Primary bone marrow lymphoma presenting with cold-type autoimmune hemolytic anemia

Area of Science:

• Hematology
• Oncology
• Immunology

Background:

• Autoimmune hemolytic anemia (AIHA) is characterized by autoantibodies against red blood cells.
• Cold-type AIHA is specifically mediated by cold agglutinins, often associated with lymphoproliferative disorders.

Observation:

• A 70-year-old woman presented with jaundice and anemia, diagnosed with cold-type AIHA.
• Bone marrow biopsy revealed diffuse large B-cell lymphoma (DLBCL) with specific immunophenotypic markers (CD20, CD79a, Bcl-2).
• Initial investigations, including imaging and endoscopy, did not reveal lymphoma lesions elsewhere.

Findings:

• The patient was diagnosed with primary bone marrow DLBCL presenting concurrently with cold-type AIHA.
• The direct antiglobulin test was positive for complement (C3b, C3d) but negative for IgG.
• High-titer cold agglutinin and absent haptoglobin confirmed the cold-type AIHA diagnosis.

Implications:

• This case highlights the importance of bone marrow examination in diagnosing primary bone marrow lymphoma, especially when associated with AIHA.
• Successful treatment with a rituximab-containing regimen achieved complete remission for both lymphoma and AIHA.
• Early diagnosis and combined treatment strategies are crucial for managing this rare presentation.