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ICD 10 Coding Alert

Reader Questions:

Read Descriptors Carefully for MZL Dx

Question: How would you code marginal zone lymphoma (MZL) with spleen involvement? My co-worker thinks it should be C88.4, but I think it should a code from the C83 category. Which of us is right?>

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Answer: The confusion here stems from the fact that both codes describe different subtypes of MZL. Clinically, mucosa-associated lymphoid tissue (MALT) lymphoma, also known as extranodal marginal zone lymphoma (EMZL), is distinct from the other types of MZL: splenic marginal zone lymphoma (SMZL) and nodal marginal zone lymphoma (NMZL). Each one has different diagnostic and genetic features, different behaviors, and different treatments (see ).

You would code EMZL with C88.4 (Extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue [MALT-lymphoma]), as the descriptor implies. But SMZL is listed as an inclusion term for the C83.0- (Small cell B-cell lymphoma) code group as you point out, making C83.07 (Small cell B-cell lymphoma, spleen) the correct choice for marginal zone lymphoma with spleen involvement.


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