Non-Hodgkin lymphoma, NOS
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See abstractor notes for definitions of splenic B-cell lymphoma/leukemia, unclassifiable and the two most common types.
WHO assigns the non-Hodgkin lymphoma, NOS code to a number of neoplasms, one of which is splenic B-cell lymphoma/leukemia, unclassifiable, a term that describes a number of variably well-defined entities involving the spleen that do not fall into any of the other types of B-cell lymphoid neoplasms. When using this code to describe splenic B-cell lymphoma/leukemia do the necessary text documentation to differentiate this disease from the non-Hodgkin, NOS. The two best defined of these splenic B-cell variants are splenic diffuse red pulp small B-cell lymphoma and hairy cell leukemia-variant (all coded to 9591/3). All of these diagnoses are non-specific. When a more specific B-cell neoplasm is defined, the histology code should be changed to the more specific diagnosis. Splenic diffuse red pulp small B-cell lymphoma is a rare disorder. All cases are diagnosed at clinical stage IV with spleen, BM and PB involvement. Peripheral lymph node involvement is rare. There is diffuse involvement of the red pulp of the spleen.
Another neoplasm coded to the NHL, NOS code is thrombocytic variant of hairy cell leukemia/hairy cell leukemia variant (Do not confuse with hairy cell leukemia. This neoplasm must be described as a VARIANT.) HCL-v accounts for about 10% of hairy cell leukemia cases. Spleen, BM and PB are involved. Hepatomegaly and lymphadenopathy is uncommon. Involvement of other solid tissue is rare. For hairy cell leukemia variant, code primary site to C42.1 with histology code 9591/3.
This code is also used for lymphomatosis cerebri because there is no ICD-O-3 code for lymphomatosis cerebri. Lymphomatosis cerebri (LC) is a rare primary nervous system lymphoma (PCNSL). This disease presents as a diffuse, infiltrating process without the formation of a mass. Radiographs show diffuse white matter involvement without enhancement.