplenic T-cell lymphoma (HSTL) is a rare, aggressive non-Hodgkin lymphoma primarily affecting the spleen, liver, and bone marrow.
• It is characterized by the infiltration of neoplastic lymphocytes within the sinusoidal spaces of these organs.
• Myelofibrosis (MF) is a myeloproliferative neoplasm involving bone marrow scarring, which can be primary or secondary to other conditions.

Observation:

• A patient presented with a left axillary lymph node biopsy showing features consistent with HSTL.
• Bone marrow biopsy revealed simultaneous infiltration by neoplastic T-cells and marked myelofibrosis.
• This represents a rare co-occurrence, with limited prior reports of even mild bone marrow fibrosis alongside T-cell lymphoma.

Findings:

• The study describes a unique case of HSTL associated with significant primary myelofibrosis.
• Histological and immunohistochemical analyses confirmed the diagnoses in the lymph node and bone marrow.
• This combination of HSTL and marked myelofibrosis has not been previously reported in the literature.

Implications:

• The co-existence of primary myelofibrosis may significantly impact the prognosis of Hepatosplenic T-cell lymphoma.
• This case underscores the need for thorough bone marrow evaluation in HSTL patients to identify potential coexisting myeloproliferative neoplasms.
• Further research is warranted to understand the pathogenesis and clinical implications of this rare dual hematologic malignancy.