Diffuse large B-cell lymphoma (DLBCL), NOS
Help me code for diagnosis year :
1. DLBCL of the CNS: Approximately 60% are supratentorial. 20-40% have multiple lesions. Approximately 20% have intraocular lesions. Many of the intraocular lesions are contralateral, and the patient may develop parenchymal lesions. Sporadic systemic recurrences may affect any organ but relatively frequently the testis and breast.
2. DLBCL associated with chronic inflammation: common sites are pleural cavity, bone (especially femur) joint, and periarticular soft tissue. Tumor mass >10 cm in most case of pleural cavity disease (pyothorax-associated lymphoma or PAL).
3. EBV positive DLBCL of the elderly: Occurs in patients who are greater than 50 years old and have no known immunodeficiency or history of lymphoma. 70% have extranodal involvement, most commonly skin, lung, tonsil, and stomach with or without LN involvement. The remaining 30% present with LN involvement only.
4. Primary cutaneous DLBCL, leg type: these lymphomas usually affect the lower leg but 10-15% arise in other sites. Patient presents with red or bluish-red tumors on one or both of the lower legs. Frequently disseminates to other sites.
5. B-cell lymphoma, unclassifiable with features intermediate between DLBCL and Burkitt lymphoma: more than half present with widespread extranodal disease. Bone marrow and peripheral blood may be involved. Patients present with lymphadenopathy or mass lesions in extranodal sites.
Code the primary site to skin when there is skin infiltration with large B-cell lymphoma; B-cell lymphoma, large cell type or large cell lymphoma and the lymphoma is limited to skin and the regional lymph nodes. If there is involvement of lymph node(s) that are not regional for the skin site involved, or involvement of bone marrow or organ(s), do not code skin as the primary site. In this situation, skin is probably a metastatic site.
Richter syndrome is a term that is used to describe the transformation from either CLL/SLL or hairy cell leukemia to DLBCL.