AHA Coding Clinic® for ICD-10-CM and ICD-10-PCS - 2016 Issue 4; ICD-10-CM NEW/REVISED CODES

Castleman Disease

Subcategory D47.Z, Other specified neoplasms of uncertain behavior of lymphoid, hematopoietic and related tissue, has been expanded to specifically identify Castleman disease (D47.Z2). Castleman disease describes a group of related lymphoproliferative disorders involving proliferation of morphologically benign lymphocytes due to excessive proinflammatory hypercytokinemia, most notably of interleukin-6. Castleman disease is diagnosed when lymph node histopathology reveals regression of germinal centers, abnormal vascularity, and a range of hyaline vascular changes and/or polytypic plasma cell proliferation. It can occur in a single lymph node region (unicentric) or in multiple lymph node regions (multicentric). Clinical features and management vary significantly...

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