[Lymphoplasmacytic lymphoma accompanied by severe myelofibrosis]

Area of Science:

• Hematology
• Oncology
• Immunology

Background:

• Lymphoplasmacytic lymphoma (LPL) is a rare B-cell malignancy.
• Pancytopenia and febrile neutropenia can be presenting symptoms of LPL.
• Bone marrow (BM) involvement is common in LPL, often accompanied by fibrosis.

Observation:

• A 61-year-old female presented with pancytopenia and febrile neutropenia.
• Imaging revealed hepatosplenomegaly and an intra-abdominal abscess, but no lymphadenopathy.
• Bone marrow examination showed severe fibrosis and atypical lymphocytes with specific immunophenotypes (CD20+, CD138+/-).

Findings:

• Genetic testing confirmed MYD88 mutation and IgH rearrangement, excluding JAK2/CALR mutations.
• Diagnosis was BM infiltration of LPL.
• Rituximab monotherapy led to complete disappearance of lymphoma cells and near-resolution of myelofibrosis.
• Post-treatment BM analysis showed negativity for the MYD88 mutation.

Implications:

• Rituximab is an effective treatment for lymphoplasmacytic lymphoma.
• Treatment can reverse bone marrow fibrosis associated with LPL.
• Resolution of MYD88 mutation post-treatment suggests therapeutic efficacy.