Primary bone marrow T-cell anaplastic large cell lymphoma with triple M gradient

Area of Science:

• Hematology
• Oncology
• Immunology

Background:

• Anaplastic large cell lymphoma (ALCL) is a distinct type of T-cell non-Hodgkin lymphoma.
• Primary bone marrow involvement without lymphadenopathy or splenomegaly is uncommon.

Observation:

• A 60-year-old male presented with anemia and fever, later found to have a triple M gradient with cryoglobulinemia.
• Bone marrow biopsy revealed massive CD30-positive, ALK-negative T-cell lymphoma with anaplastic morphology.
• PCR identified a B/T hybrid genotype disorder, excluding common ALCL translocations.

Findings:

• The patient achieved remission with CHOP chemotherapy.
• Despite initial response, the patient developed severe humoral immunodeficiency.
• The patient ultimately died from sepsis secondary to immunodeficiency.

Implications:

• This case highlights a rare presentation of ALCL primarily in the bone marrow.
• It underscores the potential for severe immunodeficiency in ALCL, even with chemotherapy response.
• Understanding complex genotypes in ALCL is crucial for prognosis and treatment strategies.